Hyperfiltration during early childhood precedes albuminuria in pediatric sickle cell nephropathy

Am J Hematol. 2019 Apr;94(4):417-423. doi: 10.1002/ajh.25390. Epub 2019 Jan 8.

Abstract

Background: In patients with diabetes mellitus, hyperfiltration precedes the development of albuminuria. Pediatric sickle cell anemia (SCA) patients have a high prevalence of hyperfiltration and albuminuria during early childhood and adolescence. We tested the hypothesis that hyperfiltration precedes the development of albuminuria in a longitudinal pediatric SCA cohort.

Methods: We identified 91 participants with HbSS or SB0 thalassemia 5-21 years of age enrolled in a longitudinal sickle cell nephropathy cohort study who had a cystatin C measured during early childhood (4-10 years of age). Early hyperfiltration was defined as a mean eGFR >180 mL/min/1.73m2 using cystatin C obtained from 4 to 10 years of age. Persistent albuminuria was defined as an albumin to creatinine ratio > 30 mg/g on two of three untimed urine specimens. Time to event analysis estimated survival curves for participants with and without hyperfiltration using Kaplan-Meier curves and used logrank test for categorical variables to assess the association with time to development of the first episode persistent albuminuria.

Results: Persistent albuminuria occurred more often and at an earlier age in participants with early hyperfiltration compared to those without early hyperfiltration (log-rank, P = .004). Participants who developed albuminuria have a significant increase in their eGFR during childhood (P = .003) as compared to participants who have not yet progressed to albuminuria (P = .26). For every 1 g/dL increase in hemoglobin, the hazard ratio for developing persistent proteinuria decreased by 0.56 (95% CI: 0.3, 1.06, P = .07).

Conclusion: Hyperfiltration precedes the development of persistent proteinuria in pediatric SCA patients. Intervention strategies should target lowering eGFR during early childhood.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Albuminuria* / etiology
  • Albuminuria* / metabolism
  • Albuminuria* / mortality
  • Albuminuria* / physiopathology
  • Anemia, Sickle Cell* / complications
  • Anemia, Sickle Cell* / metabolism
  • Anemia, Sickle Cell* / mortality
  • Anemia, Sickle Cell* / physiopathology
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Glomerular Filtration Rate*
  • Humans
  • Kidney Diseases* / etiology
  • Kidney Diseases* / metabolism
  • Kidney Diseases* / mortality
  • Kidney Diseases* / physiopathology
  • Male
  • Prospective Studies
  • Survival Rate