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孤立性垂体前叶发育不全伴异常关联。

Isolated aplasia of the anterior pituitary gland with unusual associations.

作者信息

Sultan Z, Gnanaratnam J, Sharief N

机构信息

Neonatal Unit, Basildon Hospital, Essex, UK.

出版信息

Clin Dysmorphol. 1996 Oct;5(4):347-50.

PMID:8905201
Abstract

A male infant was found to have cleft lip and palate with micropenis at birth. He suffered recurrent episodes of hypoglycaemia in the first 3 days. CT scan showed possible incomplete division of the frontal lobes and endocrinological investigations confirmed hypopituitarism. He developed renal and respiratory failure and died at the age of 10 days. Post mortem showed an absent adenohypophysis, an intact neurohypophysis, absent olfactory tracts and polysplenia.

摘要

一名男婴出生时被发现患有唇腭裂和小阴茎。他在出生后的头3天里反复出现低血糖发作。CT扫描显示额叶可能存在不完全分隔,内分泌检查证实存在垂体功能减退。他随后出现肾衰竭和呼吸衰竭,并于10日龄时死亡。尸检显示腺垂体缺如,神经垂体完整,嗅束缺如,且存在多脾症。

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