Article Text

Download PDFPDF
Nephrotic syndrome on sickle cell disease: the impact of Hydroxyurea
  1. Margarida Cunha,
  2. Carla Simão,
  3. Anabela Ferrão and
  4. Maria João Palaré
  1. Pediatria, Hospital de Santa Maria, Lisboa, Lisboa, Portugal
  1. Correspondence to Dr Margarida Cunha; cunhapmargarida{at}


Nephropathy is a common under-recognised complication of sickle cell disease (SCD) and one of the main factors of poor prognosis in these patients. The association between nephrotic syndrome and SCD in children is rare. Strategies for sickle cell nephropathy prevention are still poorly established. Blood pressure control as well as monitoring of microalbuminuria and renal function are mandatory. The use of antiproteinuric drugs, such as anti-ACE inhibitors (ACEis) and hydroxyurea, should be considered in early stages. Here, we report a case of a female adolescent with SCD and inaugural nephrotic syndrome who, after an initial treatment failure with corticotherapy, had a remarkable recovery after treatment with hydroxyurea and ACEis.

  • sickle cell disease
  • nephrotic syndrome
  • paediatrics
  • haematology (drugs and medicines)

Statistics from


  • Contributors Conception and design: MC, CS and MJP. Acquisition of data and drafting the article: MC. Analysis and interpretation of data, contribute with intellectual content, final approval of the version published, and agreement to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved: MC, CS, AF, MJP. Critical revision: CS, AF and MJP.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.