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PKU and Pterin Defects
OverviewPhenylketonuria (PKU) is a recessive disorder caused by deficiency in phenylalanine hydroxylase (PAH), the enzyme that converts the amino acid phenylalanine to tyrosine, leading to accumulation of phenylalanine in the body. This accumulation is toxic to the development and functioning of the central nervous system and leads to intellectual disability. Phenylalanine is present in almost all foods containing proteins, and treatment of PKU involves a restrictive diet with reduction of phenylalanine and addition tyrosine, which becomes essential in this condition. This requires special foods, including medical formulas that contain all amino acids except phenylalanine and special low-protein foods.
Most children with PKU are identified by newborn screening. With treatment and early introduction and maintenance of the special diet, normal IQ and development can be expected. Without treatment, symptoms in classic PKU begin by about 6 months of age.
Initial symptoms may include:
- A musty or "mousy" odor of the body and urine
- Developmental delays in sitting, crawling, and standing
- Acquired microcephaly
- Decreased skin and hair pigmentation (due to lack of tyrosine)
- Eczema
- Seizures
- Profound intellectual disability
Some patients, particularly those with mild PKU, respond to therapy with sapropterin, a synthetic form of tetrahydrobiopterin, the cofactor of phenylalanine hydroxylase. Patients with mild phenylketonuria are more likely to respond to this therapy.
Elevated phenylalanine levels can also be caused by defects in the synthesis or recycling of tetrahydrobiopterin, an essential cofactor of phenylalanine hydroxylase and other hydroxylases involved in neurotransmitter synthesis, or deficiency of DNAJC12, a co-chaperone of phenylalanine and other hydroxylases. In addition to elevated phenylalanine levels, these patients can have neurotransmitter deficiencies, and their treatment differs from that of phenylketonuria.
As part of the initial evaluation of a child with high phenylalanine level in newborn screening, metabolic geneticists will exclude a defect in the tetrahydrobiopterin pathway by measurement of the pterin profile in urine and evaluation of the activity of an enzyme, dihydropteridin reductase (DHPR), in red blood cells spotted on filter paper. Therapy in these conditions includes the administration of a synthetic form of tetrahydrobiopterin that can normalize plasma phenylalanine level. These patients also require the administration of neurotransmitter precursors (Levodopa and 5-hydroxytryptophan). Abnormalities in DNAJC12 have also been identified in patients with autism without or with only mild hyperphenylalaninemia.
Dietary protein restriction needs to be continued for life in patients with PKU, and repeated monitoring of plasma phenylalanine and tyrosine levels are needed to make sure that they remain within the therapeutic range (phenylalanine 45-360 micromolar, Tyrosine 30-120 micromolar).
Other Names & Coding
Phenylalanine hydroxylase deficiency
E70.0, Classical phenylketonuria
E70.1, Other hyperphenylalaninemias
ICD-10 for Classical Phenylketonuria (icd10data.com) and ICD-10 for Other Hyperphenylalaninemias (icd10data.com) provide further coding details.
PrevalencePKU caused by PAH deficiency occurs in about 1:16,500 live births in the US. [Therrell: 2014] The incidence varies greatly in other populations: Turks - 1:2,600; Irish - 1:4,500; African - 1:100,000; Japanese - 1:143,000; Finnish and Ashkenazi Jewish - 1:200,000. [Günther: 2008]
GeneticsAll hyperphenylalaninemias are Inherited as autosomal recessive traits. More than 600 different pathogenic variants in the phenylalanine hydroxylase (PAH) gene located on chromosome 12 have been identified in patients with phenylketonuria. There is a correlation between the type of mutations, residual enzyme activity, and highest phenylalanine level. DNA testing can fully confirm a diagnosis of phenylketonuria and, with biochemical testing, help to exclude defects in biopterin synthesis or DNAJC12. Confirmation of the diagnosis of defects in biopterin metabolism (GTPCH, PTPS, DHPR, Carbinolamine dehydratase) or DNAJC12 requires DNA testing. For this reason, gene panels have been developed to identify pathogenic variants in all the genes listed above, and results are useful in designing an individualized care plan and formulating a prognosis.
PrognosisPrognosis is good for individuals with PKU who remain on the diet. Relaxation of diet is associated with executive function deficits and an increased risk of attention deficit disorder and problems in school. Patients with defects in tetrahydrobiopterin synthesis or recycling or DNAJC12 deficiency might develop movement disorders and developmental delays. These can be improved by therapy, but in the most severe forms therapy is not completely effective.
Practice Guidelines
Camp KM, Parisi MA, Acosta PB, Berry GT, Bilder DA, Blau N, Bodamer OA, Brosco JP, et al.
Phenylketonuria Scientific Review Conference: state of the science and future research needs.
Mol Genet Metab. 2014;112(2):87-122. PubMed abstract
Vockley J, Andersson HC, Antshel KM, Braverman NE, Burton BK, Frazier DM, Mitchell J, Smith WE, Thompson BH, Berry SA.
Phenylalanine hydroxylase deficiency: diagnosis and management guideline.
Genet Med. 2014;16(2):188-200. PubMed abstract / Full Text
Singh RH, Rohr F, Frazier D, Cunningham A, Mofidi S, Ogata B, Splett PL, Moseley K, Huntington K, Acosta PB, Vockley J, Van
Calcar SC.
Recommendations for the nutrition management of phenylalanine hydroxylase deficiency.
Genet Med. 2014;16(2):121-31. PubMed abstract / Full Text
Roles of the Medical HomeChildren with phenylketonuria (PKU) will have periodic visits with the metabolic genetics team in co-management with the Medical Home clinician. Phenylalanine levels will be monitored periodically. A metabolic nutritionist will work with the family to devise an optimal approach to dietary management. The Medical Home clinician should support maintenance of the phenylalanine-restricted diet and supplementation of tyrosine and essential amino acids as recommended by the metabolic geneticist. For those identified after irreversible consequences, the Medical Home clinician should assist in management, particularly with developmental and educational interventions.
Clinical Assessment
OverviewMost children with phenylketonuria (PKU) are now identified by newborn screening or in families at known risk. A positive newborn screen for PKU reflects elevated phenylalanine. False positives occur; all initially positive newborn screens need to be confirmed through additional testing. The Medical Home clinician and the metabolic genetics team should work together to contact the family and arrange further testing. The infant will be treated as needed for symptoms and started as soon as possible on the special PKU diet. See the Portal's Phenylketonuria (PKU) for additional information.
Pearls & Alerts for AssessmentThe diagnosis of PKU must be confirmed by quantitative plasma amino acids.
