Familial atrial tachyarrhythmia with short PR interval. A family had an unusual and perhaps unique familial dysrhythmia. The proband had a short PR interval with normal QRS and chronic recurrent paroxysmal atrial tachycardia (Lown-Ganong-Levine syndrome). The arrhythmia produced left ventricular dysfunction. Both paroxysmal atrial tachycardia (PAT) and left ventricular dysfunction were reversed with administration of digoxin and propranolol hydrochloride. Three family members had paroxysmal or chronic atrial fibrillation, first diagnosed at a relatively young age (23 years, 38 years, and early 40s, respectively). Five additional family members had short PR intervals with normal QRS, and eight other family members had borderline short PR intervals. The mode of inheritance appeared to be autosomal dominant with varying expressivity. We have described a familial syndrome characterized by PAT or atrial fibrillation in its advanced form with short PR interval as a possible identifying trait. The future course of members with isolated short PR is unknown.


Anemia in hemodialysis patients.

Anemia in hemodialysis patients. The association between anemia and chronic renal failure has been recognized since the early 19th century. With the introduction of regular dialysis treatment, an understanding of all aspects of this uremic complication has become of great importance, including an appreciation of the hazards of multiple blood transfusions. This analysis of hemoglobin levels and transfusion requirements in 84 dialysis patients focuses specific attention on hemolytic mechanisms, blood loss, and the effect of bilateral nephrectomy on erythropoiesis. Because no replacement for renal erythropoietin is available, particular attention must be paid to less important, but partially correctable factors that contribute to anemia. Blood transfusion requirements can then be reduced to a minimum, together with the risks of hypersplenism, hepatitis, and sensitization of the patient to alloantigens.


Anemia and intestinal dysfunction in former residents of the Caribbean. A group of subjects had common features of megaloblastic anemia due to vitamin B12 deficiency, normal or high serum folate, and evidence of intestinal malabsorption. They were all former residents of the Caribbean area now living in New York City. Despite similar symptoms and diagnostic studies, two subjects were found to have pernicious anemia and three to have tropical sprue. Achlorhydria, serum anti-intrinsic factor antibody, the severity of small intestinal abnormalities, and posttherapy Schilling tests were helpful differential factors. These subjects illustrate the problems that may be encountered in differentiating tropical sprue and pernicious anemia.


Single-voided urine metanephrine assays in screening for pheochromocytoma. To facilitate the screening for pheochromocytoma, we have validated the use of single-voided, spot urine specimens for the determination of total metanephrines. Metanephrine excretion was found to be quite constant throughout the day and night in ten patients with essential hypertension and seven patients with pheochromocytoma. The levels in single-voided specimens were closely correlated to those in 24-hour specimens in 100 hypertensive subjects. The mean +/- 2 SD metanephrine excretion in single-voided urine specimens from 500 hypertensive subjects was 0.351 +/- 0.356 mug/mg of creatinine.


The pathophysiology and clinical characteristics of severe hypophosphatemia. Hypophosphatemia, with or without phosphorus deficiency, is found commonly in patients with a variety of disease states. Severe hypophosphatemia is most often observed in chronic alcoholics, patients recovering from diabetic ketoacidosis, and those being therapeutically refed after severe weight loss. Its consequences may be severe and contribute to mortality. The following represents a selective review of available literature on this subject published over the past 50 years and a limited number of observations on patients and experimental animals. An attempt is made to examine mechanisms by which hypophosphatemia and phosphorus depletion probably occur, outline the resulting clinical disturbances, and suggest a plan of treatment. Finally, this review will hopefully point out areas of this interesting deficiency state that desperately need further investigation.


The malaise in internal medicine.

The malaise in internal medicine. Internists today are discomforted by uncertainty of identity, governmental interference with practice, total responsibility for patients' health, and by waning of faith in science. As personal "caring" physicians, internists are secure in primary care but should maintain their distinctive scholarly leadership as master clinicians and consultants. Humanism and science are one in patient care. Future practice patterns depend on physicians themselves participating in policy decisions for inevitable controls and rationing of government financed health services. The public must understand that good health depends not only on physicians but also on a better society and what people are willing to do for themselves. Western culture has been shaken by the cruel paradoxes of progress and technology. But, human choice not science is at fault, and only wisdom in the use of science will save us.


Synthesis, storage and degradation of polyglucose in Chlorobium thiosulfatophilum. Cultures of Chlorobium thiosulfatophilum form polyglucose during growth. The polyglucose is laid down within the cells as rosette-like granules, which are made up from smaller grains. The size of each granule appears to be limited to less than 30 nm, since an increase in polyglucose content leads to more granules being formed rather than an increase in granule size. The polyglucose in washed cells is fermented in the dark to acetate, propionate, caproate and succinate, of which acetate by far comprises the largest fraction (68%). During incubation of washed cells without hydrogen donor, the level of polyglucose decreases regardless of whether the cells are incubated in the dark or in the light. Since the products formed from polyglucose under the two different conditions are not the same, it is suggested that polyglucose in the dark serves as an energy source, whereas when in the light the role of polyglucose is mainly to provide the cell with reducing power.


Isolation and characterization of cytoplasmic and chloroplastic ribosomes and their ribosomal RNAs from the diatom Cylindrotheca fusiformis. The cytoplasmic and chloroplast ribosomes from the marine diatom Cylindrotheca fusiformis were isolated and characterized. The cytoplasmic ribosomes sedimented in sucrose at 84S and dissociated into subunits of 64S and 42S in the absence of Mg2+. It contained ribosomal RNAs with molecular weights of 1.31 X 10(6) and 0.70 X 10(6). The chloroplast ribosomes sedimented at 70S only in the presence of high Mg2+ concentrations (25-100 mM). No stable subunits were routinely observed and at very high levels of Mg2+ (greater than 100 mM) the 70S species was converted to a form sedimenting at 55S. At 4 degrees C ribosomal RNAs with molecular weights of 1.1 X 10(6) and 0.40 X 10(6) were detected on polyacrylamide gel electrophoresis. When the RNAs were resolved at room temperature the large molecular weight component disappeared while RNA with molecular weights of 0.65 X 10(6) and 0.53 X 10(6) were observed. Apparently the large chloroplast RNAs dissociated into two pieces of unequal molecular weight. These properties of the diatom's chloroplast ribosomes are very similar to those of the counterparts in unicellular green algae, which suggests that both types of algae have a common phylogenetic ancestor.


Electron-cytochemical demonstration of acid phosphatase in saprophytic Claviceps purpurea. p-Nitrophenylphosphate in combination with lead salt technique was used for the cytochemical localization of acid phosphatase (EC 3.1.3.2) in saprophytic submerged culture of Claviceps purpurea Tul. The lead reaction product was found in capsular fibrils, in the newly formed parts of the cell wall and in the vacuoles of aged cells (autolysosomes). Phosphatase activity was present also in particulate intra-cytoplasmatic organelles. The concentric layering of lead deposits in these organelles indicates their relationship to endoplasmic reticulum membranes.


Is disordered thinking unique to schizophrenia? To provide data on several issues related to disordered thinking in schizophrenia, 200 acutely ill psychiatric patients (including 55 schizophrenics) were evaluated on four instruments to assess thought pathology (the Object Sorting Test, the Rorschach Test, a social comprehension test, and a proverbs test). Patients were assessed at two phases of their disorders (the acute phase and the phase of partial recovery). The results suggest (1) disordered thinking is not unique to schizophrenia; (2) distinctions between mild and severe levels of thought pathology are important; and (3) disordered thinking is influenced by acute psychopathology and acute upset. Inferential evidence suggests (4) disordered thinking fits along a continuum with normal thinking; (5) "thought disorders" are not a discrete, separate entity, standing apart from other aspects of thinking; and (6) older concepts about primary symptoms in schizophrenia need reexamination.


Very short-term memory dysfunction in schizophrenia. Defective short time constant information processing in schizophrenia. Disordered, very short-term memory (VSTM) has been hypothesized as the fundamental cognitive deficit in schizophrenia. We describe a method that measures VSTM using self-stimulated auditory average evoked potentials. This paradigm allows the VSTM hyothesis to be tested relatively free of superficial attentional and motivational artifacts. The experimental results are consistent with a VSTM dysfunction in schizophrenia. Very short-term memory dysfunction is discussed in light of recent blink reflex evidence that there is a short time constant information processing system with a time base similar to VSTM (ie, 1 to 1,000 msec). This leads to new testable hypotheses about information processing and VSTM in schizophrenia. It also lays the basis for interpreting this phenomenon as a pathologic exaggeration of an adaptive neurophysiologic mechanism.


Motoric laterality imbalance in schizophrenia. A possible concomitant of left hemisphere dysfunction. Two hundred schizophrenics were compared to 200 normal controls on a measure of laterality that included handedness, footedness, and eye dominance scales. Schizophrenics showed more left-sidedness on the laterality score. The established relationship between motoric and cognitive aspects of functional brain asymmetry, found in neurological and normal populations, suggests that the leftward tendency of schizophrenics may be manifested in cognitive and conative functions as well. These results seem to corroborate previous findings indicating that schizophrenia might be related to left hemisphere dysfunction. No relationship was found between handedness and eye dominance either in the schizophrenic or the normal groups. This finding questions the assumption that eyedness-handedness nonconcordance is a pathological sign.


Consistency of Kurt Schneider-oriented diagnosis over 40 years. Diagnostic rates of routine hospital first and total admissions in various categories of functional mental illness in three strongly Schneider-oriented German psychiatric clinics over 40 years were examined. No matter how centers and categories were compared, statistically significant inconsistency was almost always found. Only first admissions for schizophrenia and mania in Munich and Heidelberg, as well as total schizophrenic admissions in Munich and Homburg/Saar, showed no significant diagnostic differences. However, from a practical point of view, actual percentage differences found in schizophrenia and mania were really not that great; indeed, rather similar diagnostic rates were apparent. This was in contrast to actual large percentage discrepancies shown between Munich on the one hand and Heidelberg and Homburg/Saar on the other with respect to manic-depressive illness and psychotic depression.


