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Pediatric Nephrology

, Volume 21, Issue 6, pp 877–879 | Cite as

Renal amyloidosis in a child with sickle cell anemia

  • Behçet ŞimşekEmail author
  • Aysun K. Bayazit
  • Melek Ergin
  • Mustafa Soran
  • Hasan Dursun
  • Yurdanur Kilinc
Brief Report

Abstract

The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood.

Keywords

Sickle cell anemia Amyloidosis Nephrotic syndrome 

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Copyright information

© IPNA 2006

Authors and Affiliations

  • Behçet Şimşek
    • 1
    Email author
  • Aysun K. Bayazit
    • 1
  • Melek Ergin
    • 2
  • Mustafa Soran
    • 1
  • Hasan Dursun
    • 1
  • Yurdanur Kilinc
    • 3
  1. 1.Department of Pediatric NephrologyCukurova UniversityAdanaTurkey
  2. 2.Department of PathologyCukurova University, School of MedicineAdana,Turkey
  3. 3.Department of Pediatric HematologyCukurova University, School of MedicineAdana,Turkey

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