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Kidney Disease among Patients with Sickle Cell Disease, Hemoglobin SS and SC.

Abstract

BACKGROUND AND OBJECTIVES

Sickle cell disease (SCD) is an inherited anemia that afflicts millions worldwide. Kidney disease is a major contributor to its morbidity and mortality. We examined contemporary and historical SCD populations to understand how renal disease behaved in hemoglobin SS (HbSS) compared with HbSC.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS

Kidney function was examined in the multicentered Treatment of Pulmonary Hypertension and Sickle Cell Disease with Sildenafil Therapy (Walk-PHaSST) Trial (HbSS=463; HbSC=127; years 2007-2009) and historical comparator populations from the Cooperative Study of Sickle Cell Disease (CSSCD; HbSS=708) and the Multicenter Study of Hydroxyurea in Sickle Cell Disease (MSH; HbSS=299).

RESULTS

In adults with SCD, eGFR was lower among older individuals: -1.78 ml/min per 1.73 m(2) per year of age (95% confidence interval [95% CI], -2.06 to -1.50; Walk-PHaSST Trial), -1.75 ml/min per 1.73 m(2) per year of age (95% CI, -2.05 to -1.44; MSH), and -1.69 ml/min per 1.73 m(2) per year of age (95% CI, -2.00 to -1.38; CSSCD) in HbSS compared with -1.09 ml/min per 1.73 m(2) per year of age (95% CI, -1.39 to -0.75) in HbSC (Walk-PHaSST Trial). Macroalbuminuria was seen in 20% of participants with SCD (HbSS or HbSC; P=0.45; Walk-PHaSST Trial), but microalbuminuria was more prevalent in HbSS (44% versus 23% in HbSC; P<0.002). In the Walk-PHaSST Trial, albuminuria was associated with hemolysis (higher lactate dehydrogenase, P<0.001; higher absolute reticulocyte count, P<0.02; and lower Hb, P=0.07) and elevated systolic BP (P<0.001) in HbSS. One half of all participants with HbSS (20 of 39) versus one fifth without (41 of 228) elevated tricuspid regurgitant jet velocity (≥3 m/s; adverse prognostic indicator in SCD) had macroalbuminuria (P<0.001). In the CSSCD, overt proteinuria, detected (less sensitively) by urine dipstick, associated with higher 3-year mortality (odds ratio, 2.48; 95% CI, 1.07 to 5.77). Serum bicarbonate was lower in HbSS (23.8 versus 24.8 mEq/dl in HbSC; P<0.05) and associated with reticulocytopenic anemia and decreased renal function.

CONCLUSIONS

In SCD, albuminuria or proteinuria was highly prevalent, in HbSS more than in HbSC. Proteinuria associated with mortality in HbSS. Older individuals had a lower than expected eGFR, and this was more prominent in HbSS. Current management does not routinely address renal complications in SCD, which could plausibly reduce morbidity and mortality.

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  • Authors+Show Affiliations

    ,

    Division of Renal Diseases and Hypertension, University of Minnesota, Minneapolis, Minnesota;

    ,

    Divisions of Hematology and Oncology, and.

    ,

    Center for Sickle Cell Disease, Howard University, Washington, DC;

    ,

    Division of Hematology/Oncology, University of Illinois, Chicago, Illinois; and.

    ,

    Comprehensive Sickle Cell Center.

    ,

    Nephrology and Hypertension,University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, Ohio;

    ,

    Vascular Medicine Institute and Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania.

    Divisions of Hematology and Oncology, and Jane.Little@uhhospitals.org.

    Source

    MeSH

    Acid-Base Equilibrium
    Adult
    Albuminuria
    Anemia, Sickle Cell
    Biomarkers
    Clinical Trials as Topic
    Female
    Glomerular Filtration Rate
    Hemoglobin C
    Hemoglobin SC Disease
    Hemoglobin, Sickle
    Historically Controlled Study
    Humans
    Kidney
    Kidney Diseases
    Male
    Middle Aged
    Multicenter Studies as Topic
    North America
    Observational Studies as Topic
    Phenotype
    Prevalence
    Risk Factors
    United Kingdom

    Pub Type(s)

    Comparative Study
    Journal Article
    Research Support, N.I.H., Extramural
    Research Support, Non-U.S. Gov't