Communication patterns and role structure in families of male schizophrenics a study using automated techniques. Observers used automated methods to code who spoke to whom (Who-to-Whom) while families (mother, father, son) of 30 schizophrenics, 13 psychiatrically hospitalized nonschizophrenic controls, and 26 normal controls interacted on four tasks. From the Who-to-Whom data, measures of activity, channels of communication, and other variables were derived. Clear patterns of dominance were found in families of schizophrenics with fathers most and sons least active, while activity was more evenly distributed among family members in both control groups. The mother-son channel of communication was used more in both control groups than in families of schizophrenics. Differences on the Who-to-Whom measures were found among families of schizophrenic sons differing on premorbid, working and paranoid status, but social class had little effect.


Parental transactional style deviance as a possible indicator of risk for schizophrenia. The presence of a pattern of parental transactional style deviance on the Thematic Apperception Test (TAT) (a significant attribute of parents of offspring with schizophrenia spectrum disorders) was used to identify a group of disturbed nonpsychotic adolescents hypothesized to be at high risk for subsequently developing schizophreniform psychopathology. High-risk male adolescents came from two symptom groups, withdrawn adolescents and adolescents in active family conflict, which are symptom patterns similar to the premorbid pictures of two schizophrenia subtypes. High-risk parents also tended to show transactional style deviance in direct interaction with their child and in a written statement describing their child's problem. The degree of risk was significantly related to the amount of therapy in which the family was subsequently engaged and, at a four-year follow-up, to the level of adjustment of the adolescents seen earlier in the project.


Hospital vs community (foster) care for psychiatric patients. The aim of this study was to determine the effectiveness of foster care preparation and placement. Five hundred seventy-two patients from five hospitals were randomly assigned to foster care preparation (experimentals) or continued hospitalization (controls). They were studied before assignment, at placement of experimental subjects, and four months later regarding social functioning, mood, activity, and overall adjustment. Hospitals averaged two months preparing experimental subjects, resulting in 73% placed in foster care. Little change was observed between referral and placement. However, four months after placement, experimental subjects were significantly improved over controls, particularly in social functioning and adjustment. After four months, 88% of the foster care subjects were in the community. Findings suggest that attention should be given to selection criteria, that lengthy preparation may be unnecessary, and that foster care is superior to hospitalization for patients who cannot return to their own homes.


Biofeedback as an experimental treatment for postural hypotension in a patient with a spinal cord lesion. A man having severe postural hypotension resulting from a spinal cord lesion at level T3, was trained to raise and lower his blood pressure with the use of biofeedback. The procedure consisted of 11 learning sessions during which the subject's task was to effect specific changes of his blood pressure without skeletal muscle or respiratory involvement. Blood pressure was continuously monitored and reported to the subject, and positive verbal reinforcement for correct changes was also supplied. Analysis of the results revealed that the subject had learned to produce large voluntary changes in pressure. This learned ability was then applied in a standing position resulting in counteracting the postural hypotension. Suggestions for further research and implications for clinical applications are discussed.


Conditioning bladder responses in patients with spinal cord lesions. Two patients with spinal cord lesions and spastic neurogenic bladders were treated with classical conditioning techniques to reinstate independent bladder function utilizing electrical stimulation of the abdomen as the unconditioned stimulus and mild electrical stimulation of the thigh as the conditioned stimulus. Successful conditioning was achieved in both cases. Urination was brought under the control of a previously neutral stimulus with a high rate of responding.


Electromyographic study of the phasic activity of peroneus longus and brevis. Surface electrodes were utilized to pick up the electromyographic signals of the peroneus longus and brevis while foot switches recorded the gait cycle. The phasic activity of the two muscles was then  recorded while the subject walked on a treadmill, first with it placed on the level and then at 5% incline. The two muscles were found to work synchronously. This was later verified by using fine wire electrodes.


Fluidotherapy: evaluation of a new heat modality. The development of a new heat therapy modality (fluidotherapy) consisting of a bed of solid particles suspended in air is reported. Measurements of time integrated heat absorption by a hand immersed in the fluidotherapy unit at 120F (48.9C), a whirlpool at 105.2F, and a paraffin bath at 126F show the average heat absorption to be 17.2, 5.6 and 4.8 BTU respectively during a 15-minute treatment.


Pneumatic and standard double upright orthoses: comparison of their biomechanical function in three patients with spinal cord injuries. A detailed analysis of the biomechanical and physiological function of pneumatic orthoses comparing them to the standard double upright knee ankle orthoses was performed in three patients with different lesion levels. The results suggest that the main value of the long pneumatic orthosis is for early mobilization of the patient. In addition, the long pneumatic orthosis provides hip and trunk stabilization for marginal walkers who walk for exercise purposes. It is likely that most long-term functional ambulators will choose the standard knee ankle orthosis (KAO). The study dose not represent a statistical sample; therefore generalization must be limited.


Age and male erectile responsiveness.

Age and male erectile responsiveness. The purpose of the research was to determine (1) the difference in the rate of penile circumference increase per unit time between a group of young and a group of old normal males when exposed to the same erotic movie and (2) the correlation coefficient between penile shaft temperature increase and penile circumference increase. Ten males aged 19-30 years and ten males aged 48-65 years served as subjects. Each subject placed a thermistor and strain gauge on his penis and both outputs were recorded simultaneously on a two-channel recorder. The younger group responded at an erection rate 5.8 times faster than that of the older group. The correlation coefficient, based on the percent approach to oral temperature (98.6 degrees F) and the percent increase in penile circumference, was 0.75.


Treatment of male sexual deviation by use of a vibrator: case report. A new technique of enhancement of heterosexual responsiveness is described. This uses a classical conditioning paradigm with heterosexual photographic material as the CS and erections elicited by a vibrator as the UCS. A 44-year-old fetishist was treated in this manner. His response to treatment was favorable and details of change are presented. The timing of physiological and diary measure changes is in line with an explanation in terms of the treatment procedure. However, attitude changes began before treatment commenced.


Factor analysis of the sexual interaction inventory. Oblique and orthogonal factor analyses were performed on a data set produced by the administration of the Sexual Interaction Inventory to 32 former clients of a sexual dysfunction clinic. The analyses resulted in factor sets which are multiple and essentially psychologically meaningless. On the basis of the administration and the factor analysis results, several difficulties with the instrument are discussed. These are concerned with the question format, the distribution of the data, and the logical structure of the instrument.


Acute symbiotic psychosis in a postoperative transsexual. An unexpected complication of sex-reassignment surgery in a 23-year-old male-to-female transsexual is presented. Having been periodically depressed but never psychotic before surgery, the patient experienced an acute decompensation 3 days after operation. Symptoms of this time-limited illness included hallucinations and wish-fulfilling delusions, but most prominent was the demand to be in continuous contact with other people. Historical data revealed a symbiotic relationship with the grandmother ending precipitously with her death when the patient was 6. It is hypothesized that the unresolved grief reappeared after the surgery, owing to the unconscious linkage between becoming "female" and regaining the lost symbiotic object. Recompensation, possibly through the use of another person as a transitional object, was noted during the illness. This was felt to be a repetition of the pattern of reaction to previous losses.


A comparison of the measurement characteristics of two circumferential penile transducers. The measurement characteristics of two transducers which record penile circumference changes were compared. The subject wore two transducers simultaneously while viewing a section of an erotic film. Examination of the data revealed discrepancies in measurement between the two units, but these differences were shown to be statistically nonsignificant. Various measurement characteristics of the devices such as durability, ease of handling, and cost are discussed and recommendations are made to guide the worker in choosing a unit most suitable for clinical and research applications.


"Sex-role preference" as an explanatory variable in homosexual behavior. Sex-role preference is an important neglected variable in statistically controlled studies of homosexual behavior. This variable must accompany controls for degree of psychopathology of respondents and degree of heterosexual and homosexual behavior experienced by respondents. Cross-cultural differences exist. Mexican males have rigidly defined insertor-insertee roles, with earlier life events serving as predictors of these sex-role preferences. Greece is comparable to Mexico. In Turkey, stigmatization accompanies passive homosexuality. Role playing may be age graded, as in the Southwest Pacific. In lower socioeconomic classes of the United States, sex roles for homosexual males are more stereo-typically and unequivocably defined. Chicanos generally have strong sex-role preferences when involved in homosexual encounters; their attitude is similar to that found in Mexico. Among middle-class Anglo-American males, few or no sex-role feelings are associated with types of sex acts by most homosexually behaving males. This may be related to a focus on oral-genital rather than anal sex acts. The sharply dichotomized gender roles and the cultural formulation linking effeminacy and homosexuality appear to provide the necessary conditions for the development of sex-role preferences in many societies.


Sexual aggression: a second look at the offended female. A replication of earlier studies of male sex aggression conducted during the mid 1950s basically finds little modification in incidence and frequency. Approximately 50% of a sample of university women report being victims of sexual aggression during the academic year. Major changes from the earlier research appear to be focused around the nature of the pair relationships and the characteristics of the offended females.


Sexual counseling for cerebral palsied adults: case report and further suggestions. A mildly cerebral palsied woman was seen for premarital counseling regarding sexuality and the bearing and rearing of children. A multidisciplinary approach was used; sexual counselors, a gynecologist, an orthopedist, and a geneticist met with the client for a total of 8 hr counseling time. This approach is suggested for sexual counseling for the more severely handicapped, as well as in cases of mild and moderate disability.


Hepatic trauma: pitfalls in management.

Hepatic trauma: pitfalls in management. We reviewed 233 traumatic injuries to the liver. The mortality was 11%. Deaths were due to either uncontrolled intraoperative hemorrhage or the postoperative complications of multiple organ injuries. The majority of liver injuries can be safely managed with manual compression, porta hepatic control, simple sutures, or drainage. Liver injuries involving the hepatic veins or retrohepatic vena cava continue to be highly lethal despite the use of vascular isolation and hepatic resection.


