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전격성 간부전의 경과를 보인 원발성 신 아밀로이드 증 1예
박건태 ( Geon Tae Park ),조성 ( Seong Cho ),김성록 ( Sung Rok Kim ),권오언 ( Oh Wen Kwon ),우재곤 ( Jae Gon Woo ),이지은 ( Ji Eun Yi ),정경원 ( Gyung Won Jung ) 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.5
We report the case of a patient with primary renal amyloidosis, who was progressed to fulminant hepatic failure. A 68-year-old man visited our hospital for evaluation of anorexia, weight loss and hepatomegaly. Laboratory examination revealed proteinuria and azotemia. He was confirmed to have primary amyloidosis by renal biopsy. His renal function progressed to deteriorate rapidly with marked deterioration of liver function with cholestasis. Peritoneal dialysis and steroid therapy were started for reduction of urine output and rapid progression of azotemia, and aggravation of liver function. Azotemia was improved after peritoneal dialysis but the liver function worsened leading to hepatic failure, causing death. If jaundice has progressed in any patients with primary amyloidosis, liver transplantation and stem cell transplantation should be considered for preparation of hepatic failure.
Propylthiouracil 복용 후 발생한 ANCA positive RPGN
정경원 ( Gyung Won Jung ),조성 ( Seong Cho ),김성록 ( Sung Rok Kim ),권오언 ( Oh Wen Kwon ),우재곤 ( Jae Gon Woo ),이지은 ( Ji Eun Yi ) 대한신장학회 2010 Kidney Research and Clinical Practice Vol.29 No.3
Anti neutrophil cytoplasmic antibody (ANCA)-positive vasculitis and crescentic glomerulonephritis has been rarely reported in patients suffering from Graves` disease and treated with Propylthiouracil. We experienced a case of ANCA-positive crescentic glomerulonephritis presenting good prognosis after discontinuing Propylthiouracil. A 40-year-old female visited due to the proteinuria and hematuria in urinalysis. She had been medicated Propylthiouracil for 3 years. Blood pressure was 100/60 mmHg. BUN and serum creatinine were 24.7 mg/dL, and 1.9 mg/dL, respectively. Urinalysis revealed protein 1481 mg/day, many RBC`s/HPF (dysmorphic 80%), Serological ANCA was positive, anti-myeloperoxidase (MPO) antibody 1,922 AAU/ mL (normal <150 AAU/mL). The histologic finding showed crescentic glomerulonephritis on light microscopy, but no immuno deposit on immunofluorescence and light microscopy. So we diagnosed ANCA positive pauci-immune glomerulonephritis. Propylthiouracil was discontinued and steroid, cyclophosphamide was medicated within about 1 month, but stopped due to cytopenia. Patient`s creatinine level was maintained 1.3 mg/dL and showed stable progress for about over 18 months. We report this case that showed good prognosis after discontinuation of Propylthiouracil.