尿崩症、糖尿病、视神经萎缩及耳聋综合征中的生长激素缺乏与空蝶鞍：一项内分泌学研究
Growth hormone deficiency and empty sella in DIDMOAD syndrome: an endocrine study.
作者信息
Soliman A T, Bappal B, Darwish A, Rajab A, Asfour M
机构信息
Department of Paediatrics, Royal Hospital, Muscat, Oman.
出版信息
Arch Dis Child. 1995 Sep;73(3):251-3. doi: 10.1136/adc.73.3.251.
Abstract
Two girls with DIDMOAD syndrome are presented. One also had severe megaloblastic-sideroblastic anaemia and the other several neurological manifestations. Both were short with defective growth hormone secretion. Computed tomography revealed empty sella in both girls; one had widespread atrophic cortical and cerebellar changes. High doses of thiamine improved the anaemia in the first case, increased C peptide secretion in both, but had no effect on the neurological abnormalities.
摘要
本文报告了两名患有尿崩症、糖尿病、视神经萎缩及耳聋综合征（DIDMOAD综合征）的女孩。其中一名女孩还患有严重的巨幼细胞性-铁粒幼细胞性贫血，另一名女孩有多种神经学表现。两人均身材矮小，生长激素分泌不足。计算机断层扫描显示两名女孩均存在空蝶鞍；其中一名女孩还有广泛的皮质和小脑萎缩性改变。大剂量硫胺素改善了第一例患者的贫血状况，增加了两人的C肽分泌，但对神经学异常没有影响。
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