婴儿持续性高胰岛素血症性低血糖症12例临床分析

Persistent hyperinsulinemic hypoglycemia of infancy: clinical analysis of 12 cases

摘要:

目的：探讨婴儿持续性高胰岛素血症性低血糖症（PHHI）的早期诊断和治疗问题。方法：回顾性分析12例PHHI患儿的临床资料。结果：12例患儿临床表现除抽搐外,尚有发绀、嗜睡、拒奶、易激惹、出冷汗等,实验室检查有持续性低血糖和高胰岛素血症,胰高血糖素试验阳性，尿酮阴性。7例给予二氮嗪治疗(每天5~15 mg/kg)，4例治疗有效。1例行胰腺次全切除术，术后血糖正常。6例随访有精神运动发育迟缓等后遗症,3例失访，3例正在随访中。结论：PHHI可通过血糖监测、血胰岛素和尿酮检查做出早期诊断,部分患儿二氮嗪治疗有效。［中国当代儿科杂志，2009，11（10）：809-812］

Abstract:

OBJECTIVE: To study the diagnosis and treatment of persistent hyperinsulinemic hypoglycemia of infancy. METHODS: The clinical data of 12 infants with persistent hyperinsulinemic hypoglycemia were retrospectively reviewed. RESULTS: Convulsion, cyanosis, lethargy, refusing milk sucking, irritability and sweating were common symptoms. The laboratory findings displayed persistent hypoglycemia and hyperinsulinism in all of the 12 infants. The glucagon test showed positive and no urine ketones were detected in all of the 12 infants. Seven infants were treated with oral diazoxide (5-15 mg/kg daily) and 4 infants showed effective to the therapy. One patient was given subtotal pancreatectomy and the blood glucose level was restored to normal after operation. Of the 12 infants, 6 presented psychomotor retardation in a follow-up of 2 months to 67 months, 3 had loss to follow-up and 3 were still in a follow-up. CONCLUSIONS: The measurement of blood glucose, blood insulin and urinary ketons is helpful in the diagnosis of persistent hyperinsulinemic hypoglycemia of infancy. Diazoxide therapy is effective in some of patients.［Chin J Contemp Pediatr, 2009, 11 (10):809-812］