Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A Case of Hypocomplementemic Henoch-Schönlein Purpura Presenting Features of Membranoproliferative Glomerulonephritis

저 보체 혈증 및 막성 증식성 사구체 신염의 임상 상을 보인 Henoch-Schönlein (Purpura) Nephritis 1례

  • Lee, Kyong-A (Department of Pediatrics, College of Medicine, Chungbuk National University) ;
  • Ha, Tae-Sun (Department of Pediatrics, College of Medicine, Chungbuk National University)
  • 이경아 (충북대학교 의과대학 소아과학교실) ;
  • 하태선 (충북대학교 의과대학 소아과학교실)
  • Received : 2004.08.10
  • Accepted : 2004.09.23
  • Published : 2005.01.15
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Abstract

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis that can affect multiple organs predominantly the skin, joints, gastrointestinal tract and kidney. Although the specific pathogenesis of HSP is not known, there are several hypotheses. Although the importance of the complement activation in glomerular injury in HSP has been suggested, the complement levels and the blood pressure in those patients are usually normal and massive proteinuria is not common. And pathologic renal changes also have been reported to show a large variety of glomerular changes. However, to our knowledge, a membranoproliferative glomerulo-nephritis (MPGN) is a rare renal clinicopathologic manifestation of HSP. We report a 6-year-old boy with HSP who developed MPGN with hypertension, massive proteinuria, and hypo-complementemia revealed activation of the classical complement pathway, although we could not exclude the possibility of other hypocomplementemic glomerulonephritis including post-streptococcal acute glomerulonephritis.

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP)는 피부, 관절, 위장관 그리고 신장을 침범하는 혈관염으로 혈소판 감소증이 없는 자반증을 전형적인 증상으로 하는 질환으로 그 원인에 대하여 여러 가지 가설들이 있지만 아직 확실치 않다. HSP에서 신장 침범은 이차적으로 오는 혈관염으로 임상적으로 혈뇨와 단백뇨의 증상을 보이고 병리학적 소견은 IgA 신염과 매우 유사하다. 그 외에도 HSP 신염은 다양한 형태의 사구체 신염을 보이는데 막성 사구체 신염은 드물다. 이에 저자들은 HSP의 임상 양상을 가지면서 혈청 내 보체의 감소, 단백뇨 및 고혈압을 보이고 조직학적으로 막성 사구체 신염 소견을 보인 6세 환아를 경험하였기에 이를 문헌 고찰과 함께 보고하는 바이다.

Keywords

Acknowledgement

Supported by : Chungbuk National University

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