Valine, isoleucine, and leucine: a new treatment for phenylketonuria

Article Abstract:

Phenylketonuria (PKU) is a genetic disease which results in progressive mental deterioration of the patient if it is not treated. The condition is a metabolic disorder in which the enzyme that converts phenylalanine to tyrosine (both amino acids) is deficient or absent. The resultant accumulation of phenylalanine in the blood causes behavioral and intellectual defects and may lead to brain damage. PKU treatment involves a special diet which restricts phenylalanine; for this treatment to be effective, it must begin before the age of three months. Previous reports have indicated that administration of the amino acids valine, isoleucine, and leucine reduced phenylalanine levels in children with PKU. A study was conducted which measured blood serum phenylalanine levels before and after treatment with valine, isoleucine, and leucine in 16 subjects with PKU who were between the ages of 10 and 23 years old; measurements were taken over four three-month periods. At the end of each time frame, tests were also conducted to assess the neurological and psychological functioning of the subjects. Measurements taken included attention span, memory, and language functioning. All subjects had been diagnosed with PKU before three months of age and began a modified diet which was continued for three years. The results indicated improved performances during the treatment period on the tests that required substantial attention and mental processing. It was concluded that treatment with valine, isoleucine, and leucine is a beneficial supplement in PKU patients who are unable to maintain low phenylalanine levels through diet alone. No adverse effects of this long-term treatment were evident. However, a high rate of noncompliance was observed, with 25 percent of the subjects dropping out before the study was half over. (Consumer Summary produced by Reliance Medical Information, Inc.)

Phenylketonuria, Leucine, Branched chain amino acids

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The value of early treatment of deer tick bites for the prevention of Lyme disease

Article Abstract:

Early antibiotic treatment of deer tick bites may not be of value in preventing Lyme disease. Lyme disease is an infection with the bacterium Borrelia burgdorferi. The infection is transmitted to humans by deer ticks. From 1988 to 1991, researchers followed 179 children between three and 19 years old who were bitten by a deer tick. They were randomly assigned to be treatment with an antibiotic (89 patients) or a placebo, an inactive substance (90 patients). Their blood was tested for antibodies to Borrelia burgdorferi at the initial visit and six weeks later. Four patients in the placebo group had a weakly positive blood test at six weeks. One of them had clinical Lyme diseases, one had a flu-like illness and two had no symptoms. None of the patients developed late manifestations of Lyme disease, such as nerve disorders, arthritis and heart rhythm disorders.

Antibiotics, Prevention, Lyme disease, Bites and stings, Bites (Injuries), Stings (Injuries)

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Acrodermatitis enteropathica-like syndrome secondary to isoleucine deficiency during treatment of maple syrup urine disease

Article Abstract:

A condition similar to acrodermatitis enteropathica may occur in infants being treated for maple syrup urine disease (MSUD). MSUD is an inherited enzyme deficiency involving a defect in amino acid metabolism. Acrodermatitis enteropathica is a syndrome associated with zinc deficiency and characterized by skin lesions and diarrhea. A 10-day-old infant was diagnosed with MSUD and treated with a branched-chain amino acid-free formula. Within three weeks he developed skin lesions and diarrhea. Blood tests showed normal zinc levels but abnormal amino acid levels. The amino acid-free formula caused an isoleucine deficiency that led to high blood levels of leucine. This amino acid imbalance was corrected with isoleucine supplementation and the skin lesions and diarrhea resolved after seven days.

Diagnosis, Complications and side effects, Physiological aspects, Amino acids, Amino acid synthesis, Maple syrup urine disease, Acrodermatitis enteropathica

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