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1TABLE 8-2 -- Emerging Patterns of Behavior During the 1st Year of Life [*]NEONATAL PERIOD (1ST 4 WK)Prone: Lies in flexed attitude; turns head from side to side; head sags on ventral suspensionSupine: Generally flexed and a little stiffVisual: May fixate face on light in line of vision;doll's-eye movement of eyes on turning of the bodyReflex: Moro response active; stepping and placing reflexes; grasp reflex activeSocial: Visual preference for human faceAT 1 MOProne: Legs more extended; holds chin up; turns head; head lifted momentarily to plane of body on ventral suspensionSupine: Tonic neck posture predominates; supple and relaxed; head lags when pulled to sitting positionVisual: Watches person; follows moving objectSocial: Body movements in cadence with voice of other in social contact; beginning to smileAT 2 MOProne: Raises head slightly farther; head sustained in plane of body on ventral suspensionSupine: Tonic neck posture predominates; head lags when pulled to sitting positionVisual: Follows moving object 180 degreesSocial: Smiles on social contact; listens to voice and coosAT 3 MOProne: Lifts head and chest with arms extended; head above plane of body on ventral suspensionSupine: Tonic neck posture predominates; reaches toward and misses objects; waves at toySitting: Head lag partially compensated when pulled to sitting position; early head control with bobbing motion; back roundedReflex: Typical Moro response has not persisted; makes defensive movements or selective withdrawal reactionsSocial: Sustained social contact; listens to music; says aah, ngahAT 4 MOProne: Lifts head and chest, with head in approximately vertical axis; legs extendedSupine: Symmetric posture predominates, hands in midline; reaches and grasps objects and brings them to mouthSitting: No head lag when pulled to sitting position; head steady, tipped forward; enjoys sitting with full truncal supportStanding: When held erect, pushes with feetAdaptive: Sees pellet, but makes no move to reach for itSocial: Laughs out loud; may show displeasure if social contact is broken; excited at sight of foodAT 7 MOProne: Rolls over; pivots;crawls or creep-crawls (Knobloch)Supine: Lifts head; rolls over; squirmsSitting: Sits briefly, with support of pelvis; leans forward on hands; back roundedStanding: May support most of weight; bounces activelyAdaptive: Reaches out for and grasps large object; transfers objects from hand to hand; grasp uses radial palm; rakes at pelletLanguage: Forms polysyllabic vowel soundsSocial: Prefers mother; babbles;enjoys mirror; responds to changes in emotional content of social contactAT 10 MOSitting: Sits up alone and indefinitely without support, with back straightStanding: Pulls to standing position;cruises or walks holding on to furnitureMotor: Creeps or crawlsAdaptive: Grasps objects with thumb and forefinger; pokes at things with forefinger; picks up pellet with assisted pincer movement; uncovers hidden toy; attempts to retrieve dropped object; releases object grasped by other personLanguage: Repetitive consonant sounds (mama, dada)Social: Responds to sound of name; plays peek-a-boo or pat-a-cake;waves bye-byeAT 1 YRMotor: Walks with one hand held (48 wk); rises independently, takes several steps (Knobloch)Adaptive: Picks up pellet with unassisted pincer movement of forefinger and thumb; releases object to other person on request or gestureLanguage: Says a few words besides mama, dada
2Social: Plays simple ball game; makes postural adjustment to dressing
15 MOMotor: Walks alone; crawls up stairsAdaptive: Makes tower of 3 cubes; makes a line with crayon; inserts raisin in bottleLanguage: Jargon;follows simple commands; may name a familiar object (e.g., ball)Social: Indicates some desires or needs by pointing; hugs parents18 MOMotor: Runs stiffly; sits on small chair; walks up stairs with one hand held; explores drawers and wastebasketsAdaptive: Makes tower of 4 cubes; imitates scribbling; imitates vertical stroke; dumps raisin from bottleLanguage: 10 words (average); names pictures; identifies one or more parts of bodySocial: Feeds self; seeks help when in trouble; may complain when wet or soiled; kisses parent with pucker24 MOMotor: Runs well, walks up and down stairs, one step at a time; opens doors; climbs on furniture; jumpsAdaptive: Makes tower of 7 cubes (6 at 21 mo); scribbles in circular pattern; imitates horizontal stroke; folds paper once imitativelyLanguage: Puts 3 words together (subject, verb, object)Social: Handles spoon well; often tells about immediate experiences; helps to undress; listens to stories when shown pictures30 MOMotor: Goes up stairs alternating feetAdaptive: Makes tower of 9 cubes; makes vertical and horizontal strokes, but generally will not join them to make cross; imitates circular stroke, forming closed figureLanguage: Refers to self by pronoun I; knows full nameSocial: Helps put things away; pretends in play36 MOMotor: Rides tricycle; stands momentarily on one footAdaptive: Makes tower of 10 cubes; imitates construction of bridge of 3 cubes; copies circle; imitates crossLanguage: Knows age and sex; counts 3 objects correctly; repeats 3 numbers or a sentence of 6 syllablesSocial: Plays simple games (in parallel with other children); helps in dressing (unbuttons clothing and puts on shoes); washes hands48 MOMotor: Hops on one foot; throws ball overhand; uses scissors to cut out pictures; climbs wellAdaptive: Copies bridge from model; imitates construction of gate of 5 cubes; copies cross and square; draws man with 2 to 4 parts besides head; identifies longer of 2 linesLanguage: Counts 4 pennies accurately; tells storySocial: Plays with several children, with beginning of social interaction and role-playing;goes to toilet alone60 MOMotor: SkipsAdaptive: Draws triangle from copy; names heavier of 2 weightsLanguage: Names 4 colors; repeats sentence of 10 syllables; counts 10 pennies correctlySocial: Dresses and undresses; asks questions about meaning of words; engages in domestic role-playing
3TABLE 591-1 -- Screening Scheme for Developmental Delay: Upper RangeAGE(MO) GROSS MOTOR FINE MOTOR SOCIAL SKILLS LANGUAGE3 Supports weight on forearms Opens hands spontaneously Smiles appropriately Coos, laughs6 Sits momentarily Transfers objects Shows likes and dislikes Babbles9 Pulls to stand Pincer grasp Plays pat-a-cake, peek-a- Imitates sounds boo12 Walks with one hand held Releases an object on Comes when called 12 meaningful command words18 Walks upstairs with Feeds from a spoon Mimics actions of others At least 6 words assistance24 Runs Builds a tower of 6 blocks Plays with others 23 word sentences
Infants regain or exceed birth weight by 2 wk of age and should grow at approximately 30 g (1 oz)/day during the 1st mo. Crying normally peaks at about 6 wk of age, when healthy infants cry up to 3 hr/day, then decreases to 1 hr or less by 3 mo. By the end of the 2nd year, somatic and brain growth slows, with corresponding decreases in nutritional requirements and appetite, and the emergence of picky eating habits. Expect a gain of approximately 2 kg (45 lb) in weight and 78 cm (23 in) in height per year. Birthweight quadruples by 2 yr of age. An average 4 yr old weighs 40 lb and is 40 in tall. The head will grow only an additional 5 cm between ages 3 and 18 yr. Language development occurs most rapidly between 2 and 5 yr of age. Vocabulary increases from 50100 words to more than 2,000. Sentence structure advances from telegraphic phrases (Baby cry) to sentences incorporating all of the major grammatical components. As a rule of thumb, between the ages of 2 and 5 yr, the number of words in a typical sentence equals the child's age (2 by age 2 yr, 3 by age 3 yr, and so on.) By 21 mo to 2 yr, most children are using possessives (My ball), progressives (the -ing construction, as in I playing), questions, and negatives. By 4 yr of age, most children can count to 4 and use the past tense; by 5 yr of age, they can use the future tense.
Primitive ReflexesTABLE 591-2 -- Timing of Selected Primitive ReflexesREFLEX ONSET FULLY DURATION DEVELOPED
Palmar grasp 28 wk 32 wk 23 mo
Moro 2832 wk 37 wk 56 mo
Tonic neck 35 wk 1 mo 67 mo
The Moro reflex is obtained by placing the infant in a semi-upright position. The head is momentarily allowed to fall backward, with immediate resupport by the examiner's hand. The child symmetrically abducts and extends the arms and flexes the thumbs, followed by flexion and adduction of the upper extremities. An asymmetric response may signify a fractured clavicle, brachial plexus injury, or a hemiparesis. Absence of the Moro reflex in a term newborn is ominous, suggesting significant dysfunction of the CNS. The grasp response is elicited by placing a finger or object in the open palm of each hand. Normal infants grasp the object, and with attempted removal, the grip is reinforced. The tonic neck reflex is produced by manually turning the head to one side while supine. Extension of the arm occurs on that side of the body corresponding to the direction of the face, while flexion develops in the contralateral extremities. An obligatory tonic neck response, by which the infant remains locked in the fencer's position, is always abnormal and implies a CNS disorder. The parachute reflex is demonstrated by suspending the child by the trunk and by suddenly producing forward flexion as if the child were to fall. The child spontaneously extends the upper extremities as a protective mechanism. The parachute reflex appears before the onset of walking.
4Skeletal Growth TABLE 671-2 -- Skeletal Growth Considerations
Abnormal stature can be assessed as proportionate or disproportionate based on comparing the ratio of sitting height with subischial height (lower limbs). Normally the arm span is almost equal to standing height. The head is disproportionately large at birth and ratio of head height to total height is approximately 1 : 4 at birth, which changes to 1 : 7.5 at skeletal maturity. Lower extremities account for about 15% of height at birth and 30% at skeletal maturity. The rate of height and growth increase is not constant and varies with growth spurts. By age 5 yr, birth height usually doubles and the child is approximately 60% of adult height. The child is about 80% of final height at 9 yr. During puberty, the standing height increases by approximately 1 cm per month. Bone age is more important than chronological age in determining future growth potential.
GaitIn the beginning of ambulation, the child usually has a wide-based gait with hyperflexion of hips and knees, no reciprocal arm swing, and aninitial contact with heel. By the age of 2 yr, the wide gait diminishes, reciprocal arm swing begins, and the initial contact is with the heel withincreased step length and velocity. Adult kinematic patterns usually start developing by 3 yr and the time-distance parameters reach adult valuesby 7 yr, with development of a fully mature gait pattern.
Early walking is not associated with advanced development in other domains. Infants initially toddle with a wide-based gait, with the knees bentand the arms flexed at the elbow; the entire torso rotates with each stride; the toes may point in or out, and the feet strike the floor flat. Theappearance is that of genu varus (bowleg). Subsequent refinement leads to greater steadiness and energy efficiency. After several months ofpractice, the center of gravity shifts back and the torso stays more stable, while the knees extend and the arms swing at the sides for balance. Thefeet are held in better alignment, and the child is able to stop, pivot, and stoop without toppling over .
Most children walk with a mature gait and run steadily before the end of their 3rd yr.
Adolescent DevelopmentTABLE 12-2 -- Classification of Sex Maturity States in GirlsSMR PUBIC HAIR BREASTSSTAGE1 Preadolescent Preadolescent2 Sparse, lightly pigmented, straight, medial border of Breast and papilla elevated as small mound; diameter of areola labia increased3 Darker, beginning to curl, increased amount Breast and areola enlarged, no contour separation4 Coarse, curly, abundant, but less than in adult Areola and papilla form secondary mound5 Adult feminine triangle, spread to medial surface of Mature, nipple projects, areola part of general breast contour thighs
In girls, the first visible sign of puberty and the hallmark of SMR2 is the appearance of breast buds, between 8 and 12 yr of age. Mensestypically begins 22 yr later, during SMR34 (median age, 12 yr; normal range, 916 yr), around the peak height velocity. Less obviouschanges include enlargement of the ovaries, uterus, labia, and clitoris, and thickening of the endometrium and vaginal mucosa.
5In boys, the first visible sign of puberty and the hallmark of SMR2 is testicular enlargement, beginning as early as 9 yr. This is followed bypenile growth during SMR3. Peak growth occurs when testis volumes reach approximately 910 cm3 during SMR4. Under the influence of LHand testosterone, the seminiferous tubules, epididymis, seminal vesicles, and prostate enlarge. The left testis normally is lower than the right.Some degree of breast hypertrophy, typically bilateral, occurs in 4065% of boys during SMR23 due to a relative excess of estrogenicstimulation. Gynecomastia sufficient to cause embarrassment and social disability occurs in fewer than 10% of boys. Breast swelling <4 cm indiameter has a 90% chance of spontaneous resolution within 3 yr. Gynecomastia presenting later in puberty, occurring in the prepubertal period,or occurring in the absence of signs of pubertal development may be pathologic and requires a work-up, including a thorough medication (e.g.,H2-blockers, psychotropics), drug (e.g., anabolic steroids), and medical history (e.g., Klinefelter syndrome, testicular failure, thyroid disease,tumor).
For both sexes, growth acceleration begins in early adolescence, but peak growth velocities are not reached until SMR34. Boys typically peak23 yr later than girls, begin this growth at a later SMR stage, and continue their linear growth for approximately 23 yr after girls have stopped.The asymmetric growth spurt begins distally, with enlargement of the hands and feet, followed by the arms and legs, and finally, the trunk andchest, giving young adolescents a gawky appearance. Rapid enlargement of the larynx, pharynx, and lungs leads to changes in vocal quality,typically preceded by vocal instability (voice cracking). Elongation of the optic globe often results in nearsightedness. Dental changes includejaw growth, loss of the final deciduous teeth, and eruption of the permanent cuspids, premolars, and finally, molars. Orthodontic appliances maybe needed.
GrowthTABLE 14-2 -- Formulas for Approximate Average Height and Weight of Normal Infants and ChildrenWEIGHT KILOGRAMS (POUNDS)At birth 3.25 (7)312 mo (age [mo] + 11)
Body proportions follow a predictable sequence of changes with development. The head and trunk are relatively large at birth, with progressivelengthening of the limbs throughout development, particularly during puberty. The lower body segment is defined as the length from thesymphysis pubis to the floor, and the upper body segment is the height minus the lower body segment. The ratio of upper body segmentdivided by lower body segment (U/L ratio) equals approximately 1.7 at birth, 1.3 at 3 yr of age, and 1.0 after 7 yr of age. Higher U/L ratios arecharacteristic of short-limb dwarfism or bone disorders, such as rickets.
Reference standards for bone maturation facilitate estimation of bone age. Bone age correlates well with stage of pubertal development and canbe helpful in predicting adult height in early- or late-maturing adolescents. In familial short stature, the bone age is normal (comparable tochronological age). In constitutional delay, endocrinologic short stature, and undernutrition, the bone age is low and comparable to the heightage. Skeletal maturation is linked more closely to sexual maturity rating than to chronological age. It is more rapid and less variable in girls thanin boys.
Dental development includes mineralization, eruption, and exfoliation. Initial mineralization begins as early as the 2nd trimester (mean age forcentral incisors, 14 wk) and continues through 3 yr of age for the primary (deciduous) teeth and 25 yr of age for the permanent teeth.Mineralization begins at the crown and progresses toward the root. Eruption begins with the central incisors and progresses laterally. Exfoliationbegins at about 6 yr of age and continues through 12 yr of age. Eruption of the permanent teeth may follow exfoliation immediately or may lagby 45 mo. The timing of dental development is poorly correlated with other processes of growth and maturation. Delayed eruption is usuallyconsidered when there are no teeth by approximately 13 mo of age (mean + 3 standard deviations). Common causes include hypothyroid,hypoparathyroid, familial, and (the most common) idiopathic. Individual teeth may fail to erupt because of mechanical blockage (crowding, gum
7fibrosis). Causes of early exfoliation include histiocytosis X, cyclic neutropenia, leukemia, trauma, and idiopathic factors. Nutritional andmetabolic disturbances, prolonged illness, and certain medications (tetracycline) commonly result in discoloration or malformations of the dentalenamel. A discrete line of pitting on the enamel suggests a time-limited insult.
