Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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A Patient with Henoch-Schönlein Purpura with Intussusception and intractable Nephritis

  • Seo, Min Kyoung (Department of Pediatrics, Ajou University School of Medicine) ;
  • Hong, Jeong (Department of Pediatric Surgery, Ajou University School of Medicine) ;
  • Yim, Hyun Ee (Department of Pathology, Ajou University School of Medicine) ;
  • Pai, Ki Soo (Department of Pediatrics, Ajou University School of Medicine)
  • Received : 2016.09.15
  • Accepted : 2016.10.19
  • Published : 2016.10.30
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Abstract

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is the most common vasculitis in children, mainly affecting the small vessels of the skin, joints, gastrointestinal tract, and kidneys. Although most cases of HSP resolve spontaneously without sequelae, serious nephrological and intestinal problems may occur in some cases. We experienced a case of HSP complicated by simultaneous intussusception and nephritis in a 14-year-old boy who developed a sudden abdominal pain and gross hematuria on the 11th day after onset of the disease. Imaging studies revealed intussusception that required emergency laparotomy. Despite treatment with steroid and angiotensin-converting enzyme inhibitors, nephritis and nephrosis progressed for 4 weeks, and renal biopsy was performed to confirm the diagnosis. Cyclosporin A therapy was started, and remission of proteinuria was achieved after 5 months. However, the nephritis recurred and worsened to end-stage renal failure during 15 years of follow-up.

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References

  1. Tizard E, Hamilton-Ayres M. Henoch-Schonlein purpura. Archives of disease in childhood-Education & practice edition 2008;93:1-8. https://doi.org/10.1136/adc.2004.066035
  2. Lai HC. Henoch-Schonlein purpura with intussusception: a case report. Pediatrics and neonatology 2010;51:65-7. https://doi.org/10.1016/S1875-9572(10)60013-7
  3. Rai A, Nast C, Adler S. Henoch-Schonlein purpura nephritis. Journal of the American Society of Nephrology 1999;10:2637-44.
  4. Lee K-Y. A common immunopathogenesis mechanism for infectious diseases: the protein-homeostasis-system hypothesis. Infection & chemotherapy 2015;47:12-26. https://doi.org/10.3947/ic.2015.47.1.12
  5. Chang WL, Yang YH, Lin YT, Chiang BL. Gastrointestinal manifestations in Henoch-Schonlein purpura: a review of 261 patients. Acta paediatrica 2004;93:1427-31. https://doi.org/10.1111/j.1651-2227.2004.tb02623.x
  6. Ebert EC. Gastrointestinal manifestations of Henoch-Schonlein purpura. Digestive diseases and sciences 2008;53:2011-9. https://doi.org/10.1007/s10620-007-0147-0
  7. Saulsbury FT. Henoch-Schonlein Purpura in Children: Report of 100 Patients and Review of the Literature. Medicine 1999;78:395-409. https://doi.org/10.1097/00005792-199911000-00005
  8. Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schonlein purpura. Journal of paediatrics and child health 1998;34:405-9. https://doi.org/10.1046/j.1440-1754.1998.00263.x
  9. Lippl F, Huber W, Werner M, Nekarda H, Berger H, Weigert N. Life-threatening gastrointestinal bleeding due to a jejunal lesion of Henoch-Schonlein purpura. Endoscopy 2001;33:811-3. https://doi.org/10.1055/s-2001-16529
  10. Sohagia AB, Gunturu SG, Tong TR, Hertan HI. Henoch-Schonlein purpura-a case report and review of the literature. Gastroenterology research and practice 2010;2010.
  11. Almeida JLJd, Campos LMA, Paim LB, Leone C, Koch VHK, Silva CAA. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of initial prognostic factors. Jornal de pediatria 2007;83:259-66.
  12. Goldstein AR, White RH, Akuse R, Chantler C. Long-term followup of childhood Henoch-Schonlein nephritis. The Lancet 1992;339:280-2. https://doi.org/10.1016/0140-6736(92)91341-5
  13. Zaffanello M, Brugnara M, Franchini M. Therapy for children with henoch-schonlein purpura nephritis: a systematic review. The Scientific World Journal 2007;7:20-30. https://doi.org/10.1100/tsw.2007.23

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  2. A Case of Henoch-Schönlein Purpura with Fulminant Complications and Its Long-term Outcome vol.23, pp.2, 2019, https://doi.org/10.3339/jkspn.2019.23.2.128