Nephrotic syndrome on sickle cell disease: the impact of Hydroxyurea. Issue 3 (5th March 2021)

• Record Type: Journal Article
• Authors: Cunha, Margarida; Simão, Carla; Ferrão, Anabela; Palaré, Maria João
• Is Part Of: BMJ case reports. Volume 14:Issue 3(2021)
• Publication Date: 2021-03-05
• Subjects: sickle cell disease -- nephrotic syndrome -- paediatrics -- haematology (drugs and medicines); Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2020-237545
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : 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.