title: Glomerular hyperfiltration in adult sickle cell anemia: a frequent hemolysis associated feature.
abstract: OBJECTIVE
  Sickle cell anemia-associated nephropathy is a growing matter of concern because renal failure affects most aging sickle cell anemia patients. Glomerular damage is a common feature revealed by a microalbuminuria or a macroalbuminuria. Although glomerular hyperfiltration has been described for decades in this population, its prevalence in young adults is unknown.
  
  
  METHODS
  To address this issue, as well as the clinical and biologic correlates of hyperfiltration, a single-center, cross-sectional study of 280 homozygous SS disease patients was performed.
  
  
  RESULTS
  The prevalence of hyperfiltration assessed by Modification of Diet in Renal Disease estimated GFR was 51%. Among patients with hyperfiltration, 49% had hyperfiltration alone, whereas 36% and 15% had an associated microalbuminuria or macroalbuminuria, respectively. Estimated GFR sensitivity and specificity for hyperfiltration were 94% and 63%, respectively, in a selected subgroup of 48 patients (measured GFR was assessed by urinary (51)Cr EDTA clearance). In patients with no albuminuria, hyperfiltration status was significantly associated with a young age (years), the absence of alpha thalassemia, a lower hemoglobin level (g/dl), and a lower fetal hemoglobin. The role of chronic hemolysis was further strengthened by multivariate analysis showing a correlation between estimated GFR and a low plasma fetal hemoglobin level, a young age, and a high reticulocyte count (r(2) = 0.54).
  
  
  CONCLUSIONS
  Together, the data suggest that the pathophysiology of hyperfiltration would rather be attributable to the hemolysis-associated vasculopathy rather than a viscosity-vaso-occlusive process.
authors.affiliation: Université Pierre et Marie Curie, Université Paris 6, Institut National de la Santé et de la Recherche Médicale Unit 702, Physiology, Paris, France. jean-philippe.haymann@tnn.aphp.fr
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Haymann
authors.forename: Katia
authors.initials: K
authors.lastname: Stankovic
authors.forename: Pierre
authors.initials: P
authors.lastname: Levy
authors.forename: Virginie
authors.initials: V
authors.lastname: Avellino
authors.forename: Pierre-Louis
authors.initials: PL
authors.lastname: Tharaux
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Letavernier
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
authors.forename: Laurent
authors.initials: L
authors.lastname: Baud
authors.forename: Robert
authors.initials: R
authors.lastname: Girot
authors.forename: François
authors.initials: F
authors.lastname: Lionnet
mesh_terms: D000293:Adolescent; D000328:Adult; D000419:Albuminuria / Q000097:blood / Q000453:epidemiology* / Q000503:physiopathology; D000755:Anemia, Sickle Cell / Q000097:blood / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D003430:Cross-Sectional Studies; D005260:Female; D005602:France / Q000453:epidemiology; D005919:Glomerular Filtration Rate; D006451:Hemoglobin, Sickle / Q000235:genetics; D006461:Hemolysis; D006720:Homozygote; D006801:Humans; D007668:Kidney / Q000503:physiopathology*; D016014:Linear Models; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D051437:Renal Insufficiency / Q000097:blood / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D018570:Risk Assessment; D012307:Risk Factors; D055815:Young Adult
chemical_list: D006451:Hemoglobin, Sickle