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单中心肢端肥大症患者的临床表现、发病率及治疗结果

Presentation, Morbidity and Treatment Outcome of Acromegaly Patients at a Single Centre.

作者信息

Wani Rayees Ul Hamid, Misgar Raiz Ahmad, Bhat Moomin Hussain, Bhat Javaid Ahmad, Masoodi Shariq Rashid, Bashir Mir Iftikhar, Wani Arshad Iqbal

机构信息

Department of Endocrinology, Sheri Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India.

出版信息

Indian J Endocrinol Metab. 2019 Jul-Aug;23(4):433-437. doi: 10.4103/ijem.IJEM_132_19.

DOI:10.4103/ijem.IJEM_132_19
PMID:31741902
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6844178/
Abstract

INTRODUCTION

The management of acromegaly, a rare and potentially curable disease, has undergone a paradigm shift in the past few decades. Many of the treatment modalities recommended for acromegaly are either too expensive or not available in many parts of India. There is a dearth of treatment and outcome data in Indian patients.

AIM

Our aim was to study the clinical presentation, hormonal profile, radiology, management, and outcome of the disease at our center.

MATERIALS AND METHODS

Fifty one patients with acromegaly who attended the Department of Endocrinology, SKIMS, Srinagar, between October 2015 and April 2017, were included in the study. Clinical and hormonal profiles, comorbidities, treatment modalities, and outcome were evaluated.

RESULTS

The gender distribution was equal with the mean age of 42.3 ± 10.9 years at diagnosis. The majority (41) of the patients had macroadenoma. The most common presenting manifestations were acral enlargement and headache. Hypertension was present in 23, musculoskeletal manifestations in 19, and diabetes mellitus in 11 patients. Surgery was the most common method of treatment. Preoperatively only one patient with micro-adenoma had hypocortisolism, which was persistent in postoperative period, while no patient had preoperative or postoperative hypothyroidism or hypogonadism. As per the present consensus criteria, 23.7% patients achieved disease control (40% with microadenoma and only 19.5% with macroadenoma). The surgical complications occurred in 5 patients-CSF leak in 3 meningitis in 2 patients all except one having macroadenoma.

CONCLUSIONS

The presentation of disease was generally comparable to that reported in literature. Cure rates were significantly lower than those reported from many large centers.

摘要

引言

肢端肥大症是一种罕见但有可能治愈的疾病,在过去几十年中其治疗模式发生了范式转变。许多推荐用于肢端肥大症的治疗方法要么过于昂贵,要么在印度的许多地区无法获得。印度患者缺乏治疗和预后数据。

目的

我们的目的是研究我院该疾病的临床表现、激素水平、放射学检查、治疗及预后情况。

材料与方法

纳入2015年10月至2017年4月在斯利那加SKIMS内分泌科就诊的51例肢端肥大症患者。对患者的临床和激素水平、合并症、治疗方式及预后进行评估。

结果

性别分布均衡,诊断时平均年龄为42.3±10.9岁。大多数(41例)患者患有大腺瘤。最常见的临床表现是肢端肥大和头痛。23例患者有高血压,19例有肌肉骨骼表现,11例有糖尿病。手术是最常见的治疗方法。术前仅1例微腺瘤患者存在皮质醇减退,术后仍持续存在,而无患者术前或术后出现甲状腺功能减退或性腺功能减退。根据目前的共识标准,23.7%的患者疾病得到控制(微腺瘤患者为40%,大腺瘤患者仅为19.5%)。5例患者发生手术并发症——3例脑脊液漏,2例脑膜炎,除1例大腺瘤患者外均出现并发症。

结论

该疾病的表现总体上与文献报道相当。治愈率显著低于许多大型中心报道的治愈率。

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Endocrinological outcomes following endoscopic and microscopic transsphenoidal surgery in 113 patients with acromegaly.
113例肢端肥大症患者接受内镜和显微镜经蝶窦手术后的内分泌学结果。
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Growth hormone receptor variants and response to pegvisomant in monotherapy or in combination with somatostatin analogs in acromegalic patients: a multicenter study.生长激素受体变异体与培维索孟单药或联合生长抑素类似物治疗肢端肥大症患者的反应:一项多中心研究。
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