Szöke G, Vizkelety T L, Rényi-Vámos A, Elek E
Department of Orthopaedic Surgery, Semmelweis University, Budapest, Hungary.
Clin Orthop Relat Res. 1995 Jan(310):120-9.
Idiopathic carpotarsal osteolysis is 1 of the rare types of disappearing bone diseases characterized by painful limitation of movement and deformity of the wrist and tarsal joints, and associated with frequent episodes of joint inflammation and a radiographic appearance of osteolysis. Reported here is an 8-year-old girl with idiopathic carpotarsal osteolysis and Bartter's syndrome with significantly decreased re-absorption of chloride at distal renal tubules, low level of serum potassium, a metabolic alkalosis, and high levels of aldosterone and renin. Bartter's syndrome associated with idiopathic carpotarsal osteolysis has not been published previously. The typical plantar nodule in this patient confirmed the presence of fibromatosis with aggressive histologic signs in light and electron microscopic examination.
特发性腕跗骨溶解症是罕见的骨质消失性疾病类型之一，其特征为腕关节和跗骨关节活动受限伴疼痛、畸形，并伴有频繁的关节炎症发作及骨质溶解的影像学表现。本文报道了一名8岁女童，患有特发性腕跗骨溶解症及巴特综合征，其远端肾小管对氯化物的重吸收显著减少，血钾水平低，存在代谢性碱中毒，醛固酮和肾素水平升高。此前尚未有关于巴特综合征合并特发性腕跗骨溶解症的报道。该患者典型的足底结节在光镜和电镜检查中证实存在具有侵袭性组织学特征的纤维瘤病。