NutritionThe Feeding of Infants and ChildrenTABLE 42-2 -- Important Principles for Weaning Begin at 6 mo of age Avoid foods with high allergenic potential (cow's milk, eggs, fish, nuts, soybeans). At the proper age, encourage a cup rather than a bottle. Introduce 1 food at a time. Energy density should exceed that of breast milk. Iron-containing foods (meat, iron-supplemented cereals) are required. Zinc intake should be encouraged with foods such as meat, dairy products, wheat, and rice. Phytate intake should be low to enhance mineral absorption. Breast milk should continue to 12 mo; formula or cow's milk is then substituted.Give no more than 24 oz/day of cow's milk. Fluids other than breast milk, formula, and water should be discouraged. Give no more than 46 oz/day of fruit juices. No soda. Allergy prevention: promotion of breast-feeding with maternal exclusion of peanut and nut products from the mother's diet and delay in introducing major allergenic foods: cow's milk until 1 yr of age; egg until 1824 mo of age; and peanut, tree nuts, and seafood until 3 yr of age
8TABLE 45-1 -- Vitamins A, B Complex, and CNAMES AND SYNONYMS CHARACTERISTICS BIOCHEMICAL ACTION EFFECTS OF DEFICIENCY EFFECTS OF EXCESS SOURCESVITAMIN ARetinol (vitamin A1); Fat-soluble;heat- In vision, as retinal, for synthesis of Nyctalopia;photophobia, xerophthalmia, Anorexia, slow growth, Liver, fish liver oils, dairy stable;destroyed oxidation, the visual pigments rhodopsin and Bitot spots, conjunctivitis, keratomalacia drying and cracking of products, except skim1 g retinol = 3.3 IU by vitamin A = drying; bile necessary for iodopsin; in growth, reproduction, leading to blindness; faulty epiphyseal bone skin, enlargement of liver milk; egg yolk, fortified1 RAE. absorption; stored in liver; embryonic and fetal development, formation; defective tooth enamel; and spleen, swelling and margarines and fortifiedProvitamins A:the plant pigments -, protected by vitamin E bone growth, immune and epithelial keratinization of mucous membranes and pain of long bones, bone skim milk; carotenoids-, and -carotenes and functions, via retinoic acid as a skin; retarded growth; impaired resistance fragility, increased from plants: greencryptoxanthin have partial retinol ligand for specific nuclear to infection, anemia, reproductive failure, intracranial pressure, vegetables, yellow fruitsactivity: 12 g -carotene, or 24 g transcription factors, regulating fetal abnormalities alopecia, carotenemia; and vegetablesof other provitamin A carotenoids = genes involved in many fundamental fetal abnormalities1 g retinol cellular processes
VITAMIN B COMPLEXThiamin:vitamin B1; (antiberiberi Water and alcohol soluble; fat- Component of thiamine Beriberi, fatigue, irritability, anorexia, None from oral intake Meat, especially pork;vitamin) insoluble;stable in slightly acid pyrophosphate involved in oxidative constipation, headache, insomnia, whole-grain or enriched solution; labile to heat, alkali, decarboxylation of -keto acids, tachycardia, polyneuritis, cardiac failure, cereals; legumes; nuts, sulfites such as pyruvate, and in edema, elevated pyruvic acid in blood wheat germ; liver transketolation reactionsRiboflavin:vitamin B2 Sparingly soluble in water; Constituent of flavoprotein enzymes Ariboflavinosis;photophobia blurred vision, Not harmful Milk, cheese; whole-grain sensitive to light and alkali; important in oxidation-reduction burning and itching of eyes, corneal or enriched grains; meat, stable to heat, alkali, oxidation, reactions: amino acid, fatty acid, and vascularization, poor growth, cheilosis fish; eggs;green leafy acid carbohyrate metabolism and cellular vegetables; liver and other respiration organ meatsNiacin:nicotinamide;nicotinc acid Water- and alcohol- Constituent of NAD and NADP, Pellagra, multiple B-vitamin deficiency Nicotinic acid (not the Meat, fish, poultry; whole-(antipellagra vitamin) soluble;stable to acid, alkali, coenzymes in numerous oxidation- syndrome, diarrhea, dementia, dermatitis amide) is vasodilator; grain and enriched cereals; light, heat, oxidation reduction reactions Irritability, convulsions, hypochromic skin flushing and itching; green vegetables; peanuts; anemia; peripheral neuritis in patients hepatopathy liver;also from conversion receiving isoniazid; oxaluria of trytophan to niacinVitamin B6 active forms: pyridoxine, Water-soluble;destroyed by Constituent of coenzymes for Sensory neuropathy Meat, fish, poultry; whole-pyridoxal, pyridoxamine ultraviolet light and by heat decarboxylation, transamination, (from high-dose grain and fortified cereals; trans-sulfuration;fatty acid supplements, not food) soybeans;nuts; potatoes; metabolism; heme synthesis; noncitrus fruits; liver and homocysteine metabolism kidneyBiotin Crystallized from yeast; soluble Coenzyme carboxylases; involved in Dermatitis, seborrhea; inactivated by avidin Unknown Widely distributed in in water CO2 transfer in raw egg white foods; animal products, yeast, liverPantothenic acid Limited data on stability during Component of coenzyme A and acyl Experimentally produced deficiency in Unknown Widely distributed in cooking and food processing carrier protein involved in fatty acid humans: irritability, fatigue, gastric foods; beef, poultry, whole metabolism complaints, numbness, paresthesias, muscle grains, liver and kidney, cramps yeast, egg yolks
9NAMES AND SYNONYMS CHARACTERISTICS BIOCHEMICAL ACTION EFFECTS OF DEFICIENCY EFFECTS OF EXCESS SOURCESFolate:folic acid, folacin; a group of Slightly soluble in water: labile Concerned with formation and Megaloblastic anemia (infancy, pregnancy) Unknown Green vegetables, enrichedrelated compounds containing to heat, light, acid metabolism of 1-carbon units; usually secondary to malabsorption disease, grain products, orangespteridine ring, para-amino benzoic participates in synthesis of purines, glossitis, pharyngeal ulcers, impaired and other fruits, legumes,acid, and glutamic acid; pyrimidines, nucleoproteins, immunity nuts, liver, yeastpteroylglutamic acid homocysteine metabolismVitamin B12:cyanocobalamin Slightly soluble in water; stable Transfer of 1-carbon units in purine Pernicious anemia due to defect in Unknown Animal foods: muscle and to heat in neutral solution; and labile methyl group metabolism; absorption rather than dietary lack; also organ meats, fish; labile in acid or alkaline ones; essential for maturation of red blood secondary to gastrectomy, celiac disease, eggs;milk;cheese; fortified destroyed by light; castle cells in bone marrow; metabolism of inflammatory lesions of small bowel, long- cereal products; fortified intrinsic factor of the stomach nervous tissue; homocysteine term drug therapy (PAS, neomycin); soy products required for absorption metabolism; Adenosylcobalamin is methylmalonic aciduria; homocystinuria coenzyme for methylmalonyl coenzyme A mutaseVITAMIN CAscorbic acid, antiscorbutic vitamin Water-soluble;easily oxidized, As an antioxidant, maintains Fe and Scurvy:poor wound healing, bleeding gums, Adverse effects usually Citrus fruits, tomatoes, accelerated by heat, light, Cu ions in reduced state in petechiae, ecchymoses, follicular not serious; may include berries, cantaloupe, alkali, oxidative enzymes, hydroxylases involved in collagen hyperkeratosis, arthralgia osmotic diarrhea, other cabbage, broccoli, traces of copper or iron synthesis, metabolism of cholesterol gastrointestinal cauliflower, spinach, and neurotransmitters; may be symptoms; oxaluria potatoes; cooking has needed to maintain folate in a destructive effect reduced form; facilitates non-heme Fe absorption and Fe transfer from tansferritin to ferritinNAD(P), Nicotinamide adenine dinucleotide (phosphate); PAS, para-aminosalicylic acid
TABLE 48-1 -- Physical and Metabolic Properties and Food Sources of the Vitamins (D, E, and K) EFFECTS OFNAMES AND SYNONYMS CHARACTERISTICS BIOCHEMICAL ACTION DEFICIENCY EFFECTS OF EXCESS SOURCESVITAMIN DVitamin D3 (3-cholecalciferol), Fat-soluble, stable to heat, acid alkali, Necessary for gastrointestinal Rickets in growing Hypercalcemia, which may cause Exposure to sunlightwhich is synthesized in the skin, and oxidation; bile necessary for absorption of calcium; also increases children; osteomalacia; emesis, anorexia, pancreatitis, (ultraviolet light); fishand vitamin D2 (from plants or absorption; hydroxylation in the liver and absorption of phosphate; direct hypocalcemia may cause hypertension, arrhythmias, central oils, fatty fish, egg yolks,yeast) are biologically equivalent; 1 kidney necessary for actions on bone, including mediating tetany and seizures nervous system effects, polyuria, and vitamin Dfortifiedg = 40 IU vitamin D biologic resorption nephrolithiasis, and renal failure formula, milk, cereals,activity and breadVITAMIN EGroup of related compounds with Fat-soluble;readily oxidized by oxygen, Antioxidant;protection of cell Red cell hemolysis in Unknown Vegetable oils, seeds,similar biologic activities; - iron, rancid fats; bile acids necessary for membranes from lipid peroxidation premature infants; nuts, green leafytocopherol is the most potent and absorption and formation of free radicals posterior column and vegetables, andthe most common form cerebellar dysfunction; margarine pigmentary retinopathy
10 EFFECTS OFNAMES AND SYNONYMS CHARACTERISTICS BIOCHEMICAL ACTION DEFICIENCY EFFECTS OF EXCESS SOURCESVITAMIN KGroup of naphthoquinones with Natural compounds are fat-soluble; Vitamin Kdependent proteins Hemorrhagic Not established; analogues (no Green leafy vegetables,similar biologic activities; K1 stable to heat and reducing agents; labile include coagulation factors II, VII, manifestations; long-term longer used) caused hemolytic liver, and certain legumes(phylloquinone) from diet; K2 to oxidizing agent, strong acids, alkali, IX, and X;proteins C, S, Z; matrix Gla bone and vascular health anemia, jaundice, kernicterus, and and plant oils; widely(menaquinones) from intestinal light; bile salts necessary for intestinal protein, osteocalcin death distributedbacteria absorption
11Fluids, Electrolytes, and Acid BaseAcid-Base DisordersTABLE 52-11 -- Appropriate Compensation During Simple Acid-Base DisordersDISORDER EXPECTED COMPENSATIONMetabolic acidosis PCO2 = 1.5 [HCO3-] + 8 2Metabolic alkalosis PCO2 increases by 7 mm Hg for each 10 mEq/L increase in serum -[HCO3 ]Respiratory acidosisAcute [HCO3-] increases by 1 for each 10-mm Hg increase in PCO2Chronic [HCO3-] increases by 3.5 for each 10-mm Hg increase in PCO2Respiratory alkalosisAcute [HCO3-] falls by 2 for each 10-mm Hg decrease in PCO2 [HCO -Chronic 3 ] falls by 4 for each 10-mm Hg decrease in PCO2
DehydrationTABLE 54-1 -- Clinical Evaluation of Dehydration Mild dehydration (<5% in an infant; <3% in an older child or adult): normal or increased pulse; decreased urine output; thirsty; normal physical findings Moderate dehydration (510% in an infant; 36% in an older child or adult): tachycardia; little or no urine output; irritable/lethargic;sunken eyes and fontanel; decreased tears; dry mucous membranes; mild delay in elasticity (skin turgor); delayed capillary refill (>1.5 sec); cool and pale Severe dehydration (>10% in an infant; >6% in an older child or adult): rapid and weak or absent peripheral pulses; decreased blood pressure; no urine output; very sunken eyes and fontanel; no tears; parched mucous membranes; delayed elasticity (poor skin turgor); very delayed capillary refill (>3 sec); cold and mottled; limp, depressed consciousness
Rapid volume repletion: 20 mL/kg normal saline or Ringer Lactate (maximum = 1 L) over 2 hr Calculate 24-hr fluid needs: maintenance + deficit volume Subtract isotonic fluid already administered from 24 hr fluid needs Administer remaining volume over 24 hr using D5 normal saline + 20 mEq/L KCl Replace ongoing losses as they occur
12Ludans Method
< 2 y/oMild 5% wt loss 50 ml/kgMod 10% wt loss 100 ml/kgSevere 15% wt loss 150 ml/kg
PoisoningsTABLE 58-2 -- Historical and Physical Findings in PoisoningODORBitter almonds CyanideAcetone Isopropyl alcohol, methanol, paraldehyde, salicylatesAlcohol EthanolWintergreen Methyl salicylateGarlic Arsenic, thallium, organophosphatesOCULAR SIGNSMiosis Narcotics (except meperidine), organophosphates, muscarinic mushrooms, clonidine, phenothiazines, chloral hydrate, barbiturates (late), PCPMydriasis Atropine, alcohol, cocaine, amphetamines, antihistamines, cyclic antidepressants, cyanide, carbon monoxideNystagmus Phenytoin, barbiturates, ethanol, carbon monoxideLacrimation Organophosphates, irritant gas or vaporsRetinal hyperemia MethanolPoor vision Methanol, botulism, carbon monoxideCUTANEOUS SIGNSNeedle tracks Heroin, PCP, amphetaminesBullae Carbon monoxide, barbituratesDry, hot skin Anticholinergic agents, botulism
13Diaphoresis Organophosphates, nitrates, muscarinic mushrooms, aspirin, cocaineAlopecia Thallium, arsenic, lead, mercuryErythema Boric acid, mercury, cyanide, anticholinergicsORAL SIGNSSalivation Organophosphates, salicylates, corrosives, strychnineDry mouth Amphetamines, anticholinergics, antihistamineBurns Corrosives, oxalate-containing plantsGum lines Lead, mercury, arsenicDysphagia Corrosives, botulismINTESTINAL SIGNSCramps Arsenic, lead, thallium, organophosphatesDiarrhea Antimicrobials, arsenic, iron, boric acidConstipation Lead, narcotics, botulismHematemesis Aminophylline, corrosives, iron, salicylatesCARDIAC SIGNSTachycardia Atropine, aspirin, amphetamines, cocaine, cyclic antidepressants, theophyllineBradycardia Digitalis, narcotics, mushrooms, clonidine, organophosphates, blockers, calcium channel blockersHypertension Amphetamines, LSD, cocaine, PCPHypotension Phenothiazines, barbiturates, cyclic antidepressants, iron, blockers, calcium channel blockersRESPIRATORY SIGNSDepressed Alcohol, narcotics, barbituratesrespirationIncreased Amphetamines, aspirin, ethylene glycol, carbon monoxide, cyaniderespirationPulmonary edema Hydrocarbons, heroin, organophosphates, aspirinCNS SIGNSAtaxia Alcohol, antidepressants, barbiturates, anticholinergics, phenytoin, narcoticsComa Sedatives, narcotics, barbiturates, PCP, organophosphates, salicylates, cyanide, carbon monoxide, cyclic antidepressants, leadHyperpyrexia Anticholinergics, quinine, salicylates, LSD, phenothiazines, amphetamines, cocaineMuscle fasciculation Organophosphates, theophyllineMuscle rigidity Cyclic antidepressants, PCP, phenothiazines, haloperidolParesthesia Cocaine, camphor, PCP, MSGPeripheral Lead, arsenic, mercury, organophosphatesneuropathyAltered behavior LSD, PCP, amphetamines, cocaine, alcohol, anticholinergics, camphor
15POISON SYNDROME ASSOCIATED SIGNS POSSIBLE TOXINSRenal failure Oliguria or anuria Carbon tetrachloride Hematuria Ethylene glycol Myoglobinuria Methanol Mushrooms Oxalates
Oropharyngeal pain and Lip, mouth, and pharyngeal ulcerations and burning pain Paraquat[*]ulcerations Dyspnea and hemoptysis secondary to pulmonary edema or Diquat hemorrhage; can progress to pulmonary fibrosis over days to weeks Caustics (i.e., acids and alkalis) Inorganic mercuric salts Mustards (e.g., sulfur)
Cellular hypoxia Mild: Nausea, vomiting, and headache Cyanide[*] (e.g., hydrogen cyanide gas or sodium cyanide) Severe: Altered mental status, dyspnea, hypotension, seizures, and metabolic acidosis Sodium monofluoroacetate [] (SMFA)[*] Bitter almond odor Carbon monoxide Hypocalcemia or hypokalemia Hydrogen sulfide Sodium azide Methemoglobin-causing agents
Peripheral neuropathy and/or Peripheral neuropathy signs and symptoms: Muscle Mercury (organic)[*]neurocognitive effects weakness and atrophy,glove and stocking sensory loss, and depressed or absent deep tendon reflexes Arsenic (inorganic)[*]
Severe gastrointestinal illness, Abdominal pain, vomiting, profuse diarrhea (possibly Arsenic[*]dehydration bloody), and hypotension, possibly followed by multisystem organ failure Ricin[*]
Serotonin Altered mental status (agitation, confusion, coma), autonomi SSRIs, antidepressants, some opioids instability (tachycardia, hyper- or hypotension), hyperkinetic (meperidine), tramadol, St. John's wort, neuromuscular (tremor, clonus, hyperreflexia), mydriasis, MAOIs diaphoresis, increased bowel motilityWithdrawal Abdominal cramps, diarrhea, lacrimation, sweating,goose flesh, Cessation of alcohol, barbiturates, yawning, tachycardia, restlessness, hallucinations benzodiazepines, narcotics
16TABLE 58-4 -- Screening Laboratory Clues Chloral hydrateMETABOLIC ACIDOSIS (MNEMONIC = MUDPIES) Cocaine Cyanide Methanol,[*] carbon monoxide Cyclic antidepressants Uremia[*] Digitalis Diabetes mellitus[*] Freon Paraldehyde,[*] phenformin Phenothiazines Isoniazid, iron Physostigmine Ethanol,[*] ethylene glycol[*] Propranolol Salicylates, starvation, seizures Quinine, quinidine
HYPOGLYCEMIA Theophylline
CAUSES OF COMA Ethanol Isoniazid Alcohol Insulin Anticholinergics
Propranolol Antihistamines Barbiturates Oral hypoglycemic agents Carbon monoxide Salicylates ClonidineHYPERGLYCEMIA Cyanide Salicylates Cyclic antidepressants Isoniazid Hypoglycemic agents
Iron Lead
Phenothiazines Lithium Methemoglobinemia[*] Sympathomimetics MethyldopaHYPOCALCEMIA Narcotics Oxalate Phencyclidine Ethylene glycol Phenothiazines Fluoride Salicylates
17* Causes of methemoglobinemia: amyl nitrite, aniline dyes, TABLE 709-4 -- Summary of Recommendations for Children benzocaine, bismuth subnitrate, dapsone, primaquone, quinones, with Confirmed (Venous) Elevated Blood Lead Concentrations spinach, sulfonamides. CONCENTRATION RECOMMENDATIONS 1014 g/dL Lead educationTABLE 58-6 -- Common Antidotes for PoisoningANTIDOTE POISONING DietaryN-Acetylcysteine Acetaminophen;carbon tetrachloride and Environmental(Mucomyst) chloroform (experimental) Follow-up blood lead monitoring in 13 moN-Acetylcysteine Acetaminophen 1519 g/dL Lead education(Acetodote) DietaryAtropine Organophosphate and carbamate Environmental pesticides; bradycardia due to atrioventricular conduction defects, - Follow-up blood lead monitoring in 12 mo blocking agents Proceed according to actions for 2024 g/dLBAL in oil (dimercaprol) Arsenic, mercury, other metals if A follow-up blood lead concentration is in this range at least 3 mo after initial venousBenztropine (Cogentin) Acute dystonic reactions test; or Blood lead concentration increasesCyanide antidote kit Cyanide 2044 g/dL Lead education Hydrogen sulfide (nitrites only) Dietary EnvironmentalDeferoxamine (Desferal) Iron Follow-up blood lead monitoring in 1 wk1 mo (sooner if higher) Complete history and physical examinationDigoxin-specific Digitalis glycosides (synthetic or natural) Laboratory studiesFab antibodies (Digibind) Hemoglobin or hematocrit Iron statusDimercaptosuccinic acid Lead and probably mercury, arsenic, and(succimer, DMSA, perhaps other metals Environmental investigationChemet) Lead hazard reductionDiphenhydramine Extrapyramidal symptoms, acute dystonic Neurodevelopmental monitoring(Benadryl) reactions, allergic reactions Abdominal radiography (if particulate leadEDTA, calcium (calcium Lead, manganese, nickel, zinc, and ingestion is suspected) with boweldisodium, Versenate) perhaps chromium decontamination if indicatedEthanol (ethyl alcohol) Methanol, ethylene glycol 4569 g/dL Lead educationFlumazenil (Romazicon) Benzodiazepines Dietary Environmental Follow-up blood lead monitoringFomepizole (4- Ethylene glycol, methanol Complete history and physical examinationmethylpyrazole, Antizole) Laboratory studies Hemoglobin or hematocritGlucagon Blockers, calcium channel blockers, Iron status hypoglycemic agents Free EP or ZPPMethylene blue Methemoglobinemia Environmental investigationNaloxone (Narcan) Narcotics Lead hazard reduction Clonidine (inconsistent response) Neurodevelopmental monitoringOctreotide Sulfonylureas Abdominal radiography with bowel decontamination if indicated ChelationPhysostigmine Anticholinergic agents therapy(Antilirium) 70 g/dL Hospitalize and commence chelation therapy Proceed according to actions for 4569 g/dLPralidoxime (2-PAM, Organophosphate insecticidesProtopam) NOT RECOMMENDED AT ANY BLOOD LEAD CONCENTRATIONPyridoxine (Vitamin B6) Isoniazid, Gyromitra mushrooms Searching for gingival lead lines Ethylene glycol (investigational) Evaluation of renal function (except during chelation withOxygen Carbon monoxide CaNa2EDTA)Vitamin K Coumarin Testing of hair, teeth, or fingernails for leadBAL, British antilewisite; DMSA, dimercaptosuccinic acid; EDTA, Radiographic imaging of long bonesethylene diamine tetraacetic acid; ET, endotracheal; IM, intramuscular;IV, intravenous; max, maximum; NS, normal saline; PO, Oral. X-ray fluorescence of long bones
18TABLE 58-9 -- Classic Stages in the Clinical Course of Acetaminophen ToxicitySTAGE TIME AFTER CHARACTERISTICS INGESTIONI 0.524 hr Anorexia, nausea, vomiting, malaise, pallor, diaphoresisII 2448 hr Resolution of earlier symptoms; right upper quadrant abdominal pain and tenderness; elevated bilirubin, prothrombin time, hepatic enzymes; oliguriaIII 7296 hr Peak liver function abnormalities; anorexia, nausea, vomiting, and malaise may reappearIV 4 days2 wk Resolution of hepatic dysfunction or complete liver failure
19TABLE 66-5 -- Glasgow Coma ScaleEYE OPENING (TOTAL POINTS 4)Spontaneous 4To voice 3To pain 2None 1VERBAL RESPONSE (TOTAL POINTS 5)Older Children Infants and Young ChildrenOriented 5 Appropriate words; smiles, fixes, and follows 5Confused 4 Consolable crying 4Inappropriate 3 Persistently irritable 3Incomprehensible 2 Restless, agitated 2None 1 None 1MOTOR RESPONSE (TOTAL POINTS 6)Obeys 6Localizes pain 5Withdraws 4Flexion 3Extension 2None 1
Children 1 wk2 mo of age: fulfillment of the criteria listed in Table 67-2 in 2 separate examinations at least 48 hr apart, or 1 clinical examination followed by an isoelectric EEG at least 48 hr later Children 2 mo1 yr: fulfillment of the clinical criteria described in Table 67-2 in 2 separate examinations, at least 24 hr apart, or 1 clinical examination followed by an isoelectric EEG or negative results of a cerebral blood flow study at least 24 hr later Children >1 yr: fulfillment of the clinical criteria described in Table 67-2 in 2 separate examinations at 12 hr apart
1. A recognized cause of coma, sufficient to explain the History, clinical examination, laboratory, technical investigations irreversible cessation of all brain function 2. Potentially reversible causes of coma must be excluded:
d. Uncontrolled hypotension e. Surgically remediable intracranial conditions f. Any other sign that suggests a potentially reversible cause of coma
1. Absence of higher brain function Lack of consciousness, voluntary movement or responsiveness except for spinal reflexes (stimuli applied to any body region may not elicit a motor response within the cranial nerve distribution); preferably test in a cranial (trigeminal) dermatome rather than a spinal dermatome; no decorticate or decerebrate posturing, no convulsions
a. Absence of sympathetic and parasympathetic regulation of the Pupils in midposition or dilated, showing neither direct nor indirect reactionpupils to light b. Disruption of the pathways controlling eye movement in the Absence of spontaneous eye movement, absence of reaction to injection ofbrainstem iced water into the ear (vestibulo-ocular reflex), absence of doll's eye phenomenon (oculocephalic reflexes) c. Disruption of the afferent trigeminal and efferent facial nerve Absence of blink response to (careful) corneal stimulationpathways d. Disruption of afferent and efferent pathways of cranial nerves Absence of gag response to stimulation of the posterior pharynx, absence ofIX and X in the medulla oblongata cough on suctioning of the tracheae. Absence of vagal efferent activity No significant increase of heart rate on administration of intravenous atropine or on pressure applied to the eyeballs (oculocardiac reflex) f. Disruption of the respiratory control centers of the medulla No respiratory movement (as assessed by observation capnography) at aoblongata PaCO2 above a set limit during standardized apnea testingCONFIRMATORY TESTS
1. Confirmation of absence of higher brain function Electrocerebral silence on EEG during at least 30 min
2. Confirmation of complete infarction of the brain and Four-vessel contrast angiography or radionuclide imaging brainstem by confirmation of absence of blood flow
OBSERVATIONConfirmation of irreversibility Observation during a set time, repeat formal physical examination and confirmatory tests
TraumaPEDIATRIC TRAUMA SCORE[]COMPONENT CATEGORY +2 +1 -1Size >20 kg 1020 kg <10 kgAirway Normal Maintainable UnmaintainableSystolic BP >90 mm Hg 9050 mm Hg <50 mm HgCNS Awake Obtunded/LOC Coma/decerebrateOpen wound None Minor Major/penetratingSkeletal None Closed fracture Open/multiple fractures
21Genetics and MetabolismTABLE 81-7 -- Microdeletion/Contiguous Gene Syndromes and Their Clinical ManifestationsDELETION SYNDROME CLINICAL MANIFESTATIONS7q11.23 Williams Round face with full cheeks and lips, stellate pattern in iris, strabismus, supravalvular aortic stenosis and other cardiac malformations, varying degrees of mental retardation, friendly personality11p13 WAGR Hypernephroma (Wilms tumor), aniridia, male genital hypoplasia of varying degrees, gonadoblastoma, long face, upward slanting palpebral fissures, ptosis, beaked nose, low-set poorly formed auricles, mental retardation15q11-q13 Prader-Willi Severe hypotonia at birth, obesity, short stature (responsive to growth(pat) hormone), small hands and feet, hypogonadism, mental retardation15q11-q13 Angelman Hypotonia, fair hair, midface hypoplasia, prognathism, seizures,(mat) jerky ataxic movements, uncontrollable bouts of laughter, severe mental retardation20p12 Alagille syndrome Bile duct paucity with cholestasis;heart defects, particularly pulmonary artery stenosis;ocular abnormalities (posterior embryo toxin);skeletal defects such as butterfly vertebrae;long nose with broad mid-nose22q11.2 Velocardiofacial- Hypoplasia or agenesis of the thymus and parathyroid glands, DiGeorge syndrome hypoplasia of auricle and external auditory canal, conotruncal cardiac anomalies, cleft palate, short stature, behavioral problemsTABLE 84-3 -- Inborn Errors of Amino Acid Metabolism Associated with Peculiar OdorINBORN ERROR OF METABOLISM URINE ODORGlutaric acidemia (type II) Sweaty feet, acridHawkinsinuria Swimming poolIsovaleric acidemia Sweaty feet, acridMaple syrup urine disease Maple syrupHypermethioninemia Boiled cabbageMultiple carboxylase deficiency Tomcat urineOasthouse urine disease Hops-likePhenylketonuria Mousey or mustyTrimethylaminuria Rotting fishTyrosinemia Boiled cabbage, rancid butter
22NeonatologyThe FetusTABLE 6-4 -- Milestones of Prenatal DevelopmentWK DEVELOPMENTAL EVENTS1 Fertilization and implantation; beginning of embryonic period2 Endoderm and ectoderm appear (bilaminar embryo)3 First missed menstrual period; mesoderm appears (trilaminar embryo); somites begin to form4 Neural folds fuse; folding of embryo into human-like shape; arm and leg buds appear; crown-rump length 45 mm5 Lens placodes, primitive mouth, digital rays on hands6 Primitive nose, philtrum, primary palate; crown-rump length 2123 mm7 Eyelids begin8 Ovaries and testes distinguishable9 Fetal period begins; crown-rump length 5 cm; weight 9 g10 External genitals distinguishable20 Usual lower limit of viability; weight 460 g; length 19 cm25 Third trimester begins; weight 900 g; length 25 cm28 Eyes open; fetus turns head down; weight 1,000 g38 Term
By 12 wk, the gender of the external genitals becomes clearly distinguishable. Lung development proceeds, with the budding of bronchi, bronchioles, and successively smaller divisions. By 2024 wk, primitive alveoli have formed and surfactant production has begun; before that time, the absence of alveoli renders the lungs useless as organs of gas exchange. Muscle contractions first appear around 8 wk, soon followed by lateral flexion movements. By 1314 wk, breathing and swallowing motions appear and tactile stimulation elicits graceful movements. The grasp reflex appears at 17 wk and is well developed by 27 wk. Eye opening occurs around 26 wk. If a single ultrasound examination is performed, the most information can be obtained with a scan at 1820 wk, when both gestational age and fetal anatomy can be evaluated. Determination of the extent of calcification by ultrasound (placental maturity index), detection of the 1st audible fetal heart tones (1618 wk), and observation of the initial fetal movements (1820 wk) may also aid in evaluating the maturity of a fetus. Second-trimester screening (1518 wk) of maternal serum -fetoprotein (MSAFP) levels is used to screen for open neural tube defects. About 90% of affected pregnancies can be detected by an elevated MSAFP level. Gastroschisis, omphalocele, congenital nephrosis, twins, and other abnormal conditions can also be identified. Low MSAFP is associated with incorrect gestational age estimates, trisomy 18 or 21, and intrauterine growth restriction.
