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Chronic Renal Failure in Sickle Cell Disease: Risk Factors, Clinical Course, and Mortality

Darleen R. Powars, MD; Donna D. Elliott-Mills, MD; Linda Chan, PhD; Joyce Niland, PhD; Alan L. Hiti, MD, PhD; Lawrence M. Opas, MD; and Cage Johnson, MD
[+] Article, Author, and Disclosure Information

Grant Support: In part by the National Heart, Lung, and Blood Institute grant #HL 15162 and by the Sickle Cell Disease Research Foundation of Southern California.

Requests for Reprints: Darleen Powars, MD, University of Southern California School of Medicine, Pediatric Pavilion, 1129 North State Street, Room 2E19, Los Angeles, CA 90033.

Current Author Addresses: Drs. Powars, Elliott-Mills, Chan, Niland, Hiti, Opas, and Johnson: Los Angeles County and University of Southern California School of Medicine, 1129 North State Street, Los Angeles, CA 90033.

© 1991 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1991;115(8):614-620. doi:10.7326/0003-4819-115-8-614
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Objective: To determine the incidence, clinical course, and risk factors associated with the onset of chronic renal failure in sickle cell anemia and sickle C disease.

Design: A prospective, 25-year longitudinal demographic and clinical cohort study. A matched case-control study was conducted to determine risk factors.

Patients: A total of 725 patients with sickle cell anemia and 209 patients with sickle C disease who received medical care from the hematology service in a large municipal hospital. Most were observed from birth or early childhood.

Measurements: Thirty-six patients developed sickle renal failure: 4.2% of patients with sickle cell anemia and 2.4% of patients with sickle C disease. The median age of disease onset for these patients was 23.1 and 49.9 years, respectively. Survival time for patients with sickle cell anemia after the diagnosis of sickle renal failure, despite dialysis, was 4 years, and the median age at the time of death was 27 years. Relative risk for mortality was 1.42 (95% Cl, 1.12 to 1.81; P = 0.02) compared with patients who did not develop renal insufficiency.

Main Results: Histopathologic studies showed characteristic lesions of glomerular "drop out" and glomerulosclerosis. Case-control analysis showed that ineffective erythropoiesis with increasingly severe anemia, hypertension, proteinuria, the nephrotic syndrome, and microscopic hematuria were significant pre-azotemic predictors of chronic renal failure. The risk for sickle renal failure was increased in patients who had inherited the Central African Republic βs-gene cluster haplotype.

Conclusions: The pre-azotemic manifestations of hypertension, proteinuria, and increasingly severe anemia predict end-stage renal failure in patients with sickle cell disease. The rate of progression of renal insufficiency is genetically determined. Treatment of the uremic phase has been dismal, underscoring the need for the development of useful pre-azotemic therapeutic modalities.





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