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성인 원발성 신증후군의 임상소견 및 치료성적에 대한 고찰

Other Titles
 Clinical characteristics and therapeutic response of primary nephrotic syndrome in adults 
Issue Date
1988
Description
의학과/석사
Abstract
[한글] 원발성 신증후군은 이차적 원인 및 전신질환을 제외한 신 사구체병변으로 심한 단백뇨, 저알부민혈증, 전신부종 및 고지혈증 등의 임상양상율 보이는 증후군으로 신생검을 통한 조직병리 소견에 따라 미세변화형 (minimal change), 막성신증 (membranous nephropathy), 맥증식성 사구체신염 (mesangio-proliferative GN), 국소분절성 사구체경화증 (focal segmental glomerulosclerosis), 막증식성 사구체신염 (membranoproliferative GN) 등으로 분류된다. 그러나 각 원인질환에 따른 임상상, 치료반응및 경과에 대해서는 많은 논란이 되고 있다. 이에 저자는 1979년 1월부터 1987년 12월까지 5년동안 연세대학교 외과대학 세브란스병원 내과에 입원하여 원발성 신증후군으로 진단받고 경피 신생검으로 원인질환이 확진된 188 예를 대상으로 그 임상상, 치료에 대한 반응및 경과를 분석하여 다음과 같은 결과를 얻었다. 1. 원발성 신증후군 188 예의 원인질환 분포는 미세변화형이 131 예(59.61)로 가장 많았고 막성신증 31예 (16.5%), 맥증식성 사구체신염 11예(5.9%), 막증식성 사구체신염 10예 (5.3%),국소분절성 사구체경화증 5예 (2.7%)의 순이었다. 2. 대상환자의 남여비는 2.5:1로 남자에 많았으며, 평균연령은 32.1세였다. 3. 고혈압의 빈도는 막성신증 (48.4%)과 막증식성 사구체신염 (70%)에서 미세변화형 (16.8%)에 비해 높았으며, 현미경적 혈뇨는 모든 군의 50%이상에서 관찰되었고, 선택적 단백뇨는 미세변화형 (50.8%)에서 높게 관찰되었다. B형 간염 표면항원 양성율은 미세변화 형 (12.1%)에 비해 막성신증 (45.27)에서 높았다. 혈청 Ig G는 모든 군에서 정상치보다 낮게 관찰되었으며 Ig A 및 Ig M 은 정상치였다. 4. 2개월간 초치료 반응에서 완전관해율은 미세변화형이 78.8%,맥증식성 사구체신염이 71.4%, 막성신증이 4.7%로 나타났고, 무반응율은 국소분절성 사구체경화증이 100%, 막성신증이 66.7%, 막증식성 사구체신염이 55.5%, 맥증식성 사구체신염이 14.2%, 미세변화형이 7.9%순 이었다. 5. 평균 22개월의 추적관찰 후의 상황에서, 미세변화형은 완전관해율이 69.9%, 부분관해율 10.7%, 신증후군으로 재발된 상태가 18.4%로 예후가 가장 좋았으며, 막증식성 사구체신염과 국소분절성 사구체경화증 각 1예에서 사망을 나타내었다. 6. 합병증은 대상환자의 23.4%에서 관찰되었는데 감염, 혈전색전증, 급성신부전 등이었다. CLINICAL CHARCTERISTICS AND THERAPEUTIC RESPONSE OF PRIMARY NEPHR0TIC SYNDROME IN ADULTS Hyon Young Joo, M. D. Department of Medical Science The Graduate School, Yonsei University (Directed by Prof. Dae Suk Han, M. D.) Primary nephrotic syndrome is characterized by massive proteinuria, hypoalbuminemia, generalized edema and hyperlipidemia, and caused by primary glomerular damage excluding of secondary known causes. It is classified according to the histopathologic features found on renal biopsy into minimal change, mesangi O-proliferative glomerulonephritis, focal segmental glomerulosclerosis, membranous nephropathy and membranoproliferative glomerulonephritis. However, the clinical characteristics and the response to treatment of each disease are variable. To ascertain useful clinicopathological correlationship of each disease entities and to understand long-term clinical courses, studies on clinicopathologic observations as well as responses to treatment and follow-up status were evaluated on 188 cases of primary nephrotic syndrome who were admitted to the Severance Hospital, Yonsei University Medical Center during past 9 years from January 1979 to December 1987 and following results were obtained. 1. Pathologic findings of 188 patients were classified into minimal change(69.6%), membranous nephropathy (16.5%), mesangi 0-proliferative GN(5.9%), membranoproliferative GN(5.3%) and focal segmental glomerulosclerosis(2.7%). 2. Sex ratio was 2.5 to 1 with male preponderance. Mean age was 32.1 3. Incidence rate of hypertension was significantly higher in the group of membranous nephropathy(48.4%) and membranoproliferative GN(70%) than that of minimal change(16.8%). Microscopic he-maturia was noticed above 60% in all groups and selective proteinuria in 50.8% of minimal change. Positive rate of HBsAg was significantly higher in the group of membranous nephropathy(45.2%) than that of minimal change(12.1%)1. Decrease of Ig G level was observed in all groups. 