Common causes of false positive newborn screens for PKU include the administration of total parenteral nutrition, liver disease, and extreme prematurity. In these cases, elevation of several amino acids will be found, not just phenylalanine. Rapid verification, in collaboration with a metabolic specialist, is necessary to enable dietary intervention to begin before two weeks of age.Attention problems and mood disorders
Patients with phenylketonuria have an increased incidence of attention problems and mood disorders. In general, maintenance of phenylalanine levels below 360 micromolar for life is associated with better outcomes. Unfortunately, as children become more social and are exposed to new foods, levels of phenylalanine tend to increase. Working with caregivers and school systems to provide acceptable dietary choices and ongoing education on protein and phenylalanine content of foods is very important. Perpetual surveillance for attention problems may help detect poor dietary compliance. Children with PKU who also have attention deficit disorder should respond to commonly used stimulants and other medications, assuming that phenylalanine levels are well managed.
For the ConditionAll infants born in the United States and many other countries are now screened as newborns for phenylketonuria (PKU). Completion of screening should be confirmed for every newborn. Family members do not need screening for the condition but may discuss screening for carrier status with the metabolic genetics team. Routine screening for attention problems or academic performance is not recommended, but these should be monitored as reflections of inadequate maintenance of the therapeutic diet.
Of Family MembersFamily members of individuals with PKU should have the normal newborn screening and have quantitative plasma amino acids measured at 24 h of life to speed up potential treatment. Thy should receive a normal diet until diagnosis is confirmed.
For ComplicationsDevelopmental surveillance and periodic screening, particularly related to speech and language development are important to detect any early signs of delay.
PresentationsChildren born in the USA, Europe, Japan or Australia are rarely missed by neonatal screening. By contrast, immigrants from other countries might not be diagnosed at birth. They will have early delayed development and will usually present with nonspecific intellectual disability and, in many cases, seizures. They can have the odor of phenylacetic acid, but it is not always easy to appreciate. If left untreated, many will develop physical disabilities as well. Older patients with PKU who do not adhere to treatment are at higher risk for psychiatric symptoms.
Diagnostic CriteriaMeasurement of elevated phenylalanine levels in plasma (plasma amino acids) confirms the diagnosis. Measurement of urine pterins and red blood cell DHPR activity excludes disorders of biopterin synthesis and recycling. DNA testing can be used to confirm biochemical data and to exclude DNAJC12 deficiency that cannot be differentiated by biochemical testing.
Clinical ClassificationDifferent degrees of severity of PKU caused by phenylalanine hydroxylase deficiency are defined by the highest levels of phenylalanine measured in blood at equilibrium and tolerance to dietary phenylalanine. Some patients, particularly those with mild PKU, respond to therapy with sapropterin, a synthetic form of tetrahydrobiopterin, the cofactor of phenylalanine hydroxylase. [Blau: 2011]
- In benign hyperphenylalaninemia, the increase in phenylalanine levels are minimal (phenylalanine 120-360 micromolar, normal 30-90 micromolar) and require no treatment.
- In mild PKU, there is a mild increase in phenylalanine levels (360-1,200 micromolar, normal 30-90 micromolar) that is more easily controlled by diet or pharmacological therapy.
- In classic PKU, there is substantial elevation of phenylalanine levels (>1,200 micromolar, normal 30-90 micromolar) that requires strict dietary therapy and is less likely to respond to pharmacological therapy.
Differential DiagnosisMetabolic geneticists will differentiate hyperphenylalaninemia due to PAH deficiency from a defect in tetrahydrobiopterin synthesis or recycling or DNAJC12 deficiency.
Comorbid & Secondary ConditionsChildren with treated PKU, particularly those who have not maintained low serum phenylalanine levels, may have symptoms of Attention-Deficit/Hyperactivity Disorder (ADHD). Defects in executive function are frequent in adolescents with marginal levels of phenylalanine as are psychiatric and mood disorders in adults.
History & Examination
Current & Past Medical HistoryFamilies should be questioned about how well they have managed to stay on the diet and continue the prescribed supplements. Barriers to diet compliance should be explored and overcome if possible. Some children with a very high level of phenylalanine may have an increased incidence of Gastroesophageal Reflux Disease.
Family HistoryPKU is a recessive disorder, and usually there is no history of this condition in the family. In rare instances, there may be relatives with PKU or unexplained intellectual disability in previous generations before newborn screening for PKU was initiated in the 1960s or if the family is from a country that does not screen for PKU in newborns or in which consanguinity is frequent. PKU is screened for in the United States, Europe, and most other nations. For an interesting discussion of the history of PKU screening, see History of Newborn PKU Screening (National Human Genome Research Institute).
Developmental & Educational ProgressChildren who are not maintained on the strict diet may show symptoms of attention deficit hyperactivity disorder, mood disorders, and if non-compliance is severe, a drop in their intellectual functioning. The Medical Home clinician should follow developmental and academic progress closely.
Social & Family FunctioningLong-term adherence to the diet into adulthood is difficult for families to achieve for many reasons.
Physical Exam
Laboratory TestingQuantitative plasma amino acid analysis, red blood cell DHPR assay, and a urine pterin profile are performed to confirm the diagnosis of an infant with a high phenylalanine on newborn screening. Plasma amino acid testing will show a high phenylalanine without an increase in tyrosine. The red blood cell DHPR assay and urine pterin profile will identify defects in the synthesis or recycling of tetrahydropterin, which is the cofactor for phenylalanine hydroxylase. Blood levels of phenylalanine should be followed periodically. This is generally done by or in collaboration with the metabolic genetics team.
Genetic TestingQuantitative plasma amino acid analysis, red blood cell DHPR assay, and a urine pterin profile are performed to confirm the diagnosis of an infant with a high phenylalanine on newborn screening. Plasma amino acid testing will show a high phenylalanine without an increase in tyrosine. The red blood cell DHPR assay and urine pterin profile will identify defects in the synthesis or recycling of tetrahydropterin, which is the cofactor for phenylalanine hydroxylase. PAH mutation analysis will also be ordered by the metabolic genetics team if the information is needed to define the possible response to pharmacological intervention of estimated needs for dietary phenylalanine restriction or genetic counseling. This can also exclude DNAJC12 deficiency.
Specialty Collaborations & Other Services
Biochemical Genetics (Metabolics) (see ID providers [2])
Will be involved in the diagnosis and co-management of children with PKU and variants
Newborn Screening Services (see ID providers [22])
Will assist the Medical Home clinician with the response to a high phenylalanine on a newborn screen
Nutrition, Metabolic (see ID providers [13])
Is part of the metabolic genetics team and will assist the family with initiating feeding with low phenylalanine infant formula, which is started immediately upon confirmation of the diagnosis. The nutrition team will assist families in transitioning from a low phenylalanine formula to a low-phenylalanine diet when appropriate.
