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垂体巨人症:一例报告

Pituitary gigantism: a case report.

作者信息

Bhattacharjee Rana, Roy Ajitesh, Goswami Soumik, Selvan Chitra, Chakraborty Partha P, Ghosh Sujoy, Biswas Dibakar, Dasgupta Ranen, Mukhopadhyay Satinath, Chowdhury Subhankar

机构信息

Department of Endocrinology and Metabolism, SSKM and IPGMER, Kolkata, India.

出版信息

Indian J Endocrinol Metab. 2012 Dec;16(Suppl 2):S285-7. doi: 10.4103/2230-8210.104061.

Abstract

OBJECTIVE

To present a rare case of gigantism.

CASE REPORT

A 25-year-old lady presented with increased statural growth and enlarged body parts noticed since the age of 14 years, primary amenorrhea, and frontal headache for the last 2 years. She has also been suffering from non-inflammatory low back pain with progressive kyphosis and pain in the knees, ankles, and elbows for the last 5 years. There was no history of visual disturbance, vomiting, galactorrhoea, cold intolerance. She had no siblings. Family history was non-contributory. Blood pressure was normal. Height 221 cm, weight 138 kg, body mass index (BMI)28. There was coarsening of facial features along with frontal bossing and prognathism, large hands and feet, and small goitre. Patient had severe kyphosis and osteoarthritis of knees. Confrontation perimetry suggested bitemporal hemianopia. Breast and pubic hair were of Tanner stage 1. Serum insulin like growth factor-1 (IGF1) was 703 ng/ml with all glucose suppressedgrowth hormone (GH)values of >40 ng/ml. Prolactin was 174 ng/ml. Basal serum Lutenising Hormone (LH), follicle stimulating Hormone (FSH) was low. Oral glucose tolerance test (OGTT), liver and renal function tests, basal cortisol and thyroid profile, Calcium, phosphorus and Intact Parathyroid hormone (iPTH) were normal. Computed tomographyscan of brain showed large pituitary macroadenoma. Automated perimetry confirmed bitemporal hemianopia. A diagnosis of gigantism due to GH secreting pituitary macroadenoma with hypogonadotrophichypogonadism was made. Debulking pituitary surgery followed by somatostatin analogue therapy with gonadal steroid replacement had been planned, but the patient refused further treatment.

摘要

目的

报告一例罕见的巨人症病例。

病例报告

一名25岁女性,自14岁起身高增长加速且身体各部位增大,原发性闭经,近2年出现前额头痛。近5年她还一直患有非炎性下腰痛伴进行性脊柱后凸,以及膝关节、踝关节和肘关节疼痛。无视力障碍、呕吐、溢乳、不耐寒病史。她没有兄弟姐妹。家族史无特殊意义。血压正常。身高221厘米,体重138千克,体重指数(BMI)28。面部特征粗糙,伴有前额突出和下颌前突,手脚粗大,甲状腺肿大。患者有严重的脊柱后凸和膝关节骨关节炎。视野检查提示双颞侧偏盲。乳房和阴毛处于坦纳1期。血清胰岛素样生长因子-1(IGF1)为703纳克/毫升,所有葡萄糖抑制生长激素(GH)值均>40纳克/毫升。催乳素为174纳克/毫升。基础血清促黄体生成素(LH)、促卵泡生成素(FSH)较低。口服葡萄糖耐量试验(OGTT)、肝肾功能检查、基础皮质醇和甲状腺功能检查、钙、磷和完整甲状旁腺激素(iPTH)均正常。脑部计算机断层扫描显示巨大垂体大腺瘤。自动视野检查证实为双颞侧偏盲。诊断为生长激素分泌型垂体大腺瘤导致的巨人症伴低促性腺激素性性腺功能减退。计划行垂体肿瘤减积手术,随后进行生长抑素类似物治疗并给予性腺类固醇替代治疗,但患者拒绝进一步治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9cfe/3603049/98fbc85d64d2/IJEM-16-285-g001.jpg

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