23Neonatal JaundiceTABLE 102-2 -- Risk Factors for Development of SevereHyperbilirubinemia in Infants of 35 or More Week'sGestation (in Approximate Order of Importance) Necrotizing EnterocolitisMajor risk factors The clinical syndrome has been classified into stages byPredischarge TSB or TcB level in the high-risk zone Walsh and Kliegman (1986) to include systemic, intestinal, and radiographic findings.Jaundice observed in the first 24 hr Blood group incompatibility with positive direct antiglobulin A. Stage I: suspected NECtest, other known hemolytic disease (G6PD deficiency), 1. Systemic signs are nonspecific, including apnea,elevated ETCOc bradycardia, lethargy, and temperature instability. 2. Intestinal findings include feeding intolerance, recurrentGestational age 3536 wk gastric residuals, and guaiacpositive stools.Previous sibling received phototherapy 3. Radiographic findings are normal or nonspecific.
24Blood DisordersTABLE 103-4 -- Hemorrhagic Disease of the Newborn EARLY ONSET CLASSIC DISEASE LATE ONSETAge 024 hr 27 days 16 moSite of Cephalohematoma Gastrointestinal Intracranialhemorrhage Subgaleal Ear-nose-throatmucosal Gastrointestinal Intracranial Intracranial Cutaneous Gastrointestinal Circumcision Ear-nose-throatmucosal Umbilicus Cutaneous Injection sites Intra-abdominal Gastrointestinal Thoracic Injection sitesEtiology/risks Maternal drugs (phenobarbital, Vitamin K deficiency Cholestasismalabsorption phenytoin, warfarin, rifampin, of vitamin K (biliary atresia, isoniazid) that interfere with cystic fibrosis, hepatitis) vitamin K Inherited coagulopathy Breast-feeding Abetalipoprotein deficiency Idiopathic in Asian breast- fed infants Warfarin ingestionPrevention Posible vitamin K at birth or to Prevented by parenteral Prevented by parenteral and mother (20 mg) before birth vitamin K at birth. Oral high-dose oral vitamin K vitamin K regimens require during periods of repeated dosing over time malabsorption or cholestasis Avoid high-risk medicationsIncidence Very rare 2% if not given vitamin K Dependent on primary disease The swallowed blood syndrome, in which blood or bloody stools are passed, usually on the 2nd or 3rd day of life, may be confused with hemorrhage from the gastrointestinal tract. The blood may be swallowed during delivery or from a fissure in the mother's nipple. Differentiation from gastrointestinal hemorrhage is based on the fact that the infant's blood contains mostly fetal hemoglobin, which is alkali-resistant, whereas swallowed blood from a maternal source contains adult hemoglobin, which is promptly changed to alkaline hematin after the addition of alkali. Apt devised the following test for this differentiation: (1) Rinse a blood-stained diaper or some grossly bloody (red) stool with a suitable amount of water to obtain a distinctly pink supernatant hemoglobin solution. (2) Centrifuge the mixture and decant the supernatant solution. (3) To five parts of the supernatant fluid add one part of 0.25 N (1%) sodium hydroxide. Within 12 min a color reaction takes place: A yellow-brown color indicates that the blood is maternal in origin; a persistent pink indicates that it is from the infant. A control test with known adult or infant blood, or both, is advisable. A physiologic decrease in hemoglobin content is noticed at 812 wk in term infants (hemoglobin, 11 g/dL) and at about 6 wk in premature infants (710 g/dL). Early ultrasonographic signs of hydrops include organomegaly (liver, spleen, heart), the doublebowel wall sign (bowel edema), and placental thickening. Progression to polyhydramnios, ascites, pleural or pericardial effusions, and skin or scalp edema may then follow.
25Nervous System Disorders Convulsions should be distinguished from the jitteriness that may be present in normal newborns, in infants of diabetic mothers, in those who experienced birth asphyxia or drug withdrawal, and in polycythemic neonates. Jitteriness resembling simple tremors may be stopped by holding the infant's extremity; it often depends on sensory stimuli and occurs when the infant is active, and it is not associated with abnormal eye movements. Tremors are often more rapid with a smaller amplitude than are tonic clonic seizures. Seizures in premature infants are often subtle and associated with abnormal eye (fluttering, deviation, stare) or facial (chewing, tongue thrusting) movements; the motor component is often that of tonic extension of the limbs, neck, and trunk. Term infants may have focal or multifocal, clonic or myoclonic movements, but they may also have more subtle seizure activity. Apnea may be the 1st manifestation of seizure activity, particularly in a premature infant.
Intracranial bleeding may be associated with disseminated intravascular coagulopathy, isoimmune thrombocytopenia, and neonatal vitamin K deficiency, especially in infants born to mothers receiving phenobarbital or phenytoin. TABLE 99-4 -- Hypoxic-Ischemic Encephalopathy in Term Infants SIGNS STAGE 1 STAGE 2 Level of consciousness Hyperalert Lethargic
A low Apgar score at 20 min, absence of spontaneous respirations at 20 min of age, and persistence of abnormal neurologic signs at 2 wk of age also predict death or severe cognitive and motor deficits. Spinal cord injury occurs most commonly at the level of the 4th cervical vertebra with cephalic presentations and the lower cervicalupper thoracic vertebrae with breech presentations. In Erb-Duchenne paralysis, the injury is limited to the 5th and 6th cervical nerves. The infant loses the power to abduct the arm from the shoulder, rotate the arm externally, and supinate the forearm. The characteristic position consists of adduction and internal rotation of the arm with pronation of the forearm. Power to extend the forearm is retained, but the biceps reflex is absent; the Moro reflex is absent on the affected side. Klumpke paralysis is a rare form of brachial palsy; injury to the 7th and 8th cervical nerves and the 1st thoracic nerve produces a paralyzed hand and ipsilateral ptosis and miosis (Horner syndrome) if the sympathetic fibers of the 1st thoracic root are also injured.
The Umbilicus The umbilical cord usually dries and separates within 68 days after birth. The raw surface becomes covered by a thin layer of skin; scar tissue forms, and the wound is usually healed within 1215 days. The presence of saprophytic organisms delays separation of the cord and increases the possibility of invasion by pathogenic organisms. Mild infection or incomplete epithelialization may result in a moist granulating area at the base of the cord with a slight mucoid or mucopurulent discharge. Good results are usually obtained by cleansing with alcohol several times daily. Most umbilical hernias that appear before the age of 6 mo disappear spontaneously by 1 yr of age. Even large hernias (56 cm in all dimensions) have been known to disappear spontaneously by 56 yr of age. Strangulation is extremely rare. It is generally agreed that strapping is ineffective. Surgery is not advised unless the hernia persists to the age of 45 yr, causes symptoms, becomes strangulated, or becomes progressively larger after the age of 12 yrj. Defects exceeding 2 cm are less likely to close spontaneously.
26ImmunologyEvaluation of immune function should be initiated for children with clinical manifestations of a specific immunedisorder or with unusual, chronic, or recurrent infections such as (1) one or more systemic bacterial infections (sepsis,meningitis); (2) two or more serious respiratory or documented bacterial infections (cellulitis, draining otitis media,pneumonia, lymphadenitis) within 1 yr; (3) serious infections occurring at unusual sites (liver, brain abscess); (4)infections with unusual pathogens (Aspergillus, Serratia marcescens, Nocardia, Burkholderia cepacia); and (5)infections with common childhood pathogens but of unusual severity. Additional clues to immunodeficiency include:>8 ear infections per yr; >2 serious sinus infections per yr; >2 mo treatment with antibiotics with poor results; failureto thrive with or without chronic diarrhea; and the need for intravenous antibiotics to successfully treat an infectionusually treated with oral antibiotics.
27TABLE 121-3 -- Characteristic Features of Primary ImmunodeficiencyCHARACTERISTIC PREDOMINANT T-CELL DEFECT PREDOMINANT B-CELL DEFECT GRANULOCYTE DEFECT COMPLEMENT DEFECTAge at the onset of Early onset, usually 26 mo of age Onset after maternal antibodies diminish, Early onset Onset at any ageinfection usually after 57 mo of age, later childhood to adulthoodSpecific pathogens Bacteria: mycobacteria Bacteria: streptococci, staphylococci, Bacteria: staphylococci, Bacteria: Neisseria, Escherichiainvolved Haemophilus, Campylobacter Pseudomonas, Serratia, coli Klebsiella Viruses: CMV, EBV, adenovirus, parainfluenza Viruses: enterovirus[*] 3, varicella, enterovirus Fungi and parasites: Candida; opportunistic Fungi and parasites: giardia, cryptosporidia Fungi and parasites: Candida; infection, PCP Nocardia, AspergillusAffected organs Failure to thrive, protracted diarrhea, extensive Recurrent sinopulmonary infections, Skin abscesses: Infections: meningitis, arthritis, mucocutaneous candidiasis chronic gastrointestinal symptoms, dermatitis, impetigo, septicemia, recurrent malabsorption, arthritis, enteroviral cellulitis sinopulmonary infections meningoencephalitis[*] Lymph nodes: suppurative adenitis Oral cavity: periodontitis, ulcers internal organs, abscesses, osteomyelitis
Special features Graft-versus-host disease caused by maternal Autoimmunity, lymphoreticular Prolonged attachment of Rheumatoid disorders: SLE, engraftment or nonirradiated blood transfusion; malignancy: lymphoma, thymoma; umbilical cord, poor wound vasculitis, dermatomyositis, Postvaccination, disseminated BCG or varicella; postvaccination paralytic polio healing scleroderma, glomerulonephritis, hypocalcemic tetany in infancy[] angioedema
28Allergic DisordersAsthmaTABLE 143-8 -- Classification of Asthma Severity FOR ADULTS AND CHILDREN AGE > 5 YEARS WHO CAN USE A DAYS WITH NIGHTS WITH SPIROMETER OR PEAK FLOWCLASSIFICATION STEP SYMPTOMS SYMPTOMS METER FEV1 or PEF[*] % PEF Variability Predicted Normal (%)Severe persistent 4 Continual Frequent 60 >30Moderate persistent 3 Daily >1/wk >60<80 >30Mild persistent 2 >2/wk, but <1 >2/mo 80 2030 time/dayMild intermittent 1 2/wk <2/mo 80 <20
2930Rheumatic DiseasesJuvenile Rhuematoid ArthritisTABLE 154-1 -- Criteria for the Classification of Juvenile Rheumatoid ArthritisAge at onset: <16 yrArthritis (swelling or effusion, or the presence of 2 or more of the following signs: limitation of range of motion, tenderness orpain on motion, increased heat) in 1 jointsDuration of disease: 6 wkOnset type defined by type of articular involvement in the 1st 6 mo after onset:Polyarthritis: 5 inflamed jointsOligoarthritis: 4 inflamed jointsSystemic disease: arthritis with a characteristic intermittent feverExclusion of other forms of juvenile arthritisOligoarthritis (pauciarticular disease) predominantly affects the joints of the lower extremities, such as the knees and ankles (Fig. 154-3 ). Often, only a single joint is involved at onset. Isolated involvement of upper extremity large joints is notcharacteristic of this type of onset. Involvement of the hip is almost never a presenting sign of JRA. Hip disease may occur later,particularly in polyarticular JRA, and is often a component of a deteriorating functional course.
Polyarthritis (polyarticular disease) is generally characterized by involvement of both large and small joints of both upper andlower extremities. As many as 2040 joints may be affected in the more severely involved child, although inflammation of only5 joints is required as a criterion for classification of this type of onset. Polyarticular disease may resemble the characteristicpresentation of adult rheumatoid arthritis and the HLA profile is often similar. Rheumatoid nodules on the extensor surfaces ofthe elbows and over the Achilles tendons, while unusual, are associated with a more severe course. Micrognathia reflects chronictemporomandibular joint disease. Cervical spine involvement of the apophyseal joints occurs frequently with a risk of atlantoaxialsubluxation and potential neurologic sequelae.
Systemic-onset disease is characterized by arthritis and prominent visceral involvement that includes hepatosplenomegaly,lymphadenopathy, and serositis, such as a pericardial effusion. It is characterized by a quotidian fever with temperatures to 39C,sometimes followed by mildly hypothermic temperatures for 2 wk. Each febrile episode is frequently accompanied by acharacteristic faint, erythematous, macular rash; these evanescent salmon-colored lesions may be linear or circular, from 25 mmin size, and are often distributed in groups with a linear distribution most commonly over the trunk and proximal extremities. Thisrash is not pruritic. Its most diagnostic feature is its transient nature, with a group of lesions usually lasting <1 hr. The Koebnerphenomenon, which is cutaneous hypersensitivity to superficial trauma resulting in a localized recurrence of the rash, issuggestive, but not diagnostic, of systemic-onset disease. Heat, such as a warm bath, also evokes a reappearance of the rash.
Kawasaki DiseaseTABLE 165-1 -- Clinical and Laboratory Features of Kawasaki DiseaseEPIDEMIOLOGIC CASE DEFINITION (CLASSIC CLINICAL CRITERIA)[*]Fever persisting at least 5 days[]Presence of at least 4 principal features: Changes in extremities Acute: Erythema of palms, soles; edema of hands, feet Subacute: Periungual peeling of fingers, toes in weeks 2 and 3 Polymorphous exanthem Bilateral bulbar conjunctival injection without exudate Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae Cervical lymphadenopathy (>1.5 cm diameter), usually unilateralExclusion of other diseases with similar findings[]
31Systemic Lupus ErythematosusTABLE 157-2 -- 1997 Revised Classification Criteria for Systemic Lupus ErythematosusCRITERION[*] DEFINITIONMalar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial foldsDiscoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesionsPhotosensitivity Rash as a result of unusual reaction to sunlight (elicited by patient history or physician observation)Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by a physicianArthritis Non-erosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusionSerositis Pleuritis:convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion or Pericarditis:documented by ECG or rub or evidence of pericardial effusionRenal disorder Persistent proteinuria >0.5 g/day or >3-plus (+ + +) if quantitation not performed or Cellular casts: may be red blood cell, hemoglobin, granular, tubular, or mixedNeurologic Seizures:in the absence of offending drugs or known metabolic derangements (e.g., uremia, ketoacidosis, or electrolytedisorder imbalance) or Psychosis:in the absence of offending drugs or known metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance)Hematologic Hemolytic anemia, with reticulocytosisdisorder Leukopenia: <4,000/mm3 total on two or more occasions Lymphopenia: <1,500/mm3 on two or more occasions Thrombocytopenia: <100,000/mm3Immunologic Anti-DNA antibody to native DNA in abnormal titerdisorder or Anti-Smith:presence of antibody to Smith nuclear antigen or Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of lgG or lgM anticardiolipin antibodies;(2) a positive test result for lupus anticoagulant using a standard method, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 mo and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test (FTA-ABS), Standard methods should be used in testing for the presence of antiphospholipid.Antinuclear An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any time and in the absence ofantibody drugs known to be associated with drug-induced lupus syndromeAn approved modification deletes the positive LE cell preparation from the immunologic disorder criteria and substitutes the presence of abiologic false-positive test for syphilis.* The proposed classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person shall be said to have SLEif any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observa-tion.