Treatment & Management
OverviewPhenylalanine levels should be maintained between 40 and 360 micromolar until the teenage years. There is no consensus on the value to be targeted in adults, with the levels above being recommended by some clinics and levels up to 900 micromolar deemed adequate by others.
Pearls & Alerts for Treatment & ManagementDietary treatment
Initiate dietary treatment before 2 weeks of age and continued for life. Though maintaining the phenylalanine-restricted diet is difficult for many to achieve it is essential to avoid the sequelae and complications of high blood phenylalanine. [MacDonald: 2010]Avoid sugar substitutes
Aspartame (e.g., NutraSweet, Equal, Sweet Mate, Canderel) is commonly found in diet drinks and medications and a rich source of phenylalanine; it must be avoided.Pregnant women with PKU
Women with PKU who become pregnant should maintain levels of phenylalanine below 300 micromolar throughout pregnancy since phenylalanine is a powerful teratogenic agent. High levels of phenylalanine have been associated with increased risk of miscarriage, congenital heart disease, cleft lip and palate, microcephaly, and profound intellectual disability. Dietary control is recommended from least 2 months before becoming pregnant.
How should common problems be managed differently in children with PKU and Pterin Defects?
Development (Cognitive, Motor, Language, Social-Emotional)Follow speech and development for signs of deficits in executive function.
As the infant grows, the metabolic genetics team will help the child and family transition from low phenylalanine formula to a low phenylalanine diet. Additional supplements are added as needed to ensure that essential nutrients are included. Sapropterin, a stable form of tetrahydropterin, may be helpful for some children. [Lee: 2008] [Vernon: 2010] [Trefz: 2010]
Specialty Collaborations & Other Services
Biochemical Genetics (Metabolics) (see ID providers [2])
Will co-manage children with PKU and periodic visits should be scheduled
Nutrition, Metabolic (see ID providers [13])
Is part of the metabolic genetics team and will provide education and support for families with children on the PKU diet
The child is monitored for normal growth and maintenance of adequate phenylalanine levels. The family is instructed on how to spot blood on filter paper, which is then dried and mailed to the health department for phenylalanine and tyrosine determination. This is obtained initially from a heel stick and then a fingerstick as the child gets older. Monitoring is performed weekly for the first year of life. Subsequently, the frequency decreases to every other week and then to monthly when the child enters school age and is in excellent metabolic control. The frequency is adjusted by the metabolic clinic in response to the phenylalanine levels and the severity of the patient's PKU, with more frequent monitoring in those with poor metabolic control and persistently elevated phenylalanine levels.
The child should be given progressive control over the diet to assure that, by the teenage years, there is a good understanding of how to maintain phenylalanine levels within the therapeutic range. Unfortunately, the diet is difficult to maintain and levels of phenylalanine increase as children get older. Sapropterin, a synthetic form of tetrahydrobiopterin, can reduce phenylalanine levels in a select group of patients with PKU. It is given as a trial for about one month, measuring phenylalanine levels before and during the trial. Individuals are considered responsive if phenylalanine levels drop 30% or more. The drug seems very safe.
Specialty Collaborations & Other Services
Nutrition, Metabolic (see ID providers [13])
Is part of the metabolic genetics team and will provide ongoing education and support for families with children on the PKU diet.
Pharmacy & Medications
PEGylated phenylalanine ammonia lyase (pegvalyase, Palinziq) is a new injectable medication used in PKU. This bacterial enzyme transforms circulating phenylalanine in ammonia and cinnamic acid. The latter is then excreted in urine as hyppuric acid after conjugation with glycine. Treatment with pegvaliase does not require a specific diet, but it can cause immune reactions related to the bacterial origin of the injectable drug. [Longo: 2014] [Zori: 2018] [Longo: 2018]
Issues Related to PKU and Pterin Defects
Ask the Specialist
Can mothers breastfeed a baby who has just been diagnosed with phenylketonuria (PKU)?
It is often possible to breastfeed an infant with PKU, but this should be discussed in detail with the metabolic genetics team. Adjustments in the baby's diet may need to be made.
How expensive are medical foods and other special supplements children with PKU will need?
Medical foods are relatively expensive. Insurance will usually provide formula and supplements for children with PKU if they are prescribed by your doctor. However, preauthorization might be necessary. In addition, some insurance plans stop covering them as children become adults.
Resources for Clinicians
On the Web
PKU - Information for Professionals (STAR-G)
Structured list of information about the condition with links to more information; Screening, Technology, and Research in Genetics.
Phenylalanine Hydroxylase Deficiency (GeneReviews)
Detailed information addressing clinical characteristics, diagnosis/testing, management, genetic counseling, and molecular pathogenesis; from the University of Washington and the National Library of Medicine.
Helpful Articles
PubMed search for phenylketonurias in children and adolescents, last 2 years
Blau N, van Spronsen FJ, Levy HL.
Lancet. 2010;376(9750):1417-27. PubMed abstract / Full Text
Viau KS, Wengreen HJ, Ernst SL, Cantor NL, Furtado LV, Longo N.
Correlation of age-specific phenylalanine levels with intellectual outcome in patients with phenylketonuria.
J Inherit Metab Dis. 2011. PubMed abstract
van Spronsen FJ, Himmelreich N, Rüfenacht V, Shen N, Vliet DV, Al-Owain M, Ramzan K, Alkhalifi SM, Lunsing RJ, Heiner-Fokkema
RM, Rassi A, Gemperle-Britschgi C, Hoffmann GF, Blau N, Thöny B.
Heterogeneous clinical spectrum of DNAJC12-deficient hyperphenylalaninemia: from attention deficit to severe dystonia and intellectual disability.
J Med Genet. 2017. PubMed abstract
Clinical Tools
Care Processes & Protocols
Confirmatory Algorithms for Phenylalanine Elevated (ACMG) ( 174 KB)
An algorithm of the basic steps involved in determining the final diagnosis of an infant with a positive newborn screen; American College of Medical Genetics.
ACT Sheet for Increased Phenylalanine (ACMG) ( 351 KB)
Contains short-term recommendations for clinical follow-up of the newborn who has screened positive; American College of Medical Genetics.
Resources for Patients & Families
Information on the Web
PKU - Information for Parents (STAR-G)
A fact sheet, written by a genetic counselor and reviewed by metabolic and genetic specialists, for families who have received an initial diagnosis of a newborn disorder; Screening, Technology and Research in Genetics.
Phenylketonuria (MedlinePlus)
Information for families that includes description, frequency, causes, inheritance, other names, and additional resources; from the National Library of Medicine.
Resources for PKU (Disease InfoSearch)
Compilation of information, articles, and links to support; from Genetic Alliance.
