The diagnosis of hyperinsulinism in a child in the absence of hyperinsulinemia.

スポンサーリンク

概要

• 論文の詳細を見る

• This report concerns a 15-year-old Japanese girl with hyperinsulinism whose fasting serum immunoreactive insulin (IRI) levels were invariably within normal range. She developed frequent seizures in association with brief fasting at 9 months of age. The anticonvulsant therapy was instituted without benefit. At 5 years of age, she was admitted to our hospital for evaluation of hypoglycemia. Despite profound hypoglycemia her basal serum IRI levels remained normal, and various endocrine tests failed to demonstrate hyperinsulinemia unequivocally. Diagnosis of hyperinsulinism was suspected by consistently lowered serum non-esterified fatty acid (NEFA) and lack of ketonuria during hypoglycemia, and was confirmed by a dramatic response to diazoxide therapy. During the past 9 years, she has remained asymptomatic with fasting blood sugar of 74-87mg/dl on diazoxide (2.5mg/kg/day).

• The Keio Journal of Medicineの論文

著者

• 松尾 宣武

  Department of Pediatrics, School of Medicine, Keio University

• 永井 敏郎

  Department of Pediatrics, Tokyo Metropolitan Children's Hospital

• 土屋 裕

  Department of Pediatrics, Tokyo Metropolitan Children's Hospital

• 津崎 さゆ美

  Department of Pediatrics, School of Medicine, Keio University

• 長 秀男

  Department of Pediatrics, Tokyo Metropolitan Children's Hospital

• 佐藤 清二

  Department of Pediatrics, School of Medicine, Keio University

関連論文

• A 27-year-old Woman with Alstrom Syndrome Who Had Liver Cirrhosis
• The diagnosis of hyperinsulinism in a child in the absence of hyperinsulinemia.
• Hyperphenylalaninemia syndromes: Current status of diagnosis and management.

スポンサーリンク