Juvenile Dermatomyositis o Heliotrope rash, Gottron papules rash is often florid and is usually palpable over joints, especially the metacarpal phalangeal, intercarpal phalangeal (Gottron papules), knees, elbows, and medial malleoli of the ankles rash often has onset in sun-exposed areas and develops as the first symptom in 50% of cases, and concomitantly with weakness in 25% of cases. The characteristic periorbital violaceous erythema (heliotrope)
32Infectious DiseaseImmunization Vaccine types o whole inactivated microorganisms (polio and hepatitis A) o parts of the organism (acellular pertussis, human papillomavirus [HPV], and HepB) o polysaccharide capsules (pneumococcal and meningococcal polysaccharide vaccines) o polysaccharide capsules conjugated to protein carriers (Hib, pneumococcal, and meningococcal conjugate vaccines) o live attenuated microorganisms (measles, mumps, rubella, varicella, rotavirus, and live attenuated influenza vaccines) o toxoids (tetanus and diphtheria) Vaccine routes o Most inactivated vaccines, including DTaP, hepatitis A, HepB, Hib, TIV, PCV7, MCV4, and Tdap, are administered intramuscularly (IM). o In contrast, the commonly used live attenuated vaccines, MMR and varicella, should be dispensed subcutaneously (SC). o IPV and PPS23 (pneumococcal polysaccharide vaccine) can be given IM or SC. Pneumococcal o Immunologic responsiveness and efficacy following administration of pneumococcal polysaccharide vaccines is unpredictable in children <2 yr of age. Two licensed pneumococcal vaccines contain purified polysaccharide of 23 valent pneumococcal serotypes (PPV23) responsible for >95% of cases of invasive disease. The clinical efficacy of these vaccines is controversial, and studies have yielded conflicting results. Polysaccharide antigens 6A, 14, 19F, and 23F frequently produce childhood disease and are poorly immunogenic in children <5 yr of age. o In contrast, pneumococcal polysaccharide vaccines conjugated to various proteins (heptavalent pneumococcal polysaccharide conjugated to CRM197) provoke protective antibody responses in 90% of infants given these vaccines at 2, 4, and 6 mo of age, and greatly enhanced responses (immunologic memory) are apparent after booster doses given at 1215 mo of age. In addition, protein conjugate-polysaccharide vaccines reduce nasopharyngeal carriage of vaccine serotypes by up to 6070%. The currently available heptavalent vaccine (PCV7) contains conjugated capsular polysaccharides of serotypes 4, 6B, 9V, 14, 19F, 23F, and 18C. Adverse events after the administration of PCV7 have included local swelling and redness and slightly increased rates of fever, when used in conjunction with other childhood vaccines. o Immunization with PCV7 is recommended for all infants in a schedule of 4 doses administered at 2, 4, 6, and 1215 mo of age. High-risk children 2 yr of age (see Table 181-1 ), such as those with asplenia, sickle cell disease, some types of immune deficiency (antibody deficiencies), HIV infection, or chronic lung, heart, or kidney disease (including nephrotic syndrome), may benefit also from PPV23 administered after 2 yr of age and following priming with the scheduled doses of PCV7. After the initial immunization, a single supplemental dose of PPV23 may be used 3 yr after the 1st dose for children <10 yr of age at the time of revaccination, or it may be used at 5 yr after the 1st dose for children 10 yr of age or older at the time of revaccination. Diphtheria o Universal immunization with diphtheria toxoid throughout life, to provide constant protective antitoxin levels and to reduce severity of C. diphtheriae disease, is the only effective control measure. Although immunization does not preclude subsequent respiratory or cutaneous carriage of toxigenic C. diphtheriae, it decreases local tissue spread, prevents toxic complications, diminishes transmission of the organism, and provides herd immunity when at least 7080% of a population is immunized. o The higher-potency (D) formulation of toxoid is used for primary series and booster doses for children through 6 yr of age because of superior immunogenicity and minimal reactogenicity. For individuals 7 years of age or older, dT is recommended for the primary series and booster doses because the lower concentration of diphtheria toxoid is adequately immunogenic and because increasing the content of diphtheria toxoid heightens reactogenicity with increasing age. o For children from 6 wk to 7 yr of age, five 0.5-mL doses of diphtheria-containing (D) vaccine are given in a primary series, including 3 doses at 2, 4, and 6 mo of age, with a 4th dose, an integral part of the primary series, 912 mo after the third dose. A booster dose is given at 46 yr of age (unless the 4th primary dose was administered after the 4th birthday). For persons 7 yr of age and older, three 0.5 mL doses of diphtheria-containing (d) vaccine are given in a primary series of 2 doses 48 wk apart and a 3rd dose 612 mo after the 2nd dose. The only contraindication to tetanus and diphtheria toxoid is a history of neurologic or severe hypersensitivity reaction after a previous dose. For children <7 yr of age in whom pertussis immunization is contraindicated, DT is used. Those begun with DTaP or DT before 1 yr of age should have a total of five 0.5 mL doses of diphtheria-containing (D) vaccines by 6 yr of age. For those beginning at around 1 yr of age, the primary series is three 0.5 mL doses of diphtheria-containing (D) vaccine, with a booster given at 46 yr, unless the 3rd dose was given after the 4th birthday. o A booster dose, consisting of an adult preparation of Tdap, is recommended at 1112 yr of age. Adolescents 1318 yr of age who missed the 1112 year old Td or Tdap booster dose or in whom it has been 5 yr since the Td booster dose also should receive a single dose of Tdap if they have completed the DTP/DTaP series. o There is no association of DT or dT with convulsions. Local adverse effects alone do not preclude continued use. Persons who experience Arthus-type hypersensitivity reactions or a temperature >103F (39.4C) after a dose of dT, which is rare, usually have high serum tetanus antitoxin levels and should not be given dT more frequently than every 10 yr, even if a significant tetanus-prone injury is sustained. DT or dT preparation can be given concurrently with other vaccines. Haemophilus influenzae conjugate vaccines containing diphtheria toxoid (PRP-D) or the variant of diphtheria toxin, CRM197 protein (HbOC), are not substitutes for diphtheria toxoid immunization and do not affect reactogenicity. Meningococcal o A quadrivalent vaccine composed of capsular polysaccharides of meningococcal groups A, C, Y, and W135 (MPSV4) was the primary meningococcal vaccine available in the United States until January 2005, with the approval of a diphtheria toxoidbased protein-conjugate meningococcal vaccine (MCV4) for use in persons 1155 yr of age. MPSV4 is immunogenic in adults but is unreliable in children <2 yr of age. It remains the only approved vaccine in the United States for children 210 yr of age and adults >55 yr of age. About 75% of meningococcal disease among persons 11 yrs of age in the United States is caused by serogroups C, Y, or W-135, and thus is potentially vaccine preventable.
33 o MCV4 and MPSV4 produce similar titers of anticapsular bactericidal antibodies against each of the 4 serogroups in the vaccines at 1 mo after vaccination (in age-appropriate patients), with 4-fold increases in titer with both in >90% of vaccinees against serogroups A, C, and W-135 and >80% against serogroup Y. Protective titers (1 : 128) are induced in >97% of recipients of either vaccine against all 4 serogroups in age-appropriate patients. MCV4 causes transient fever and local redness, pain, or swelling slightly more frequently than MPSV4, which is attributed to the diphtheria toxoid component of MCV4. o The efficacy of MPSV4 is 85% for serogroups A and C and likely similar for serogroups W-135 and Y. Efficacy of MCV4 is expected to be similar to MPSV4 since bactericidal antibodies confer immunity and the titers of these induced by MCV4 are noninferior to those of MPSV4. Use of a serogroup Cdiphtheria CRM197 conjugate vaccine in the United Kingdom since 1999 has reduced serogroup C disease by about 95% in children in that country, and similar results with use of this vaccine have been reported from Quebec. Reduced nasopharyngeal carriage of serogroup C strains among serogroup Cdiphtheria CRM197 vaccinees and community immunity (herd immunity) among nonvaccinees have been demonstrated in the U.K. o Duration of immunity from MPSV4 is at most 35 yr, such that revaccination of persons with ongoing risk of meningococcal infections can be considered within this time frame for past MPSV4 recipients. Protection from MCV4 is expected to be longer, but the full duration and potential need for revaccination are not yet known. Immune responses to conjugate meningococcal vaccines are boostable (T cell dependent), whereas those to MPSV4 are not (T cell independent). Conjugate vaccines appear to be immunogenic and safe in infants but have not yet been approved or recommended for this age group in the United States. o MCV4, as a single dose, is the preferred vaccine in the United States for persons 1155 yr of age for whom meningococcal vaccination is recommended. MPSV4 remains an acceptable alternative for this age range when MCV4 is unavailable and is recommended for children 210 yr of age and adults >55 yr of age, pending further evaluation and approval of MCV4 in these age groups. The monovalent serogroup Cdiphtheria CRM197 conjugate vaccine currently is used in much of Europe, Canada, Australia, and Brazil. A 3 dose series of this vaccine can be given to infants starting at 2 mo of age, with 1 dose recommended for persons 1 yr of age. o MCV4 is routinely recommended for all adolescents at 1112 yr at the pre-adolescent visit, and adolescents at age 15 yr or high school entry if not previously vaccinated. The goal is routine vaccination of all 11 yr old adolescents beginning in 2008. MCV4 and the Tdap (tetanus and diphtheria toxoids and acellular pertussis booster) vaccine should be administered to adolescents during the same visit if both vaccines are indicated. If this is not feasible, MCV4 and Tdap can be administered in either sequence with a minimum interval of 1 mo between vaccines. MCV4 is also recommended for all incoming college freshmen living in dormitories. Many colleges and universities, and some states, have mandated meningococcal immunization of all matriculating freshmen. MCV4 is also indicated for other adolescents who wish to decrease their risk for meningococcal disease. o Tetravalent meningococcal vaccines also are recommended for U.S. children 2 yr of age who have anatomic or functional asplenia, complement component deficiencies that prevent terminal attack complex formation, or travel plans to areas with hyperendemic or epidemic meningococcal infection rates such as sub-Saharan Africa during the DecemberJune dry season. o Guillain-Barr syndrome (GBS) has been reported to be temporally related to administration of meningococcal vaccine, although the rate among vaccine recipients is similar to that among the general population (see Chapter 615 ). Persons with previously diagnosed GBS should not receive conjugate meningococcal vaccine. Pertussis o Three (primary) doses of DTaP should be administered during the 1st year of life, generally at ages 2, 4, and 6 mo of age. A 4th dose (1st booster) is recommended for children at 1518 mo of age, at least 6 mo after the 3rd dose, to maintain adequate immunity during the preschool years. The 4th dose may be administered as early as 12 mo of age, provided 6 mo have elapsed since the 3rd dose and the child is unlikely to return at 1518 mo of age. The 5th dose (2nd booster) is recommended for children at 46 yr of age to confer continued protection against disease during the early years of schooling. A 5th dose is not necessary if the 4th dose in the series is administered on or after the 4th birthday. o In 2005, 2 tetanus toxoid, reduced diphtheria toxoid and acellular pertussis vaccine, adsorbed (Tdap) products were licensed for use in older individuals as single-dose booster vaccines to provide protection against tetanus, diphtheria, and pertussis. The preferred age for Tdap vaccination is 1112 yr. All adolescents 1118 yr of age who received Td, but not Tdap, should receive a single dose of Tdap to provide protection against pertussis if they have completed the recommended childhood DTP/DTaP vaccination series. An interval of at least 5 yr between Td and Tdap is encouraged in routine situations to reduce the risk for local and systemic reactions after Tdap vaccination. However, an interval of <5 yr between Td and Tdap can be used. Both Tdap and tetravalent meningococcal conjugate vaccine should be administered to adolescents 1118 yr of age during the same visit if both vaccines are indicated and available. Adults 1964 yr of age should receive a single dose of Tdap to replace their next Td booster dose. Priority for Tdap vaccine should be given for health care workers who have contact with children and for eligible family contacts of neonates. Typhoid o Globally, 2 vaccines are currently available for potential use in children. An oral, live-attenuated preparation of the Ty21a strain of S. Typhi has been shown to have good efficacy (6782%) for up to 5 years. Significant adverse effects are rare. The Vi capsular polysaccharide can be used in people 2 yr of age. It is given as a single intramuscular dose, with a booster every 2 yr and has a protective efficacy of 7080%. MMR o Immunization with the live mumps vaccine is the primary mode of prevention used in the United States. It is given as part of the MMR 2 dose vaccine schedule, at 1215 mo of age for the 1st dose and 46 yr of age for the 2nd dose. If not given at 46 yr, the 2nd dose should be given before children enter puberty. Antibody develops in 95% of vaccinees after 1 dose. One study showed vaccine effectiveness of 88% for 2 doses of MMR vaccine compared with 64% for a single dose. Immunity appears to be long lasting, with existing serologic and epidemiologic evidence indicating protection for >25 yr. As a live-virus vaccine, MMR should not be administered to pregnant women or severely immunodeficient or immunosuppressed individuals. HIV-infected patients not severely immunocompromised may receive the vaccine since the risk for severe infection with mumps outweighs the risk for serious reaction to the vaccine. Individuals with anaphylactoid reactions to egg or neomycin may be at risk for immediate- type hypersensitivity reactions to the vaccine. Persons with other types of reactions to egg or reactions to other components of the vaccine are not restricted from receiving the vaccine. Varicella Varicella is a vaccine-preventable disease. Live virus varicella vaccine is available as a monovalent vaccine and is also available in combination with measles, mumps, and rubella (MMRV) vaccines. Administration of varicella vaccine within 4 wk of MMR vaccine has been associated with a higher risk for breakthrough disease; therefore, it is recommended that the vaccines either be administered
34 simultaneously at different sites or be given at least 4 wk apart. Simultaneous administration of the vaccines may also be accomplished by immunizing with the MMRV vaccine. Varicella vaccine is recommended for routine administration to children at 1218 mo and at 46 yr of age. Catch-up vaccination with the second dose is recommended for children and adolescents who received only 1 dose. Vaccination with 2 doses, separated by at least 4 wk, is also recommended for children, adolescents, and adults without evidence of immunity. Breakthrough disease may be more common in children vaccinated with a single dose younger than 15 mo. Varicella vaccine is contraindicated in children with cell-mediated immune deficiencies, although the vaccine may be administered to children with acute lymphoblastic leukemia who are in remission and who meet enrollment criteria under a research protocol, and the vaccine may also be considered for HIV-infected children with a CD4 count greater than 15%. Both these groups should receive 2 doses of vaccine, 3 months apart. Specific guidelines for immunizing these children should be reviewed before vaccination. Children with isolated humoral immune deficiencies may receive VZV vaccine. Vaccine virus establishes latent infection; however, the risk for developing subsequent herpes zoster is lower after vaccine than after natural VZV infection among immunocompromised children. Postlicensure data also suggest the same trend in healthy vaccinees. A new VZV vaccine formulation was licensed in 2006 for use as a single immunization of individuals >60 yr of age for prevention of herpes zoster reactivation and to decrease the frequency of postherpetic neuralgia. It is not indicated for the treatment of zoster or postherpetic neuralgia. Influenza TABLE 255-3 -- Indications for Annual Influenza VaccinationPERSONS AT HIGH RISK FOR COMPLICATIONS FROM THE FLU
Because of the decreased potential for causing febrile reactions, only the split-virus vaccine is recommended for children <12 yr of age. Two doses of vaccine (0.25 mL for 636 mo of age; 0.5 mL for 38 yr of age) at least 1 mo apart are recommended for primary immunization of children <9 yr of age. Live-attenuated vaccines that are administered intranasally are in clinical trials and have been demonstrated to have an efficacy comparable with that of inactivated vaccine in adults. Trials in children have shown efficacy of 90%. These vaccines are currently licensed for use in ages 5 and above. Their ease of administration could serve to increase influenza vaccination among children.
Post-Exposure Prophylaxis Measles o Susceptible individuals exposed to measles may be protected from infection either by vaccine administration or immunization with immunoglobulin. The vaccine is effective in prevention or modification of measles if given within 72 hr of exposure. Immune globulin may be given up to 6 days following exposure to prevent or modify infection. Immunocompetent children should receive 0.25 mL/kg intramuscularly and immunocompromised children should receive 0.5 mL/kg. Immune globulin is indicated for susceptible household contacts of measles patients, especially infants <6 mo of age, pregnant women, and immunocompromised persons. Varicella Vaccine given to normal children within 35 days after exposure (as soon as possible is preferred) is effective in preventing or modifying varicella, especially in a household setting where exposure is very likely to result in infection. Varicella vaccine is now recommended for postexposure use, for outbreak control. Oral acyclovir administered late in the incubation period may modify subsequent varicella in the normal child; however, its use in this manner is not recommended until it can be further evaluated. High-titer anti-VZV immune globulin as postexposure prophylaxis is recommended for immunocompromised children, pregnant women, and newborns exposed to maternal varicella to be administered intramuscularly within 96 hr of exposure. Although licensed pooled
35 intravenous immune globulin (IVIG) preparations contain antivaricella antibodies, the titer varies from lot to lot. Newborns whose mothers develop varicella 5 days before to 2 days after delivery should receive 1 vial of VariZIG. Diphtheria o All household contacts and those who have had intimate respiratory or habitual physical contact with a patient are closely monitored for illness through the 7 day incubation period. Cultures of the nose, throat, and any cutaneous lesions are performed. Antimicrobial prophylaxis is presumed effective and is administered regardless of immunization status using erythromycin (40 50 mg/kg/day divided qid PO for 7 days; maximum 2 g/day) or a single injection of benzathine penicillin G (600,000 U IM for <30 kg, 1,200,000 U IM for 30 kg). Diphtheria toxoid vaccine, in age-appropriate form, is given to immunized individuals who have not received a booster dose within 5 yr. Children who have not received their 4th dose should be vaccinated. Those who have received fewer than 3 doses of diphtheria toxoid or who have uncertain immunization status are immunized with an age-appropriate preparation on a primary schedule. Meningococcemia o Close contacts of patients with meningococcal disease are at increased risk for infection and should be carefully monitored and brought to medical attention if fever develops. Antibiotic prophylaxis is indicated as soon as possible for household, daycare, and nursery school contacts and for those who have had contact with the patient's oral secretions during the 7 days before onset of illness. Prophylaxis is not routinely recommended for medical personnel except those with intimate exposure, such as with mouth-to-mouth resuscitation, intubation, or suctioning before antibiotic therapy was begun. Children may be given rifampin (10 mg/kg orally every 12 hr for a total of 4 doses; maximum dose 600 mg; 5 mg/kg/dose for infants <1 mo of age) or ceftriaxone (125 mg in a single dose IM for children <12 yr of age; 250 mg in a single dose IM for those >12 yr of age). Ciprofloxacin (500 mg orally as a single dose) may be given to persons 18 yr of age. Penicillin does not eradicate nasopharyngeal carriage; patients treated with penicillin should receive prophylaxis before hospital discharge. H. influenza o For prophylaxis, children should be given rifampin orally (01 mo of age, 10 mg/kg/dose; >1 mo of age, 20 mg/kg/dose, not to exceed 600 mg/dose) once a day for 4 consecutive days. The adult dose is 600 mg once daily. Rifampin prophylaxis is not recommended for pregnant women. Pertussis TABLE 194-2 -- Recommended Antimicrobial Treatment and Postexposure Prophylaxis for Pertussis, by Age Group PRIMARY AGENTS ALTERNATE AGENT[*]
Use if azithromycin is unavailable; 4050 mg/kg/day in 4 divided doses for 14 days.
TMP 8 mg/kg/day, SMZ 40 mg/kg/day in 2 divided doses for 14 days
Infants (aged 6 10 mg/kg in a single dose on 4050 mg/kg/day 15 mg/kg/day in 2 TMP 8 mg/kg/day, mo) and day 1 then 5 mg/kg/day (maximum 2 g/day) in 4 divided doses SMZ 40 mg/kg/day in 2 children (maximum 500 mg) on days divided doses for 14 days. (maximum 1 g/day) divided doses for 14 25 for 7 days days
Adults 500 mg in a single dose on 2 g/day in 4 divided doses 1 g/day in 2 divided TMP 320 mg/day, SMZ day 1 then 250 mg/day on for 14 days. doses for 7 days 1,600 mg/day in 2 days 25 divided doses for 14 days From Centers for Disease Control and Prevention: Recommended antimicrobial agents for treatment and postexposure prophylaxis of pertussis. 2005 CDC Guidelines. MMWR 2005;54:116. * Trimethoprim-sulfamethoxazole (TMP-SMZ) can be used as an alternative agent to macrolides in patients aged 2 mo who are allergic to macrolides, who cannot tolerate macrolides, or who are infected with a rare macrolide-resistant strain of Bordetella pertussis.
36Fever Definitions o Intermittent fever is an exaggerated circadian rhythm that includes a period of normal temperatures on most days. o Extremely wide fluctuations may be termed septic or hectic fever. o Sustained fever is persistent and does not vary by more than 0.5C/day. o Remittent fever is persistent and varies by more than 0.5C/day. o Relapsing fever is characterized by febrile periods that are separated by intervals of normal temperature; tertian fever occurs on the 1st and 3rd days (malaria caused by Plasmodium vivax), and quartan fever occurs on the 1st and 4th days (malaria caused by Plasmodium malariae). o Biphasic fever indicates a single illness with 2 distinct periods (camelback fever pattern); poliomyelitis is the classic example. A biphasic course is also characteristic of leptospirosis, dengue fever, yellow fever, Colorado tick fever, spirillary rat-bite fever (Spirillum minus), and the African hemorrhagic fevers (Marburg, Ebola, and Lassa fever). o Periodic fever is used narrowly to describe fever syndromes with a regular periodicity (cyclic neutropenia, and periodic fever, aphthous stomatitis, pharyngitis, and adenopathy [PFAPA]) or more broadly to include disorders characterized by recurrent episodes of fever that do not follow a strictly periodic pattern (familial Mediterranean fever, Hibernian fever, TNF-receptor-associated periodic syndrome [TRAPS], hyper-IgD syndrome, the Muckle- Wells syndrome).
37 chorioretinitis CMV, toxoplasmosis, and syphilis
Rubella Viral shedding from the nasopharynx begins about 10 days after infection and may be detected up to 2 wk following onset of the rash. The period of highest communicability is from 5 days before to 6 days following appearance of the rash.
Mumps Virus appears in the saliva from up to 7 days before to as long as 7 days after onset of parotid swelling. The period of maximum infectiousness is 12 days before to 5 days after parotid swelling.
Polio Poliovirus has been isolated from feces for >2 wk before paralysis to several weeks after the onset of symptoms.Enterovirus Infected children, both symptomatic and asymptomatic, frequently shed enteroviruses from the respiratory tract for <13 wk, whereas fecal shedding continues up to 711 wk postinfection.Parvovirus B19 Children with erythema infectiosum are not likely to be infectious at presentation because the rash and arthropathy represent immune-mediated, postinfectious phenomena. Isolation and exclusion from school or child care are unnecessary and ineffective after diagnosis. Children with B19-induced red cell aplasia, including the transient aplastic crisis, are infectious when they present and demonstrate a more intense viremia. Most of these children require transfusions and supportive care until their hematologic status stabilizes. They should be isolated in the hospital to prevent spread to susceptible patients and staff. Isolation should continue for at least 1 wk and until after resolution of fever. Pregnant caregivers should not be assigned to these patients. Exclusion of pregnant women from workplaces where children with erythema infectiosum may be present (e.g., primary and secondary schools) is not recommended as a general policy because it is unlikely to reduce their risk.Varicella Patients with varicella are contagious from 24 to 48 hr before the rash appears and until vesicles are crusted, usually 37 days after onset of rash.