National Urea Cycle Disorders Foundation
Support and information that includes medical lectures on urea cycle disorders, nutrition and medication resources, and information about events and conferences.
PKU in Children and Adolescents (clinicaltrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.
Services for Patients & Families in Idaho (ID)
|Service Categories
|# of providers* in:
|Other states (5) (show)
|Biochemical Genetics (Metabolics)
|Medical Genetics
|Newborn Screening Services
|Nutrition, Metabolic
For services not listed above, browse our Services categories or search our database.
* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.
Blau N, Hennermann JB, Langenbeck U, Lichter-Konecki U.
Diagnosis, classification, and genetics of phenylketonuria and tetrahydrobiopterin (BH4) deficiencies.
Mol Genet Metab. 2011;104 Suppl:S2-9. PubMed abstract
Blau N, van Spronsen FJ, Levy HL.
Lancet. 2010;376(9750):1417-27. PubMed abstract / Full Text
Camp KM, Parisi MA, Acosta PB, Berry GT, Bilder DA, Blau N, Bodamer OA, Brosco JP, et al.
Phenylketonuria Scientific Review Conference: state of the science and future research needs.
Mol Genet Metab. 2014;112(2):87-122. PubMed abstract
Though its title suggests a focus on research, this also represents a consensus on best approaches to care.
Günther T, Schreiber C, Noebauer C, Eicken A, Lange R.
Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: a 30-year review.
Pediatr Cardiol. 2008;29(6):1071-6. PubMed abstract
Lee P, Treacy EP, Crombez E, Wasserstein M, Waber L, Wolff J, Wendel U, Dorenbaum A, Bebchuk J, Christ-Schmidt H, Seashore
M, Giovannini M, Burton BK, Morris AA.
Safety and efficacy of 22 weeks of treatment with sapropterin dihydrochloride in patients with phenylketonuria.
Am J Med Genet A. 2008;146A(22):2851-9. PubMed abstract
Longo N, Harding CO, Burton BK, Grange DK, Vockley J, Wasserstein M, Rice GM, Dorenbaum A, Neuenburg JK, Musson DG, Gu Z,
Sile S.
Single-dose, subcutaneous recombinant phenylalanine ammonia lyase conjugated with polyethylene glycol in adult patients with phenylketonuria: an open-label, multicentre, phase 1 dose-escalation trial.
Lancet. 2014;384(9937):37-44. PubMed abstract / Full Text
Longo N, Zori R, Wasserstein MP, Vockley J, Burton BK, Decker C, Li M, Lau K, Jiang J, Larimore K, Thomas JA.
Long-term safety and efficacy of pegvaliase for the treatment of phenylketonuria in adults: combined phase 2 outcomes through PAL-003 extension study.
Orphanet J Rare Dis. 2018;13(1):108. PubMed abstract / Full Text
MacDonald A, Gokmen-Ozel H, van Rijn M, Burgard P.
The reality of dietary compliance in the management of phenylketonuria.
J Inherit Metab Dis. 2010;33(6):665-70. PubMed abstract
Singh RH, Rohr F, Frazier D, Cunningham A, Mofidi S, Ogata B, Splett PL, Moseley K, Huntington K, Acosta PB, Vockley J, Van
Calcar SC.
Recommendations for the nutrition management of phenylalanine hydroxylase deficiency.
Genet Med. 2014;16(2):121-31. PubMed abstract / Full Text
Therrell BL Jr, Lloyd-Puryear MA, Camp KM, Mann MY.
Inborn errors of metabolism identified via newborn screening: Ten-year incidence data and costs of nutritional interventions for research agenda planning.
Mol Genet Metab. 2014;113(1-2):14-26. PubMed abstract / Full Text
Trefz FK, Belanger-Quintana A.
Sapropterin dihydrochloride: a new drug and a new concept in the management of phenylketonuria.
Drugs Today (Barc). 2010;46(8):589-600. PubMed abstract
Vernon HJ, Koerner CB, Johnson MR, Bergner A, Hamosh A.
Introduction of sapropterin dihydrochloride as standard of care in patients with phenylketonuria.
Mol Genet Metab. 2010;100(3):229-33. PubMed abstract
Viau KS, Wengreen HJ, Ernst SL, Cantor NL, Furtado LV, Longo N.
Correlation of age-specific phenylalanine levels with intellectual outcome in patients with phenylketonuria.
J Inherit Metab Dis. 2011. PubMed abstract
Vockley J, Andersson HC, Antshel KM, Braverman NE, Burton BK, Frazier DM, Mitchell J, Smith WE, Thompson BH, Berry SA.
Phenylalanine hydroxylase deficiency: diagnosis and management guideline.
Genet Med. 2014;16(2):188-200. PubMed abstract / Full Text
Zori R, Thomas JA, Shur N, Rizzo WB, Decker C, Rosen O, Li M, Schweighardt B, Larimore K, Longo N.
Induction, titration, and maintenance dosing regimen in a phase 2 study of pegvaliase for control of blood phenylalanine in adults with phenylketonuria.
Mol Genet Metab. 2018. PubMed abstract
van Spronsen FJ, Burgard P.
The truth of treating patients with phenylketonuria after childhood: the need for a new guideline.
J Inherit Metab Dis. 2008;31(6):673-9. PubMed abstract
van Spronsen FJ, Himmelreich N, Rüfenacht V, Shen N, Vliet DV, Al-Owain M, Ramzan K, Alkhalifi SM, Lunsing RJ, Heiner-Fokkema
RM, Rassi A, Gemperle-Britschgi C, Hoffmann GF, Blau N, Thöny B.
Heterogeneous clinical spectrum of DNAJC12-deficient hyperphenylalaninemia: from attention deficit to severe dystonia and intellectual disability.
J Med Genet. 2017. PubMed abstract


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Remittance Trends in Central America
Recent years have seen an impressive surge in official remittance flows to Central America. The Inter-American Development Bank (IADB) estimates the region received US$7.8 billion through official channels in 2004, a 17 percent increase from the 2003 figure of US$6.7 billion.
Although a huge portion of remittance flows to Latin America and the Caribbean (LAC) still goes to countries outside of Central America — almost 60 percent reportedly just to Mexico, Brazil, and Colombia — Central America's slice of the remittance pie has nonetheless been growing. It now accounts for 17 percent of the official flow to LAC, up from just 15 percent in 2001 according to IADB.
Reporting almost US$2.7 billion in official flows in 2004, Guatemala topped the list, followed closely by El Salvador, with US$2.5 billion (see Table 1). These two countries, which account for nearly two-thirds of the two million Central Americans counted in the 2000 US census, receive almost 64 percent of total remittance flows to Central America, and they are the fourth- and fifth-largest remittance-receiving countries in LAC. Remittance growth in Guatemala tripled from 2001 to 2004.
