頁籤選單縮合
題名 | Idiopathic Multicentric Osteolysis with Nephropathy=特發性多發性骨頭溶解症併腎病變 |
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作者 | 張弘偉; 朱宗信; 蔡建誠; 謝博生; | 書刊名 | 臺灣醫學會雜誌 |
卷期 | 99:3 2000.03[民89.03] |
頁次 | 頁243-247 |
分類號 | 416.261 |
關鍵詞 | 特發性多發性骨頭溶解症; 腎病變; Idiopathic multicentric osteolysis; Nephropathy; Glomerulosclerosis; |
語文 | 英文(English) |
英文摘要 | Idiopathic multicentric osteolysis is a rare syndrome that manifests with progressive loss of carpal and tarsal bones in childhood. Affected children have arthritic-like episodes, followed by progressive deformities, radiographic osteolytic changes, and variable degrees of disability. A rare form of this disease (type III, sporadic) is associated with serious nephropathy. We present the first reported case of type III idiopathic multicentric osteolysis in a Chinese woman. The patient, a 34-year- old woman with normal mental development and no family history of bone or kidney disease, presented with a 4-day history of nausea and vomiting. She had shortening and swelling of the hands, which had occurred in childhood and persisted at the time of admission. X-ray studies showed disappearance of the carpal bones, and multiple osseous erosions of the tarsal bones. Hypertension, severe azotemia, and metabolic acidosis were also noted. Advanced renal disease was documented after a series of investigations, including renal biopsy. She is now dialysis-dependent. This case illustrates the importance of early diagnosis and management of idiopathic multicentric osteolysis with nephropathy. |
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