38 Incubation periods Diphtheria 2-7 days
Genital gonorrhea 2-5 days in men; 5-10 days in women; 1-4 days after birth in opthalmia neonatorum
Typhoid fever 4-14 days; usually 7-14 days (range 3-30 days)
Enteroviruses The incubation period of enterovirus infections is typically 36 days, except for acute hemorrhagic conjunctivitis, which has an incubation period of 13 days.
Streptococcus pneumoniae o S. pneumoniae is the most frequent cause of bacteremia, bacterial pneumonia, and otitis media, and the second most common cause of meningitis in children.
39 TABLE 181-1 -- Children at High or Moderate Risk of Invasive Pneumococcal Infection High risk (incidence of invasive pneumococcal disease =150 cases/100,000 people per year)
Children with: Sickle cell disease, congenital or acquired asplenia, or splenic dysfunction
Cochlear implants
Children with: Congenital immune deficiency; some B-(humoral) or T-lymphocyte deficiencies, complement deficiencies (particularly C1, C2, C3, and C4), or phagocytic disorders (excluding chronic granulomatous disease)
Chronic cardiac disease (particularly cyanotic congenital heart disease and cardiac failure)
Chronic pulmonary disease (including asthma treated with high-dose oral corticosteroid therapy) Cerebrospinal leaks from a congenital malformation, skull fracture, or neurologic procedure
Diseases associated with immunosuppressive therapy or radiation therapy (including malignant neoplasms, leukemias, lymphomas, and Hodgkin disease) and solid organ transplantation
Diabetes mellitus
Moderate risk (incidence of invasive pneumococcal disease =20 cases/100,000 people Per year)
o Immunization with PCV7 is recommended for all infants in a schedule of 4 doses administered at 2, 4, 6, and 1215 mo of age. High-risk children 2 yr of age such as those with asplenia, sickle cell disease, some types of immune deficiency (antibody deficiencies), HIV infection, or chronic lung, heart, or kidney disease (including nephrotic syndrome), may benefit also from PPV23 administered after 2 yr of age and following priming with the scheduled doses of PCV7. After the initial immunization, a single supplemental dose of PPV23 may be used 3 yr after the 1st dose for children <10 yr of age at the time of revaccination, or it may be used at 5 yr after the 1st dose for children 10 yr of age or older at the time of revaccination.
Group A Streptococcus o Severe invasive GAS infection Varicella is the most commonly identified risk factor in children. In most cases, portal of entry is believed to be skin or mucous membrane. Severe invasive disease rarely follows GAS pharyngitis.
Scarlet fever o The rash appears within 2448 hr after onset of symptoms, although it may appear with the 1st signs of illness . It often begins around the neck and spreads over the trunk and extremities. It is a diffuse, finely papular, erythematous eruption producing a bright red discoloration of the skin, which blanches on pressure. It is often more intense along the creases of the elbows, axillae, and groin. The skin has a goose-pimple appearance and feels rough. The face is usually spared, although the cheeks may be erythematous with pallor around the mouth. o After 34 days, the rash begins to fade and is followed by desquamation, 1st on the face, progressing downward, and often resembling that seen subsequent to a mild sunburn. Occasionally, sheetlike desquamation may occur around the free margins of the fingernails, the palms, and the soles. Examination of the pharynx of a patient with scarlet fever reveals essentially the same findings as with GAS pharyngitis. o In addition, the tongue is usually coated and the papillae are swollen. After desquamation, the reddened papillae are prominent, giving the tongue a strawberry appearance.
Why can glomerulonephritis occur after both throat and skin infections while rheumatic fever occurs only after throat infections? o GAS can be subdivided into >100 serotypes on the basis of the M protein antigen, which is located on the cell surface and in fimbriae that project from the outer edge of the cell. o The M types commonly associated with pharyngitis rarely cause skin infections, and the M types commonly associated with skin infections rarely cause pharyngitis.
40 o A few of the pharyngeal strains (M type 12) have been associated with glomerulonephritis, but far more of the skin strains (M types 49, 55, 57, and 60) have been considered nephritogenic. A few of the pharyngeal serotypes, but none of the skin strains, have been associated with acute rheumatic fever. o Rheumatogenic potential is not solely dependent on the serotype but is a characteristic of specific strains within several serotypes.
Group B Streptococcus o TABLE 183-2 -- Recommended Duration of Therapy for Manifestations of GBS Disease TREATMENT DURATION
Meningitis 23 wk
Ventriculitis 4 wk
Osteomyelitis 4 wk
o GBS prophylaxis Vaginorectal GBS screening cultures should be performed for all pregnant women at 3537 wk gestation. Women whose culture status is unknown (culture not done, incomplete, or results unknown) and who deliver prematurely (<37 wk gestation) or experience prolonged rupture of membranes (18 hr) or intrapartum fever (38.0C) should also receive intrapartum chemoprophylaxis. If amnionitis is suspected, broad-spectrum antibiotic therapy that includes an agent active against GBS should replace GBS prophylaxis. Drugs Penicillin Cefazolin if penicillin intolerant Clindamycin or erythromycin for penicillin allergic at high risk for anaphylaxis
DiphtheriaAsymptomatic Case Contacts: All household contacts and those who have had intimate respiratory or habitual physical contact with a patient areclosely monitored for illness through the 7 day incubation period. Cultures of the nose, throat, and any cutaneous lesions are performed.Antimicrobial prophylaxis is presumed effective and is administered regardless of immunization status using erythromycin (4050 mg/kg/daydivided qid PO for 7 days; maximum 2 g/day) or a single injection of benzathine penicillin G (600,000 U IM for <30 kg, 1,200,000 U IM for30 kg). Diphtheria toxoid vaccine, in age-appropriate form, is given to immunized individuals who have not received a booster dose within 5yr. Children who have not received their 4th dose should be vaccinated. Those who have received fewer than 3 doses of diphtheria toxoid or whohave uncertain immunization status are immunized with an age-appropriate preparation on a primary schedule.Asymptomatic Carriers: When an asymptomatic carrier is identified, antimicrobial prophylaxis is given for 7 days and an age-appropriatepreparation of diphtheria toxoid is administered immediately if a booster has not been given within 1 yr. Individuals are placed on dropletprecautions (respiratory tract colonization) or contact precautions (cutaneous colonization only) until at least 2 subsequent cultures obtained 24hr apart after cessation of therapy are negative. Repeat cultures are performed about 2 wk after completion of therapy for cases and carriers, and,if positive, an additional 10 day course of oral erythromycin should be given and follow-up cultures performed. Susceptibility testing of isolatesshould be performed as erythromycin resistance is reported. Neither antimicrobial agent eradicates carriage in 100% of individuals. In 1 report,21% of carriers failed a single course of therapy. Antitoxin is not recommended for asymptomatic close contacts or carriers, even if inadequatelyimmunized. Transmission of diphtheria in modern hospitals is rare. Only those with an unusual contact with respiratory or oral secretions shouldbe managed as contacts. Investigation of the casual contacts of patients and carriers or persons in the community without known exposure hasyielded extremely low carriage rates and is not routinely recommended.
Convalescent 2 weeks Salmonella typhi o Individuals who excrete S. Typhi for 3 mo after infection are regarded as chronic carriers. Shigella o Shigellae require very low inocula to cause illness. Ingestion of as few as 10 S. dysenteriae serotype 1 organisms can cause dysentery in some susceptible individuals. This is in contrast to organisms such as Vibrio cholerae, which require ingestion of 1081010 organisms to cause illness. The inoculum effect explains the ease of person-to-person transmission (major mechanism) of shigellae in contrast to V. cholerae. o Colon is the target organ (most intense changes in the distal colon) o Greater severity and risk for complications with S. dysenteriae serotype 1 infection (this type tends to occur in epidemics)
41Viral Infections Measles o Koplik spots appear 1-4 days prior to the onset of the rash o Cough is the symptom that lasts the longest (often up to 10 days) Labs: reduced total WBC with lymphocytes more decreased than neutrophils; normal ESR and CRP o Susceptible individuals exposed to measles may be protected from infection either by vaccine administration or immunization with immunoglobulin. The vaccine is effective in prevention or modification of measles if given within 72 hr of exposure. Immune globulin may be given up to 6 days following exposure to prevent or modify infection. Immunocompetent children should receive 0.25 mL/kg intramuscularly and immunocompromised children should receive 0.5 mL/kg. Immune globulin is indicated for susceptible household contacts of measles patients, especially infants <6 mo of age, pregnant women, and immunocompromised persons. Rubella o Centrifugal rash o Labs: leukopenia, neutropenia, mild thrombocytopenia o Most common clinical manifestations of CRS: ocular (cataracts or retinopathy in 71%) and deafness (67%) o Patients with postnatal infection should be isolated from susceptible individuals for 7 days after onset of the rash. Standard plus droplet precautions are recommended for hospitalized patients. Children with CRS may excrete the virus in respiratory secretions up to 1 yr of age and should be maintained in contact precautions until then unless repeated cultures of urine and pharyngeal secretions are negative. Similar precautions apply to CRS patients with regard to attendance in school and out-of-home child care. Enteroviruses o Manifestations of nonpolio enteroviruses: nonspecific febrile illness, hand-foot-mouth disease, herpangina, pleurodynia, URTI, acute hemorrhagic conjunctivitis, myocarditis and pericarditis, diarrhea, meningitis, myositis, arthritis, pancreatitis, orchitis o Poliovirus In suspected cases of acute flaccid paralysis, 2 stool specimens should be collected 2448 hr apart, as soon as possible after the diagnosis of poliomyelitis is suspected. Poliovirus concentrations are high in the stool in the 1st week after the onset of paralysis, which is the optimal time for collection of stool specimens. Polioviruses may be isolated from 8090% of acutely ill patients, whereas <20% may yield virus within 34 wk after onset of paralysis. Because most children with spinal or bulbospinal poliomyelitis have constipation, rectal straws may be used to obtain specimens; ideally a minimum of 810 g of stool should be collected. o Coxsackievirus Herpangina Characteristic lesions, present on the anterior tonsillar pillars, soft palate, uvula, tonsils, posterior pharyngeal wall, and, occasionally, the posterior buccal surfaces, are discrete 12 mm vesicles and ulcers that enlarge over 23 days to 34 mm and are surrounded by erythematous rings that vary in size up to 10 mm. Typically about 5 lesions are present, with a range of 1 to >15. Pleurodynia (Bornholm disease) is an epidemic or sporadic illness characterized by paroxysmal thoracic pain, due to myositis involving chest and abdominal wall muscles. o Echovirus (enteric cytopathic human orphan virus) Parvovirus B19 o Erythema infectiosum / fifth disease o Arthropathy o Transient aplastic crisis o Myocarditis o Papular-purpuric gloves and socks syndrome (PPGSS) Herpes o The hallmarks of common HSV infections are skin vesicles and shallow ulcers. o The vesicles may be more extensively distributed than typically seen with enteroviral herpangina. o The most common site of herpes labialis is the vermilion border of the lip. Varicella Vaccine given to normal children within 35 days after exposure (as soon as possible is preferred) is effective in preventing or modifying varicella, especially in a household setting where exposure is very likely to result in infection. Varicella vaccine is now recommended for postexposure use, for outbreak control. Oral acyclovir administered late in the incubation period may modify subsequent varicella in the normal child; however, its use in this manner is not recommended until it can be further evaluated. High-titer anti-VZV immune globulin as postexposure prophylaxis is recommended for immunocompromised children, pregnant women, and newborns exposed to maternal varicella. Human varicella-zoster immune globulin (VariZIG) is distributed by FFF Enterprises, California, (1-800-843-7477) to be administered intramuscularly within 96 hr of exposure. The recommended dose is 1 vial (125 units) for each 10 kg increment (maximum 625 units) given intramuscularly as soon as possible but within 96 hr after exposure. Although licensed pooled intravenous immune globulin (IVIG) preparations contain antivaricella antibodies, the titer varies from lot to lot. The recommended dose of IVIG for postexposure prophylaxis is 400 mg/kg administered once within 96 hr of exposure. Immunocompromised patients who have received high-dose IVIG (100400 mg/kg) for other indications within 23 wk before VZV exposure can be expected to have serum antibodies to VZV. Newborns whose mothers develop varicella 5 days before to 2 days after delivery should receive 1 vial of VariZIG. VariZIG is also indicated for pregnant women, premature infants <28 wk of gestation (<1,000 g) who were exposed to varicella, and premature infants >28 wk of gestation who are exposed to varicella and whose mother has no evidence of varicella immunity. If possible, adults should be tested for VZV IgG antibodies before VariZIG administration because many adults with no clinical history of varicella are immune. Anti- VZV antibody prophylaxis may ameliorate disease but does not eliminate the possibility of progressive disease, nor does it assure that varicella is not transmitted to close susceptible contacts; patients should be monitored and treated with acyclovir if necessary once lesions develop. Close contact between a susceptible high-risk patient and a patient with herpes zoster is also an indication for VariZIG prophylaxis. Passive antibody administration or treatment does not reduce the risk for herpes zoster or alter the clinical course of varicella or herpes zoster when given after the onset of symptoms.
42 EBV o Benign EBV-associated proliferations include oral hairy leukoplakia, primarily in adults with AIDS, and lymphoid interstitial pneumonitis, primarily in children with AIDS. Malignant EBV-associated proliferations include nasopharyngeal carcinoma, Burkitt lymphoma, Hodgkin disease, lymphoproliferative disorders, and leiomyosarcoma in immunodeficient states, including AIDS. o Epitrochlear LAD most suggestive. o Splenomegaly is typical. o "Ampicillin rash" if treated with ampicillin or amoxicillin. Very few patients with infectious mononucleosis experience complications. The most feared complication is subcapsular splenic hemorrhage or splenic rupture, which occurs most frequently during the 2nd week of the disease at a rate of <0.5% of cases in adults; the rate in children is unknown but is probably much lower. Rupture is commonly related to trauma, which often may be mild, and is rarely fatal. Swelling of the tonsils and oropharyngeal lymphoid tissue may be substantial and may cause airway obstruction that manifests as drooling, stridor, and interference with breathing. Airway compromise with progressive symptoms occurs in <5% of cases and is a common indication for hospitalization with infectious mononucleosis. It may be managed by elevating the head of the bed and administration of intravenous hydration, humidified air, and systemic corticosteroids. Respiratory distress with incipient or actual airway occlusion should be managed by tonsillo-adenoidectomy followed by endotracheal intubation for 1224 hr in an intensive care setting. Many uncommon and unusual neurologic conditions have been reported to be associated with EBV infectious mononucleosis. Headache is present in about half of cases, with severe neurologic manifestations, such as seizures and ataxia, in 15% of cases. Perceptual distortions of sizes, shapes, and spatial relationships, known as the Alice in Wonderland syndrome (metamorphopsia), may be a presenting symptom. There may be meningitis with nuchal rigidity and mononuclear cells in the cerebrospinal fluid, facial nerve palsy, transverse myelitis, and encephalitis. Guillain-Barr syndrome or Reye syndrome may follow acute illness. Hemolytic anemia, often with a positive Coombs test result and with cold agglutinins specific for red cell antigen i, occurs in 3% of cases. The onset is typically in the 1st 2 wk of illness and lasts for <1 mo. Aplastic anemia is a rare complication that usually presents 34 wk after the onset of illness, usually with recovery in 48 days, but some cases do require bone marrow transplantation. Mild thrombocytopenia and neutropenia are common, but severe thrombocytopenia (<20,000 platelets/L) or severe neutropenia (<1,000 neutrophils/L) are rare. Myocarditis or interstitial pneumonia may occur, both resolving in 34 wk. Other rare complications include pancreatitis, parotitis, and orchitis.
Roseola The prodromal period of roseola is usually asymptomatic but may include mild upper respiratory tract signs, among them minimal rhinorrhea, slight pharyngeal inflammation, and mild conjunctival redness. Mild cervical or, less frequently, occipital lymphadenopathy may be noted. Some children may have mild palpebral edema. Physical findings during the prodromal stage have no clear relationship to roseola, and may simply reflect an accompanying respiratory viral infection. Clinical illness is generally heralded by high temperature, usually ranging from 37.9 to 40C (101106F), with an average of 39C (103F). Some children may become irritable and anorexic during the febrile stage, but most behave normally despite high temperatures. Seizures may occur in 510% of children with roseola during this febrile period. Infrequent complaints include rhinorrhea, sore throat, abdominal pain, vomiting, and diarrhea. In Asian countries, ulcers at the uvulopalatoglossal junction (Nagayama spots) are common in infants with roseola. Fever persists for 35 days, and then typically resolves rather abruptly (crisis). Occasionally, the fever may gradually diminish over 2436 hours (lysis). A rash appears within 1224 hr of fever resolution. In many cases, the rash develops during defervescence or within a few hours of fever resolution. The rash of roseola is rose colored, as the name implies, and is fairly distinctive. However, it may be confused with exanthems resulting from rubella, measles, or erythema infectiosum. The roseola rash begins as discrete, small (25 mm), slightly raised pink lesions on the trunk and usually spreads to the neck, face, and proximal extremities. The rash is not usually pruritic, and no vesicles or pustules develop. Lesions typically remain discrete but occasionally may become almost confluent . After 13 days, the rash fades. Some children experience evanescent rashes that resolve within a few hours. Subtle differences in clinical presentation have been noted between roseola associated with HHV-7 compared with HHV-6. These include a slightly older age, lower mean temperature, and shorter duration of fever in HHV-7-associated cases. However, these differences are insufficient to clinically distinguish HHV-6- from HHV-7-associated roseola. There are reports of children experiencing HHV-6-associated roseola followed later by HHV-7-associated roseola.
TORCH Infections Toxoplasma
Congenital infection may present as a mild or severe neonatal disease, with onset during the 1st mo of life or with sequelae or relapse of apreviously undiagnosed, and untreated, infection presenting during infancy or even later in life. There is a wide variety of manifestations ofcongenital infection ranging from hydrops fetalis and perinatal death to small size for gestational age, prematurity, peripheral retinal scars,persistent jaundice, mild thrombocytopenia, CSF pleocytosis, and the characteristic triad of chorioretinitis, hydrocephalus, and cerebralcalcifications. More than half of congenitally infected infants are considered normal in the perinatal period, but almost all such children developocular involvement later in life if they are not treated during infancy. Neurologic signs such as convulsions, setting-sun sign with downwardgaze, and hydrocephalus with increased head circumference may be associated with or without substantial cerebral damage, or with relativelymild inflammation obstructing the aqueduct of Sylvius. If affected infants are treated promptly, signs and symptoms may resolve and they maydevelop normally.
All newborns infected with T. gondii should be treated whether or not they have clinical manifestations of the infection because treatment maybe effective in interrupting acute disease that damages vital organs. Infants should be treated for 1 yr with pyrimethamine (2 mg/kg/day for 2days, then 1 mg/kg/day for 2 or 6 mo, then 1 mg/kg given on Monday, Wednesday, and Friday, or more often, PO), sulfadiazine (100 mg/kg/daydivided bid PO), and leukovorin (510 mg given on Monday, Wednesday, and Friday, PO).
43 Rubella TABLE 244-1 -- Pathologic Findings of Congenital Rubella Syndrome SYSTEM PATHOLOGIC FINDINGS
Ventriculoseptal defect
Parenchymal necrosis
Eye Microphthalmia
Endothelial necrosis
Lobular disarray
Bile stasis
Thinning cartilage
44 Figure 244-4 Bilateral cataracts in infant with congenital rubella syndrome. A variety of late-onset manifestations of CRS have been recognized. In addition to PRP, these include diabetes mellitus (20%), thyroid dysfunction (5%), and glaucoma and visual abnormalities associated with the retinopathy, which had previously been considered benign. o If pregnant woman is exposed, test for rubella IgG ASAP, and if negative, repeat at 2-3 wks and at 6 wks after exposure. CMV: Symptomatic congenital CMV infection was originally termed cytomegalic inclusion disease. Only 5% of all congenitally infected infants have severe cytomegalic inclusion disease, another 5% have mild involvement, and 90% are born with subclinical, but still chronic, CMV infection. The characteristic signs and symptoms of clinically manifested infections include intrauterine growth restriction, prematurity, hepatosplenomegaly and jaundice, blueberry muffinlike rash, thrombocytopenia and purpura, and microcephaly and intracranial calcifications. Other neurologic problems include chorioretinitis, sensorineural hearing loss, and mild increases in cerebrospinal fluid protein. Symptomatic newborns are usually easy to identify. The most severe symptomatic congenital infect ions and those resulting in sequelae are more likely to be caused by primary rather than reactivated infections in pregnant women. Reinfection with a different strain of CMV can lead to symptomatic congenital infection. Asymptomatic congenital CMV infection is likely the leading cause of sensorineural hearing loss, which occurs in approximately 7% of all infants with congenital CMV infection, whether symptomatic at birth or not. A randomized controlled phase III study with ganciclovir (6 mg/kg/dose every 12 hr IV for the 1st 6 wk of life) concluded that treatment both prevents hearing deterioration and improves or maintains normal hearing function at 6 mo of age, and may prevent the hearing deterioration that occurs after 1 yr of age. Herpes HSV infection may be acquired in utero, during the birth process, or during the neonatal period. Intrauterine and postpartum infections are well described but occur infrequently. Postpartum transmission may be from the mother or another adult with a nongenital (typically HSV-1) infection such as herpes labialis. Most cases of neonatal herpes result from maternal infection and transmission, usually during passage through a contaminated infected birth canal of a mother with asymptomatic genital herpes. Transmission is well documented in infants delivered by cesarean section. Fewer than 30% of mothers of an infant with neonatal herpes have a history of genital herpes. The risk for infection is higher in infants born to mothers with primary genital infection (>30%) compared to recurrent genital infection (<2%). Use of scalp electrodes may also increase risk. Neonatal HSV infection is almost never asymptomatic. Its clinical presentation reflects timing of infection, portal of entry, and extent of spread. Infants with intrauterine infection typically have skin vesicles or scarring, eye findings including chorioretinitis and keratoconjunctivitis, and microcephaly or hydranencephaly that are present at delivery. Few infants survive without therapy, and those that do generally have severe sequelae. Infants infected during delivery or postpartum present with 1 of 3 patterns of disease: (1) disease localized to the skin, eyes, or mouth; (2) encephalitis with or without skin, eye, or mouth (SEM) disease; or (3) disseminated infection involving multiple organs, including the brain, lungs, liver, heart, adrenals, and skin. Infants with SEM disease generally present at 511 days of life and typically develop a few small vesicles, particularly on the presenting part or at sites of trauma such as with scalp electrode placement. If untreated, infants with SEM disease may progress to develop encephalitis or disseminated disease. Infants with encephalitis typically present at 817 days of life with clinical findings suggestive of bacterial meningitis, including irritability, lethargy, poor feeding, poor tone, and seizures. Fever is relatively uncommon, and only about 60% have skin vesicles ( Fig. 249-3 ). If untreated, 50% will die and most survivors have severe neurologic sequelae.