Honduras and Nicaragua followed at some distance, at around the US$1 billion mark, while Panama, Costa Rica, and Belize trailed with less than US$325 million in remittances in 2004. The low levels of the latter three reflect the fact that they have relatively few emigrants in the United States.
Not surprisingly, the dominant source of these remittance flows is the United States. A good proportion, however, is also believed to be sent from other LAC countries. Despite the attention given to remittances from developed countries, particularly the United States in the case of Latin America, these so-called South-South remittance flows are far from negligible.
A 2003 study of Costa Rica and Nicaragua suggests that about one-third of remittances received in Nicaragua are actually sent from Costa Rica. Given that Mexico is the second-largest destination of Guatemalan workers after the United States, it can also be assumed that at least some of the remittances going to Guatemala are coming from Mexico. Indeed, research conducted for IADB estimated that, in 2002, about US$1.5 billion of the US$32 billion remitted to LAC were actually interregional.
Table 1. Remittances to Central American Countries in Millions of U.S. Dollars, 2001 to 2004
In interpreting these numbers, it is critical to realize they are official estimates and, thus, do not take into account remittances flowing through informal channels, such as those hand carried by migrants on visits home. Given the predominance of informal transfer mechanisms in Central America, it is safe to assume these figures are, more probably than not, underestimates.
Indeed, evidence from household surveys suggests widespread use of informal remittance channels to Central America, as in many other regions. The World Bank estimates, for example, that informal remittances account for about five percent of the actual remittance flow to Guatemala and almost 15 percent to El Salvador.
Remittances and the Macroeconomy
The importance of these huge remittance flows to the economies of Central America becomes very visible once they are compared to key economic aggregates such as gross domestic product (GDP), foreign direct investment (FDI), official development assistance (ODA), and exports.
In all but three Central American countries, remittances are equivalent to at least 10 percent of GDP, suggesting a heavy dependence on remittances as an engine of economic activity. Figures from Nicaragua, El Salvador, and Honduras are particularly high (see Table 2).
Table 2. Remittances to Central America as a Percentage of GDP, FDI, ODA, and Tourism Receipts (2004)
Except in Costa Rica and Panama, remittances also far outweigh both private capital flows and official development assistance. Particularly remarkable in this regard is Guatemala, where remittances are 21 times greater than FDI and 30 times greater than ODA.
Figures from the rest of Central America are no less striking. As share of FDI and ODA, remittances also scored very high in El Salvador, Honduras, and Nicaragua. Interestingly, Belize's remittance flows, although low in absolute value, are two and a half times greater than FDI and 15 times greater than ODA. Indeed, in Guatemala, El Salvador, Honduras, and Belize, remittances are far larger than ODA and FDI combined.
Remittances also made up for the shortfall in exports of some traditional products, dwarfing coffee exports in Guatemala, El Salvador, and Nicaragua, and banana exports in Honduras and Panama. Remittances, again with the exception of Costa Rica, Panama, and Belize, are also at least three times greater than tourism receipts.
For political scientist Manuel Orozco, a long-time analyst of remittances, this dominance of remittance flows over traditional export receipts signals that Central American countries are in the midst of a transition away from "agro-exporting economies" and toward "transnationally integrated households" that are mainly exporting labor to the United States.
Remittances and Development
Despite remittances increasing in magnitude and importance for the majority of Central American countries today, it is interesting to note that their impact on development is far from clear. For decades now, the perception has lingered that remittances are used mostly for consumption by individual households and rarely, if at all, invested in productive enterprises. Thus the developmental potential of remittances is generally thought to be low. In fact, it has been argued that remittances lead not to long-term economic growth but to a passive and dangerous dependency.
Recent household surveys do show that Central Americans who receive remittances mainly use them to cover basic necessities. According to the World Bank, about 77 percent of remittances, on average, are believed to be spent on immediate needs, such as food. At 84 percent of total expenditures, consumption spending is particularly high in El Salvador while Honduras (77 percent) and Guatemala (68 percent) are not far behind.
More recently, however, an increasing number of studies suggest a more positive developmental role for remittances. Although a big chunk of the literature on this topic is concentrated on other countries and regions, a relatively small number of studies focus on some Central American countries.
For example, economist Richard Adams found that, contrary to common perception, Guatemalan households receiving remittances actually spend slightly less, at the margin, on consumption — food and consumer goods and durables — than do households receiving no remittances. For Adams, this rather surprising result may be explained by Guatemalan households' tendency to view their remittances as a temporary stream of income thus precluding more spending on consumption.
Adams further found that although remittances only had a limited role in reducing the number of poor people in Guatemala, they do reduce the depth of poverty and are therefore particularly beneficial for the poorest of the poor. According to his calculations, remittances reduced extreme poverty by almost 22 percent.
A study on child schooling in El Salvador also found that remittances have a large and significant effect on school retention. From a development standpoint, this finding is clearly promising. Since the increased investments in education contribute to human capital formation, it is likely that remittances may benefit developing countries' long-term growth prospects.
Remittances have also been found to rise when the recipient economy suffers a downturn in activity or macroeconomic shocks due to financial crisis, natural disaster, or political conflict. By compensating for foreign-exchange losses due to these events, World Bank economist Dilip Ratha has found that remittances may smooth consumption and thus contribute to the stability of recipient economies. Indeed, emigration from Central America and the remittances that soon followed were mainly a response to the political turmoil of the 1980s and early 1990s as well as to natural disasters such as Hurricane Mitch in Honduras in 1998.
Very recently, the Economic Commission for Latin America and the Caribbean (ECLAC) released the findings of a comprehensive, region-wide study of the impact of remittances on poverty and inequality. This study included the top four remittance-receivers in Central America — Guatemala, El Salvador, Honduras, and Nicaragua. In this study, however, the results were mixed and prone to different interpretations.
On one hand, the ECLAC study reported that remittances' impact on poverty, indigence rates, and income distribution is very limited. The study revealed that remittances reduced poverty rates in Central America by only 2.2 percentage points. It is important to note that this average is skewed because of El Salvador, which registered a particularly high decline of 4.5 percentage points (see Table 3).
Remittances' impact on indigence is much higher but still limited to a 2.7 percentage-point reduction. This average is skewed, once again, by El Salvador, which registered a higher reduction of 5.4 percentage points.
Remittances' effect on income distribution, though perceptible, is also very limited. El Salvador registered the most significant improvement with an almost five percent reduction in the value of the Gini index, the most commonly used measure of inequality, while Guatemala and Nicaragua were at the far lower end of the scale. Remittances were associated with a small increase in inequality in El Salvador.