"Spoon bowl" deformity of proximal femoral bypass vein graft: a cause of late graft failure on four occasions. We cite four examples of splaying of the proximal end of femoral bypass vein grafts accompanied by graft stenosis as a cause for late postoperative recurrent ischemic symptoms or graft failure. Though usually associated with dilation of the common femoral artery, one patient showed no arterial aneurysm. After appropriate angiographic views to demonstrate the "spoon bowl" deformity, corrective surgery to widen or bypass the stenotic segment and prevent further dilation of the artery, where indicated, resulted in patent grafts in all patients for at least one year. Diminishing pulses or the recurrence of ischemic symptoms after femoral bypass vein graft are indications for angiography of the graft. This should include appropriate views to demonstrate the proximal anastomosis that may show a "spoon bowl" deformity amenable to surgical correction.


Management of hereditary site-specific colon cancer. A family with site-specific colon cancer and discrete adenomatous colon polyps in certain members has provided a prototype for the study and clinical management of hereditary cancer. Analysis of this kindred showed an autosomal dominant mode of inheritance, early onset of cancer with predilection for the right colon, and frequent extraprimary cancers of the colon. Knowledge of these phenomena are critical to the management of this hereditary cancer syndrome. On our recommendation, two brothers of the family who were treated for colon cancer by hemicolectomy underwent prophylactic total colectomy. The resected colon from one of these patients had an occult adenocarcinoma in a villoglandular polyp.


Freezing and storage of breast cancer tissue for estrogen receptor protein assay: a convenient method. Sixteen patients underwent biopsy of primary or metastatic breast cancer and had equal portions of the tumors rapidly frozen by either the use of liquid nitrogen or the fluorinated hydrocarbon spray, Cryokwik. The estrogen receptor protein (ERP) assay was then performed on both specimens, utilizing the surcose gradient density method. In general, both methods of freezing gave similar results. Three patients had ERP measured after approximately three to four months of storage at -70 C. After a minimum of 14 weeks' storage, there was no statistically significant loss of activity by either method of preparation. The Cryokwik preparation method makes the accurate measurement of ERP possible for all community hospitals. The tissue may be prepared and stored until a convenient time for transportation to the appropriate laboratory.


Pathophysiology of postoperative ileus.

Pathophysiology of postoperative ileus. In eight dogs, cellotomy, rubbing the small bowel, and exposing it to air suppressed the migrating bursts of action potentials and contractions that occur in the gastrointestinal tract during fasting and greatly slowed (one to three days) the gastrointestinal transit of 7-mm plastic spheres. The operation also caused a transient one-day increase in the concentration of epinephrine and a more prolonged five-day increase in the concentration and norepinephrine in arterial and venous plasma. Phentolamine mesylate and propranolol hydrochloride prevented the inhibition of the bursts of gastric action potentials brought about by operation, but these drugs did not alter the inhibition of the small intestinal action potentials or the speed of gastrointestinal transit of spheres.


Cystic adventitial degeneration of the common femoral artery. Cystic adventitial degeneration (CAD) of the arteries is an uncommon cause of intermittent claudication in adults. The majority of reported cases have involved the popliteal artery and have occurred predominantly in men, and all had clinical evidence of occlusive vascular disease. An unusual example of CAD of the common femoral artery masqueraded clinically as an aneurysm, but the arteriogram was normal. It involved 5 cm of the artery and half its circumference, and was resected and replaced with a saphenous vein graft. The cyst contained clear mucoid fluid and a fibrous wall without a cyst lining. This is the eighth case reported of CAD involving the proximal segment of a large lower extremity artery. The etiology is unknown, but many authors think such cysts arise from primitive "arthrogenic" mesenchyme in the adventitia of blood vessels.


Iatrogenic choledochal stricture with choledochopancreatic fistula and pseudocyst. During routine cholecystectomy, a 39-year-old woman suffered an injury to the common bile duct, which resulted in a choledochal stricture and a choledochopancreatic fistula with pseudocyst formation. Treatment consisted of a choledochoduodenostomy for the common bile duct stricture and a pancreaticoduodenostomy for internal drainage of the pancreatic pseudocyst. A long-term follow-up has yielded an excellent clinical result.


Polyurethane foam (Bentley) micropore blood transfusion filter: filtration characteristics. Stored human blood of varying age was passed through polyurethane foam (Bentley) micropore blood transfusion filters. Passage through these filters resulted in decreased screen filtration pressure (SFP) of the blood and increased filter weights. Numerous microaggregates were removed and SFP returned to normal after filtration. Occlusion of the filter occurred after passage of only 2 units of whole blood. On the basis of this research, we conclude that polyurethane foam (Bentley) micropore blood transfusion filters are effective in removal of microaggregates from stored human blood. Because the filtering capacity is not great, it is recommended that when these filters are used during transfusion a new filter be used for each unit of blood administered.


Proximal radial artery-cephalic vein fistula hemodialysis. We describe a technique of proximal radial artery-cephalic vein fistula for hemodialysis. This is a useful method in patients with previously failed fistulas in the distal forearm or in diabetics in whom there is occlusive distal disease of the radial artery. This was used in 20 patients, with a patency rate of 90% over a follow-up period of 6 to 52 months and without any complications.


Neurologic aspects of hereditary hemorrhagic telangiectasia. Report of two cases. The several neurologic manifestations of hereditary hemorrhagic telangiectasia (HHT) may be caused by complications of pulmonary arteriovenous fistulae or associated central nervous system vascular malformations. The presence of skin and mucosal telangiectases should alert the clinician to the possibility of the disorder and in turn of its potential for associated neurologic disease, including cerebral hemorrhage and abscess. This report describes two cases and demonstrates that the clinical spectrum of HHT should be enlarged to include its admittedly rare, but serious, neurologic aspects.


EEG correlations with biochemical abnormalities in Reye syndrome. A patient with Reye syndrome was studied throughout the course of the illness with continuous EEG monitoring, and these patterns were correlated with serial determinations of serum ammonia and short-chain fatty acid concentrations. There was high correlation between degree of EEG abnormality, clinical symptoms, and elevations of the short-chain fatty acids, while serum ammonia concentrations correlated poorly with the EEG and with the clinical state.


Rigid spine syndrome. A type I fiber myopathy. The rigid spine syndrome is an unusual disorder characterized by nonprogressive mild axial and proximal muscle weakness, flexion contractures of the neck and spine, scoliosis, and joint contractures. The biopsy specimen from the patient described in this report shows type I fiber hypotrophy and predominance. This contrasts with previous reports that describe connective tissue proliferation as the major histologic abnormality.


Reversible cerebellocerebral disorder in primary hemochromatosis. Reversible cerebellar dysfunction is described in a patient with primary hemochromatosis. The electroencephalographic abnormalities initially recorded became normal after repeated phlebotomies. Both cerebellar dysfunction and electroencephalographic changes were present without clinical signs of hepatic failure, but may be related to the altered hepatic function tests.


Möbius syndrome due to brain stem tegmental necrosis. Möbius syndrome undoubtedly results from a variety of disorders affecting central or peripheral portions of appropriate cranial nerves or their target muscles. Pathological alterations observed in cranial nuclei are most often viewed as aplastic or dysplastic lesions. Two patients with Möbius syndrome with associated facial and skeletal malformations showed mineralized necrotic foci in multiple brain stem nuclei. Prenatal encephalomalacic lesions represent the pathological basis for some cases of congenital static Möbius syndrome.


Moya-Moya disease in black adults.

Moya-Moya disease in black adults. We describe two instances of Moya-Moya disease in black adults. Little is known concerning the origin of this disease. Currently, there is no effective treatment.


Experimental autoimmune myasthenia gravis in rats. Modification by thymectomy and prednisolone. In rats immunized with purified acetylcholine receptor protein (AChRP) from Torpedo electroplax, a defect of neuromuscular transmission physiologically identical to that seen in myasthenia gravis developed. The most sensitive index of the neuromuscular blockage was miniature end-plate potential (MEPP) amplitude. As early as 24 hours after inoculation with AChRP, the thymus showed reactive changes that are probably nonspecific. Removal of the thymus before or within three days after immunization delayed, but did not prevent, development of reduced MEPP amplitude. Prednisolone given within 35 days after immunization produced reversal of MEPP reduction within 24 hours, but had no such immediate effect when given 15 days later. It is probable that prednisolone acted by reducing the immunologic responsiveness of the animals during the developing phase of the defect of neuromuscular transmission.


Acetazolamide-induced interference with primidone absorption. Case reports and metabolic studies. Effects of acetazolamide on primidone plasma levels were studied in three patients. Apparent interaction occurred in two patients. Primidone was not detected in the plasma when given orally with acetazolamide in one patient. In another, peak serum concentration was delayed, with corresponding delays in urinary excretion of primidone and metabolites. Plasma and urine concentrations of the two metabolites, phenylethylmalonamide and phenobarbital, were also studied.


Electrical activity in voluntarily contracting muscle. The collision technique was used to determine the relationship between the electrical potential and the force produced by a voluntarily contracting first dorsal interosseus muscle. The method allows, in effect, synchronization of nerve impulses of asynchronously firing motoneurons subserving voluntary contraction. A compound muscle action potential, M (voluntary), thus recorded as an electrical corollary of muscle contraction, related linearly to the force of contraction under isometric conditions. Using M(voluntary), an approximate percentage of motoneuron pool discharging during a 10-msec period was calculated at various levels of voluntary effort. In ten healthy subjects, it increased linearly  with muscle force from 2.4% +/- 1.4% at 0.5 kg to 23.5% +/- 9.6% at 4.5 kg.


Oculopharyngodistal myopathy.

Oculopharyngodistal myopathy. An autosomal dominant, heredofamilial myopathy consisted of slowly progressive ptosis and extraocular palsy, and weakness of the masseter, facial, and bulbar muscles, as well as distal involvement of the limbs starting around 40 years of age or later. No other neurological symptoms or disturbances of other organs or tissues were observed. In one case, autopsy disclosed no remarkable change in the central and peripheral nervous system, and muscle biopsy specimens from all patients showed myopathic patterns without any specific change. A descriptive term, "oculopharyngodistal myopathy," was proposed to separate the present illness from other ocular myopathies.