Figure 249-3 Vesicular-pustular lesions on the face of a neonate with herpes simplex virus infection. See also color plates.
Infants with disseminated HSV infections generally become ill at 511 days of life. Their clinical picture is similar to bacterial sepsis with hyper- or hypothermia, irritability, poor feeding, and vomiting. They may also exhibit respiratory distress, cyanosis, apneic spells, jaundice, purpuric rash, and evidence of central nervous system infection; seizures are common. Skin vesicles are seen in about 75% of cases. If untreated, the infection causes shock and disseminated intravascular coagulation; approximately 90% of these infants die, and most survivors have severe neurologic sequelae. All infants with proven or suspected neonatal HSV infection should be begun promptly on high-dose intravenous acyclovir (60 mg/kg/day divided every 8 hr IV). Treatment may be discontinued in those infants shown by laboratory testing to not be infected. Infants with HSV disease limited to skin, eyes, and mouth should be treated for 14 days, while those with disseminated or central nervous system disease should receive 21 days of therapy. Patients receiving high-dose therapy should be monitored for neutropenia. o The risk for transmission is greatest during a primary or nonprimary 1st infection (3050%) and much lower when the exposure is during a recurrent infection (<2%). Infants born to mothers dually infected with HIV and HSV-2 are also at increased risk for acquiring HIV compared with infants born to HIV-positive mothers who are not HSV-2 infected. (Nonprimary 1st infection occurs in individuals previously infected with 1 type of HSV (HSV-1) who have become infected for the 1st time with the other HSV type (HSV-2).) o Syphilis Untreated syphilis during pregnancy has a transmission rate approaching 100%. Fetal or perinatal death occurs in 40% of affected infants. Among survivors, manifestations have traditionally been divided into early and late stages. The early signs appear during the 1st 2 yr of life, and late signs appear gradually during the 1st 2 decades. Early manifestations result from transplacental spirochetemia and are analogous to the secondary stage of acquired syphilis. Approximately 66% of infected infants are asymptomatic at the time of birth and are identified only by routine prenatal screening; if they are untreated, symptoms develop within weeks or months. The early manifestations of congenital infection are varied and involve multiple organ systems ( Table 215-1 ). Hepatosplenomegaly, jaundice, and elevated liver enzymes are common. Histologically, liver involvement includes bile stasis, fibrosis, and extramedullary hematopoiesis. Lymphadenopathy tends to be diffuse and to resolve spontaneously, although shotty nodes may persist. Coombs negative hemolytic anemia is characteristic. Thrombocytopenia is often associated with platelet trapping in an enlarged spleen. Characteristic osteochondritis and periostitis ( Fig. 215-3 ) and mucocutaneous rash ( Fig. 215-4 ), pre senting with erythematous maculopapular or bullous lesions, followed by desquamation involving hands and feet, are common. Mucous patches, rhinitis (snuffles), and condylomatous lesions ( Fig. 215-5 ) are highly characteristic features of mucous membrane involvement in congenital syphilis. Bone involvement occurs frequently. Roentgenographic abnormalities include multiple sites of osteochondritis at the wrists, elbows, ankles, and knees, and periostitis of the long bones and rarely the skull. The osteochondritis is painful and often results in irritability and refusal to move the involved extremity (pseudoparalysis of Parrot). Central nervous system (CNS) abnormalities, failure to thrive, chorioretinitis, nephritis, and nephrotic syndrome may also be seen. Clinical manifestations of renal involvement include hypertension, hematuria, proteinuria, hypoproteinemia, hypercholesterolemia, and hypocomplementemia. They appear to be related to glomerular
45 deposition of circulating immune complexes. Less common clinical manifestations of early congenital syphilis include gastroenteritis, peritonitis, pancreatitis, pneumonia, eye involvement (glaucoma and chorioretinitis), nonimmune hydrops, and testicular masses. TABLE 215-1 -- Clues That Suggest a Diagnosis of Congenital Syphilis[*] EPIDEMIOLOGIC BACKGROUND CLINICAL FINDINGS
Mother treated for syphilis during pregnancy with a drug Mucous patches other than penicillin
Hepatomegaly, splenomegaly
Generalized lymphadenopathy
Nephrotic syndrome
Syndromes/Eponyms o Reiter syndrome - S. flexneri infection
46 o Ekiri syndrome - toxic encephalopathy secondary to Shigella The rare syndrome of severe toxicity, convulsions, extreme hyperpyrexia, and headache associated with brain edema and a rapidly fatal outcome without sepsis or significant dehydration (Ekiri syndrome or lethal toxic encephalopathy) is not well understood. o Fitz-Hugh-Curtis syndrome - perihepatitis due to N. gonorrhea Dissemination from the fallopian tubes through the peritoneum to the liver capsule results in perihepatitis (Fitz-Hugh-Curtis syndrome). May also be caused by Chlamydia o Ritter's disease - staphylococcal scalded skin syndrome (+) Nikolsky sign (epidermis separates in response to gentle stroking) Abortive type resembles scarlet fever and has a negative Nikolsky sign o St. Anthony's fire - erysipelas (GABHS) Superficial severe inflammation of the skin with blanched raised sharply demarcated, irregular, rapidly advancing borders Prominent constitutional symptoms (fever, chills, vomiting, irritability) o Vincent angina Vincent angina, also known as acute necrotizing ulcerative gingivitis or trench mouth, is an acute, fulminating, mixed bacterial-spirochetal infection of the gingival margin and floor of the mouth. It is characterized by gingival pain, foul breath, and pseudomembrane formation. o Ludwig angina Ludwig angina is an acute, life-threatening cellulitis of dental origin of the sublingual and submandibular spaces. Infection spreads rapidly in the neck and may cause airway obstruction. o Lemierre syndrome Lemierre syndrome, or postanginal sepsis, is a suppurative infection of the lateral pharyngeal space that usually begins as pharyngitis. It may complicate infectious mononucleosis. It presents as septic thrombophlebitis of the jugular vein leading to multiple septic pulmonary embolizations. Clinical signs include neck swelling and pain, trismus, and dysphagia culminating with signs of sepsis and respiratory distress. Fusobacterium necrophorum is the most commonly involved organism, although polymicrobial infection is frequent. o Forchheimer spots About the time of onset of the rash, examination of the throat may reveal tiny, rose-colored lesions (Forchheimer spots) or petechial hemorrhages on the soft palate.
Associations Typhoid fever In about 25% of cases, a macular or maculopapular rash (rose spots) may be visible around the 7th10th day of the illness, and lesions may appear in crops of 1015 on the lower chest and abdomen and last 23 days. o Cer vicofacial actinomycosis - "lumpy jaw" Cervicofacial actinomycosis usually presents as a painless, slow-growing, hard mass and can produce cutaneous fistulas, a condition commonly known as lumpy jaw. o Chancroid painful genital ulceration and inguinal lymphadenopathy that is caused by Haemophilus ducreyi lymphadenopathy can become fluctuant to form buboes gram-negative coccobacilli in parallel clusters (school of fish) o Infantile hypertrophic pyloric stenosis Associated with treatment of pertussis with erythromycin in infants < 1 mos o Stepladder rise in fever - typhoid o Campylobacter jejuni - associated with GBS, Miller-Fisher syndrome (GBS variant with ataxia, areflexia and ophthalmoplegia), and reactive arthritis (also IgA nephropathy) o Yersinia - enterocolitica (mimics appendicitis), pseudotuberculosis (mesenteric lymphadenitis; may have a complication similar to Kawasaki), pestis (bubonic plague or acute febrile lymphadenitis) o Echthyma gangrenosum - characteristic skin lesion of Pseudomonas (hemorrhagic nodules --> ulcers with eschar formation) o Rope sign (polio) - an acute angulation between the chin and larynx caused by weakness of the hyoid muscles (the hyoid bone is pulled posteriorly, narrowing the hypopharyngeal inlet) o Hand foot and mouth disease - most frequently caused by coxsackievirus A16 (more severe: enterovirus 71) o "Diseases" First disease - measles Second disease - scarlet fever Third disease - rubella Fourth disease - Filatov-Dukes disease (atypical scarlet fever) Fifth disease - erythema infectiosum Sixth disease - roseola infantum / exanthem subitum
47 o Adenovirus - Pharyngoconjunctival fever is a clinically distinct syndrome that occurs typically with type 3 adenovirus. Features include a high temperature that lasts 45 days, pharyngitis, conjunctivitis, preauricular and cervical lymphadenopathy, and rhinitis. Nonpurulent conjunctivitis occurs in 75% of patients and is manifested by inflammation of both the bulbar and palpebral conjunctivae of 1 or both eyes, which often persists after the fever and other symptoms have resolved. Headache, malaise, and weakness are common, and there is considerable lethargy after the acute stage. o Tzanck smear o Balloon cells and multinucleated giant cells - herpes o Acantholytic epidermal cells - pemphigus
GastroenterologyDiarrheaTABLE 303-7 -- Mechanisms of DiarrheaPRIMARY STOOLMECHANISM DEFECT EXAMINATION EXAMPLES COMMENTSecretory Decreased Watery, normal Cholera, toxigenic E. coli; Persists during fasting; absorption, osmolality; osmoles = 2 carcinoid, VIP, neuroblastoma, bile salt malabsorption increased secretion, (Na+ + K+) congenital chloride diarrhea, may also increase electrolyte transport Clostridium difficile, intestinal water secretion; cryptosporidiosis (AIDS) no stool leukocytesOsmotic Maldigestion, Watery, acidic, and Lactase deficiency, glucose- Stops with fasting; transport defects reducing substances; galactose malabsorption, increased breath ingestion of increased osmolality; lactulose, laxative abuse hydrogen with unabsorbable osmoles >2 (Na+ + K+) carbohydrate malabsorption; no stool leukocytesIncreased motility Decreased transit Loose to normal- Irritable bowel syndrome, Infection may also time appearing stool, thyrotoxicosis, postvagotomy contribute to increased stimulated by gastrocolic dumping syndrome motility reflexDecreased Defect in Loose to normal Pseudoobstruction, blind loop Possible bacterialmotility neuromuscular appearing stool overgrowth unit(s) Stasis (bacterial overgrowth)Decreased surface Decreased Watery Short bowel syndrome, celiac May require elementalarea (osmotic, functional capacity disease, rotavirus enteritis diet plus parenteralmotility) alimentationMucosal invasion Inflammation, Blood and increased Salmonella, Shigella, infection; Dysentery evident in decreased colonic WBCs in stool amebiasis; Yersinia, blood, mucus, and WBCs reabsorption, Campylobacter infections increased motility
48TABLE 337-1 -- Food-borne Illnesses (Bacterial) INCUBATION DURATIONETIOLOGY PERIOD SIGNS AND SYMPTOMS OF ILLNESS ASSOCIATED FOODS LABORATORY TESTING TREATMENTBacillus anthracis 2 days to weeks Nausea, vomiting, malaise, Weeks Insufficiently cooked Blood Penicillin is first choice bloody diarrhea, acute abdominal contaminated meat for naturally acquired pain gastrointestinal anthrax. Ciprofloxacin is second option.
Bacillus cereus 16 hr Sudden onset of severe nausea 24 hr Improperly refrigerated Normally a clinical diagnosis. Supportive care(preformed and vomiting. Diarrhea may be cooked or fried rice, meats Clinical laboratories do notenterotoxin) present. routinely identify this organism. If indicated, send stool and food specimens to reference laboratory for culture and toxin identification.Bacillus cereus 1016 hours Abdominal cramps, watery 2448 hr Meats, stews, gravies, Testing not necessary, self- Supportive care(diarrheal toxin) diarrhea, nausea. vanilla sauce limiting (consider testing food and stool for toxin in outbreaks).Brucella abortus, B. 721 days Fever, chills, sweating, Weeks Raw milk, goat cheese Blood culture and positive Acute: Rifampin andmelitensis, and B. suis weakness, headache, muscle and made from unpasteurized serology doxycycline daily for 6 wk. joint pain, diarrhea, bloody stools milk, contaminated meats Infections with complications during acute phase require combination therapy with rifampin, tetracycline, and an aminoglycoside.Campylobacter jejuni 25 days Diarrhea, cramps, fever, and 210 days Raw and undercooked Routine stool culture; Supportive care. For severe vomiting; diarrhea may be poultry, unpasturized milk, Campylobacter requires cases, antibiotics such as bloody. contaminated water special media and incubation erythromycin and quinolones at 42C to grow. may be indicated early in the diarrheal disease. Guillain-Barr syndrome can be a sequela.Clostridium 1272 hr Vomiting, diarrhea, blurred Variable (from Home-canned foods with a Stool, serum, and food can be Supportive care. Botulismbotulinum children vision, diplopia, dysphagia, and days to low acid content, tested for toxin. Stool and antitoxin is helpful if given earlyand adults (preformed descending muscle weakness months). Can improperly canned food can also be cultured for in the course of the illness.toxin) be complicated commercial foods, home- the organism. These tests can Contact the state health by respiratory canned or fermented fish, be performed at some state department. The 24-hour number failure and herb-infused oils, baked health department for state health departments to death. potatoes in aluminium foil, laboratories and CDC. call is (700) 4887100. cheese sauce, bottled
49 INCUBATION DURATIONETIOLOGY PERIOD SIGNS AND SYMPTOMS OF ILLNESS ASSOCIATED FOODS LABORATORY TESTING TREATMENT garlic, foods held warm for extended periods of time (e.g., in a warm oven)Clostridium 330 days In infants <12 mo, lethargy, Variable Honey, home-canned Stool, serum, and food can be Supportive care. Botulismbotulinuminfants weakness, poor feeding, vegetables and fruits, corn tested for toxin. Stool and immune globulin can be constipation, hypotonia, poor syrup food can also be cultured for obtained from the Infant head control, poor gag and the organism. These tests can Botulism Prevention Program, sucking reflex be performed at some state Health and Human Services, health department California (510-540-2646), laboratories and CDC. Botulinum antitoxin is generally not recommended for infants.Clostridium 816 hr Watery diarrhea, nausea, 2448 hr Meats, poultry, gravy, Stools can be tested for Supportive care. Antibiotics notperfringens toxin abdominal cramps; fever is rare. dried or precooked foods, enterotoxin and cultured for indicated. time- and/or temperature- organism. Because abused food Clostridium perfringens can normally be found in stool, quantitative cultures must be done.Enterohemorrhagic E. 18 days Severe diarrhea that is often 510 days Undercooked beef Stool culture; E. coli O157 : Supportive care, monitor renalcoli (EHEC) including bloody, abdominal pain and especially hamburger, H7 requires special media to function, hemoglobin, andE. coli O157 : H7 and vomiting. Usually, little or no unpasteurized milk and grow. If E. coli O157 : H7 is platelets closely. E. coli O157 :other Shiga toxin fever is present. More common juice, raw fruits and suspected, specific testing H7 infection is also associatedproducing E. coli in children <4 yr old. vegetables (e.g., sprouts), must be requested. Shiga with hemolytic uremic syndrome(STEC) salami (rarely), and toxin testing may be done (HUS), which can cause lifelong contaminated water using commercial kits; complications. Studies indicate positive isolates should be that antibiotics may promote the forwarded to public health development of HUS. laboratories for confirmation and serotyping.Enterotoxigenic E. coli 13 days Watery diarrhea, abdominal 3 to >7 days Water or food Stool culture. ETEC requires Supportive care. Antibiotics are(ETEC) cramps, some vomiting contaminated with human special laboratory techniques rarely needed except in severe feces for identification. If cases. Recommended antibiotics suspected, must request include TMP-SMX and specific testing. quinolones.Listeria 948 hr for Fever, muscle aches, and nausea Variable Fresh soft cheeses, Blood or cerebrospinal fluid Supportive care and antibiotics;monocytogenes gastrointestinal or diarrhea. Pregnant women unpasteurized milk, cultures. Asymptomatic fecal Intravenous ampicillin, symptoms, 26 may have mild flu-like illness, inadequately pasteurized carriage occurs; therefore, penicillin, or TMP-SMX are wk for invasive and infection can lead to milk, ready-to-eat deli stool culture usually not recommended for invasive disease premature delivery or stillbirth. meats, hot dogs helpful. Antibody to disease.
50 INCUBATION DURATIONETIOLOGY PERIOD SIGNS AND SYMPTOMS OF ILLNESS ASSOCIATED FOODS LABORATORY TESTING TREATMENT Elderly or immunocompromised listerolysin O may be helpful patients may have bacteremia or to identify outbreak meningitis. retrospectively. At birth and Infants infected from mother at infancy risk for sepsis or meningitis.Salmonella spp. 13 days Diarrhea, fever, abdominal 47 days Contaminated eggs, Routine stool cultures Supportive care. Other than for cramps, vomiting. S. typhi and S. poultry, unpasteurized S. typhi and S. paratyphi, paratyphi produce typhoid with milk or juice, cheese, antibiotics are not indicated insidious onset characterized by contaminated raw fruits unless there is extra-intestinal fever, headache, constipation, and vegetables (alfalfa spread, or the risk of extra- malaise, chills, and myalgia; sprouts, melons). S. typhi intestinal spread, of the diarrhea is uncommon, and epidemics are often related infection. Consider ampicillin, vomiting is not usually severe. to fecal contamination of gentamicin, TMP-SMX, or water supplies or street- quinolones if indicated. A vended foods. vaccine exists for S. typhi.Shigella spp. 2448 hr Abdominal cramps, fever, and 47 days Food or water Routine stool cultures Supportive care. TMP-SMX diarrhea. Stools may contain contaminated with human recommended in the U. S. if blood and mucus. fecal material. Usually organism is susceptible; nalidixic person-to-person spread, acid or other quinolones may be fecal-oral transmission. indicated if organism is resistant, Ready-to-eat foods especially in developing touched by infected food countries. workers, e.g., raw vegetables, salads, sandwiches.Staphylococcus aureus 16 hr Sudden onset of severe nausea 2448 hrs Unrefrigerated or Normally a clinical diagnosis. Supportive care.(preformed and vomiting. Abdominal improperly refrigerated Stool, vomitus, and food canenterotoxin) cramps. Diarrhea and fever may meats, potato and egg be tested for toxin and be present. salads, cream pastries. cultured if indicated.Vibrio cholerae (toxin) 2472 hr Profuse watery diarrhea and 37 days. Contaminated water, fish, Stool culture; Vibrio cholerae Supportive care with aggressive vomiting, which can lead to Causes life- shellfish, street-vended requires special media to oral and intravenous rehydration. severe dehydration and death threatening food typically from Latin grow. If V. cholerae is In cases of confirmed cholera, within hours dehydration. America or Asia suspected, must request tetracycline or doxycycline is specific testing. recommended for adults, and TMP-SMX for children (<8 yr).Vibrio 248 hr Watery diarrhea, abdominal 25 days Undercooked or raw Stool cultures. Vibrio Supportive care. Antibiotics areparahaemolyticus cramps, nausea, vomiting. seafood, such as fish, parahaemolyticus requires recommended in severe cases: shellfish special media to grow. If V. tetracycline, doxycycline, parahaemolyticus is gentamicin, and cefotaxime.