On the other hand, however, the study also found that if the analysis focused only on remittance-receiving households, remittances enabled many recipients to escape poverty. The Central American countries in the study recorded, on average, a 20 percent decline in poverty rates among these households. El Salvador registered the highest drop in Central America with a 39 percent poverty-rate reduction and was followed by Honduras, Guatemala, and Nicaragua.
The impact of remittances on indigence in receiving households is even more dramatic. ECLAC found that 64 percent of Salvadoran households receiving remittances were lifted out of extreme poverty. Corresponding figures for Guatemala (43 percent), Honduras (28 percent) and Nicaragua (27 percent), although smaller, are still very significant.
These numbers can mean different things to different people. They can affirm what skeptics have been pointing out for decades — that the benefits of remittances do not accrue beyond the households receiving them. They can even contribute to inequality, as the case of Honduras revealed.
Others, however, might look at the same numbers and emphasize the significant number of remittance-receiving households that managed to improve their living conditions and escape poverty. Further, it can be argued that the dismal impact of remittances on aggregate poverty and indigence figures stems from the fact that the number of households receiving these transfers is still small. In other words, if more households received remittances, then the impact on poverty would be much more pronounced.
The authors of the ECLAC study clearly take the latter view. They emphasize that remittances would lift 2.5 million Latin Americans above the poverty line. They further suggest that if, as they suspect, the data utilized in their study came from a source that underreported remittances received, then several million more persons in Latin America have been lifted out of poverty.
Ultimately, however, the developmental impact of remittances on Central American economies can only be ascertained from empirical studies based on more accurate data.
Migrant, Government, and Private Sector Initiatives
As the discussion on the costs and benefits of remittances occupy the academic literature and professional circles, key stakeholders have already launched initiatives aimed at maximizing the benefits of remittances. This is hardly surprising given that the ongoing debate on the impacts of remittances is occurring at a time when the perceived developmental potential of remittances has never been greater.
One of the most well known of such initiatives started with the migrants themselves. Like their counterparts from the rest of LAC, Central Americans have organized hometown associations, or HTAs.
Members of these associations pool their financial resources and send money or goods back to their hometowns. These so-called collective remittances are used to finance infrastructure and social projects, such as remodeling churches and schools. Guatemalan HTAs, for example, have been involved in purchasing small fire trucks for their hometowns, mobilizing support for their country after the 1996 peace agreements, and even in raising money for crises such as Hurricane Mitch.
Although, at present, only one percent of all remittances in Central America come from HTAs, the International Fund for Agricultural Development recently estimated that their contribution could rise to between three and five percent in 10 years if their management and institutional capacity improves.
Central American governments have also taken initiatives to maximize the benefits from remittances. El Salvador is particularly noteworthy in this regard. Almost six years ago, it created an office that coordinates governmental outreach efforts to Salvadorans living abroad. El Salvador's decision in 2000 to adopt the U.S. dollar as its legal tender was also widely acknowledged to be related to the nation's heavy reliance on foreign exchange coming from remittances.
Taking its cue from Mexico, El Salvador also launched a $300,000 matching fund in 2003 to implement joint partnership activities with HTAs. This grant will act as an incentive for HTAs to start and/or broaden development initiatives for their hometowns. Unlike in Mexico, however, the Salvadoran government is offering a one-to-one match with HTA money. Interestingly, related initiatives in Guatemala reportedly fell short in gaining government support and never took off.
Members of civil society, including international organizations, have also taken an interest in remittances. Through its Multilateral Investment Fund (MIF), IADB has been particularly active in the region. One of its rallying cries — bank the unbanked — is especially important in Central America given that only two out of 10 Central American migrants have bank accounts. This figure is significantly lower than LAC's ratio of six out of 10.
Credit unions in El Salvador, Guatemala, Honduras, and Nicaragua were instrumental in the creation of the International Remittance Network (IRnet) in July 1999. The network, which facilitates remittance flows from the United States to Latin America, reportedly lowered remittance costs not only by generating competition but also through raising customer awareness of remittance fees.
The private sector has also become more involved. Notably, several banks in Central America have been able to raise relatively cheap and long-term financing from international capital markets via securitization of future remittance flows. The volume of remittance securitization has grown rapidly since Mexico made the first transaction in 1994.
These initiatives are just a sample of an increasing array of activities, programs, and policies different actors have taken to maximize the benefits of remittances. Unfortunately, the effectiveness of these initiatives is unknown because evaluations are either incomplete or unavailable for public consumption.
What is clear, however, is that remittances do play an increasingly significant role in Central America, a role that, given current remittance flow projections, will only increase in the years ahead. Whether remittances will be able to lift this region of almost 40 million people out of poverty is a question only time can truly answer. If empirical findings from other regions are indicative of what is happening in Central America, then the skeptics are bound to be disappointed.
Adams, Richard. 2006. "Remittances, Poverty and Investment in Guatemala." International Migration, Remittances, and the Brain Drain. Edited by Çaglar Özden and Maurice Schiff. Washington, DC: World Bank.
Cox-Edwards, Alejandro and Manuelita Ureta. 2003. "International Migration, Remittances, and Schooling: Evidence from El Salvador." Journal of Development Economics 72(2): 429–61.
Economic Commission for Latin America and the Caribbean (ECLAC). 2006. "Social Panorama of Latin America 2005: Preliminary Version." United Nations. Available online.
Inter-American Development Bank. 2005. "Transforming Labor Markets and Promoting Financial Democracy, Statistical Comparison.", November. Available online.
Fagen, Patricia and Micah N. Bump. 2006. " Remittances between Neighboring Countries in Latin America." Beyond Small Change, Making Migrant Remittances Work for Development. Edited by Donald Terry and Steven Wilson.Washington, DC: Inter-American Development Bank.
Orozco, Manuel. 2006. "Migration, Money and Markets: The New Realities for Central America" in Beyond Small Change, Making Migrant Remittances Work for Development, Edited by Donald Terry and Steven Wilson.Inter-American Development Bank Washington DC
Ratha, Dilip. 2006. "Trends, Determinants and Macroeconomic Effects of Remittance." Global Economic Prospects 2006: Economic Implications of Remittances and Migration Washington, DC: The International Bank for Reconstruction and Development / The World Bank.
Sander, Cerstin. 2003. "Migrant Remittances to Developing Countries: A Scoping Study: Overview and Introduction to Issues fro Pro-Poor Financial Services." Paper prepared for the UK Department of International Development (DFID). June.


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Wildwalks Images
The Warrimoo Track is a great walk for exploring the upper reaches of Cowan Creek. This walk wanders down the ridge to follow the bank of Cowan Creek to Bobbin Head. There are many different views to be seen and a variety of flora and fauna throughout makes this walk quite interesting. Enjoy a cool drink from the cafe at the end of the walk.