Pure motor hemiplegia due to cerebral cortical infarction. Although pure motor hemiplegia has not been reported after cerebral cortical infarction, occasional exceptions may occur. We provide three such examples. Necropsy study confirmed the site of lesion in one patient, and laboratory results (EEG and computerized axial tomography) suggested cortical involvement in the other two patients.


Cerebral arteriovenous malformation and the primitive trigeminal artery. A 27-year-old woman with a small cerebral arteriovenous malformation and a primitive trigeminal artery had subarachnoid hemorrhage. This case and a review of the literature suggest that some of the "spontaneous" subarachnoid hemorrhages reported in patients with a primitive trigeminal artery may have been due to rupture of an unrecognized small arteriovenous malformation or aneurysm.


Influenza virus antibodies in Parkinsonism. Comparison of postencephalic and idiopathic Parkinson patients and matched controls. Hemagglutination inhibiting antibodies to four influenza virus strains: A/Swine/1976/30 (HswN1), A/PR/8/34 (HON1), A/England/1/51 (H1N), and A/Singapore/1/57 (H2N2), were studied in blood serum specimens from 20 patients with postencephalitic and 55 patients with idiopathic Parkinson disease and their age- and sex-matched controls. No significant differences were observed in the distribution or the mean titers of antibodies to any of the four strains tested, when the postencephalitic patients and their controls were compared. The postencephalitic group was also similar to the idiopathic group with regard to the influenza antibodies.


Decreased basal tear production associated with general anesthesia. Basal tear production was measured by means of standardized Schirmer strips and 0.5% proparacaine hydrochloride topical anesthesia in 20 patients. Premedication with systemic diazepam (Valium) and atropine sulfate had no effect on basal tear production. General surgical anesthesia resulted in a noticeable depression of basal tear production at 10, 30, and 60 minutes following induction of the anesthesia. It is suggested that prophylactic eye care include both replacement of tears and prevention of mechanical exposure of the cornea during general anesthesia.


A new simple method for canalicular intubation. A series of ten patients had canalicular intubation with Teflon and Silastic tubing. Our experience has shown that Silastic tubing appears to be more pliable, has good capillary action, and causes less irritation within the canalicular system than polyethylene or Teflon tubing. Five patients had very good results, three patients were improved, and there were two failures. We believe that this technique is useful in selected cases where there is an obstructed canaliculus near its junction with the sac. There should be only minimal scarring in the medial canthal area for the greatest chance of success with this method.


Cautery applications to relieve punctal stenosis. Applications of cautery to the mucosa and muscle at the base of the lacrimal papillae can result in retraction of the walls of the punctum, reestablishing patency. A relationship may exist between the cause of stenosis of the lacrimal papillae and stenosis at other juncture points in the lacrimal excretory system.


Climatic droplet keratopathy in a 16-year-old boy. Primary climatic droplet degeneration of the cornea that consisted of bandshaped keratopathy and superficial spheroidal golden opacities occurred in a 16-year-old boy. Light microscopy showed proteinaceous deposits in the superficial corneal stroma. Electron microscopy confirmed the colloidal nature of this material.


Prognostic factors in small malignant melanomas of choroid and ciliary body. Two hundre seventeen small malignant melanomas, each with a volume less than 1,400/cu mm, were studied to determine what factors were useful in predicting metastasis. Using a single-factor approach with 16 risk factors, we found seven that correlated well with outcome. These were cell type, pigmentation, size (largest dimension), scleral extension, mitotic activity, location of anterior margin of the tumor, and optic nerve extension. Using a linear discriminant function, the four best factors in combination were cell type, largest dimension, scleral extension, and mitotic activity. Four variables (largest diameter, location of anterior margin, mitotic activity, and optic nerve invasion) that might be correlated with clinical observations were found to be less accurate in separating fatal and nonfatal cases than cell type alone.


Prognostic factors in choroidal and ciliary body melanomas. We reviewed 293 cases of choroidal and ciliary body melanoma with a followup period of five years or more. Nine factors influenced significantly the prognosis: Age of the patient at the time of enucleation, location of the tumor and of its anterior border, largest tumor diameter in contact with the sclera, height of the tumor, integrity of the Bruch membrane, cell type, pigmentation, and scleral infiltration by tumor cells. Some of these factors can be evaluated clinically before the enucleation. Seven sets of three factors each were determined in order to have better prognostic values based on the clinical data and the cell type. The largest tumor diameter is the single most important clinical and pathological prognostic factor. The prognosis is relatively good when this diameter is 10 mm or less and becomes poor when it exceeds 10mm.


Pseudovitelliform macular degeneration.

Pseudovitelliform macular degeneration. Three patients with vitelliform-like macular lesions had normal electroooculogram (EOG) light-peak/dark-trough ratios, unlike typical cases of vitelliform dystrophy (Best macular dystrophy). Leakage of fluorescein dye from perifoveal cappillaries implicated an increased permeability of these vessels as the probable cause for the vitelliform-appearing lesions. All patients with vitelliform lesions demonstrating normal EOG ratios should have an evaluation by fluorescein angiography to rule out a diagnosis of what we prefer to call pseudovitelliform macular degeneration.


The prevalence of HLA-B12 and HLA-B7 antigens in primary open-angle glaucoma. HLA-B12 antigen was found in 50% and HLA-B7 was present in 49% of patients with primary open-angle glaucoma. Either one of the antigens B12 or B7 was noted in 88% of such glaucoma patients. The prevalences of B12, B7, or of either antigen were much greater in patients with primary open-angle glaucoma than in the general population or in patients with normal intraocular pressure. The prevalences in patients with ocular hypertension (IOP greater than 20 mm Hg) were intermediate between the ocular normotensives and the patients with primary open-angle glaucoma. The close association between HLA-B12 and HLA-B7 and primary open-angle glaucoma suggested immunologic components of the disease and possible convenient genetic markers.


The importance of disc hemorrhage in the prognosis of chronic open angle glaucoma. The progression of field damage of 48 eyes with chronic simple glaucoma and a splinter hemorrhage on the disc was compared with that of 48 chronic simple glaucoma eyes without hemorrhage. The incidence of visual field progression was substantially higher among those with a hemorrhage. Similar visual field progression was substantially higher in 29 eyes with elevated introcular pressure and a disc hemorrhage when compared with 29 ocular hypertensive patients with no hemorrhage. The prognostic importance of a disc hemorrhage is discussed.


Closed vitreous surgery: XV. Fibrovascular ingrowth from the pars plana sclerotomy. Ten eyes developed fibrovascular ingrowth from the sclerotomy following closed vitreous surgery. The complication was detected by examining all patients postoperatively by indirect ophthalmoscopy and biomicroscopy with a three-mirror lens. The average follow-up was 13.2 months. Nine patients had proliferative retinal disease prior to surgery and related systemic disease. In six eyes, useful vision was lost due to the ingrowth. In three eyes, a stabilization occurred. In one eye, that of a 12-year-old girl with an undefined syndrome of cochlear and retinal neovascularization, the ingrowth involuted. Rubeosis iridis was observed in 6 eyes that developed vascular ingrowth as opposed to 11 eyes in a control group of 78 eyes. Eyes with rubeosis iridis should not have vitreous surgery, and careful dissection of the Tenon capsule should be done over the sclerotomy.


Clinically unsuspected phacoanaphylaxis after ocular trauma. Phacoanaphylaxis (lens-induced uveitis) is a potentially curable ocular inflammation secondary to lens truma. Recently we have seen three clinically undiagnosed enucleation specimens in our eye pathology laboratory with this entity. Our observations add an argument to the recent concept that early lens removal in cases of traumatic cataract with lens capsule rupture would ultimately lead to better visual results.


The treatment of A and V patterns by monocular surgery. Eleven patients with "A" or "V" pattern strabismus and the absence of notable oblique muscles overactions, were operated on, using the technique of monocular vertical displacement of the horizontal rectus muscles. The average correction of the A or V pattern was 16.5 delta. Subjective torsional complaints were not reported by any of the patients. Torsion was measured postoperatively with the Maddox rod, Maddox wing, and amblyoscope. The average torsional measurement, at least one month postoperatively, was 1.6 degrees with a range of 0 degrees to 7 degrees. It was concluded that monocular vertical displacement of the horizontal rectus muscles can result in satisfactory correction of the A or V pattern and produces no substantial postoperative torsional symptoms or substantial measured torsion at both distance and near fixation.


Optic nerve hypoplasia with good visual acuity and visual field defects: a study of children of diabetic mothers. Seventeen children, born of severely diabetic mothers, exhibited segmental optic nerve hypoplasia with normal visual acuity and altitudinal or sector field defects corresponding to the hypoplastic areas of the disc. This is the first group of patients with optic nerve hypoplasia in which a possible cause has been identified. Optic nerve hypoplasia is neither a rare abnormality usually associated with serious central nervous system defects, nor is it always accompanied by decreased visual acuity.


Hemorrhage from isolated aneurysm of a retinal artery: report of two cases simulating malignant melanoma. Isolated retinal arterial aneurysms or macroaneurysms of the retinal arteries may be the cause of hemorrhage in the retina or vitreous. Two cases are reported in which hemorrhages from isolated retinal arterial aneurysms clinically simulated malignant melanoma of the choroid. Previous studies of lesions simulating malignant melanoma of the uvea have shown that vitreous and retinal hemorrhage may easily mislead the clinician. It is stressed that careful prolonged observation does not materially decrease the patient's chance for survival if he does have a malignant melanoma and may prevent an unnecessary enucleation.


Aqueous humor ascorbate concentration and open-angle glaucoma. The mean value for aqueous concentration in 35 patients with open-angle glaucoma was 22.4 +/- 12.9 mg/100 ml. The mean value for aqueous ascorbate in four patients with uncomplicated senile cataract was 11.55 +/- 3.01 mg/100 ml. The results indicate that the majority of open-angle glaucomatous eyes do not involve a deficiency of ascorbate, and suggest that ascorbate has no therapeutic value in the management of primary open-angle glaucoma. The magnitude of aqueous ascorbate variation among glaucoma eyes is probably related to the factors that influence the patency of trabecular meshwork, not the metabolic activity of the ciliary processes.


