신증후군을 동반한 유전분증

We report two cases of renal amyloidosis, one of which is associated wit alpha heavy chain disease rarely reported in the literature. The first case was a young female with nephrotic syndrome and renal amyloidosis was documented by renal biopsy as a cause of syndrome. Serum immuno- electrophoresis revealed polyclonal gammopathy. She developed rapid deterioration of renal function the- reafter. At that time, renal venography showed renal vein thrombosis of the left kidney. The second case, a 73-year-old male patient, also presented with nephrotic syndrome. He complained of marked dry mouth and dry eyes simulating Sjogren syndrome. Tissue biopsy of the salivary gland, gingiva and kidney ravealed amyloidosis. Congo red stain of the fat tissue obtained by punch biopsy contributed to the diagnosis of amyloidosis. This particular patient turned out to have alpha heavy chain disease which is extreme- ly rare worldwide.