Total climbing: 280m
6.2 km One way
2 hrs 45 mins
Moderate track
This pleasant walk starts in North Turramurra and follows the Darri and Warrimoo tracks into the Ku-ring-gai Chase National Park. The walk follows the picturesque Cowan Creek through one of the most serene parts of the park, and finishes at Bobbin Head, a great place to enjoy the cafe or have a picnic lunch.
Total climbing: 457m
9.4 km One way
4 hrs 15 mins
Moderate track
This great walk starts at the Sphinx Memorial and loops around via Cowan Creek, exploring diverse environments as it changes altitude. There is an optional side trip to the Empire Marina and cafe for a recharge before the climb back up to the Sphinx. This walk provides some great water views of the Cowan Creek and the interesting rock formations along its shores.
Total climbing: 499m
9.6 km Circuit
4 hrs
Moderate track
This walk starts at the Sphinx War Memorial and follows some well maintained and popular walking tracks down to Cowan Creek. Enjoy the water views as you head through Bobbin Head and Apple Tree Bay. The walk continues through the National Park and up the hill to Mt Kuring-gai. There are plenty of great spots to stop and rest, including a cafe, kiosk and picnic area at Bobbin Head.
Total climbing: 673m
10.7 km One way
4 hrs 45 mins
Hard track
This great circuit walk enjoys a variety of landscapes in Ku-ring-gai Chase National Park. The walk starts at the historic Sphinx war memorial and follows a series of popular walking tracks that loop around Bobbin Head, including historic Aboriginal engravings, temperate rainforest and a lookout. As the track passes through Bobbin Head, it provides a great opportunity to take a break and pick up a snack at the kiosk before continuing on the return leg.
Total climbing: 619m
12.9 km Circuit
5 hrs
Hard track
Starting in North Turramurra, this walk follows the Darri and Warrimoo Tracks through Ku-ring-gai Chase National Park and down along the picturesque Cowan Creek. Bobbin Head makes a great spot to take a break and have some lunch at the cafe, kiosk or picnic area. From Bobbin Head, the track continues through Apple Tree Bay, following Cowan Creek for a little while before climbing up and out of the valley to Mt Kuring-gai station.
Total climbing: 852m
14.6 km One way
6 hrs 30 mins
Hard track


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This West Coast trio, boasting a new CD entitled "Blaze" marched on stage, donning black Stetsons, and, well, black everything else. Their persona and set list would brings to mind a mashup between Thievery Corporation, Johnny Cash and every desert mirage in-between.
Concord Music Hall
Featuring David Satori (Beats Antique), Evan Fraser (Hamsa Lila) and Mark Reveley (Jed and Lucia), Dirtwire emerges from another dimension of music.
Each member is a consummate showman. The band drums up enthusiasm with low-growling vocals, ambitious jaw harp solos and raw blues harp riffs. Exchanging instruments in a flash, they consistently brandished their versatility. Sometimes mimicking the hum of hollowed-out bamboo; other times, celebrating Africa, with the goni harp or the delicate Kalimba.
They sure weren't shy about merging politics into their rootsy/electronic fare:
"Chicago, are you ready for a revolution, my friends? Take it to the streets."
And against a litany of cantankerous, counter rhythms, Dirtwire rapped:
"Straight to Wall Street, Straight to the Pentagon. What you gonna do? Where you gonna take it?"
There were lots of devoted fans present, who rapped or chanted right along.
Dirtwire took ˜When The Levee Breaks" to a new level, which is no small feat. It was every bit as rousing as Led Zeppelin's fine version, but more. Unique with their special flair of intricate beats. Even Jimmy Page, never mastered slide banjo. The blues harp solo was rich and forceful.
"Shish Kabobs" is about the most singable song ever written, but the structure left lots of space for brilliant bass lines, too. And when push came to shove, this trio took to the drum sticks, each one outdoing his musical brother - All to the audience's delight.
"Next one is for all you earth activists, all you farmers." David teased. The dreamy, blues intro. escalated abruptly into a swamp.
"This one's all about sitting as low as you can go." David clarified. The lyrics were fun and extraordinarily playful. As for the instrumental parts, the banjo said the rest.
"Make some noise, you feeling alright?" With their natural stage presence and flair for roots and all-things contemporary, Dirtwire is the definitive crowd pleaser. They performed the last song,"Stranger" to a barrage of applause.


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New Oxford American Dictionary (3 ed.) Quick reference
"More current than its closest rival in size....This is a 'buy'. " — Booklist STARRED REVIEW
As Oxford's flagship American dictionary, the New Oxford American Dictionary sets the standard of excellence for lexicography in this country. With more than 350,000 words, phrases, and senses, and hundreds of explanatory notes, this dictionary provides the most comprehensive and accurate coverage of American English available.
The dictionary draws on the two-billion-word Oxford English Corpus and the unrivaled citation files of the world-renowned Oxford English Dictionary to provide the most accurate and richly descriptive picture of American English ever offered in any dictionary. The Third Edition offers a thoroughly updated text, with revisions throughout and approximately 2,000 new words, phrases, and meanings. Many new words relate to fast-moving areas such as computing, technology, current affairs, and ecology, while others have recently entered the popular lexicon. Usage notes have been updated in light of the most recent Corpus evidence.
One of the hallmarks of the New Oxford American Dictionary is the way it reflects the living language. Unlike in more traditional dictionaries, where meanings are ordered chronologically according to the history of the language, each entry plainly shows the principal meaning or meanings of the word, organized by importance in today's English. Thus readers can be confident that the first definition they see is the one most likely to be used by people today, and is not a sense that has been obsolete for two centuries.
Offering clear, authoritative, and precise information, with the in-depth and up-to-date coverage that users need and expect, the New Oxford American Dictionary is the benchmark by which all other American dictionaries are measured.
Australian Oxford Dictionary (2 ed.) Reference library
An authoritative guide to contemporary Australian English, produced by the Australian National Dictionary Centre at the Australian National University and Oxford University Press.
The Canadian Oxford Dictionary (2 ed.) Reference library
'a unique reference book for all Canadians', Robert MacNeil, Time Magazine
The Canadian Oxford Dictionary was the runaway bestseller of 1998, spending over a year on the Globe and Mail's bestseller list and winning the Canadian Booksellers Association's Libris awards for Non-fiction Book of the Year and Specialty Book of the Year. The dictionary combines coverage of international English with information on English as it is particularly used in Canada. Definitions are presented so that the meaning most familiar to Canadians appears first and foremost. Each of these entries is exceptionally reliable, the result of thorough research into the language and Oxford's unparalleled language resources.
