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[4例儿童尿崩症患者垂体柄不明原因的形态学异常]

[Morphological abnormalities of the pituitary stalk of unknown cause in 4 cases of diabetes insipidus in children].

作者信息

Banerjee A, Léger J, Garel C, Hassan M, Czernichow P

机构信息

Service d'endocrinologie-diabétologie, hôpital Robert-Debré, Paris, France.

出版信息

Arch Pediatr. 1994 Mar;1(3):233-40.

PMID:7994330
Abstract

BACKGROUND

Magnetic resonance imaging (MRI) is the best technique for studying hypothalamic and hypophyseal structures.

CASE REPORTS

Four children aged 2 years 5 months to 8 years with idiopathic diabetes insipidus were studied. Their condition had been identified for 2-6 years. All the patients had undergone complete clinical, neurological and endocrinological investigations during the search for the cause of their diabetes insipidus. A CT scan was performed in one patient and MRI in all four; the investigations were repeated each 6 months for 4 years.

RESULTS

The first examination showed complete lack of antidiuretic hormone (four patients) and growth hormone deficiency (two patients). An enlarged pituitary stalk was seen in three patients, one patient had a small anterior pituitary, and three patients gave no posterior pituitary hypersignal. Follow-up showed that three patients completely lacked of growth hormone. One was thyrotropin deficient, three had persistent enlarged pituitary stalks, three had sub-normal anterior pituitaries and all four patients produced no post-pituitary hypersignal. None of the patients developed signs of histiocytosis or germinoma.

CONCLUSION

The finding of abnormal pituitary stalks by MRI raises the possibility that the apparently idiopathic diabetes insipidus is due to some type of infiltrating disease of the hypothalamus and pituitary.

摘要

背景

磁共振成像(MRI)是研究下丘脑和垂体结构的最佳技术。

病例报告

对4名年龄在2岁5个月至8岁的特发性尿崩症患儿进行了研究。他们的病情已确诊2至6年。所有患者在寻找尿崩症病因的过程中均接受了全面的临床、神经学和内分泌学检查。1名患者进行了CT扫描,4名患者均进行了MRI检查;在4年的时间里,每6个月重复进行一次检查。

结果

首次检查显示所有4名患者均完全缺乏抗利尿激素,2名患者存在生长激素缺乏。3名患者可见垂体柄增粗,1名患者垂体前叶较小,3名患者垂体后叶无高信号。随访显示,3名患者完全缺乏生长激素。1名患者促甲状腺激素缺乏,3名患者垂体柄持续增粗,3名患者垂体前叶功能低下,所有4名患者垂体后叶均无高信号。所有患者均未出现组织细胞增多症或生殖细胞瘤的迹象。

结论

MRI检查发现垂体柄异常提示,看似特发性的尿崩症可能是由某种类型的下丘脑和垂体浸润性疾病引起的。

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