The glomerulopathy of sickle cell disease

Kenneth Ataga, Vimal K. Derebail, David R. Archer

Research output: Contribution to journalReview article

48 Citations (Scopus)

Abstract

Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology. While most patients with albuminuria are treated with angiotensin converting enzyme inhibitors/angiotensin receptor blockers, there are no published long-term data on the efficacy of these agents. With the improved patient survival following kidney transplantation, SCD patients with end-stage renal disease should be considered for this treatment modality. Given the high prevalence of albuminuria and its association with multiple SCD-related clinical complications, additional studies are needed to answer several clinically important questions in a bid to adequately elucidate its pathophysiology, natural history, and treatment.

Original languageEnglish (US)
Pages (from-to)907-914
Number of pages8
JournalAmerican Journal of Hematology
Volume89
Issue number9
DOIs
StatePublished - Jan 1 2014

Fingerprint

Albuminuria
Sickle Cell Anemia
Natural History
Hypertension
Angiotensin Receptor Antagonists
Reperfusion Injury
Angiotensin-Converting Enzyme Inhibitors
Kidney Transplantation
Biological Availability
Chronic Kidney Failure
Echocardiography
Nitric Oxide
Oxidative Stress
Stroke
Blood Pressure
Kidney
Survival
Therapeutics

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

The glomerulopathy of sickle cell disease. / Ataga, Kenneth; Derebail, Vimal K.; Archer, David R.

In: American Journal of Hematology, Vol. 89, No. 9, 01.01.2014, p. 907-914.

Research output: Contribution to journalReview article

Ataga, Kenneth ; Derebail, Vimal K. ; Archer, David R. / The glomerulopathy of sickle cell disease. In: American Journal of Hematology. 2014 ; Vol. 89, No. 9. pp. 907-914.
@article{689a1c5b28e9471a955a87c7752f6a9d,
title = "The glomerulopathy of sickle cell disease",
abstract = "Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68{\%} in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology. While most patients with albuminuria are treated with angiotensin converting enzyme inhibitors/angiotensin receptor blockers, there are no published long-term data on the efficacy of these agents. With the improved patient survival following kidney transplantation, SCD patients with end-stage renal disease should be considered for this treatment modality. Given the high prevalence of albuminuria and its association with multiple SCD-related clinical complications, additional studies are needed to answer several clinically important questions in a bid to adequately elucidate its pathophysiology, natural history, and treatment.",
author = "Kenneth Ataga and Derebail, {Vimal K.} and Archer, {David R.}",
year = "2014",
month = "1",
day = "1",
doi = "10.1002/ajh.23762",
language = "English (US)",
volume = "89",
pages = "907--914",
journal = "American Journal of Hematology",
issn = "0361-8609",
publisher = "Wiley-Liss Inc.",
number = "9",

}

TY - JOUR

T1 - The glomerulopathy of sickle cell disease

AU - Ataga, Kenneth

AU - Derebail, Vimal K.

AU - Archer, David R.

PY - 2014/1/1

Y1 - 2014/1/1

N2 - Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology. While most patients with albuminuria are treated with angiotensin converting enzyme inhibitors/angiotensin receptor blockers, there are no published long-term data on the efficacy of these agents. With the improved patient survival following kidney transplantation, SCD patients with end-stage renal disease should be considered for this treatment modality. Given the high prevalence of albuminuria and its association with multiple SCD-related clinical complications, additional studies are needed to answer several clinically important questions in a bid to adequately elucidate its pathophysiology, natural history, and treatment.

AB - Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology. While most patients with albuminuria are treated with angiotensin converting enzyme inhibitors/angiotensin receptor blockers, there are no published long-term data on the efficacy of these agents. With the improved patient survival following kidney transplantation, SCD patients with end-stage renal disease should be considered for this treatment modality. Given the high prevalence of albuminuria and its association with multiple SCD-related clinical complications, additional studies are needed to answer several clinically important questions in a bid to adequately elucidate its pathophysiology, natural history, and treatment.

UR - http://www.scopus.com/inward/record.url?scp=84906088536&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84906088536&partnerID=8YFLogxK

U2 - 10.1002/ajh.23762

DO - 10.1002/ajh.23762

M3 - Review article

VL - 89

SP - 907

EP - 914

JO - American Journal of Hematology

JF - American Journal of Hematology

SN - 0361-8609

IS - 9

ER -