先天性高胰岛素血症5例临床分析

目的　分析 5例先天性高胰岛素血症患儿的临床资料 ,探讨其早期诊断和治疗问题。方法　回顾性分析 5例先天性高胰岛素血症患儿的临床资料。结果　 5例临床表现除抽搐外 ,尚有发绀、呼吸暂停、饥饿感和多汗等 ,实验室检查有持续性低血糖和高胰岛素血症 ,胰腺病理均为胰岛细胞增殖。 5例经胰腺 95 %次全切除术后 ,血胰岛素和血糖逐渐恢复正常 ,4例治愈 ,随访 3例有继发性癫疒间 后遗症 ,1例失访 ;另 1例术后 1个月死于败血症。结论　先天性高胰岛素血症可通过血糖监测、血胰岛素和尿酮检查作出早期诊断 ,胰腺 95 %次全切除术治疗有效。

Early diagnosis and treatment of congenital hyperinsulinism:clinical analysis of 5 cases

Objective To investigate the early diagnosis and treatment of congenital hyperinsulinism.Methods 5 cases of congenital hyperinsulinism were studied by retrospective analysis.Results The main symptoms included convulsion,cyanosis,apnea,feeling hunger and sweating.The laboratory examination included persistent hypoglycemia and hyperinsulinism.The histologic lesions were all nesidioblastosis.5 cases had 95% subtotal pancreatectomy.Blood glucose and blood insulin returned to normal range after operation.During follow-up,4 cases recovered ,but 3 cases had subsequent epilepsy,and one case was failed to contact.Another case died from septicemia one month after operation.Conclusion Blood glucose,blood insulin and urine ketones examinations are helpful in early diagnosis of congenital hyperinsulinism.95% subtotal pancreatectomy is effective.