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Kidney Disease among Patients with Sickle Cell Disease, Hemoglobin SS and SC
Journal article   Open access   Peer reviewed

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

Paul Drawz, Sabarish Ayyappan, Mehdi Nouraie, Santosh Saraf, Victor Gordeuk, Thomas Hostetter, Mark T Gladwin and Jane Little
Clinical journal of the American Society of Nephrology, Vol.11(2), pp.207-215
02/05/2016
DOI: 10.2215/CJN.03940415
PMCID: PMC4741034
PMID: 26672090
url
https://doi.org/10.2215/CJN.03940415View
Published (Version of record) Open Access

Abstract

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

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