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題 名 | Medullary Cystic Disease: A Family Study=腎髓質囊腫病:一個家族之研究 |
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作 者 | 陳鴻鈞; 張哲銘; 蔡瑞熊; 賴永勳; | 書刊名 | 臺灣醫學會雜誌 |
卷 期 | 97:3 1998.03[民87.03] |
頁 次 | 頁210-213 |
分類號 | 415.74 |
關鍵詞 | 腎髓質囊腫病; Medullary cystic disease; Nephronophthisis; |
語 文 | 英文(English) |
英文摘要 | Medullary cystic disease of the kidney is characterized by progressive tubulointerstitial disease with medullary cyst formation and secondary glomerular sclerosis. We treated a patient with chronic renal failure and investigated the family history of renal disease. The patient, an 18-year-old woman, was admitted due to poor appetite and fatigue for several months. Findings on physical examination were normal except for a pale conjunctiva. Urinalysis revealed only mild proteinuria with clear sediment. The hemogram showed normocytic normochromic anemia with hemoglobin 86 g/L. The patient was azotemic and her creatinine clearance rate was 10.7 mL/min. Renal sonography showed contraction of both kidneys with a marked increase in cortical echogenicity One small cyst was found in the medullary area. Computed tomography (CT) and magnetic resonance imaging revealed several medullary cysts. Percutaneous renal biopsy showed focal and periglomerular sclerosis, marked tubular atrophy, and interstitial fibrosis. Ten of her family members were examined for renal function, and by sonography and CT. Five had medullary cysts, and three of the five showed abnormal renal function, Medullary cystic disease should be considered in the differential diagnosis of patients with renal disease and a positive family history. |
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