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| 신증후군과 말초 신경증으로 발현된 원발성 전신성 유전분증 1예 |
| 배윤상 , 이능아 , 이상호 , 이지혁 , 조만종 , 최선희 , 고영재 , 김병준 , 이성주 , 양윤식 |
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| Abstract |
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Primary systemic amyloidosis is a progressive disease that is frequently fatal. Nephrotic syndrome is present in almost one-third, congestive heart failure in one-quarter, and peripheral neuropathy in one-sixth of patients at the time of diagnosis. If heart or renal failure are presented, survival rate is poor. We experienced a case of a 66 year-old fernale patient who had complained lower leg edema and paresthesia of extremities for about 5 rnonths. The laboratory findings were consistent with nephrotic syndrome, but the lower leg edema was non-pitting and the cause of paresthesia was unknown. We perforrned kidney and nerve biopsy and confirmed a case of primary systemic arnyloidosis. In this case, presence of postural hypotension, probable cardiac involvement and relatively long spikes along the outside of the glomerular capillary loops on me- thenamine silver stain is suggestive of poor prognosis. We can predict chronic renal failure and congestive heart failure in the course of this case. We report a case of primary systemic amyloidosis predominantly presenting nephrotic syndrome and peripheral neuropathy with review of related lite- ratures. |
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