Periocular injection of corticosteroids: an experimental evaluation of its role in the treatment of corneal inflammation. The present experiments demonstrate that subconjunctivally injected corticosteroids are less effective in suppressing corneal inflammation than are topically instilled corticosteroids. Topical administration of 6.5 mg of prednisolone acetate over a 30-hour period reduced corneal inflammatory activity by 52%. Subconjunctival injection of 50 mg of the same steroid, also given over a 30-hour period, yielded a 15% reduction in corneal inflammation. A fourfold increase (200 mg) in the quantity of prednisolone acetate injected subconjunctivally resulted in a nonsignificant (P less than .05) increment (24%) in anti-inflammatory effect. Dexamethasone sodium phosphate achieved a 30% reduction in corneal inflammation, the maximum effect observed after subconjunctival administration. The data suggest that different modes of corneal penetration are involved after drug delivery via the two routes. Concurrent administration of corticosteroids by the topical and subconjunctival routes seemingly produced an additive anti-inflammatory effect.


New Tyndall photometer.

New Tyndall photometer. An instrument is described that can accurately measure delicate changes in the Tyndall phenomenon in the anterior chamber of the eye. An important feature is its use of an external light source and its simple adaptation to the slit lamp.


[Phlebography in fractures and dislocations of the talus (author's transl)]. Late osteonecrosis and/or osteoarthrosis occurs frequently after dislocation and fracture dislocation of the talus. The cause seems to be a primary vascular lesion additionally to the initial damage of bone and cartilage and subsequent joint incognity. We recommended phlebography 3 to 6 months after injury for the proof or exclusion of a vascular lesion. Our experience with this method has been encouraging.


[Biomechanics of vertebral segments. Clinical and prognostical interpretations (author's transl)]. The authors propose a new model to estimate the forces of the intrinsic equilibrium of the segment. Based on the theory of the so-called transient pole, it became possible to describe the momentary movement of an angle relative to its neighbour and to define the way of the intrinsic equilibrium. This transient pole fixes the centre of rotation around which the upper angle moves relative to the lower vertebral body. Normal and pathological cases can be diagnosed at once by use of a special röntgenological appliance in connection with the statistical experience. It becomes possible to calculate the forces and movements acting on a segment.


Surgical treatment of peri-prosthetic fractures after total knee replacement (author's transl). An operative procedure is described for the treatment of periprosthetic fractures of the femur after total knee replacement. The special problems of osteosynthesis are being discussed. In case of technical contraindications the partial replacement of the distal femur is being recommended as an alternative procedure.


Juvenile ankylosing spondylitis, its clinical features and HLA-B27. We have experienced six patients of juvenile ankylosing spondylitis. Their chief complaints were polyarthralgia or coxalgia, however, any pain or stiffness in lumbar regions was absent. These clinical features were atypical for those of ankylosing spondylitis, and much more like those of a peripheral arthropathy such as juvenile rheumatoid arthritis. After a long-term follow-up, all the patients showed definite clinical features of ankylosing spondylitis. HLA-B27 was found in 4 out of 6 cases. This frequency is almost equal to that of adult ankylosing spondylitis patients. HLA typing was very important in the early diagnosis.


[The problem of acetabular loosening in patients with total hip joint replacement (author's transl)]. Recently aseptic loosining of the acetabular part in patients with allo-arthroplasty has also become a numerically increasing problem. Different opinions exist regarding the etiology, they range from mechanical to tissue factors. Favourable personal experience with careful handling of the bony acetabulum during surgery appeares to confirm that the size and type of treatment of the acetabular wound during implantation of the acetabular prosthesis are significant factors for the durability of the allo-arthroplasty. A formula which was compiled indicates and clearly shows how the increase in area of the acetabular wound depends on the increase of the acetabular radius. In addition, the laws of cellular reaction from general pathology should be the reason to limit damage to living tissue to the absolutly necessary extent.


[Congenital dislocations of the knee joint (author's transl)]. The authors discuss the frequency, etiology, clinical symptomatology, classification, and treatment of congenital dislocations of the knee joint. They present 8 cases of their own material. The congenital knee joint dislocation can be treated conservatively with good results. Treatment with plaster casts does not interfere with treatment of simultaneous hip dysplasias in Pavlik bandages. Even foot deformities can be treated at the same time. They emphasise the need for earliest treatment which seems equally important for all three deformities: knee joint dislocations, hip dysplasias, and club feet.


[The Mannheimer oxide ceramic-metal composite hip prostheses (author's transl)]. Preliminary results with two alumina oxide ceramic-metal composite hip prostheses in 30 patients are presented. The indication, operative technique and postop. care as well as the postop. results are described and documented in detail.


[The operative treatment of kyphose (author's transl)]. Report is given on 17 patients with operative treatment of their kyphoses of various etiology. The correction itself has been carried out following diverse preoperative methods of correction using an anterior and/or dorsal approach to the spine. Altogether an average correction of 22.3 degree, that is 48 per cent could be obtained.


Fogarty catheter technique for removal of endobronchial foreign body. An increasing number of internists and chest physicians have become participants in the practice of bronchoesophagology. The otolaryngologist seeking to broaden his skills in the art of bronchoscopi foreign body removal  will find his take become increasingly difficult. We describe a rare case involving the aspiration of a 1.5-cm marble by a patient, resulting in marginal pulmonary function. What we beleive is a previously unreported technique for rapid removal of the object was used.


DiGeorge syndrome associated with multiple squamous cell carcinomas. Experimental evidence and clinical experiences have have shown that immune mechanisms and cancer are closely related. A case of a 42-year-old man with DiGeorge syndrome is presented as an interesting example of an impaired immune mechanism and is described in association with multiple squamous cell carcinomas of the upper respiratory system. The congenital absence of the thymus gland in DiGeorge syndrome results in an absence of a cell-mediated immune response. There are plasma cells and germinal centers in lymph nodes, but lymphocytes in the paracortical areas are sparse. Patients wiith DiGeorge syndrome have no delayed hypersensitivity, cannot be actively sensitized with dinitrochlorobenzene, reject allografts poorly, and have no lymphocytic response to phytohemagglutinin antigens.


Traumatic neuroma of the nose.

Traumatic neuroma of the nose. We report a case of a traumatic neuroma of the nose. It was an unusual site for two distinct types of tissue response to skin and soft trauma that exceeded the reparative needs of the body. In the first instance, a small cutaneous nerve was sectioned, and its inability to establish continuity distally resulted in a neuroma. In the second instance, the excision site of the neuroma was reinjured, and an abnormal connective tissue response occurred, resulting in a hypertrophic scar or keloid. Both were treated similarly with excision, and ultimately with local steroid injection with satisfactory results.


Delayed epistaxis secondary to shotgun wound to maxillary artery. Report of a case. Severe delayed epistaxis following open or closed trauma has been reported in the literature by many authors. We report a case of delayed epistaxis following a shotgun wound to the face. The evaluation of this type of patient, including the use of angiography to localize the bleeding, is illustrated. We review methods of conservative management of epistaxis and outline the surgical treatment of this patient.


The importance of tumor attachment to the carotid artery. The records of 508 patients who had undergone radical neck dissection were reviewed. Twenty-eight of these patients (5.5%) were found to have tumor attached to the carotide arterial system. Twenty-four patients (86%) died with tumor, the mean survival time being 16.9 months. Local recurrence of tumor in the neck occurred in 46% of the patients and distant metastases in 67%. Eighteen percent of those who developed local recurrence did so in the absence of distant metastases and might have benefited from a more aggressive approach toward the tumor.


Cholesteatoma in children.

Cholesteatoma in children. The 65 cholesteatomas operated on in children showed a more expansive and rapid growth than those in adults. In one fifth of the cases cholesteatoma filled the whole air-cell area, which was wide in half of the children. Fifty-two ears of these children had an attic or a posterosuperior perforation. One case was complicated by a fistula in the horizontal semicircular canal, and the ossicular chain was unbroken in 23 cases (35%). Thus, the findings support the idea of the primary soft-tissue spread of cholesteatoma in children. Five ears (8%) discharged postoperatively, and three ears (5%) were reoperated on and showed residual tympanal cholesteatoma. Cavity obliteration with canal wall down technique proved safe, even in the cases of the most extensive and active cholesteatoma.


Static compliance of the eardrum in Meniere disease. A recent study  compared the acoustic impedance values in the affected and unaffected ears of patients with unilateral Meniere disease, and suggested that the higher impedance measured in the affected ears was due to increased intralabyrinthine pressure (endolymphatic hydrops). The present study does not support these findings. This conclusion is based on the static compliance of 17 patients with unilateral Meniere disease, measured during the glycerin test. Glycerin, an osmotic diuretic, produces transient improvements in pure-tone threshold and/or speech discrimination in the affected ear only. Orally administered glycerin is thought to cause these improvements by reducing endolymphatic hydrops. We were unable to find any change in static complicance after glycerin ingestion. Also no difference between the affected and unaffected ears, either before or after glycerin, was noted.


Lateral defects in velopharyngeal insufficiency. Diagnosis and treatment. A variety of surgical techniques is available for the closure of substantial velopharyngeal defects. While the use of the centrally based pharyngeal flap remains the cornerstone of surgical treatment, there are patients who have poor lateral pharyngeal wall (LPW) mobility. After surgery that uses a centrally based flap, the speech result in such patients is less than optimal, since the poorly mobile LPW does not approximate to the central flap, thus creating a lateral defect. We have added the Towne view to our protocol for cine-speech studies to study better the lateral defects. This view is perpendicular to the velopharyngeal spincter and shows the true-purse-string nature of it. The incidence of such defects in our last 40 consecutive patients is reviewed. Based on the existence of a lateral defect, recommendations are made for a system of operations in the treatment of velopharyngeal insufficiency.


