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韦格纳肉芽肿病是儿童全垂体功能减退症的罕见病因。

Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood.

作者信息

Kara Ozlem, Demirel Fatma, Acar Banu Celikel, Cakar Nilgün

出版信息

J Pediatr Endocrinol Metab. 2013;26(9-10):959-62. doi: 10.1515/jpem-2013-0033.

Abstract

Wegener granulomatosis (WG) is a cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)-associated, multi-system, necrotizing granulomatous vasculitis. Inflammation of the nasal or oral mucosa, and lung and kidney involvements are typical in the course of the disease. In rare cases, pituitary involvement may occur and cause panhypopituitarism. Pituitary involvement is very rare, and only two pediatric case reports have been published to date out of a total of 24 cases. This is a case report of an adolescent patient who presented with panhypopituitarism symptoms and was later diagnosed with WG. A 16-year-old female patient complained of fever, headache, purulent nasal discharge and severe muscle and joint pain. Additionally, she had polyuria and polydipsia. Investigations revealed a pituitary mass and panhypopituitarism. Positivity of c-ANCA and renal biopsy result compatible with WG confirmed the diagnosis.

摘要

韦格纳肉芽肿病(WG)是一种与胞浆型抗中性粒细胞胞浆抗体(c-ANCA)相关的多系统坏死性肉芽肿性血管炎。鼻或口腔黏膜炎症以及肺和肾脏受累是该疾病过程中的典型表现。在罕见情况下,垂体可能受累并导致全垂体功能减退。垂体受累非常罕见,迄今为止,在总共24例病例中仅有两篇儿科病例报告发表。这是一例青少年患者的病例报告,该患者出现全垂体功能减退症状,后来被诊断为韦格纳肉芽肿病。一名16岁女性患者主诉发热、头痛、脓性鼻涕以及严重的肌肉和关节疼痛。此外,她有多尿和多饮症状。检查发现垂体肿块和全垂体功能减退。c-ANCA阳性以及肾活检结果与韦格纳肉芽肿病相符,从而确诊。

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