51 INCUBATION DURATIONETIOLOGY PERIOD SIGNS AND SYMPTOMS OF ILLNESS ASSOCIATED FOODS LABORATORY TESTING TREATMENT suspected, must request specific testing.Vibrio vulnificus 17 days Vomiting, diarrhea, abdominal 28 days Undercooked or raw Stool, wound, or blood Supportive care and antibiotics; pain, bactermia, and wound shellfish, especially cultures. Vibrio vulnificus tetracycline, doxycycline, and infections. More common in the oysters, other requires special media to ceftazidime are recommended. immunocompromised, or in contaminated seafood, and grow. If V. vulnificus is patients with chronic liver open wounds exposed to suspected, must request disease (presenting with bullous seawater specific testing. skin lesions). Can be fatal in patients with liver disease and the immunocompromised.Yersinia enterocolytica 2448 hr Appendicitis-like symptoms 13 wk, usually Undercooked pork, Stool, vomitus, or blood Supportive care. If septicemia orand Y. (diarrhea and vomiting, fever, self-limiting unpasteurized milk, tofu, culture. Yersinia requires other invasive disease occurs,pseudotuberculosis and abdominal pain) occur contaminated water. special media to grow. If antibiotic therapy with primarily in older children and Infection has occurred in suspected, must request gentamicin or cefotaxime young adults. May have a infants whose caregivers specific testing. Serology is (doxycycline and ciprofloxacin scarlitiniform rash or erythema handled chitterlings. available in research and also effective). nodosum with Y. reference laboratories. pseudotuberculosis.
52 INCUBATION DURATIONETIOLOGY PERIOD SIGNS AND SYMPTOMS OF ILLNESS ASSOCIATED FOODS LABORATORY TESTING TREATMENT fever. Temporary lactose intolerance may Ready-to-eat foods touched by via immunoassay Severe diarrhea may occur. Infants and children, elderly, and infected food workers (salads, require fluid and immunocompromised are especially fruits). electrolyte vulnerable. replacement.Other viral agents 1070 hr Nausea, vomiting, diarrhea, malaise, 29 days Fecally contaminated foods. Identification of the virus in Supportive care,(astroviruses, abdominal pain, headache, fever Ready-to-eat foods touched by early acute stool samples. usually mild, self-adenoviruses, infected food workers. Some Serology. Commercial ELISA limiting. Goodparvoviruses) shellfish. kits are now available for hygiene. adenoviruses and astroviruses.
53 INCUBATION DURATION ASSOCIATEDETIOLOGY PERIOD SIGNS AND SYMPTOMS OF ILLNESS FOODS LABORATORY TESTING TREATMENT weight loss food contaminated by parasitesmay need at least 3 an ill food handler after samples. cooking; drinking water.Toxoplasma 523 days Generally asymptomatic, 20% may Months Accidental ingestion of Isolation of parasites from blood or Asymptomatic healthy, but infected,gondii develop cervical lymphadenopathy contaminated other body fluids; observation of persons do not require treatment. and/or a flu-like illness. In substances (e.g., soil parasites in patient specimens via Spiramycin or pyrimethamine plus immunocompromised patients: contaminated with cat microscopy or histology. Detection sulfadiazine may be used for pregnant central nervous system (CNS) feces on fruits and of organisms is rare; serology women. Pyrimethamine plus sulfadiazine disease, myocarditis, or vegetables), raw or (reference laboratory needed) can be may be used for immunocompromised pneumonitis is often seen. partly cooked meat a useful adjunct in diagnosing persons, in specific cases. Pyrimethamine (especially pork, lamb, toxoplasmosis. However, IgM plus sulfadiazine (with or without or venison) antibodies may persist for 618 mo steroids) may be given for ocular disease and thus may not necessarily indicate when indicated. Folinic acid is given recent infection. PCR of bodily with pyrimethamine plus sulfadiazine to fluids. For congenital infection: counteract bone marrow suppression. isolation of T. gondii from placenta, umbilical cord, or infant blood. PCR of white blood cells, CSF, or amniotic fluid, or IgM and IgA serology, performed by a reference laboratory.Toxoplasma In infants at birth Treatment of the mother may Months Passed from mothergondii (congenital reduce severity and/or incidence of (who acquired acuteinfection) congenital infection. Most infected infection during infants have few symptoms at birth. pregnancy) to child Later, they will generally develop signs of congenital toxoplasmosis (mental retardation, severely impaired eyesight, cerebral palsy, seizures), unless the infection is treatedTrichinella 12 days for Acute:nausea, diarrhea, vomiting, Months Raw or undercooked Positive serology or demonstration Supportive care plus mebendazole orspiralis initial symptoms; fatigue, fever, abdominal contaminated meat, of larvae via muscle biopsy. Increase albendazole others begin 28 discomfort followed by muscle usually pork or wild in eosinophils. wk after infection soreness, weakness, and occasional game meat (e.g., bear or cardiac and neurologic moose) complications
54TABLE 337-4 -- Food-borne Illnesses (Noninfectious) INCUBATION DURATION LABORATORYETIOLOGY PERIOD SIGNS AND SYMPTOMS OF ILLNESS ASSOCIATED FOODS TESTING TREATMENTAntimony 5 min8 hr usually Vomiting, metallic taste Usually self- Metallic container Identification of metal in Supportive care <1 hr limited beverage or foodArsenic Few hours Vomiting, colic, diarrhea Several days Contaminated food Urine. May cause Gastric lavage, BAL eosinophilia (dimercaprol)Cadmium 5 min8 hr usually Nausea, vomiting, myalgia, increase in Usually self- Seafood, oysters, clams, Identification of metal in Supportive care <1 hr salivation, stomach pain limited lobster, grains, peanuts foodCiguatera fish poisoning 26 hr Gl:abdominal pain, nausea, vomiting, Days to weeks A variety of large reef fish: Radioassay for toxin in fish Supportive care, IV mannitol.(ciguatera toxin) diarrhea to months grouper, red snapper, or a consistent history Children more vulnerable. amberjack, and barracuda (most common) 3 hr Neurologic:paresthesias, reversal of hot or cold, pain, weakness 25 days Cardiovascular:bradycardia, hypotension, increase in T wave abnormalitiesCopper 5 min8 hr usually Nausea, vomiting, blue or green vomitus Usually self- Metallic container Identification of metal in Supportive care <1 hr limited beverage or foodMercury 1 wk or longer Numbness, weakness of legs, spastic May be Fish exposed to organic Analysis of blood, hair Supportive care paralysis, impaired vision, blindness, coma. protracted mercury, grains treated with Pregnant women and the developing fetus mercury fungicides are especially vulnerable.Mushroom toxins, short- <2 hr Vomiting, diarrhea, confusion, visual Self-limited Wild mushrooms (cooking may Typical syndrome and Supportive careacting (museinol, muscarine, disturbance, salivation, diaphoresis, not destroy these toxins) mushroom identified orpsilocybin, Coprius hallucinations, disulfiram-like reaction, demonstration of the toxinartemetaris, ibotenic acid) confusion, visual disturbance.Mushroom toxins, long- 48 hr diarrhea; 24 Diarrhea, abdominal cramps, leading to Often fatal Mushrooms Typical syndrome and Supportive care, life-acting (amanitin) 48 hr liver failure hepatic and renal failure mushroom identified and/or threatening, may need life demonstration of the toxin supportNitrite poisoning 12 hr Nausea, vomiting, cyanosis, headache, Usually self- Cured meats, any contaminated Analysis of the food, blood Supportive care, methylene dizziness, weakness, loss of consciousness, limited foods, spinach exposed to blue chocolate-brown colored blood excessive nitrificationPesticides (organophosphates Few min to few Nausea, vomiting, abdominal cramps, Usually self- Any contaminated food Analysis of the food, blood Atropine;2-PAMor carbamates hours diarrhea, headache, nervousness, blurred limited (Pralidoxime) is used when vision, twitching, convulsions, salivation atropine is not able to control and meiosis symptoms and is rarely necessary in carbamate poisoning.Puffer fish (tetrodotoxin) <30 min Parasthesias, vomiting, diarrhea, abdominal Death usually Puffer fish Detection of tetrodotoxin in Life-threatening, may need pain, ascending paralysis, respiratory failure in 46 hr fish respiratory support
Scombroid (histamine) 1 min3 hr Flushing, rash, burning sensation of skin, 36 hr Fish:bluefin, tuna, skipjack, Demonstration of histamine Supportive care, antihistamines mouth and throat, dizziness, urticaria, mackerel, marlin, escolar, and in food or clinical diagnosis parasthesias mahi mahiShellfish toxins (diarrheic, Diarrheic shellfish Nausea, vomiting, diarrhea, and abdominal Hours to 23 A variety of shellfish, primarily Detection of the toxin in Supportive care, generally self-neurotoxic, amnesic) poisoning (DSP) pain accompanied by chills, headache, and days mussels, oysters, scallops, and shellfish; high-pressure limiting. Elderly are especially 30 min to 2 hr fever shellfish from the Florida coast liquid chromatography sensitive to ASP and the Gulf of Mexico Neurotoxic shellfish Tingling and numbness of lips, tongue, and poisoning (NSP) throat, muscular aches, dizziness, reversal few min to hours of the sensations of hot and cold, diarrhea, and vomiting Amnesic shellfish Vomiting, diarrhea, abdominal pain and poisoning (ASP) neurologic problems such as confusion, 2448 hr memory loss, disorientation, seizure, comaShellfish toxins (paralytic 30 min3 hr Diarrhea, nausea, vomiting leading to Days Scallops, mussels, clams, Detection of toxin in food Life-threatening, may needshellfish poisoning) parasthesias of mouth, lips, weakness, cockles or water where fish are respiratory support dysphasia, dysphonia, respiratory paralysis located; high-pressure liquid chromatographySodium fluoride Few min to 2 hr Salty or soapy taste, numbness of mouth, Usually self- Dry foods (e.g., dry milk, flour, Testing of vomitus or Supportive care vomiting, diarrhea, dilated pupils, spasms, limited baking powder, cake mixes) gastric washings. Analysis pallor, shock, collapse contaminated with sodium of the food. fluoridecontaining insecticides and rodenticidesThallium Few hours Nausea, vomiting, diarrhea, painful Several days Contaminated food Urine, hair Supportive care parathesias, motor polyneuropathy, hair lossTin 5 min8 hr usually Nausea, vomiting, diarrhea Usually self- Metallic container Analysis of the food Supportive care <1 hr limitedVomitoxin Few min to 3 hr Nausea, headache, abdominal pain, Usually self- Grains such as wheat, corn, Analysis of the food Supportive care vomiting limited barleyZinc Few hours Stomach cramps, nausea, vomiting, Usually self- Metallic container Analysis of the food, blood Supportive care diarrhea, myalgias limited and feces, saliva or urine
56 Shigella o Clinical manifestations (severe abdominal pain, high fever, emesis, anorexia, generalized toxicity, urgency, and painful defecation) The diarrhea may be watery and of large volume initially, evolving into frequent small-volume, bloody mucoid stools; most children (>50%) never progress to the stage of bloody diarrhea, whereas in others the 1st stools are bloody. Significant dehydration related to the fluid and electrolyte losses in both feces and emesis can occur. Untreated diarrhea may last 12 wk; only about 10% of patients have diarrhea persisting for more than 10 days. Persistent diarrhea occurs in malnourished infants, those with AIDS, and occasionally previously normal children. Even nondysenteric disease can be complicated by persistent illness. Neurologic findings are among the most common extraintestinal manifestations of bacillary dysentery, occurring in as many as 40% of hospitalized infected children. Enteroinvasive E. coli can cause similar neurologic toxicity. Convulsions, headache, lethargy, confusion, nuchal rigidity, or hallucinations may be present before or after the onset of diarrhea. The cause of these neurologic findings is not understood. In the past, these symptoms were attributed to the neurotoxicity of Shiga toxin, but it is clear that that explanation is wrong since the organisms isolated from children with Shigella-related seizures are usually not Shiga toxin producers. Seizures sometimes occur when little fever is present, suggesting that simple febrile convulsions do not explain their appearance. Hypocalcemia or hyponatremia may be associated with seizures in a small number of patients. Although symptoms often suggest central nervous system infection, and cerebrospinal fluid pleocytosis with minimally elevated protein levels can occur, Shigella meningitis is rare. The most common complication of shigellosis is dehydration. Inappropriate secretion of antidiuretic hormone with profound hyponatremia may complicate dysentery, particularly when S. dysenteriae is the etiologic agent. Hypoglycemia and protein-losing enteropathy may occur. Other major complications, particularly in very young, malnourished children, include sepsis and disseminated intravascular coagulation. Given that shigellae penetrate the intestinal mucosal barrier, these events are surprisingly uncommon. Shigellae and sometimes other gram-negative enteric bacilli are recovered from blood cultures in 15% of patients in whom blood cultures are taken; because patients selected for blood cultures represent a biased sample, the risk for bacteremia in unselected cases of shigellosis is presumably lower. Bacteremia is more common with S. dysenteriae serotype 1 than with other shigellae; the mortality rate is high (-20%) when sepsis occurs. Neonatal shigellosis is rare. Newborns may have only low-grade fever with mild nonbloody diarrhea. However, complications occur more commonly than in older children and include septicemia, meningitis, dehydration, colonic perforation, and toxic megacolon. S. dysenteriae serotype 1 infection is commonly complicated by hemolysis, anemia, and hemolytic- uremic syndrome. This syndrome is caused by Shiga toxinmediated endothelial injury; E. coli that produce Shiga toxin (E. coli 0157 : H7, E. coli 0111 : NM, E. coli 026 : H11) also cause hemolytic- uremic syndrome (see Chapter 518 ). Rectal prolapse, toxic megacolon or pseudomembranous colitis (usually associated with S. dysenteriae), cholestatic hepatitis, conjunctivitis, iritis, corneal ulcers, pneumonia, arthritis (usually 25 wk after enteritis), reactive arthritis with uveitis, urethritis and rash, cystitis, myocarditis, and vaginitis (typically with a blood-tinged discharge associated with S. flexneri) are uncommon events. Although rare, surgical complications of shigellosis can be severe; the most common are intestinal obstruction, appendicitis, and perforation. Death is a rare outcome in well-nourished older children; malnutrition, illness in the 1st year of life, hypothermia, severe dehydration, thrombocytopenia, hyponatremia, renal failure, and bacteremia are common in children who die during bacillary dysentery. The rare syndrome of severe toxicity, convulsions, extreme hyperpyrexia, and headache associated with brain edema and a rapidly fatal outcome without sepsis or significant dehydration (Ekiri syndrome or lethal toxic encephalopathy) is not well understood. o Diagnosis Although clinical features suggest shigellosis, they are insufficiently specific to allow confident diagnosis. Infection by Campylobacter jejuni, Salmonella, enteroinvasive E. coli, Shiga toxinproducing E. coli such as E. coli 0157 : H7, Yersinia enterocolitica, Clostridium difficile, and Entamoeba histolytica, as well as inflammatory bowel disease, may cause confusion. Presumptive data supporting a diagnosis of bacillary dysentery include the finding of fecal leukocytes (confirming the presence of colitis), fecal blood, and demonstration in peripheral blood of leukocytosis with a dramatic left shift (often with more bands than segmented neutrophils). The total peripheral white blood cell count is usually 5,00015,000 cells/mm3, although leukopenia and leukemoid reactions occur. Cultures of both stool and rectal swab specimens optimize the diagnosis of Shigella infections. In children who appear to be toxic, blood cultures should be obtained; this is particularly important in very young or malnourished infants because of their increased risk for bacteremia.
57 o Treatment As with gastroenteritis from other causes, the 1st concern about a child with suspected shigellosis should be for fluid and electrolyte correction and maintenance (see Chapters 55 and 337 ). Nutrition is a key concern in areas where malnutrition is common. A high-protein diet during convalescence enhances growth in the following 6 mo. A single large dose of vitamin A (200,000 IU) lessens severity of shigellosis in areas where vitamin A deficiency is common. Drugs that retard intestinal motility (diphenoxylate hydrochloride with atropine [Lomotil] or loperamide [Imodium]) should not be used because of the risk for prolonging the illness. Although some authorities recommend withholding antibacterial therapy because of the self-limited nature of the infection, cost of drugs, and risk for emergence of resistant organisms, there is a persuasive logic in favor of empirical treatment of all children in whom shigellosis is strongly suspected. Even if not fatal, the untreated illness may cause a child to be quite ill for weeks with chronic or recurrent diarrhea. Malnutrition may develop or worsen during prolonged illness, particularly in children in developing countries. The risk for continued excretion and subsequent infection of family contacts further argues against the strategy of withholding antibiotics. Ceftriaxone (50 mg/kg/day as a single daily dose IV or IM) can be used for empirical therapy. Oral 1st and 2nd generation cephalosporins are inadequate as alternative drugs despite in vitro susceptibility. Nalidixic acid (55 mg/kg/day divided qid PO), where available, is also an acceptable alternative drug. Azithromycin (12 mg/kg/day PO for the 1st day followed by 6 mg/kg/day for the next 4 days) has proven to be an effective alternative drug for shigellosis. Quinolones such as ciprofloxacin, norfloxacin, or ofloxacin, which have been recommended for use in persons 18 yr of age, are not routinely used for children because of the putative risk for arthropathy. Use of these agents is reserved for seriously ill children with bacillary dysentery due to an organism that is suspected or known to be resistant to other agents. Treatment is typically for a 5 day course. Zinc (20 mg/day for 14 days) improves immune response to Shigella infection, at least in settings where malnutrition is common. It should probably be included in therapy of dysenteric patients in such settings. Treatment of patients suspected on clinical grounds of having Shigella infection should be initiated when they are first evaluated. Stool culture is obtained to exclude other pathogens and to assist in antibiotic changes should a child fail to respond to empirical therapy. A child who has typical dysentery and who responds to initial empirical antibiotic treatment should be continued on that drug for a full 5 day course, even if the stool culture is negative. The logic of this recommendation is based on the difficulty of culturing Shigella from stools. In a child who fails to respond to therapy of a dysenteric syndrome in the presence of initially negative stool culture results, cultures should be retaken and the child re-evaluated for other possible diagnoses. o Prevention Breastfeeding Education about handwashing and proper food preparation E. coli o Types ETEC: watery diarrhea, self-limited, traveler's diarrhea EIEC: watery diarrhea or dysentery with fever, colonic lesions with ulcerations EPEC: major cause of infant acute and persistent diarrhea STEC/EHEC: mild diarrhea to severe hemorrhagic colitis, children 6 mos-10 years, person to person / food and water transmission, low number of organisms sufficient to cause disease, affects colon most severely, HUS (ARF, thrombocytopenia, hemolytic anemia) Fever is an uncommon manifestation (vs in shigellosis or EIEC) Antibiotics should not be given (increases risk for HUS) EAEC: acute and persistent diarrhea in children > 12 mos V. cholera o Types O1 El Tor and O139 Large inoculum required to cause disease; antacids increase risk of disease o Clinical manifestations Cholera is characterized by an acute onset of copious watery diarrhea and vomiting without abdominal cramps or fever. The stools are colorless with small flecks of mucus (rice-water) and are sometimes described as having a fishy odor. At first, children may be restless or extremely thirsty, but if fluid and electrolyte losses are not replaced, they may become lethargic or unconscious. Other signs of dehydration may rapidly manifest, including poor skin turgor, sunken eyes, dry mouth and tongue, no urine output, delayed capillary refill, rapid or weak pulse, and low blood pressure ( Fig. 198-1 ). Severe dehydration, metabolic acidosis, and hypokalemia can occur in 412 hr. The fluid losses may be so rapid that the child quickly develops hypovolemic shock, hypoglycemia, coma, and seizures and is at risk of dying within a few hours of onset.
58TABLE 337-5 -- Extraintestinal Manifestations of Enteric Infections ASSOCIATED ENTERICMANIFESTATION PATHOGEN(S) ONSET AND PROGNOSISFocal infections due to systemic spread All major pathogens Onset usually during the acuteof bacterial pathogens, including can cause such direct infection, but may present subsequently.vulvovaginitis, urinary tract infection, extraintestinal Prognosis depends on infection site.endocarditis, osteomyelitis, meningitis, infections, includingpneumonia, hepatitis, peritonitis, Salmonella,chorioamnionitis, soft tissue infection,and septic thrombophlebitis Shigella, Yersinia, Campylobacter, Clostridium difficileReactive arthritis Salmonella, Shigella, Typically occurs about 13 wk after Yersinia, infection. Relapses after reinfection Campylobacter, may develop in 1550% of people but Cryptosporidium, most children recover fully within 26 Clostridium difficile mo after the 1st symptoms appear.Guillain-Barr syndrome Campylobacter Usually occurs a few weeks after the original infection. Prognosis good although 1520% may have sequelae.Glomerulonephritis Shigella, Can be of sudden onset in acute, Campylobacter, referring to a sudden attack of Yersinia inflammation, or chronic, which comes on gradually. In most cases, the kidneys heal with time.IgA nephropathy Campylobacter Characterized by recurrent episodes of blood in the urine, this condition results from deposits of the protein immunoglobulin A (IgA) in the glomeruli. IgA nephropathy can progress for years with no noticeable symptoms. Men seem more likely to develop this disorder than are women.Erythema nodosum Yersinia, Although painful, is usually benign and Campylobacter, more commonly seen in adolescents. Salmonella Resolves with 46 weeks.Hemolytic uremia syndrome Shigella dysenteriae 1, Sudden onset, short-term renal failure. Escherichia coli 0157: In severe cases, renal failure requires H7, others several sessions of dialysis to take over the kidney function, but most children recover without permanent damage to their health.Hemolytic anemia Campylobacter, Relatively rare complication and may Yersinia have a chronic course.