Parasympathetic nerve section for control of sialorrhea. Twenty mentally retarded patients with a considerable drooling problem underwent transtympanic sectioning of selected preganglionic parasympathetic nerve fibers to the major salivary glands. Following surgery, cessation of trouble-some sialorrhea occurred in 15 to 20 patients; but by six months postoperatively, only ten patients remained controlled. The best results obtained were in a subgroup of five patients who underwent bilateral Jacobson nerve and chorda tympani nerve section. In four of these patients, sialorrhea was controlled after a minimum follow-up of two years. Parasympathetic denervation of the major salivary glands via a transtympanic approach is offered as a primary modality of therapy in those mentally retarded patients judged to have considerable sialorrhea. Sacrifice of both chorda tympani nerves appears to enhance the surgical results.


Perilymph fistulas secondary to gunshot wound. Localization with a radioactive pharmaceutical. The symptoms, clinical evaluation, routes of formation, and repair of perilymph fistulas are reviewed. We report a case with oval and round window perilymph fistulas secondary to a gunshot wound. A radioactive pharmaceutical was injected into the subarachnoid space in the lumbar area. Subsequent radioactivity was detected at the sites of the fistulas. We discuss the implications and a suggested technique for fistula detection.


Frequency effects of temporary N1 depression following acoustic overload. The effect of acoustic overload on the N1 potential at different frequences was determined. The conditions of overstimulation were such as to produce temporary effects. The results of the study were: (1) the N1 potential was substantially depressed by acoustic overloads that did not alter the alternating current (AC) cochlear potential; (2) the maximum N1 depression was at one-half octave above the overstimulation frequency; (3) there was no N1 depression at frequencies below the frequency of overstimulation; and (4) the N1 depression recovered within 24 hours.


Influence of succinylcholine on middle component auditory evoked potentials. Auditory evoked potentials in the middle component time domain (post-stimulus, 8 to 50 msec) were recorded in response to 1,000-Hz tone pips in a normal-hearing adult subject. Electromyographic (EMG) responses in response to ulnar nerve shocks were recorded from the ipsilateral hypothenar muscles. With the assistance of an anesthesiologist, data were collected during a normal resting state, a state of light sedation, and a state of complete skeletal muscle paralysis from succinylcholine administration. During the paralyzed state, there was abolition of the normal EMG responses seen in the resting and sedated states. The auditory evoked potentials, however, appeared unchanged during the paralyzed state, indicating that they were not of myogenic origin.


Postnatal development of laryngeal reflexes in the dog. An investigation of laryngeal reflex maturation was undertaken in the dog to identify its period of most rapid postnatal differentiation. The canine model was selected because of its laryngeal reflex similarity to that of the human being. Observations in the dog indicate maximum differentiation to occur 1 to 1 1/2 months postnatally, based on measurements of recurrent laryngeal nerve myelination and conduction velocity. Latency and threshold analyses of evoked laryngeal responses additionally confirm this viewpoint. The importance of these observations rests in an understanding of developmental upper respiratory tract mechanisms that may be chronologically related to causes of fatal ventilatory events producing unexplained infant death. Such a correlation, requiring further confirmation, is based on the assumption that a critical period of most rapid development allows a transient susceptibility to noxious influences that can cause a disruption in organ function and even death of that organism itself.


Management of otitic cholesteatomas.

Management of otitic cholesteatomas. When otitic cholesteatomas are removed by a surgical method that preserves or reconstructs the bony canal wall and tympanic membranes, there is a risk of imcomplete removal and possible regrowth of the lesion. To study the incidence of this complication, 52 patients with cholesteatoma had elective repeaated explorations at various postoperative intervals; eight (15.4%) had early cholesteatoma regrowth even though there was no clinical evidence of it. Seven of these regrowths were small and were again srugically removed. It is advocated that conservative surgery for cholesteatoma be followed up in two years by another elective surgical exploration to check for and dispose of possible cholesteatoma regrowth. This would provide safety comparable to that achieved with radical mastoidectomy, with far better function and hearing. The method does necessitate patient understanding and cooperation and I suggest methods of achieving this.


Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease). Management of epistaxis in nine patients using systemic hormone therapy. The combination of a progestogen and an estrogen (Enovid, Enovid E) has been used successfully in the management of nine patients with hereditary hemorrhagic telangiectasia (HHT) and severe epistaxis: Such therapy was instituted when epistaxis was severe and only after other therapeutic measures had failed. Seven of our patients required the 5-mg tablet (Enovid) to achieve a satisfactory clinical result; two patients received the 2.5-mg preparation (Enovid E). The eight women received cyclic therapy; the man received therapy without interruption. Because of the possibility of serious adverse effects from the drugs, it is required that each patient receive a complete medical evaluation prior to therapy and at frequent intervals while receiving therapy. Enovid therapy for HHT is not recommended for the patient with mild bleeding that can be controlled by conventional therapy.


Auditory test results in 500 cases of acoustic neuroma. Auditory test results in the first 53 causes of this series of 500 acoustic neuromas were reported in 1964. Subsequent studies involving larger numbers of patients detailed new results based on these additional cases. Five hundred cases of surgically confirmed acoustic neuromas were analyzed. Pure-tone loss configurations, speech discrimination, auditory adaptation as measured by the modified tone decay test, Bekesy audiometry, short increment sensitivity index test, and alternate binaural loudness balance test data were evaluated. The results of this investigation in general confirm previous studies, although percentage figures for positive tests differed in the large series. Acoustic reflex results were reported for those patients evaluated more recently. Slightly more than half of the cases had inconsistent audiometric results in one or more aspects. Inconsistencies to the tests were examined in detail. Consistent responses to the tests appear to be related to the size of the lesion. The full battery of audiologic tests is of importance in the differential diagnosis of acoustic neuromas.


Solitary extramedullary plasmacytoma of the larynx. Plasmacytomas, indistinguishable histologically from plasma cell tumors arising in bone marrow, occur almost anywhere in the body-usually independently of multiple myeloma. Thus, they represent an isolated area of plasmacytic dyscrasia but occasionally can be the first evidence of multiple myeloma. From 1949 to 1974, six patients with solitary extramedullary plasmacytomas of the larynx were seen at the Mayo Clinic; during this period, approximately 3,100 patients with malignant laryngeal neoplasms were examined. For these six patients, the median age was 53 years (range, 32 to 63 years). The first symptom in five patients was hoarseness (mean duration, nine months). Excision, electrocoagulation, irradiation, or some combination of these constituted effective therapy. Follow-up ranged from 3 to 25 years. The choice of therapy depends on the size, shape, and location of the tumor. Extensive surgery is rarely necessary.


Histopathologic changes in the temporal bone resulting from measles infection. We report the pathologic changes in the temporal bones of four children who died of bronchoppenumonia secondary to acute measles infection. All suffered from severe necrotizing otitis media characterized by round cell infiltration. In one case, multilobulated giant cells (Warthin-Finkelday) were found in the middle ear. These cells appear to be similar to those found elsewhere within the body during acute measles infection. Inner ear changes were seen in two cases. Severe loss of nerve fibers and ganglion cells with atrophy of the striae vascularis was present in one case. Adhesions were seen between the Reissner membrane and the tectorial membrane in one case and the Reissner membrane and the inner spiral limbus in a second case. Such findings are characteristic of those seen in the cochlear duct of persons suffering from prenatal rubella.


Postnatal descent of the epiglottis in man. A preliminary report. The function of the epiglottis in adult man is unclear. However, during early infancy the epiglottic cartilage appears to play an important role in separating the upper respiratory tract from the upper digestive tract. This separation is accomplished by approximation of the epiglottis to the palate, providing a continuous airway from the nose through the larynx into the trachea. This structural arrangement, however, is uniquely lost in man during postnatal development. Maturational descent of the epiglottis, found to occur between 4 and 6 months of age, is verified by cineradiography. This structural change, requiring a reorganization of respiratory function, represents a discrete developmental event manifesting the potential for oral tidal respiration from a pattern of obligate nasal breathing. This period, four to six months postnatally, interestingly coincides with the peak incidence of sudden infant death syndrome (crib death), which similarly occurs at 3 to 5 months of age.


Oncocytes in the nasopharynx.

Oncocytes in the nasopharynx. A mucosal biopsy specimen taken from the anterior superior part of the nasopharynx revealed the presence of numerous oncocytes. These cells were observed in both the mucosal epithelium and in the submucosal glands. Ultrastructurally, the oncocytes were characterized by vast numbers of closely packed mitochondria, which in some cells had a rarified matrix, and by a paucity of other cytoplasmic organelles. It could not be determined whether these cells represented an early stage in the development of a nasopharyngeal oncocytoma, or whether they were the result of local reactive oncocytic metaplasia.


The cardiovascular response to acoustic stimuli. The purposes of this investigation were to determine whether adults display alterations of cardiac rate under acoustical stimulus conditions and whether such alterations are influenced by signal level. The stimulus consisted of a narrow band of noise centered at 1 000 Hz and presented at 20, 40, 60 and 80 dB SPL. The stimulus was found to produce alterations of heart rate significantly different from variation under non-stimulus conditions, indicating that cardiovascular responses occurred. However, the responses themselves were unaffected by differences of sound pressure level.


Asymptotic noise-induced temporary threshold shift in chinchilla measured by the auditory evoked response. Monaural chinchillas were exposed to octave band (4 kHz center frequency) noise at 80 dB SPL for 96 h. Auditory threshold at frequencies of 1, 2, 4, and 8 kHz was estimated by the auditory evoked response (AER) technique prior to noise exposure, and on a fixed schedule, both during exposure and following removal from the noise. Results were analyzed in terms of both threshold and threshold shift. Results indicate that the AER is adequate measure of asymptotic noise-induced temporary threshold shift. Advantages of the use of AER as opposed to behavioral means of threshold determination are discussed. Threshold during this long-term noise exposure was found to be determined by the spectral and intensity components of the noise independent of pre-exposure threshold. Implications for future research in long-term noise exposure are discussed.