4. With initial treatment, complete remission was obtained in 78.8% of minimal change, 71.4% of mesangialproliferative GN. and 4.7% of membranous nephropathy. No response were noticed in 55.5% of membranoproliferative GN, 65.7% of membranous nephropathy and all of focal segmental glomerulosclerosis. 5. During the follow-up period (mean 22-months), course of minimal change was relatively good with 69.5% in complete remission state, partial remission state in 10.7%, relapsed nephrotic syndrome in 18.41%. 6. The main complications observed were infection, thromboembolism and acute renal failure and were seen in 23.4% of the cases.
[영문] Primary nephrotic syndrome is characterized by massive proteinuria, hypoalbuminemia, generalized edema and hyperlipidemia, and caused by primary glomerular damage excluding of secondary known causes. It is classified according to the histopathologic features found on renal biopsy into minimal change, mesangi O-proliferative glomerulonephritis, focal segmental glomerulosclerosis, membranous nephropathy and membranoproliferative glomerulonephritis. However, the clinical characteristics and the response to treatment of each disease are variable. To ascertain useful clinicopathological correlationship of each disease entities and to understand long-term clinical courses, studies on clinicopathologic observations as well as responses to treatment and follow-up status were evaluated on 188 cases of primary nephrotic syndrome who were admitted to the Severance Hospital, Yonsei University Medical Center during past 9 years from January 1979 to December 1987 and following results were obtained. 1. Pathologic findings of 188 patients were classified into minimal change(69.6%), membranous nephropathy (16.5%), mesangi 0-proliferative GN(5.9%), membranoproliferative GN(5.3%) and focal segmental glomerulosclerosis(2.7%). 2. Sex ratio was 2.5 to 1 with male preponderance. Mean age was 32.1 3. Incidence rate of hypertension was significantly higher in the group of membranous nephropathy(48.4%) and membranoproliferative GN(70%) than that of minimal change(16.8%). Microscopic he-maturia was noticed above 60% in all groups and selective proteinuria in 50.8% of minimal change. Positive rate of HBsAg was significantly higher in the group of membranous nephropathy(45.2%) than that of minimal change(12.1%)1. Decrease of Ig G level was observed in all groups. 4. With initial treatment, complete remission was obtained in 78.8% of minimal change, 71.4% of mesangialproliferative GN. and 4.7% of membranous nephropathy. No response were noticed in 55.5% of membranoproliferative GN, 65.7% of membranous nephropathy and all of focal segmental glomerulosclerosis. 5. During the follow-up period (mean 22-months), course of minimal change was relatively good with 69.5% in complete remission state, partial remission state in 10.7%, relapsed nephrotic syndrome in 18.41%. 6. The main complications observed were infection, thromboembolism and acute renal failure and were seen in 23.4% of the cases.
URI
http://ir.ymlib.yonsei.ac.kr/handle/22282913/117096
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2. 학위논문 > 1. College of Medicine (의과대학) > 석사
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