Makibayashi K, Yanagita M, Kanatsu K
Department of Internal Medicine, Hyogo Prefectural Amagasaki Hospital, Japan.
Nihon Jinzo Gakkai Shi. 1997 Jul;39(5):507-11.
A 61-year-old male had nephrotic syndrome in association with minimal renal amyloidosis. The amyloid deposits were inconspicuous and had been initially overlooked, and the biopsy specimen was thought to show minimal glomerular changes. Accordingly he was diagnosed as having minimal change nephrotic syndrome (MCNS). Thereafter, administration of 40 mg/day of prednisolone was started. A few weeks after treatment, proteinuria decreased, but did not disappear. Four years later, he was readmitted for the treatment of the nephrotic syndrome. The second biopsy at 65 years of age revealed typical renal amyloidosis by light microscopy. Congo red staining and electron microscopy confirmed the presence of amyloid deposits. The serum A protein (SAA) level was 328 micrograms/ml. Furthermore, the first biopsy specimen revealed minimal renal amyloidosis by Congo red staining. At the time, 40 mg/day of prednisolone proved ineffective for the proteinuria. However, after methylprednisolone pulse therapy, the proteinuria decreased and the nephrotic syndrome improved. After discharge, administration of 20 mg/day of prednisolone was maintained. One year later, the patient showed no evidence of recurrence of the nephrotic syndrome and the SAA level decreased (from 328 micrograms/ml to 74.4 micrograms/ml). Prednisolone proved to have a beneficial effect on the reduction of proteinuria and SAA levels. We strongly recommend careful examination for amyloid deposits in all kidney biopsy specimens with the appearance of MCNS on older patients whose proteinuria does not respond to the administration of prednisolone.
一名61岁男性患有肾病综合征,伴有轻微肾淀粉样变性。淀粉样沉积物不明显,最初被忽视,活检标本被认为显示肾小球变化轻微。因此,他被诊断为微小病变性肾病综合征(MCNS)。此后,开始每天服用40毫克泼尼松龙。治疗几周后,蛋白尿减少,但未消失。四年后,他因肾病综合征再次入院治疗。65岁时的第二次活检通过光学显微镜显示为典型的肾淀粉样变性。刚果红染色和电子显微镜证实了淀粉样沉积物的存在。血清A蛋白(SAA)水平为328微克/毫升。此外,第一次活检标本经刚果红染色显示为轻微肾淀粉样变性。当时,每天40毫克泼尼松龙对蛋白尿无效。然而,甲基泼尼松龙冲击治疗后,蛋白尿减少,肾病综合征得到改善。出院后,维持每天20毫克泼尼松龙的给药。一年后,患者无肾病综合征复发迹象,SAA水平下降(从328微克/毫升降至74.4微克/毫升)。泼尼松龙被证明对降低蛋白尿和SAA水平有有益作用。我们强烈建议对所有表现为MCNS但蛋白尿对泼尼松龙治疗无反应的老年患者的肾活检标本仔细检查是否存在淀粉样沉积物。