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垂体柄缺如导致的未经治疗的垂体功能减退症且成年身高正常:两例报告

Untreated hypopituitarism due to absence of the pituitary stalk with normal adult height: report of two cases.

作者信息

Papastathopoulou Leda, Tzanela Marinella, Vlassopoulou Vania, Vassiliadi Dimitra, Thalassinos Nikolaos

机构信息

Department of Endocrinology, Diabetes & Metabolism, Evangelismos Hospital, 10676 Athens, Greece.

出版信息

Endocrine. 2006 Feb;29(1):175-9. doi: 10.1385/endo:29:1:175.

Abstract

Patients with congenital multiple pituitary hormones deficiency (MPHD) occasionally present with pituitary stalk interruption and ectopic posterior lobe on magnetic resonance imaging (MRI). Very rarely normal adult height despite growth hormone deficiency (GHD) has been described in these patients. We report two patients with evidence of congenital MPHD, who remained untreated until adulthood. They both failed to develop spontaneous puberty, and they demonstrated very low growth velocity until adulthood when they continued to grow, with a final height of 176 and 169 cm when they sought medical attention in our department at the age of 45 and 33 yr, respectively. At that time a hypoplastic pituitary, absence of pituitary stalk, and ectopic posterior pituitary lobe were found on MRI, and the laboratory investigations, including dynamic tests for pituitary hormone reserve, revealed MPHD with severe GHD. In conclusion, these cases illustrate that very rarely patients with untreated MPHD can reach normal adult height. Some postulations about the pathophysiology of this phenomenon are discussed.

摘要

先天性多种垂体激素缺乏(MPHD)患者在磁共振成像(MRI)上偶尔会出现垂体柄中断和垂体后叶异位。在这些患者中,尽管存在生长激素缺乏(GHD),但极罕见地会出现成年身高正常的情况。我们报告了两名有先天性MPHD证据的患者,他们直到成年都未接受治疗。他们均未自发进入青春期,在成年前生长速度非常缓慢,直到成年后仍持续生长,分别在45岁和33岁到我们科室就诊时,最终身高为176厘米和169厘米。当时MRI显示垂体发育不全、垂体柄缺如以及垂体后叶异位,包括垂体激素储备动态试验在内的实验室检查显示为MPHD伴严重GHD。总之,这些病例表明,未经治疗的MPHD患者极少能达到正常成年身高。本文讨论了关于这一现象病理生理学的一些推测。

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