Prevalence and Pathologic Features of Sickle Cell Nephropathy and Response to Inhibition of Angiotensin-Converting Enzyme

Falk, Ronald J.; Scheinman, Jon I.; Phillips, Grady; Orringer, Eugene P.; Johnson, Anthony; Jennette, J. Charles

doi:10.1056/nejm199204023261402

Cited by 271 publications

(242 citation statements)

References 27 publications

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“…The use of angiotensin-converting enzyme (ACE) inhibiting medications has been shown to reduce levels of proteinuria in small-scale trials and may prove to be beneficial in delaying the onset of CKD. 23 In view of the seriousness of this complication, large-scale randomized controlled trials of therapies to halt progression of SCN are urgently required.…”

Section: Tg Day Et Almentioning

confidence: 99%

Association between hemolysis and albuminuria in adults with sickle cell anemia

Day

Drašar²,

Fulford³

et al. 2011

Haematologica

104

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We have investigated renal function and markers of hemolysis in a cohort of 424 adult African-British patients with sickle cell disease. While significant associations were found in HbSS and HbSb 0 (sickle cell anemia) patients with and without controlling for covariates between hemolytic markers and albuminuria, the associations were not significant in patients with HbSC. Estimated glomerular filtration rate, a marker of renal function, correlated significantly with reticulocyte count and bilirubin. Alpha thalassemia, present in 34% of the sickle cell anaemia patients, had a protective effect against albuminuria in this group. Altogether, the incidence of hyperfiltration was 71% and microalbuminuria 37%, making nephropathy a common complication of sickle cell anemia. Haematologica 2012;97(2):201-205. doi:10.3324/haematol.2011 This is an open-access paper. ABSTRACTexcluded from the study group if they had a history of diabetes mellitus, HIV infection, malignancy or another cause of chronic kidney disease (CKD) or if they were on a blood transfusion program. Seventeen patients were on ACE (angiotensin converting enzyme) inhibitor or ARB (angiotensin II receptor blocker) therapy; these patients were also excluded from the study. Since 2006, urinary albumin/creatinine ratio (ACR) has been a routine measurement for patients attending the specialist clinic. All patients were routinely genotyped for α thalassemia using a PCR-based methodology on DNA extracted from peripheral blood.17

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Section: Tg Day Et Almentioning

confidence: 99%

Association between hemolysis and albuminuria in adults with sickle cell anemia

Day

Drašar²,

Fulford³

et al. 2011

Haematologica

104

View full text Add to dashboard Cite

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“…Renal complications are a well known cause of morbidity and mortality in sickle cell disease, and the incidence of renal failure increases as overall patient survival improves [1][2][3][4][5][6][7][8][9][10]. Early elements of sickle cell renal disease include increased renal plasma flow accompanied by glomerular hyperfiltration and increased proximal tubular function, as well as hematuria.…”

Section: Introductionmentioning

confidence: 99%

Estimated glomerular filtration rate in sickle cell anemia is associated with polymorphisms of bone morphogenetic protein receptor 1B

Nolan

Cohen

et al. 2006

American J Hematol

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Renal disease is common in sickle cell anemia. In this exploratory work, we used data from a longitudinal study of the natural history of sickle cell disease to examine the hypothesis that polymorphisms (SNPs) in selected candidate genes are associated with glomerular filtration rate (GFR). DNA samples and clinical and laboratory data were available for 1,140 patients with sickle cell anemia. GFR was estimated using the Cockcroft-Gault and Schwartz formulas for adults and children, respectively. We examined *175 haplotype tagging (ht) SNPs in about 70 genes of the TGFb/BMP pathway for their association with GFR using linear regression. Four SNPs in BMPR1B, a bone morphogenetic protein (BMP) receptor gene, yielded statistically significant associations (P values ranging from 0.015 to 0.046). Three haplotypes in this gene were also associated with GFR. The TGF-b/BMP pathway has been associated with the development of diabetic nephropathy, which has some features in common with sickle cell nephropathy. Our results suggest that, as with other subphenotypes of sickle cell disease, renal function may be genetically modulated. Am. J. Hematol. 82:179-184, 2007. V V C 2006 Wiley-Liss, Inc.

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“…Overt sickle cell disease (HbSS) is associated with lesions of glomerular hypertension, and angiotensin converting enzyme inhibitors improve proteinuria in these patients. 21 Similar pathology may occur in the HbAS state and identifying these patients would allow aggressive monitoring for albuminuria and early intervention with antiproteinuric agents.…”

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confidence: 99%