Sickle Cell Disease Nephropathy: An Update on Risk Factors and Potential Biomarkers in Pediatric Patients

Abstract

One of the major chronic complications of sickle cell disease (SCD) is sickle cell nephropathy. The aim of this review is to discuss the pathophysiology, natural history, clinical manifestations, risk factors, biomarkers and therapeutic approaches for sickle cell nephropathy, focusing on studies with pediatric patients. The earliest manifestation of renal disease is an increase in the glomerular filtration rate. A finding that may also be observed in early childhood is microalbuminuria. Nephrin, KIM-1, VGFs, chemokines and renin-angiotensin system molecules have emerged as potential early markers of renal dysfunction in SCD. In regards to a therapeutic approach, renin-angiotensin system inhibitors and angiotensin receptor blockers seem to be effective for the control of albuminuria in adults with SCD, although new studies in children are needed. The precise moment to begin renoprotection in SCD patients who should be treated remains to be determined.

Keywords:

• biomarkers
• chronic kidney disease
• hyperfiltration
• microalbuminuria
• proteinuria
• risk factor
• sickle cell anemia
• sickle cell disease
• sickle cell nephropathy

Acknowledgements

The authors thank all subjects with sickle cell disease and parents for their cooperation in our studies.

Financial & competing interests disclosure

The authors thank the financial support of Fundação de Amparo a Pesquisa do Estado de Minas Gerais (FAPEMIG - grant number APQ-00131-17, grant number: BIP-00005-18 and grant number: APQ-04261-17), Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq – grant number: PQ-301037/2016-7) and Pro-Reitoria de Pesquisa (PRPq-UFMG) to pay for English revision. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

English language support was provided by American Journal Experts and was funded by Pro-Reitoria de Pesquisa (PRPq-UFMG).

Additional information

Funding

The authors thank the financial support of Fundaööo de Amparo a Pesquisa do Estado de Minas Gerais (FAPEMIG - grant number APQ-00131-17, grant number: BIP-00005-18 and grant number: APQ-04261-17), Conselho Nacional de Desenvolvimento Cientöfico e Tecnolögico (CNPq ö grant number: PQ-301037/2016-7) and Pro-Reitoria de Pesquisa (PRPq-UFMG) to pay for English revision. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. English language support was provided by American Journal Experts and was funded by Pro-Reitoria de Pesquisa (PRPq-UFMG).