    Language

    eng

    PubMed ID

    26672090

    Citation

    Drawz, Paul, et al. "Kidney Disease Among Patients With Sickle Cell Disease, Hemoglobin SS and SC." Clinical Journal of the American Society of Nephrology : CJASN, vol. 11, no. 2, 2016, pp. 207-15.
    Drawz P, Ayyappan S, Nouraie M, et al. Kidney Disease among Patients with Sickle Cell Disease, Hemoglobin SS and SC. Clin J Am Soc Nephrol. 2016;11(2):207-15.
    Drawz, P., Ayyappan, S., Nouraie, M., Saraf, S., Gordeuk, V., Hostetter, T., ... Little, J. (2016). Kidney Disease among Patients with Sickle Cell Disease, Hemoglobin SS and SC. Clinical Journal of the American Society of Nephrology : CJASN, 11(2), pp. 207-15. doi:10.2215/CJN.03940415.
    Drawz P, et al. Kidney Disease Among Patients With Sickle Cell Disease, Hemoglobin SS and SC. Clin J Am Soc Nephrol. 2016 Feb 5;11(2):207-15. PubMed PMID: 26672090.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Kidney Disease among Patients with Sickle Cell Disease, Hemoglobin SS and SC. AU - Drawz,Paul, AU - Ayyappan,Sabarish, AU - Nouraie,Mehdi, AU - Saraf,Santosh, AU - Gordeuk,Victor, AU - Hostetter,Thomas, AU - Gladwin,Mark T, AU - Little,Jane, Y1 - 2015/12/15/ PY - 2015/04/09/received PY - 2015/10/30/accepted PY - 2015/12/17/entrez PY - 2015/12/17/pubmed PY - 2017/1/11/medline KW - acidosis KW - albuminuria KW - anemia, sickle cell KW - blood pressure KW - chronic kidney disease KW - glomerular filtration rate KW - humans KW - hydroxyurea KW - hypertension, pulmonary SP - 207 EP - 15 JF - Clinical journal of the American Society of Nephrology : CJASN JO - Clin J Am Soc Nephrol VL - 11 IS - 2 N2 - BACKGROUND AND OBJECTIVES: Sickle cell disease (SCD) is an inherited anemia that afflicts millions worldwide. Kidney disease is a major contributor to its morbidity and mortality. We examined contemporary and historical SCD populations to understand how renal disease behaved in hemoglobin SS (HbSS) compared with HbSC. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Kidney function was examined in the multicentered Treatment of Pulmonary Hypertension and Sickle Cell Disease with Sildenafil Therapy (Walk-PHaSST) Trial (HbSS=463; HbSC=127; years 2007-2009) and historical comparator populations from the Cooperative Study of Sickle Cell Disease (CSSCD; HbSS=708) and the Multicenter Study of Hydroxyurea in Sickle Cell Disease (MSH; HbSS=299). RESULTS: In adults with SCD, eGFR was lower among older individuals: -1.78 ml/min per 1.73 m(2) per year of age (95% confidence interval [95% CI], -2.06 to -1.50; Walk-PHaSST Trial), -1.75 ml/min per 1.73 m(2) per year of age (95% CI, -2.05 to -1.44; MSH), and -1.69 ml/min per 1.73 m(2) per year of age (95% CI, -2.00 to -1.38; CSSCD) in HbSS compared with -1.09 ml/min per 1.73 m(2) per year of age (95% CI, -1.39 to -0.75) in HbSC (Walk-PHaSST Trial). Macroalbuminuria was seen in 20% of participants with SCD (HbSS or HbSC; P=0.45; Walk-PHaSST Trial), but microalbuminuria was more prevalent in HbSS (44% versus 23% in HbSC; P<0.002). In the Walk-PHaSST Trial, albuminuria was associated with hemolysis (higher lactate dehydrogenase, P<0.001; higher absolute reticulocyte count, P<0.02; and lower Hb, P=0.07) and elevated systolic BP (P<0.001) in HbSS. One half of all participants with HbSS (20 of 39) versus one fifth without (41 of 228) elevated tricuspid regurgitant jet velocity (≥3 m/s; adverse prognostic indicator in SCD) had macroalbuminuria (P<0.001). In the CSSCD, overt proteinuria, detected (less sensitively) by urine dipstick, associated with higher 3-year mortality (odds ratio, 2.48; 95% CI, 1.07 to 5.77). Serum bicarbonate was lower in HbSS (23.8 versus 24.8 mEq/dl in HbSC; P<0.05) and associated with reticulocytopenic anemia and decreased renal function. CONCLUSIONS: In SCD, albuminuria or proteinuria was highly prevalent, in HbSS more than in HbSC. Proteinuria associated with mortality in HbSS. Older individuals had a lower than expected eGFR, and this was more prominent in HbSS. Current management does not routinely address renal complications in SCD, which could plausibly reduce morbidity and mortality. SN - 1555-905X UR - https://www.unboundmedicine.com/medline/citation/26672090/Kidney_Disease_among_Patients_with_Sickle_Cell_Disease_Hemoglobin_SS_and_SC_ L2 - http://cjasn.asnjournals.org/cgi/pmidlookup?view=long&amp;pmid=26672090 DB - PRIME DP - Unbound Medicine ER -