Middle components of human auditory averaged electroencephalic response: waveform variations during averaging. The bases for variations of the middle-component (8-50 ms) auditory averaged electroencephalic response (AER) to clicks during the averaging process were explored by examining (1) the trend of the mean variance of the averaged waveform, (2) averaged waveforms generated by successive blocks of stimuli fractioned from a total of 512 stimuli, and (3) averaged waveforms generated by successive, but partially overlapping, blocks of responses recorded for a train of 512 stimuli. These analyses indicated no systematic changes in peak latencies or peak-to-peak amplitudes as stimulation progressed. Fluctuations in middle AER waveform are more readily explained by non-stationarity of the background electrophysiologic noise. Previously reported amplitude reduction with increasing stimulus number possibly can be explained by progressive reduction of the background noise on which the consistent-amplitude middle components are superimposed.


[Stapedial reflexes in presbyacusis and occupational deafness]. Acoustic reflex thresholds were measured on 251 subjects (418 ears) with varying degrees of sensorineural hearing loss. The subjects are divided in three groups according to the pathology involved: presbyacusis and noise-induced hearing loss and combined hearing loss caused by both presbyacusis and noise exposure. The dynamic range of hearing was found to be reduced for all cases and at all frequencies. The decrease in dynamic range was proportional to the severity of the hearing loss. The sensation levels of the acoustic reflex thresholds seem to be dependent on the degree of hearing loss and not on the aetiology. Practical conclusions are drawn concerning the prediction of thresholds of hearing and the diagnosis of recruitment based on reflex measurements.


Audiometric and ophthalmological findings in rubella deafness. In an attempt to study the relevance of ophthalmological changes to the diagnosis of cochlear hearing impairment due to fetal rubella infection, a survey of the literature combined with a clinical investigation was carried out. Based on the investigation of 57 patients, 31% had congenital hearing impairment due to fetal rubella infection. Of these patients, 61% had typical rubella retinal changes. In the remaining 69%, ophthalmoscopy revealed no abnormality, except in 1 patient. As the cause of the hearing impairment in this patient was unknown, it was concluded that the mother had suffered from subclinical rubella infection in the first trimester of her pregnancy. It is concluded that rubella retinitis is found with such a high incidence in rubella children that it can be used as a tool in the diagnosis of cochlear hearing impairment due to fetal rubella infection. Patients with congenital hearing impairment ought to undergo a routine ophthalmoscopy which will detect eventual pigmentary changes.


Pseudoauditory air conduction thresholds in the deaf. Pure-tone air conduction thresholds, as well as speech detection thresholds, were obtained from both ears of 10 profoundly deaf individuals before and after the administration of a local anesthetic in two conditions: under earphones, and while wearing a conventional-type hearing aid. The results indicated only minimal differences between the pre-anesthetic and post-anesthetic thresholds. These findings support the contention that high intensity, low frequency thresholds in the profoundly deaf are primarily auditory in nature and do not contain significant vibratory components. The continued use of hearing aids with profoundly deaf children is recommended.


The relative importance of recovery time in compression hearing aids. Recovery time (RT) in compression hearing aids was studied using six aids of identical characteristics except for factory-modified RT. Recordings of CID W-22 lists were made through the aids and played to normally hearing and otopathologic (sensorineural) subjects. Speech intelligibility was measurably influenced by RT variation with attack time held constant (10 ms). The effect was much more pronounced with the normal than with the hearing-impaired listeners, a phenomenon not unexpected when testing such a subtle feature in a low-fidelity transmission system. When the RTs of the aids were respecified according to a proposed standard (IEC) the data suggested that RTs for English in the vicinity of 90 ms may be most desirable. Additional work is required using more complex stimuli and background noise.


A comparison of hearing aids with amplitude compression. Intelligibility scores with a modified rhyme test were obtained for normally hearing subjects and subjects with sensorineural hearing impairment using several commercial hearing aids. These hearing aids differed mainly in values of time constants of compression and of harmonic distortion during overshoots. In general, better performance was obtained for the shorter time constants of compression, but performance seemed also to be affected by the occurrence of higher levels of harmonic distortion and of peak-clipping during overshoots.


Progressive systemic sclerosis in a family: case report of a mother and son and review of the literature. Progressive systemic sclerosis (PSS) developed in a 6-year-old boy and in his 38-year-old mother 9 years later. Both parent and child had Raynaud's phenomenon, integumental sclerosis, and pulmonary involvement, but they differed in other aspects of their disease. Previous descriptions of the familial occurrence of PSS are reviewed and compared to the present case reports. Environmental and genetic factors of possible etiologic significance are discussed.


Resources for the care of arthritics in a nonmetropolitan community. Redding, California. All professional, institutional, and administrative resources available to persons with musculoskeletal disabilities, including arthritis, were surveyed in a large nonmetropolitan community. Access to such resources and their residual capacity to serve local arthritics was determined. The resources appeared adequate as was the access, but the referral pattern was poorly developed. Most of the physicians in the community did not perceive a need for a trained rheumatologist practicing in the community.


Comparative effects of adrenal regeneration hypertension on non-arteriosclerotic and arteriosclerotic Sprague-Dawley vs spontaneously hypertensive rats. Adrenal regeneration hypertension (ARH) was induced in virgin and breeder, spontaneously hypertensive (SHR) and Sprague-Dawley (SD) rats. The blood pressure of the previously normotensive, virgin, SD rats and the SD breeder rats with preexistent mild hypertension became greatly elevated. ARH caused an increase in the preexisted severe hypertension in SHR virgin and breeder rats. Serum enzymes, e.g., CPK, SGOT and LDH, were greatly elevated concomitant with the finding of old and new foci of myocardial necrosis. ARH produced a dichotomous metabolic effect, i.e., elevated cholesterol, glucose, and corticosterone levels in SD rats but reduced levels in SHR rats. The zonae glomerulosae of the the regenerated adrenal glands of SD rats were devoid of lipid whereas the zonae glomerulosae of SHR rats were full of lipid. Intact SHR breeder rats develop arterial lesions confined to their reproductive organs but after ARH treatment, they were found to have aortic, coronary and renal arterial lesions which were similar to those which occur, spontaneously, in SD breeder rats. It is suggested that changes in the spectrum of adrenal steroids produced during ARH may contribute to the diverse metabolic changes and the alterations in the usual cardiovascular degenerative changes found in these two strains.


Glucocorticosteroid inhibition of nonphagocytic discharge of lysosomal enzymes from human neutrophils. Glucocorticosteroids and mineralocorticosteroids were tested for their capacity to inhibit the nonphagocytic discharge of two lysosomal enzymes--a cartilage matrix-degrading neutral protease and beta-glucuronidase--from highly purified human neutrophils. Lysosomal enzyme discharge from neutrophils adherent to nonphagocytizable, immobilized, heat-aggregated IgG was inhibited by the four glucocorticosteroids--methylprednisolone sodium succinate, triamcinolone acetonide hemisuccinate, para methasone acetate, and hydrocortisone sodium succinate. These glucocorticoids also inhibited zymosan-induced release of beta-glucuronidase from neutrophils that had been pretreated with cytochalasin B in order to completely prevent the onset of phagocytosis. Inhibition by the glucocorticoids of lysosomal enzyme discharge provoked by a soluble divalent cation ionophore was also observed. Neither desoxycorticosterone acetate nor aldosterone hemisuccinate, two mineralocorticosteroids, inhibited lysosomal enzyme release. Similarly, the salt moieties of some of the steroids tested, such as sodium succinate and sodium acetate, failed to elicit an effect on enzyme release. Therefore interference with lysosomal enzyme discharge was restricted to the glucocorticosteroid ring structure. Because interference either with the adherence of neutrophils to immune reactants or with the activities of the discharged lysosomal enzymes by the glucocorticoids could be interpreted as inhibition of lysosomal enzyme release, steroidal effects on these parameters were examined. None of the glucocorticoids tested elicited any significant effects on neutrophil adherence or lysosomal enzyme activity. Thus it appears that glucocorticosteroids are capable of inhibiting directly the nonphagocytic discharge of lysosomal enzymes from human neutrophils.


Lactoferrin and lysozyme levels in synovial fluid: differential indices of articular inflammation and degradation. Lysozyme and lactoferrin levels were measured in 71 synovial fluids (SF) of patients with traumatic effusions, osteoarthritis, rheumatoid arthritis, pseudogout, septic arthritis, and gout, as well as in 91 synovial fluids graded according to their neutrophil count. Elevated lysozyme levels were found in all the inflammatory arthritides and also in osteoarthritis. Lactoferrin levels were not increased in osteoarthritis but displayed a close correlation to the extent of the inflammatory response as judged by SF neutrophilia. The ratio of lysozyme to lactoferrin decreased progressively with increasing SF neutrophilia. In vitro experiments showed that lactoferrin is released from neutrophils isochronously with lysozyme and beta-glucuronidase. Lactoferrin was not found in hyaline cartilage, a tissue known to contain lysozyme. These results are consistent with belief that SF lysozyme has a major derivation from both cartilage and neutrophils, and that lactoferrin arises only from neutrophils. These findings indicate that the simultaneous measurement of lysozyme and lactoferrin provides a potentially useful index of both joint inflammation and cartilage degradation.


The effect of continuous heparin infusion for one year on serum cholesterol and triglyceride concentrations in the dog. Fourteen normal dogs received continuous infusions of intravenous heparin for one year by means of an implantable infusion pump. Heparin wad admistered at an overall mean rate of 666 units/kg/day, a dose sufficient to prolong the Lee-White clotting time to greater than twice normal. Eight control, animals, under the same dietary and activity regimen, received continuous infusions of bacteriostatic water for one year by means of implanted pumps. Serum cholesterol concentrations rose to 50% above control values after one month of heparin infusion, and remained significantly (P less than 0.05) elevated at this level for the remaining 11 months. Serum triglyceride levels were unchanged. A possible mechanism for this elevation resides in the known effect of heparin to increase plasma free fatty acid concentrations by its activation of lipoprotein lipase. These results may have implications for the long-term use of heparin anticoagulation in the treatment of atherosclerotic states in man.


