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先天性肾上腺皮质增生症患者的“腕骨消失”——诊断难题

'Vanishing carpal bones' in a patient with congenital adrenal hyperplasia - A diagnostic dilemma.

作者信息

Siddarth Kamath K, Jayakrishnan K N, Acharya Ashwath, Bhat Anil K

机构信息

Department of Orthopaedics, Kasturba Medical College, Manipal Academy of Higher Education, India.

出版信息

J Orthop. 2020 Oct 9;22:493-496. doi: 10.1016/j.jor.2020.10.001. eCollection 2020 Nov-Dec.

Abstract

The vanishing bone disease, also known as Gorhams disease usually involves long bones. Isolated carpal bone involvement is uncommon. A 14 year old male presented with pain in the right wrist joint since 4 months. He was a known case of Congenital Adrenal Hyperplasia on oral steroids for 3 years. X-rays showed deformed arthritic scaphoid, lunate and capitate. A proximal row carpectomy was performed to provide functional wrist mobility to the patient. Histopathological examination was consistent with Gorham's disease. In a patient with systemic disease receiving steroid therapy Gorhams disease poses a diagnostic challenge.

摘要

骨质消失症,也称为戈勒姆病,通常累及长骨。孤立的腕骨受累并不常见。一名14岁男性自4个月前开始出现右腕关节疼痛。他是一名已知的先天性肾上腺皮质增生症患者,口服类固醇药物3年。X线显示舟状骨、月骨和头状骨有变形的关节炎表现。进行了近端排腕骨切除术以恢复患者腕关节的功能活动度。组织病理学检查结果符合戈勒姆病。对于正在接受类固醇治疗的全身性疾病患者,戈勒姆病带来了诊断挑战。

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