This new edition is completely up to date with 5,000 new words and senses. It features 2,200 uniquely Canadian words and senses, 350 usage notes, 7,000 idiomatic expressions, 5,500 biographical entries, and over 5,600 place names.
Oxford Dictionary of English (3 ed.) Quick reference
The foremost single volume authority on the English language, the Oxford Dictionary of English is at the forefront of language research, focusing on English as it is used today. It is informed by the most up-to-date evidence from the largest language research programme in the world, including the two-billion-word Oxford English Corpus.
This new edition includes thousands of brand-new words and senses, as well as up-to-date encyclopedic information, and extensive appendices covering topics such as countries, heads of state, and chemical elements.
Ideal for anyone who needs a comprehensive and authoritative dictionary of current English; for professionals, students, academics, and for use at work or at home.
The New Zealand Oxford Dictionary Reference library
This dictionary is the first large-scale English dictionary especially prepared for New Zealand users. Compiled at the New Zealand Dictionary Centre in Wellington, it reflects research into New Zealand words and their use, including new words, and new uses of older words that have evolved in New Zealand. The dictionary also reflects research into international English conducted by Oxford dictionary centres worldwide.
The New Zealand Oxford Dictionary contains over 100,000 definitions, including over 12,000 New Zealand entries and a wide range of encyclopedic information.
Oxford Paperback Thesaurus (4 ed.) Quick reference
300,000 synonyms and antonyms with thousands of usage examples from real English to show how synonyms are used in context.


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Jump to navigation Jump to search
Ward Vandewege
I'm a (part-time) CTO for the Free Software Foundation. We have a Campaign for a free BIOS, because a Free BIOS is essential to achieve true computing freedom.
Where possible, we only buy hardware capable of running coreboot. Currently, we are standardizing on AMD-based systems: in terms of servers we have some Tyan systems and more recently we've purchased Silicon Mechanics A236 and A266 servers. We also have a bunch of Gigabyte M57SLI-S4 based machines, a few PC Engines ALIX boards, and a couple Asus M2A-VM boards.
You can reach me at firstname.lastname@example.com.


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Student Sues School District Over Illegal Search of Her Facebook Page
A 12-year-old Minnesota girl has joined forces with the ACLU to sue her school district. Minnewaska School officials penalized the girl, known only as "R.S.," for information she posted off of school property to her private Facebook page. At one point, she was "intimidated" into giving up her password so that administrators could see the rest of her posts.
The lawsuit claims that her First Amendment rights were violated by employees at Minnewaska Area Middle School, in west-central Minnesota, as well as her Fourth Amendment rights regarding unreasonable search and seizure.
Nothing R.S. posted on her Facebook page seems that damaging — her initial punishment was for calling one of the school's adult hall monitors "mean" — but she also used an expletive and discussed sex. (Details not provided.)
While the school district contends that no one stepped out of line, the ACLU believes R.S. had her First Amendment rights violated. In a statement released by Charles Samuelson, executive director of the ACLU in Minnesota, he notes that, "Students do not shed their First Amendment rights at the school house gate." And they're not required to shed their Facebook passwords either.


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Set your calendars now, Kevin Hart is coming to the BJC on Sunday March 29, at 7:30.
Tickets are $47.50 and go on-sale this Friday at 10am at the Bryce Jordan Center, Eisenhower Auditorium, Penn State Downtown Theatre, Altoona Campus Ticket Outlet, online at ticketmaster.com, or by calling 800-745-3000. VIP tickets are also available.
UNIVERSITY PARK STUDENTS – A special UPAC presale will begin this Wednesday, January 28 at Noon at the HUB Lounge, Bryce Jordan Center, Eisenhower Auditorium, Penn State Downtown Theatre, and online athttps://oss.ticketmaster.com/aps/psubjc/EN/account/login. A limited amount of tickets will be priced at $19.50 courtesy of UPAC. Regular student pricing will be $34.50
If there's one thing KEVIN HART can do, it's sell shoes. If there's one thing Kevin Hart can do better than sell shoes, it's explode into one of the foremost comedians and entertainers in the industry today.
Hart has continued to work non-stop and 2015 is no exception. Kevin will be seen in three comedies – Screen Gem's The Wedding Ringer was just released January 16, Get Hard, opposite Will Farrell will be released March 27, and Ride Along 2, the sequel to last year's one-hundred-million-dollar blockbuster hit of the same name will be released later this year. The consummate worker, Hart is a force in television too, Executive Producing the show, Real Husbands of Hollywood which premiered its third season last September. He also recently hosted Saturday Night Live on NBC. With his passion for stand-up and entertainment Kevin also continues to tour his live stand-up to sold-out audiences around the country.
Kevin Hart began his career sizing men and women for footwear, when a chance, electrifying performance at amateur night in a Philadelphia comedy club changed his life. Kevin quit his shoe salesman job and began performing full time at such venues as The Boston Comedy Club, Caroline's, Stand-Up NY, The Laugh Factory, and The Comedy Store in Los Angeles. However, it was his first appearance at the "Montreal Just for Laughs Comedy Festival" that led Kevin into roles in feature films such as Paper Soldiers, Scary Movie 3, and Along Came Polly opposite Ben Stiller and Jennifer Aniston.
In 2012, Hart was tapped to host the 2012 MTV VMA's, garnering much industry praise for his appearance, before his next comedy tour, "Let Me Explain" took him to 90 American cities along with Europe and Africa. Records have not withstood under his LME tour either, as Kevin became only the second American in history to sell out London's O2 Arena. Kevin spent the fall of 2012 filming two movies back to back: Screen Gem's re-make of Sexual Perversity titled About Last Night, and Universal's buddy cop movie Ride Along, opposite Ice Cube. Kevin continued his incredible run with a starring role in Screen Gem's Think Like A Man, a comedy based on Steve Harvey's bestselling book, which grossed $95 Million worldwide, and had a supporting role in the Universal, Nick Stoller comedy, Five Year Engagement, produced by Judd Apatow.
In September, 2011 Hart released "Laugh At My Pain," the feature film version of his comedy tour (under the same name). The movie grossed over $7 million, and was 2011's most successful film of those released in less than 300 theaters. The LAMP tour was so successful, it catapulted Hart to 2011's number one comedian on Ticketmaster, and in February, 2011, he sold out the Nokia Theater for two nights in a row, breaking the record previously set by Eddie Murphy. This lead to the LAMP DVD hitting double platinum in February 2012, after being on sale for only a month.
In 2009, Hart's one-hour Comedy Central special I'm A Grown Little Man became one of the highest rated specials for the network, and in 2010, Hart's DVD Seriously…Funny was one of the fastest selling DVDs, going triple Platinum, aided by and the Comedy Central special of the same name, which was the highest rated comedy special of 2010.