Relapsing polychondritis: an ultrastructural study. Ear cartilage from a typical case of relapsing polychondritis was examined with the electron microscope. A large number of dense granules and vesicles, which were compatible with matrix vesicles or lysosomes, surrounded the affected chondrocytes. In less severely damaged chondrocytes, these granules and vesicles appeared to be formed by pinching off of the cytoplasmic processes or by budding from the processes. Calcification of the granules was minimal. In severely damaged chondrocytes, an admixture of these granules and cytoplasmic organelles occurred. It is speculated that many of these dense granules are lysosomal in nature and that they may produce inflammation and reduce the proteoglycan content of cartilage.


Lyme arthritis: an epidemic of oligoarticular arthritis in children and adults in three connecticut communities. An epidemic form of arthritis has been occurring in eastern Connecticut at least since 1972, with the peak incidence of new cases in the summer and early fall. Its identification has been possible because of tight geographic clustering in some areas, and because of a characteristic preceding skin lesion in some patients. The authors studied 51 residents of three contiguous Connecticut communities -- 39 children and 12 adults -- who developed an illness characterized by recurrent attacks of asymmetric swelling and pain in a few large joints, especially the knee. Attacks were usually short (median: 1 week) with much longer intervening periods of complete remission (median: 2.5 months), but some attacks lasted for months. To date the typical patient has had three recurrences, but 16 patients have had none. A median of 4 weeks (range: 1-24) before the onset of arthritis, 13 patients (25%) noted an erythematous papule that developed into an expanding, red, annular lesion, as much as 50 cm in diameter. Only 2 of 159 family members of patients had such a lesion and did not develop arthritis (P less than 0.000001). The overalll prevalence of the arthritis was 4.3 cases per 1,000 residents, but the prevalence among children living on four roads was 1 in 10. Six families had more than 1 affected member. Nine of 20 symptomatic patients had low serum C3 levels, compared to none of 31 asymptomatic patients (P less than 0.005); no patient had iridocyclitis or a positive test for antinuclear antibodies. Neither cultures of synovium and synovial fluid nor serologic tests were positive for agents known to cause arthritis. "Lynne arthritis" is thought to be a previously unrecognized clinical entity, the epidemiology of which suggests transmission by an arthropod vector.


Fatty acid composition of adipose tissue in patients with coronary heart disease. In order to study the relationship between the fatty acid composition of adipose tissue and coronary heart disease (CHD), 34 consecutive male patients with acute myocardial infarction and 33 hospitalized men free of CHD were compared. Patients with diabetes mellitus, endocrine disorders, liver and kidney diseases, recent changes in body weight and deviations from the "normal", customary diet were exlcuded. A statistically significant difference between the two groups was observed only in stearic acid, its proportion being lower in CHD patients (3.25% vs. 4.13%). Using multivariate discriminant analysis, age discriminated best between the groups, followed by stearic acid. The signs observed were positive for the former and negative for the latter. All other acids, relative body weight, and skinfold measurements did not significantly contribute to the discrimination. Age did not correlate with the proportion of stearic acid. Blood lipids from samples taken within 24 h of admission did not significantly differ between the groups. Three months later they had risen considerably in the infarct patients. The metabolic basis of the relationship between CHD and stearic acid is not clear at present. Additional studies are necessary to substantiate the importance of this acid as an indicator of CHD.


The time course of metabolic changes induced by dietary cholesterol in guinea pigs. The time course of the effects of dietary cholesterol on the lipid composition of liver, plasma and red cells of guinea pigs, of the pathological changes of tissues and of hematological parameters was studied. The purpose was to identify the primary injury and so to contribute to the eludication of the mechanism(s) for the development of the hemolytic anemia observed in this species after long-term cholesterol supplementation. The results showed that the initial effects observed within 1 week occur in the liver lipids and histology accompanied by changes in plasma and RBC lipids. These events were followed by further, slower increases of tissue lipids without major qualitative changes. The earliest signs of an anemia were observed between weeks 5 and 7. We conclude that the primary insult of cholesterol is liver damage leading to the production of abnormal plasma lipoproteins which in turn cause a net increase of RBC-cholesterol that is accompanied by their morphological abnormalities. The hemolytic anemia does not seem to be caused directly by either the altered composition of RBC nor their altered morphology.


Serum 25-hydroxycalciferol in myocardial infarction. Oral intake of calciferol (vitamin D) was higher in patients with myocardial infarction than in controls. Having established an assay for 25-hydroxycalciferol (25-OH-D), the principal circulating form of the vitamin, we measured this compound in control subjects and patients with myocardial infarction. In controls, 25-OH-D varied with the season: levels were high in summer and low in winter. Furthermore, levels were low in control subjects above 60 years of age. In patients with myocardial infarction, normal and low values for 25-OH-D were found. It is concluded that in this region patients with myocardial infarction do not consume greater amounts of vitamin D.


Inhibition of lipoprotein lipase activity by a monoclonal immunoglobulin in autoimmune hyperlipidemia. Autoimmune hyperlipidemia (AIH) may be induced a variety of antibodies which inhibit different stages of the lipolytic process by which the lipid load is removed from the circulating lipoproteins. In a patient having a monoclonal gammopathy and a nephrotic syndrome with a glomerulonephritis and a marked hypertriglyceridemia, it was found previously that the monoclonal IgG gamma Lac. reacted with human VLDL as well as with human serum albumin. Here it is demonstrated that the purified IgG gamma inhibits the lipolysis of triglyceride substrates by reacting with a substance (Lac. S) necessary for lipoprotein lipase activity. The interaction of IgG lambda Lac. with serum or HDL-activated triglyceride substrates inhibits the lipolytic activity of human and rat plasma post heparin and also adipose tissue lipases. It slightly inhibits the activity of swine pancreatic lipases. The Lac S. which reacts with IgG Lac. is associated to whole and delipidated VLDL and HDL and not to LDL or purified APo-A. It may be an Apo-C or a non-peptidic co-factor of the lipases which remains bound to the apoprotein core after delipidation. Its lack of species specificity and its presence as traces in HSA preparations favors the latter hypothesis. The Lac. substances is different from the Pg and As substances which were found to react with IgA anti-Pg and IgG anti-As antibodies in previously reported antilipoprotein AIH.


Anti-inflammatory agents in experimental atherosclerosis. Part 2. Failure of antihypertensive drugs to exacerbate atherosclerotic plaque formation. Hypertension is associated with an increased incidence of generalized vascular disease. Antihypertensive drug therapy, while decreasing overall mortality due to cerebral hemorrhage, myocardial hypertrophy or renal failure, paradoxically does not appear to reduce the incidence of coronary atherosclerosis. This study investigates whether the drugs, as a possible side effect, may have an adverse influence on the development of atherosclerotic plaques. Groups of rabbits were fed an atherogenic diet containing 1% cholesterol for 12 weeks. Two commonly used antihypertensive agents (methyldopa and chlorthalidone) were added to the diet of some groups at levels of 100 mg and 10 mg per day respectively. No significant increase in total atherosclerotic plaque area was produced by either of the drugs tested singly or in combination. Plasma renin levels were only mildly elevated and in this experimental system there was no correlation between renin activity and atherosclerotic plaque intensity. There is thus no evidence from this study that antihypertensive drugs have any adverse effects on atherosclerotic plaque formation. While the ineffectiveness of these drugs against coronary atherosclerosis may indicate that normalization of pressure cannot arrest changes already initiated, it also supports the possibility that association of atherosclerosis with hypertension may be symptomatic of a common underlying defect not correlated by normalizing blood pressure.


Variation in proteins of single lesions from the intima of the aorta from a human patient with severe atherosclerosis. A method is presented for grinding and extracting very small samples of tough fibrous tissue from single atherosclerotic lesions of human aortas. Grinding to a powder was easily accomplished while the samples were frozen in liquid nitrogen in a porcelain micromortar. Extractions of the powder were made first in the mortar and then in tapered centrifuge tubes. Salt soluble, dodecyl sulfate--mercaptoethanol--urea soluble and hot alkali soluble fractions were obtained, in addition to a hot alkali insoluble elastin residue from each sample. Variation in the protein composition of 23 samples from the lumenal surface of the severely atherosclerotic aorta of a 58-year-old human male were determined. The proteins soluble in the buffered-saline and the dodecyl sulfate-urea soluble polypeptides from each sample were analyzed by dodecyl sulfate--acrylamide gel electrophoresis. The amino acid compositions of the insoluble elastin fractions were determined. The 5 grossly normal intima samples had very similar gel electrophoresis band patterns and amino acid compositions. The 3 samples of necrotic gruel had markedly different dodecyl sulfate--urea soluble polypeptides than either normal or calcified tissue; they also had elastin fractions whose amino acid compositions were unique in that they contained 10 times more serine than elastin fractions from grossly normal intima. The 3 calcified samples had less saline or dodecyl sulfate soluble protein than either grossly normal or necrotic gruel samples, and had very altered elastin fraction compositions characterized by much higher contents of hydroxylysine than grossly normal intima. The elastin fractions of necrotic gruel and calcified tissue samples had little or no isodesmosine or desmosine, suggesting that little of the elastin found in healthy aorta tissue was present.


The association of elevated plasma cortisol and early atherosclerosis as demonstrated by coronary angiography. To study the association of plasma cortisol and coronary atherosclerosis, we elected 71 male outpatients who had coronary angiography as part of their evaluation at our facility. Forty-eight percent of the angiograms showed no evidence of coronary artery disease (CAD), 20% showed mild CAD, and 32% showed moderate to severe CAD. We found significant correlations between elevated serial morning plasma cortisols and moderate to severe coronary atherosclerosis. Using the odds ratio, we compared plasma cortisol to the major risk factors for coronary artery disease. Plasma cortisol was second only to serum cholesterol as a discriminator in our patient population between diseased and non-diseased patients. We found a significant correlation between plasma cortisol and cholesterol, blood pressure, and smoking- the three cardinal risk factors for CAD. The highest degree of correlation was found between cortisol and cholesterol. The possible significance of the association of cortisol and the major risk factors for CAD is discussed.