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[成胰岛细胞增生症。关于12例新病例]

[Nesidioblastosis. Apropos of 12 new cases].

作者信息

Samoud A, Dey D, Brauner R, Doagi M, Kallel H, Boubaker S, Osman R, Ben Dridi M F

机构信息

Service de Pédiatrie, Hôpital la Rabta, TUNIS, Tunisie.

出版信息

Ann Pediatr (Paris). 1990 Dec;37(10):651-5.

PMID:2291591
Abstract

Two new cases of diffuse hyperplasia of the pancreas are reported. This infrequent condition is caused by intermittent and variable insulin hypersecretion. The hyperinsulinism is responsible for severe, lasting and intractable hypoglycemia that causes seizures and mental retardation. Onset usually occurs in the neonatal period. The diagnosis of hyperinsulinism rests on four criteria: the presence of increased insulin levels in the face of hypoglycemia, the low urinary excretion of ketone bodies during hypoglycemic episodes, the need for more than 15/mg/kg/min glucose to maintain the serum glucose level above 2 mmol/l, and a positive response to glucagon. The topographic diagnosis is often disappointing. Medical treatment of the hypoglycemia with diazoxide is a transient measure. Subtotal pancreatectomy is indispensable. Postoperative results are variable. Insulin deficiency diabetes mellitus is common and unusual in that insulin induces an exaggerated response. Recovery can be observed. If hypoglycemia recurs, diazoxide is often effective.

摘要

报告了两例胰腺弥漫性增生的新病例。这种罕见病症是由间歇性和波动性胰岛素分泌过多引起的。高胰岛素血症导致严重、持久且难以控制的低血糖,进而引发癫痫和智力发育迟缓。发病通常发生在新生儿期。高胰岛素血症的诊断基于四个标准:低血糖时胰岛素水平升高、低血糖发作期间酮体尿排泄量低、需要超过15毫克/千克/分钟的葡萄糖才能将血糖水平维持在2毫摩尔/升以上,以及对胰高血糖素的阳性反应。定位诊断往往不尽人意。用二氮嗪治疗低血糖是一种临时措施。胰腺次全切除术是必不可少的。术后结果各不相同。胰岛素缺乏性糖尿病很常见,其不同寻常之处在于胰岛素会引发过度反应。可以观察到恢复情况。如果低血糖复发,二氮嗪通常有效。

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