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Biomedical subjects

J Bieri

Publications and source records attributed to J Bieri.

8 recordsLinked to original sources

[Near-drowning in children].

From 1971 to 1977 a total of 32 nearly-drowned children were admitted to the University Pediatric Hospital, Berne, and the Children's Hospital, Lucerne. 5 died within 15 h to 10 days. Neurological damage was seen in 5 cases. 20 children were under 5 years of age and only 8 over 7 years. All cases with fatal consequences or severe cerebral sequel had cardiac arrest on admission. A maximal possible submersion time for survival without consequences has not been found. The pathophysiology of fresh water drowning and current treatment are summarized. Situations leading to near drowning are specified and some aspects of prognosis discussed.

Adolescent

Atypical phenylketonuria caused by 7, 8-dihydrobiopterin synthetase deficiency.

A patient with atypical phenylketonuria and normal liver dihydropteridine reductase and phenylalanine-4-hydroxylase activities excreted neopterin but not biopterin or dihydrobiopterin in urine. The oral administration of L-sepiapterin (1 mg/kg body weight) lowered serum-henylalanine from 17.1 to 1.1 mg/dl within 6 h. Comparable responses were observed after oral administration of L-erythro-7, 8-dihydrobiopterin or L-erythro-5, 6, 7, 8-tetrahydrobiopterin (each given in a dose of 2.5 mg/kg body weight). The results indicate a 7, 8-dihydrobiopterin synthetase deficiency in the patient.

Alcohol Oxidoreductases