title: [Leucine sensitive hypoglycemia. Follow-up studies under treatment with diazoxide (author's transl)].
abstract: Since 1969 leucine sensitive hypoglycemia has been diagnosed in 4 infants. Elevated serum insulin levels suggested hyperinsulinism; in 1 infant islet cell hyperplasia was demonstrated by morphological examination of pancreatic tissue. All cases were treated with diazoxide and have been followed under therapy for periods ranging from 4 months to 6 1/4 years. The upper limit of dosage was 15 mg/kg. The blood glucose values promptly became normal in 2 infants under this regimen. The development of these patients was normal. In the 2 other infants treatment was effective only after the additional restriction of daily protein intake to 2-2.5 g/kg. Severe cerebral damage occurred in one of these infants due to recurrent episodes of hypoglycemia, causing further development to be considerably restricted; the other one developed satisfactorily but with moderate mental retardation. Both children are suffering from cerebral convulsions. In all patients hypertrichosis was the only side effect.
mesh_terms: D001786:Blood Glucose / Q000032:analysis; D003981:Diazoxide / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D004044:Dietary Proteins; D004342:Drug Hypersensitivity; D004827:Epilepsy / Q000150:complications; D005260:Female; D006801:Humans; D006983:Hypertrichosis / Q000139:chemically induced; D007003:Hypoglycemia / Q000097:blood / Q000139:chemically induced / Q000150:complications / Q000178:diet therapy / Q000188:drug therapy*; D007223:Infant; D007231:Infant, Newborn; D007328:Insulin / Q000097:blood; D008607:Intellectual Disability / Q000150:complications; D007930:Leucine; D008297:Male
chemical_list: D001786:Blood Glucose; D004044:Dietary Proteins; D007328:Insulin; D007930:Leucine; D003981:Diazoxide
vernacular_title: Leucinempfindliche Hypoglykämie. Verlaufsbeobachtungen unter Behandlung mit Diazoxid