본문 바로가기
KERD
논문KCI 등재

초점분절사구체경화증에서 발세포병증과 형태 변화

Podocytopathy and Morphologic Changes in Focal Segmental Glomerulosclerosis

정현주
대한소아신장학회 (The Korean Society Of Pediatric Nephrology)|2013.04|Childhood Kidney Diseases (Childhood Kidney diseases)|제17권 제1호|pp. 13-18

2

조회

0

다운로드

0

인용

0

참고문헌

키워드

MorphologySubtypesFocal segmental glomerulosclerosis
주제분류소아과학

초록

발세포병증은 발세포의 손상을 특징으로 하는 사구체질환이다. 발세포의 손상은 여러 사구체질환에서 관찰될 수 있으나, 미소변화질환과 FSGS에서 주요 병인으로 작용한다. 이 글에서는 FSGS에서 발세포 손상의 형태 변화와 분절경화의 유형을 설명하고자 한다. 발세포가 손상되면 형태 변화로 발돌기의 소실, 발세포 세포질 내 공포, 발세포하 낭 등이 관찰되며, 심하면 발세포의 탈락 및 자멸사가 관찰된다. 그러나 분절경화가 초래되기까지에는 일정 수준 이상의 발세포의 소실이 있어야 하며, 손상된 발세포는 동일한 사구체 소엽 내 주변 발세포로 손상을 전파하여 병변이 커지게 된다. FSGS는 광학현미경 소견을 기초로 NOS형, perihilar형, cellular형, tip형, collapsing형의 다섯 가지 유형으로 나뉜다. 각 아형에 따라 임상 경과나 스테로이드 치료에 대한 반응이 다르고 흔히 동반되는 임상 조건들도 다르다고 보고되었으나 이에 대하여는 아직도 논란이 있는 실정이다. 앞으로 FSGS 발생에 관여하는 유전 정보와 혈액 내 투과인자의 성분 등 관련된 인자들에 대한 체계적인 연구가 이루어지면 FSGS에서 관찰되는 조직 변화나 병태생리를 더 잘 설명할 수 있을 것으로 기대해 본다.

Abstract

Podocytopathy is glomerular lesions characterized by podocyte injury. It is observed in various glomerular diseases, but minimal change disease and focal segmental glomerulosclerosis (FSGS) are the prototypes. In this review, morphologic features of podocyte injury and subtypes of FSGS will be reviewed briefly. Effacement of podocyte foot processes is the most common feature of podocyte injury. As podocytic injury progresses, intracytoplasmic vacuoles, subpodocytic cyst, detachment of podocytes from the glomerular basement membrane and apoptosis develop. Glomerular capillary loops in epithelium-denuded area undergo capillary collapse. Synechia and hyalinosis may accompany this lesion. To manifest segmental sclerosis, podocyte loss above a threshold level may be required. Injured podocytes can injure neighboring intact podocytes, and thereby spread injury within the same lobule. FSGS can be categorized into five subtypes by morphologic characteristics; not otherwise specified (NOS), perihilar, cellular, tip, and collapsing types. Each subtype has been reported to show different clinical courses and associated conditions, but there are controversies on its significance. With recent progress in the discovery of genetic abnormalities causing FSGS and plasma permeability factors, we expect to unravel pathophysiology of FSGS and to understand histological sequences leading to FSGS in near future.

참고문헌 (32)

