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与唇腭裂相关的下丘脑 - 垂体激素不足

Hypothalamo-pituitary hormone insufficiency associated with cleft lip and palate.

作者信息

Roitman A, Laron Z

出版信息

Arch Dis Child. 1978 Dec;53(12):952-5. doi: 10.1136/adc.53.12.952.

Abstract

Two male patients with congenital cleft lip and palate first seen at ages 10.2 and 21.5 years presented with typical signs of hypothalamic-interior pituitary hormone deficiencies. They were found to lack GH, LH, and FSH and to be partially deficient in TSH and ACTH. Several congenital defects may explain this rare syndrome affecting midline structures in the proximity of the maldeveloped palate, including Rathke's pouch, which migrates distally to develop into the anterior pituitary.

摘要

两名先天性唇腭裂男性患者首次就诊时年龄分别为10.2岁和21.5岁,表现出典型的下丘脑-垂体前叶激素缺乏症状。他们被发现缺乏生长激素(GH)、促黄体生成素(LH)和促卵泡生成素(FSH),促甲状腺激素(TSH)和促肾上腺皮质激素(ACTH)部分缺乏。几种先天性缺陷可能解释了这种影响发育不良腭部附近中线结构的罕见综合征,包括Rathke囊,它向远端迁移并发育成垂体前叶。

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Pituitary insufficiency in cleft palate or lip.唇腭裂中的垂体功能不全。
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Gastroschisis associated with cleft lip and palate.腹裂合并唇腭裂。
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引用本文的文献

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Cleft palate and gonadotrophin deficiency.腭裂与促性腺激素缺乏症。
Arch Dis Child. 1984 Jun;59(6):580-2. doi: 10.1136/adc.59.6.580.

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