0

The full content of Annals is available to subscribers

Subscribe/Learn More  >
Articles |

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 and Author 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
Text Size: A A A

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.

Figures

Tables

References

Letters

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Comments

Submit a Comment
Submit a Comment

Summary for Patients

Clinical Slide Sets

Terms of Use

The In the Clinic® slide sets are owned and copyrighted by the American College of Physicians (ACP). All text, graphics, trademarks, and other intellectual property incorporated into the slide sets remain the sole and exclusive property of the ACP. The slide sets may be used only by the person who downloads or purchases them and only for the purpose of presenting them during not-for-profit educational activities. Users may incorporate the entire slide set or selected individual slides into their own teaching presentations but may not alter the content of the slides in any way or remove the ACP copyright notice. Users may make print copies for use as hand-outs for the audience the user is personally addressing but may not otherwise reproduce or distribute the slides by any means or media, including but not limited to sending them as e-mail attachments, posting them on Internet or Intranet sites, publishing them in meeting proceedings, or making them available for sale or distribution in any unauthorized form, without the express written permission of the ACP. Unauthorized use of the In the Clinic slide sets will constitute copyright infringement.

Toolkit

Buy Now

to gain full access to the content and tools.

Want to Subscribe?

Learn more about subscription options

Advertisement
Related Articles
Related Point of Care
Topic Collections
PubMed Articles
Forgot your password?
Enter your username and email address. We'll send you a reminder to the email address on record.
(Required)
(Required)