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[胰岛细胞增殖症的临床症状、诊断与治疗]

[Clinical aspects, diagnosis and therapy of nesidioblastosis].

作者信息

Dein E, Scheerschmidt G, Menzel K, Gottschalk E

机构信息

Klinik und Poliklinik für Kindermedizin, Medizinischen Akademie Erfurt.

出版信息

Kinderarztl Prax. 1990 Jan;58(1):29-35.

PMID:2332945
Abstract

Hyperinsulinemia due to an excessive secretion of insulin independent on normal regulation is the most frequent cause of persistent neonatal hypoglycemia. We report on clinical course, diagnostic procedures and treatment of nesidioblastosis in three patients. Main symptoms observed in newborn period were hypoglycemia, respiratory embarrassment, cyanosis and convulsions. Primary treatment was started by continuous infusion of glucose, administration of diazoxide and prednisolone or glucagon. Most important investigations were performed simultaneously. In all three children subtotal resection of pancreas was necessary, because there was no constant blood glucose level. Histological specimens confirmed diagnosis. In two of three patients pancreatectomy followed. One suffers from diabetes mellitus, the other one fed normally, has stable blood glucose level possibly due to existence of extrapancreatic insulin producing cells.

摘要

由于胰岛素分泌过多且不受正常调节导致的高胰岛素血症是持续性新生儿低血糖最常见的原因。我们报告了3例胰岛细胞增殖症患者的临床病程、诊断方法及治疗情况。新生儿期观察到的主要症状为低血糖、呼吸窘迫、发绀和惊厥。初始治疗通过持续输注葡萄糖、给予二氮嗪和泼尼松龙或胰高血糖素开始。最重要的检查同时进行。在所有3例患儿中,由于血糖水平不稳定,均需要进行胰腺次全切除术。组织学标本确诊。3例患者中有2例行胰腺切除术。其中1例患糖尿病,另1例喂养正常,血糖水平稳定,可能是由于存在胰腺外产胰岛素细胞。

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