  1. Neal CR, "Threedimensional reconstruction of glomeruli by electron microscopy reveals a distinct restrictive urinary subpodocyte space", J Am Soc Nephrol, 16, pp.1223~1235 (2005)
  2. Cohen AH, "Glomerular podocyte degeneration in human renal disease: an ultrastructural study", Lab Invest, 37, pp.30~42 (1977)
  3. Grishman E, "Focal glomerular sclerosis in nephrotic patients: an electron microscopic study of glomerular podocytes", Kidney Int, 7, pp.111~122 (1975)
  4. Wharram BL, "Podocyte depletion causes glomerulosclerosis: diphtheria toxin-induced podocyte depletion in rats expressing human diphtheria toxin receptor transgene", J Am Soc Nephrol, 16, pp.2941~2952 (2005)
  5. Matsusaka T, "Podocyte injury damages other podocytes", J Am Soc Nephrol, 22, pp.1275~1285 (2011)
  6. Howie AJ, "The glomerular tip lesion: a previously undescribed type of segmental glomerular abnormality", J Pathol, 142, pp.205~220 (1984)
  7. Beaman M, "The glomerular tip lesion: a steroid responsive nephrotic syndrome", Clin Nephrol, 27, pp.217~221 (1987)
  8. Schwartz MM, "Focal segmental glomerular sclerosis: the cellular lesion", Kidney Int, 28, pp.968~974 (1985)
  9. Weiss MA, "Nephrotic syndrome, progressive irreversible renal failure, and glomerular "collapse": a new clinicopathologic entity?", Am J Kidney Dis, 7, pp.20~28 (1986)
  10. Ito H, "Twenty-seven children with focal segmental glomerulosclerosis: correlation between the segmental location of the glomerular lesions and prognosis", Clin Nephrol, 22, pp.9~14 (1984)
  11. Morita M, "The clinical significance of the glomerular location of segmental lesions in focal segmental glomerulosclerosis", Clin Nephrol, 33, pp.211~219 (1990)
  12. Meyrier AY, "Collapsing glomerulopathy: expanding interest in a shrinking tuft", Am J Kidney Dis, 33, pp.801~803 (1999)
  13. Detwiler RK, "Collapsing glomerulopathy: a clinically and pathologically distinctvariant of focal segmental glomerulosclerosis", Kidney Int, 45, pp.1416~1424 (1994)
  14. Shimamura T, "A collapsing form of glomerulopathy", Pathol Int, 45, pp.520~523 (1995)
  15. Valeri A, "Idiopathic collapsing focal segmental glomerulosclerosis: a clinicopathologic study", Kidney Int, 50, pp.1734~1746 (1996)
  16. Grcevska L, "Collapsing glomerulopathy: clinical characteristics and follow-up", Am J Kidney Dis, 33, pp.652~657 (1999)
  17. Schwartz MM, "Primary focal segmental glomerular sclerosis in adults: prognostic value of histologic variants", Am J Kidney Dis, 25, pp.845~852 (1995)
  18. Kambham N, "Obesity-related glomerulopathy: an emerging epidemic", Kidney Int, 59, pp.1498~1509 (2001)
  19. D'Agati VD, "Pathologic classification of focal segmental glomerulosclerosis: a working proposal", Am J Kidney Dis, 43, pp.368~382 (2004)
  20. Thomas DB, "Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants", Kidney Int, 69, pp.920~926 (2006)
  21. Stokes MB, "Cellular focal segmental glomerulosclerosis: Clinical and pathologic features", Kidney Int, 70, pp.1783~1792 (2006)
  22. Deegens JK, "Pathological variants of focal segmental glomerulosclerosis in an adult Dutch population--epidemiology and outcome", Nephrol Dial Transplant, 23, pp.186~192 (2008)
  23. 정현주, "한국인 성인에서 국소분절사구체경화증의 아형에 따른 분석", Journal of Pathology and Translational Medicine, 44(6), pp.589~596 (2010), 대한병리학회
  24. D'Agati VD, "The spectrum of focal segmental glomerulosclerosis: new insights", Curr Opin Nephrol Hypertens, 17, pp.271~281 (2008)
  25. Barisoni L, "The dysregulated podocyte phenotype: a novel concept in the pathogenesis of collapsing idiopathic focal segmental glomerulosclerosis and HIV-associated nephropathy", J Am Soc Nephrol, 10, pp.51~61 (1999)
  26. Bariety J, "Podocytes undergo phenotypic changes and express macrophagic-associated markers in idiopathic collapsing glomerulopathy", Kidney Int, 53, pp.918~928 (1998)
  27. Nagata M, "Cell cycle regulation and differentiation in the huma podocyte lineage", Am J Pathol, 153, pp.1511~1520 (1998)
  28. Dijkman H, "The parietal epithelial cell is crucially involved in human idiopathic focal segmental glomerulosclerosis", Kidney Int, 68, pp.1562~1572 (2005)
  29. Howie AJ, "Evolution of nephrotic-associated focal segmental glomerulosclerosis and relation to the glomerular tip lesion", Kidney Int, 67, pp.987~1001 (2005)
  30. Canaud G, "Recurrence of nephrotic syndrome after transplantation in a mixed population of children and adults: course of glomerular lesions and value of the Columbia classification of histological variants of focal and segmental glomerulosclerosis (FSGS)", Nephrol Dial Transplant, 25, pp.1321~1328 (2010)
  31. Santin S, "Clinical utility of genetic testing in children and adults with steroid-resistant nephrotic syndrome", Clin J Am Soc Nephrol, 6, pp.1139~1148 (2011)
  32. Wei C, "Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis", Nature Med, 17, pp.952~960 (2011)

관련 논문AI추천

초점성 분절성 사구체 경화증의 병리와 분류김용진대한소아신장학회 · Childhood Kidney Diseases2012 · 제16권 제1호한국인 성인에서 국소분절사구체경화증의 아형에 따른 분석김도경, 김민주, 임범진, 정현주대한병리학회 · Journal of Pathology and Translational Medicine2010 · 제44권 제6호Pathogenesis of Focal Segmental Glomerulosclerosis도우성, 양재원, 임범진, Agnes B. Fogo대한병리학회 · Journal of Pathology and Translational Medicine2016 · 제50권 제6호Alport 증후군에서 사구체 기저막의 형태학적 변화와 사구체 상피세포의 구조 및 임상지표와의 관계김기혁, 김영기, 엄혜진, 이재승, 정현주, 홍성진대한소아신장학회 · Childhood Kidney Diseases2010 · 제14권 제1호사구체신염의 병리 - 표본제작 및 기본병변 -김용진대한소아신장학회 · Childhood Kidney Diseases2011 · 제15권 제1호Significance of Osteopontin Expression in the Progression of Human Focal Segmental Glomerulosclerosis김태숙, 윤길숙대한병리학회 · Journal of Pathology and Translational Medicine2010 · 제44권 제5호

이 간행물의 다른 논문 (4건)

C3 신염의 병리김용진pp. 1-5혈전성 미세혈관병증의 병리설미영pp. 6-12소아의 지역사회 획득 장구균 요로감염의 임상 양상임택진, 김성헌, 김혜영, 이혁, 김수영, 박수은pp. 19-24A Female Infant with Segmental Multicystic Dysplastic Kidney이준호, 김문규pp. 25-28
원문 보기