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| 신유전분증의 임상적 특징 (Renal Amyloidosis) |
| 허우성 , 오국환 , 김연수 , 윤형진 , 안규리 , 한진석 , 김성권 , 이정상 , 이현순 , 김용일 ( Woo Seong Huh , Kook Hwan Oh , Yon Su Kim , Hyung Jin Yoon , Curie Ahn , Jin Suk Han , Suhng Won Kim , Jung Sang Lee ) |
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| Abstract |
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Renal involvment is the most common clinical problem in amyloidosis, affecting more than one third of the patients at presentation, and renal amyloidosis accounts for 3-9% of nephrotic syndrome in adults. However, there were several case reports in Korea. We retrospectively analyzed the clinical and labora tory data of 17 patients with biopsyproved renal amyloidosis examined at Seoul National University Hospital. And the results were as follows; 1) The underlying diseases of renal amylodiosis were multiple myeloma (5 patients), Castlemans disease (1 patient), and ankylosing spondylitis (1 patient). The underlying diseases of 10 patients could not be found. 2) 12 patients (71%) were over age of 50 years at diagnosis. 3) The renal manifestations were nephrotic syndrome (11 patients), chroinc nephritic syndrome (5 patients), and asymptomatic urinary abnormality (1 patient). 4) A monoclonal protein was found n 10 patients (59%). The lambda to kappa ratio was 9: 1 5) The median survival of patients with renal amyloidosis was 14 months. These findings suggest that amyloidosis should be considered in nephrotic syndrome over age of 50 years. |
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