59TABLE 337-6 -- Symptoms Associated with Dehydration MINIMAL OR NO DEHYDRATION (<3% MILD TO MODERATE SEVERE DEHYDRATION LOSS OF BODY DEHYDRATION (39% (>9% LOSS OF BODYSYMPTOM WEIGHT) LOSS OF BODY WEIGHT) WEIGHT)Mental status Well;alert Normal, fatigued or restless, Apathetic, lethargic, irritable unconsciousThirst Drinks normally; might Thirsty;eager to drink Drinks poorly; unable to drink refuse liquidsHeart rate Normal Normal to increased Tachycardia, with bradycardia in most severe casesQuality of Normal Normal to decreased Weak, thready, or impalpablepulsesBreathing Normal Normal;fast DeepEyes Normal Slightly sunken Deeply sunkenTears Present Decreased AbsentMouth and Moist Dry ParchedtongueSkinfold Instant recoil Recoil in <2 sec Recoil in >2 secCapillary Normal Prolonged Prolonged;minimalrefillExtremities Warm Cool Cold;mottled;cyanoticUrine output Normal to decreased Decreased Minimal
60DEGREE OF REPLACEMENT OFDEHYDRATION REHYDRATION THERAPY LOSSES NUTRITION maintenance fluid rates
ConstipationTABLE 329-1 -- Distinguishing Features of Hirschsprung Disease and Functional ConstipationVARIABLE FUNCTIONAL (ACQUIRED) HIRSCHSPRUNG DISEASEOnset of constipation After 2 yr of age At birthEncopresis Common Very rareFailure to thrive Uncommon PossibleEnterocolitis None PossibleForced bowel Usual NonetrainingAbdominal Uncommon CommondistentionPoor weight gain Rare CommonAnal tone Normal NormalRectal examination Stool in ampulla Ampulla emptyMalnutrition None PossibleAnorectal Distention of the rectum causes relaxation of the internal No sphincter relaxation or paradoxical increase in
61VARIABLE FUNCTIONAL (ACQUIRED) HIRSCHSPRUNG DISEASEmanometry sphincter pressureRectal biopsy Normal No ganglion cells, increased acetylcholinesterase stainingBarium enema Massive amounts of stool, no transition zone Transition zone, delayed evacuation (>24 hr)
Respiratory DisordersCough (and other respiratory symptoms)TABLE 381-1 -- Indicators of Serious Chronic Lower Respiratory Tract Disease in Children Persistent fever Ongoing limitation of activity Failure to grow Failure to gain weight appropriately Clubbing of the digits Persistent tachypnea and labored ventilation Chronic purulent sputum Persistent hyperinflation Substantial and sustained hypoxemia Refractory infiltrates on chest x-ray Persistent pulmonary function abnormalities Family history of heritable lung disease Cyanosis and hypercarbiaTABLE 381-3 -- Characteristics of a Chronic Cough and Their Etiologic SignificanceTYPE OF COUGH LIKELY RESPONSIBLE CONDITIONLoose (discontinuous), productive Bronchitis, asthmatic bronchitis, cystic fibrosis, bronchiectasisBrassy Tracheitis, habit coughWith stridor Laryngeal obstruction, pertussisParoxysmal (with or without gagging) Cystic fibrosis, pertussis syndrome, and foreign bodyStaccato Chlamydial pneumonitisNocturnal Upper or lower respiratory tract allergic reaction, or both, sinusitisMost severe on awaking in morning Cystic fibrosis, bronchiectasis, chronic bronchitisWith vigorous exercise Exercise-induced asthma, cystic fibrosis, bronchiectasisDisappears with sleep Habit cough, mild hypersecretory states such as in cystic fibrosis and asthmaTight (wheezy) Reactive airways
62CHARACTERISTIC THINK OFAbrupt onset Foreign body, pulmonary embolismFollows exercise Reactive airways diseaseAccompanies eating, drinking Aspiration, gastroesophageal reflux, tracheoesophageal fistulaThroat clearing Postnasal dripProductive (sputum) InfectionNight cough Sinusitis, reactive airways diseaseSeasonal Allergic rhinitis, reactive airways diseaseImmunosuppressed patient Bacterial pneumonia, Pneumocystis carinii, Mycobacterium tuberculosis, Mycobacterium avium intracellulare, cytomegalovirusDyspnea Hypoxia, hypercarbiaAnimal exposure Chlamydia psittaci (birds), Yersinia pestis (rodents), Francisella tularensis (rabbits), Q fever (sheep, cattle), hantavirus (rodents), histoplasmosis (pigeons)Geographic Histoplasmosis (Mississippi, Missouri, Ohio River Valley), coccidioidomycosis (southwest), blastomycosis (north and midwest)Workdays with clearing on Occupational exposuredays offTABLE 418-1 -- Respiratory Signs and Symptoms Originating from Outside the Respiratory TractSIGN NONRESPIRATORYSYMPTOM CAUSES PATHOPHYSIOLOGY CLUES TO DIAGNOSISChest pain Cardiac disease Inflammation (pericarditis), ischemia Precordial pain, friction rub on (anomalous coronary artery, vascular examination; exertional pain, radiation disease) to arm or neckChest pain Gastroesophageal reflux Esophageal inflammation and/or spasm Heartburn, abdominal pain diseaseCyanosis Congenital heart disease Right-to-left shunt Neonatal onset, lack of response to oxygenCyanosis Methemoglobinemia Increased levels of metHgb interfere Drug or toxin exposure, lack of with delivery of oxygen to tissues response to oxygenDyspnea Toxin exposure, drug side Variable, but often metabolic acidosis Drug or toxin exposure confirmed by effect, or overdose history or toxicology screen, normal Spo2Dyspnea Anxiety, panic disorder Increased respiratory drive and Occurs during stressful situation, other increased perception of respiratory symptoms of anxiety or depression effortsExercise Anemia Inadequate oxygen deliver to tissues Pallor, tachycardia, history of bleeding,intolerance history of inadequate dietExercise Deconditioning Self-explanatory History of inactivity, obesityintoleranceHemoptysis Nasal bleeding Posterior flow of bleeding causes History and physical examination appearance of pulmonary origin suggest nasal source, normal chest examination, and chest radiographyHemoptysis Upper gastrointestinal tract Hematemesis mimics hemoptysis History and physical examination bleeding suggest gastrointestinal source, normal chest examination and chest radiographyWheezing, Congenital or acquired Pulmonary overcirculation (ASD, Murmurcough, dyspnea cardiac disease VSD, PDA), left ventricular dysfunction Refractory to bronchodilators Radiographic changes (prominent pulmonary vasculature, pulmonary
63SIGN NONRESPIRATORYSYMPTOM CAUSES PATHOPHYSIOLOGY CLUES TO DIAGNOSIS edema)Wheezing, Gastroesophagel reflux Laryngeal and bronchial response to Emesis, pain, heartburncough disease stomach contents ?Vagally mediated bronchoconstriction Refractory to bronchodilators
64Pleural EffusionTABLE 397-5 -- Differentiation of Pleural Fluid TRANSUDATE EXUDATE COMPLICATED EMPYEMAAppearance Clear Cloudy PurulentCell count <1000 >1000 >5000Cell type Lymphocytes, monocytes PMNs PMNsLDH <200 U/L >200 U/L >1000 U/LPleural/serum LDH ration <0.6 >0.6 >0.6Protein >3 g Unusual Common CommonPleural/serum protein ratio <0.5 >0.5 >0.5 [*]Glucose Normal Low Very low[*](<40 mg/dL)pH[*] Normal (7.407.60) 7.207.40 <7.20, chest tube placement requiredGram stain Negative Usually positive >85% positive unless patient received prior antibiotics
CardiologyRheumatic Fever TABLE 182-2 -- Guidelines for the Diagnosis of Initial Attack of Rheumatic Fever (Jones Criteria, Updated 1992) MAJOR MANIFESTATIONS[*] MINOR MANIFESTATIONS
Polyarthritis Arthralgia
C-reactive protein
Prolonged PR interval
Chorea From Jones criteria, updated 1992. JAMA 1992;268:20692073. Copyright American Medical Association. * The presence of 2 major or of 1 major and 2 minor manifestations indicates a high probability of acute rheumatic fever if supported by evidence of preceding group A streptococcal infection. Diagnosis can be made without fulfilling the Jones criteria in 3 instances. o Chorea may occur as the only manifestation of acute rheumatic fever. o Indolent carditis may be the only manifestation in patients who 1st come to medical attention months after the onset of acute rheumatic fever. o Although most patients with recurrences of acute rheumatic fever fulfill the Jones criteria, some may not. Clinical maneuvers to elicit features of chorea o (1) demonstration of milkmaid's grip (irregular contractions of the muscles of the hands while squeezing the examiner's fingers) o (2) spooning and pronation of the hands when the patient's arms are extended o (3) wormian darting movements of the tongue upon protrusion o (4) examination of handwriting to evaluate fine motor movements.
65 TABLE 182-5 -- Duration of Prophylaxis for People Who Have Had Acute Rheumatic Fever: Recommendations of the American Heart Association CATEGORY DURATION
Rheumatic fever with carditis but without residual heart 10 yr or well into adulthood, whichever is longer disease (no valvular disease[*])
Rheumatic fever with carditis and residual heart disease At least 10 yr since last episode and at least until 40 yr of age; (persistent valvular disease[*]) sometimes lifelong prophylaxis From the American Academy of Pediatrics: Red Book: 2006 Report of the Committee on Infectious Diseases,27th ed. Elk Grove Village, IL, American Academy of Pediatrics, 2006, p 619. * Clinical or echocardiographic evidence.
66Hct/Hgb RESPIRATORY SUPPORT AND/OR SYMPTOMS TRANSFUSION VOLUMEHct Infants requiring minimal respiratory support (any mechanical ventilation or 15 mL/kg PRBCs over period of 24 hr30/Hgb endotracheal/nasal CPAP > 6 cm H2O and FIO2 0.4)10Hct Infants not requiring mechanical ventilation but who are receiving supplemental O 2 or 20 mL/kg PRBCs over period of 24 hr25/Hgb 8 CPAP with an FIO2 0.4 and in whom 1 or more of the following is present: (divide into 210 mL/kg volumes if fluid sensitive)
Hct Asymptomatic and an absolute reticulocyte count <100,000 cells/L 20 mL/kg PRBCs over period of 24 hr20/Hgb 7 (210 mL/kg volumes)
TABLE 473-1 -- Guidelines for Pediatric Fresh Frozen Plasma Transfusions [*]INFANTS, CHILDREN, AND ADOLESCENTS
67Solid TumorsTABLE 499-3 -- Differential Diagnosis of Abdominal and Pelvic Tumors in Infants and ChildrenTUMOR AGE CLINICAL SIGNS LABORATORY FINDINGSWilms Preschool Unilateral flank mass, aniridia, Hematuria;bone scintigraphy (clear cell hemihypertrophy sarcoma)Neuroblastoma Preschool GI/GU obstruction, raccoon eyes, myoclonus- Increased VMA;increased HVA;increased opsoclonus, diarrhea, skin nodules (infants) ferritin; stippled calcification in mass. Bone marrow positiveNon-Hodgkin >1 yr Intussusception in >2-yr-old Increased urate; bone marrow positivelymphomaRhabdomyosarcoma All GI/GU obstruction, sarcoma botryoides, vaginal bleeding, paratesticular massGerm cell/teratoma Preschool, Girls:abdominal pain, vaginal bleeding Increased hCG;Increased AFP teens Boys:testicular mass, new-onset hydrocele Sacrococcygeal mass/dimpleHepatoblastoma Birth3 yr Large, firm liver Increased AFPHepatoma School age, Large, firm liver; hepatitis B, cirrhosis Increased AFP teensTABLE 501-1 -- Comparison of Features of Osteosarcoma and the Ewing Family of TumorsFEATURE OSTEOSARCOMA EWING FAMILY OF TUMORSAge Second decade Second decadeRace All races Primarily whitesSex (M : F) 1.5 : 1 1.5 : 1Cell Spindle cellproducing osteoid Undifferentiated small round cell, probably of neural originPredisposition Retinoblastoma, Li-Fraumeni syndrome, Paget None known disease, radiotherapySite Metaphyses of long bones Diaphyses of long bones, flat bonesPresentation Local pain and swelling; often, history of injury Local pain and swelling; feverRadiographic Sclerotic destruction (less commonly lytic); Primarily lytic, multilaminar periosteal reaction (onionfindings sunburst pattern skinning)Differential Ewing sarcoma, osteomyelitis Osteomyelitis, eosinophilic granuloma, lymphoma,diagnosis neuroblastoma, rhabdomyosarcomaMetastasis Lungs, bones Lung, bonesTreatment Chemotherapy Chemotherapy Ablative surgery of primary tumor Radiotherapy and/or surgery of primary tumorOutcome Without metastases: 70% cured; with metastases at Without metastases: 60% cured; with metastases at diagnosis, 20% survival diagnosis, 2030% survival
68Urologic DisordersTABLE 545-1 -- Differential Diagnosis of Scrotal Masses in Boys and AdolescentsPAINFUL PAINLESSTesticular torsion HydroceleTorsion of appendix testis Inguinal hernia[*]Epididymitis Varicocele[*]Trauma:ruptured testis, hematocele Spermatocele[*]Inguinal hernia (incarcerated) Testicular tumor [*]Mumps orchitis Henoch-Schnlein purpura[*] Idiopathic scrotal edema* May be associated with discomfort.
Neurologic DisordersHeadacheTABLE 595-2 -- Indications for Neuroimaging in a Child with Headaches Abnormal neurologic signs Recent school failure, behavioral change, fall-off in linear growth rate Headache awakens child during sleep; early morning headache, with increase in frequency and severity Periodic headaches and seizures coincide, especially if seizure has a focal onset Migraine and seizure occur in the same episode, and vascular symptoms precede the seizure (20 50% risk of tumor or arteriovenous malformation) Cluster headaches in child; any child <56 yr whose principal complaint is a headache Focal neurologic symptoms or signs developing during a headache (i.e., complicated migraine) Focal neurologic symptoms or signs (except classic visual symptoms of migraine) develop during the aura, with fixed laterality; focal signs of the aura persisting or recurring in the headache phase Visual graying-out occurring at the peak of a headache instead of the aura Brief cough headache in a child or adolescent
69CNS InfectionsTABLE 602-1 -- Cerebrospinal Fluid Findings in Central Nervous System Disorders PRESSURE PROTEINCONDITION (MM H2O) LEUKOCYTES (MM3) (MG/DL) GLUCOSE (MG/DL) COMMENTSNormal 5080 <5, 75% lymphocytes 2045 >50 (or 75% serum glucose)COMMON FORMS OF MENINGITISAcute bacterial meningitis Usually elevated 10010,000 or more; usually 3002,000; PMNs Usually 100500 Decreased, usually <40 (or Organisms usually seen on Gram stain and recovered (100300) predominate <50% serum glucose) by culture.Partially treated bacterial Normal or 510,000; PMNs usual but mononuclear cells may Usually 100500 Normal or decreased Organisms may be seen on Gram stain. Pretreatmentmeningitis elevated predominate if pretreated for extended period of time may render CSF sterile. Antigen may be detected by agglutination testViral meningitis or Normal or Rarely >1,000 cells. Eastern equine encephalitis and Usually 50200 Generally normal; may be HSV encephalitis is suggested by focal seizures or bymeningoencephalitis slightly elevated lymphocytic choriomeningitis (LCM) may have cell decreased to <40 in some viral focal findings on CT or MRI scans or EEG. (80150) counts of several thousand. PMNs early but diseases, particularly mumps Enteroviruses and HSV infrequently recovered from mononuclear cells predominate through most of the (1520% of cases) CSF. HSV and enteroviruses may be detected by PCR course of CSFUNCOMMON FORMS OF MENINGITISTuberculous meningitis Usually elevated 10500; PMNs early, but lymphocytes predominate 1003,000;may <50 in most cases; decreases Acid-fast organisms almost never seen on smear. through most of the course be higher in with time if treatment is not Organisms may be recovered in culture of large presence of block provided volumes of CSF. Mycobacterium tuberculosis may be detected by PCR of CSFFungal meningitis Usually elevated 5500; PMNs early but mononuclear cells 25500 <50;decreases with time if Budding yeast may be seen. Organisms may be predominate through most of the course. Cryptococcal treatment is not provided recovered in culture. Cryptococcal antigen (CSF and meningitis may have no cellular inflammatory serum) may be positive in cryptococcal infection responseSyphilis (acute) and Usually elevated 50500;lymphocytes predominate 50200 Usually normal Positive CSF serology. Spirochetes not demonstrableleptospirosis by usual techniques of smear or culture; darkfield examination may be positiveAmebic (Naegleria) Elevated 1,00010,000 or more; PMNs predominate 50500 Normal or slightly decreased Mobile amebae may be seen by hanging-drop examination of CSF at room temperatureBRAIN AND PARAMENINGEAL ABSCESSESBrain abscess Usually elevated 5200; CSF rarely acellular; lymphocytes 75500 Normal unless abscess ruptures No organisms on smear or culture unless abscess (100300) predominate; if abscess ruptures into ventricle, PMNs into ventricular system ruptures into ventricular system predominate and cell count may reach >100,000Subdural empyema Usually elevated 1005,000; PMNs predominate 100500 Normal No organisms on smear or culture of CSF unless (100300) meningitis also present; organisms found on tap of subdural fluidCerebral epidural abscess Normal to 10500;lymphocytes predominate 50200 Normal No organisms on smear or culture of CSF
70 PRESSURE PROTEINCONDITION (MM H2O) LEUKOCYTES (MM3) (MG/DL) GLUCOSE (MG/DL) COMMENTS slightly elevatedSpinal epidural abscess Usually low, 10100;lymphocytes predominate 50400 Normal No organisms on smear or culture of CSF with spinal blockChemical (drugs, dermoid Usually elevated 1001,000 or more; PMNs predominate 50100 Normal or slightly decreased Epithelial cells may be seen within CSF by use ofsysts, myelography dye) polarized light in some children with dermoidsNONINFECTIOUS CAUSESSarcoidosis Normal or 0100;mononuclear 40100 Normal No specific findings elevated slightlySystemic lupus Slightly elevated 0500; PMNs usually predominate; lymphocytes may 100 Normal or slightly decreased No organisms on smear or culture. Positive neuronalerythematosus with CNS be present and ribosomal P protein antibodies in CSFinvolvementTumor, leukemia Slightly elevated 0100 or more; mononuclear or blast cells 501,000 Normal to decreased (2040) Cytology may be positive to very high
71Effects of In Utero Positioning o Normal full term newborns can have 20-30 degree hip and knee flexion contractures Resolve at 4-6 months o Changes due to positioning Hip externally rotates 80-90 degrees Limited internal rotation 0-10 degrees Lower leg has inward rotation and feet are supinated Top leg shows more changes than bottom leg Face may be distorted Spine and upper ex less affected o Effects completely resolve by 3-4 years
Start with knees and hips flexed Start with knees and hips flexed
Adduct thigh and direct force posteriorly Lift greater trochanter and abduct the hip o Most reliable sign of a dislocated hip: limitation of abduction o Shortening of the thigh, the Galeazzi sign, is best appreciated by placing both hips in 90 degrees of flexion and comparing the height of the knees, looking for asymmetry ( Fig. 677-5 ). Asymmetry of thigh and gluteal skin folds may be present in 10% of normal infants but is suggestive of DDH. Another helpful test is the Klisic test, in which the examiner places the 3rd finger over the greater trochanter and the index finger of the same hand on the anterior superior iliac spine. In a normal hip, an imaginary line drawn between the two fingers points to the umbilicus. In the dislocated hip, the trochanter is elevated, and the line projects halfway between the umbilicus and the pubis ( Fig. 677-6 ).
72 Slipped capital femoral epiphysis o Displacement of the CFE from the metaphysis; displacement of the femoral head from the femoral neck o May be acute or chronic o Older children and adolescents (10-17 years) - epiphysis of the femoral head slips away from the femoral neck because of weakened epiphyseal plate; painful limp; pain may be referred to the knee; obese limping adolescent complaining of knee pain o Possible causes: increased shearing stress, endocrine factors o Tx: immobilization, osteotomies
Fingertip amputation When referring, all amputated parts should be saved, wrapped in saline-soaked gauze, placed into a watertight bag, and then placed in ice water. Ice should never directly contact the part, as it can cause severe osmotic and thermal injury.
Fractures The differences in the pediatric skeletal system predispose children to injuries different from those of adults. The important differences are the presence of periosseous cartilage, physes, and a thicker, stronger, more osteogenic periosteum that produces new bone called callus more rapidly and in greater amounts. The other factors are a disproportionately large head, pliable rib cage, unprotected large and small bowel, and open epiphyseal plates in children. The pediatric bone has low density and more porosity. The low density is due to lower mineral content and the increased porosity due to increased number of haversian canals and vascular channels. These differences result in a comparatively lower modulus of elasticity and lower bending strength. The bone in children may fail either in tension or in compression; the fracture lines do not propagate as in adults, and hence there is less chance of comminuted fractures.
The Spine Scoliosis o Thoracic curves < 30 deg rarely progress o Lumbar curves more likely to progress Congenital scoliosis o GU abnormalities in 20-40%; renal UTZ should be done