Sickle cell disease: Renal manifestations and mechanisms

Karl A Nath, Robert P. Hebbel

Research output: Contribution to journalArticle

104 Citations (Scopus)

Abstract

Sickle cell disease (SCD) substantially alters renal structure and function, and causes various renal syndromes and diseases. Such diverse renal outcomes reflect the uniquely complex vascular pathobiology of SCD and the propensity of red blood cells to sickle in the renal medulla because of its hypoxic, acidotic, and hyperosmolar conditions. Renal complications and involvement in sickle cell nephropathy (SCN) include altered haemodynamics, hypertrophy, assorted glomerulopathies, chronic kidney disease, acute kidney injury, impaired urinary concentrating ability, distal nephron dysfunction, haematuria, and increased risks of urinary tract infections and renal medullary carcinoma. SCN largely reflects an underlying vasculopathy characterized by cortical hyperperfusion, medullary hypoperfusion, and an increased, stress-induced vasoconstrictive response. Renal involvement is usually more severe in homozygous disease (sickle cell anaemia, HbSS) than in compound heterozygous types of SCD (for example HbSC and HbSβ + -thalassaemia), and is typically mild, albeit prevalent, in the heterozygous state (sickle cell trait, HbAS). Renal involvement contributes substantially to the diminished life expectancy of patients with SCD, accounting for 16-18% of mortality. As improved clinical care promotes survival into adulthood, SCN imposes a growing burden on both individual health and health system costs. This Review addresses the renal manifestations of SCD and focuses on their underlying mechanisms.

Original languageEnglish (US)
Pages (from-to)161-171
Number of pages11
JournalNature Reviews Nephrology
Volume11
Issue number3
DOIs
StatePublished - Mar 27 2015

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Sickle Cell Anemia
Kidney
Sickle Cell Trait
Medullary Carcinoma
Thalassemia
Nephrons
Hematuria
Life Expectancy
Chronic Renal Insufficiency
Acute Kidney Injury
Urinary Tract Infections
Health Care Costs
Hypertrophy
Blood Vessels
Erythrocytes
Hemodynamics
Survival
Mortality
Health

ASJC Scopus subject areas

  • Nephrology

Cite this

Sickle cell disease : Renal manifestations and mechanisms. / Nath, Karl A; Hebbel, Robert P.

In: Nature Reviews Nephrology, Vol. 11, No. 3, 27.03.2015, p. 161-171.

Research output: Contribution to journalArticle

Nath, Karl A ; Hebbel, Robert P. / Sickle cell disease : Renal manifestations and mechanisms. In: Nature Reviews Nephrology. 2015 ; Vol. 11, No. 3. pp. 161-171.
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