title: Clinicopathological features and prognosis of membranoproliferative-like Henoch-Schönlein purpura nephritis in children.
abstract: BACKGROUND
  The aim of this retrospective study was to define the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI.
  
  
  METHODS
  Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males = 5, females = 4, age: 9.5 ± 2.03 years, mean ± SD). The clinical features, laboratory and pathological findings, treatment and outcome of the 9 patients were retrospectively analyzed.
  
  
  RESULTS
  Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (> 50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients.
  
  
  CONCLUSIONS
  The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.
authors.affiliation: , Zhengzhou, China. huangyanjie69@hotmail.com.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China. huangyanjie69@hotmail.com.
authors.forename: Yan-Jie
authors.initials: YJ
authors.lastname: Huang
authors.affiliation: , Zhengzhou, China.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
authors.forename: Xiao-Qing
authors.initials: XQ
authors.lastname: Yang
authors.affiliation: , Zhengzhou, China.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
authors.forename: Wen-Sheng
authors.initials: WS
authors.lastname: Zhai
authors.affiliation: , Zhengzhou, China.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
authors.forename: Xian-Qing
authors.initials: XQ
authors.lastname: Ren
authors.affiliation: , Zhengzhou, China.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
authors.forename: Qing-Yin
authors.initials: QY
authors.lastname: Guo
authors.affiliation: , Zhengzhou, China.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
authors.forename: Xia
authors.initials: X
authors.lastname: Zhang
authors.affiliation: , Zhengzhou, China.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
authors.forename: Meng
authors.initials: M
authors.lastname: Yang
authors.affiliation: , Zhengzhou, China.|Second Department of Medicine, Numazu City Hospital, Numazu, Japan.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Yamamoto
authors.affiliation: , Zhengzhou, China.|Department of Anesthesia, Stanford University School of Medicine, California, USA.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Sun
authors.affiliation: , Zhengzhou, China.|Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Ding
mesh_terms: D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006027:Glycosides / Q000627:therapeutic use; D006417:Hematuria / Q000209:etiology; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications* / Q000188:drug therapy* / Q000473:pathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D009393:Nephritis / Q000209:etiology; D009404:Nephrotic Syndrome / Q000209:etiology; D011379:Prognosis; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome; D029757:Tripterygium
chemical_list: D015415:Biomarkers; D006027:Glycosides; D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide
keywords: Henoch-Schönlein purpura nephritis; clinicopathological features; prognosis