Nonphenylketonuric hyperphenylalaninemia

Article Abstract:

Phenylketonuria (PKU) is a disorder caused by the lack of the enzyme needed to convert phenylalanine, an amino acid essential for growth and protein metabolism. Children born with this defect are unable to rid the body of phenylalanine. Phenylalanine can build up in the blood, and high levels can damage brain tissue. Patients with PKU are given a diet free of phenylalanine to prevent mental retardation. A milder form of high phenylalanine in the blood is termed non-PKU hyperphenylalanemia. The notion of initiating a diet free of phenylalanemia was arrived at arbitrarily, on the basis of the amount of phenylalanine in the blood. Few studies have shown the effectiveness of such diets in non-PKU hyperphenylalanemia. A follow-up study of 16 patients with non-PKU hyperphenylalanemia was done to assess mental and physical health of patients with and without phenylalanine-free diets. A diet free of phenylalanine did not offer any benefits over the nonrestrictive diets. This may imply that there is a threshold level of phenylalanine in the blood which does not cause mental retardation. Most of the patients have been in good health, and only two children have had emotional difficulties. It is not clear whether the psychotic condition diagnosed in one patient was the result of hyperphenylalanemia. Although 12 of the patients tested above the acceptable levels phenylalanine, the problems typically associated with untreated classic PKU were not present. Dietary restrictions for patients with mild forms of hyperphenylalanemia are not justified, according to these results. Women planning to have children, however, should be monitored carefully as phenylalanine passes through the placenta to the fetus. A diet free of phenylalanine is suggested for pregnant women with non-PKU hyperphenylalanemia. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Physiological aspects, Phenylketonuria, Diet therapy, Phenylalanine, Low-phenylalanine diet, Amino acid metabolism, Inborn errors of, Low phenylalanine diet, Inborn errors of amino acid metabolism

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Arteriovenous malformation

Article Abstract:

An arteriovenous malformation (AVM) may present in children as central nervous system signs and rapid deterioration of neurologic status. An AVM is a congenital abnormal blood vessel formation. A previously health nine-year-old boy was admitted to the emergency department because of inappropriate behavior. He claimed to have ingested a variety of his grandmother's medications; however, no pill fragments were recovered from his stomach. During the stomach-pumping procedure, his heart rate slowed, and he turned blue. He was successfully resuscitated, but his neurologic responses deteriorated and brain death was diagnosed on following an electroencephalogram. A CT scan performed with and without contrast medium showed an AVM.

Case studies, Cerebral arteriovenous malformations

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A New Kind of Prohibition

Article Abstract:

The Supreme Court is expected to rule shortly on whether or not Oklahoma can ban alcohol advertising from electronic media that originates in other states. Oklahoma only wants to enforce its law against broadcasters and cable operators. The implications of Oklahoma's ban on the electronic media are discussed.

Advertising, Oklahoma, Courts of last resort

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