Abstract

BackgroundSickle cell disease (SCD) is an autosomal recessive disease resulting in hemolytic anemia and recurrent vaso-occlusive events. Consequently, it can result in a broad range of functional and structural renal and cardiac alterations. Chronic kidney disease (CKD), in SCD, is associated with proteinuria, microalbuminuria, and hemoglobinuria. Cardiac complications in SCD include pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, and sudden death. In patients with advancing age, cardio-renal dysfunction can have substantial effects on morbidity and mortality. Our primary aim was to compare the incidence of major adverse cardiac events (MACE) and all-cause mortality in sickle cell nephropathy (SCN).MethodsIn this retrospective study, we used International Classification of Diseases (ICD)-10 codes to identify admissions in 2019 with a diagnosis of MACE with a prior diagnosis of SCD and/or SCN. Our search of the HCA Healthcare Enterprise Data Warehouse for adult patients >18 years yielded 6,693 patients with SCD, of which 658 patients (9.8%) had SCN. Primary endpoints were incidence of MACE and all-cause mortality. Patients with MACE encompassed those with nonfatal stroke, nonfatal myocardial infarction, and congestive heart failure (CHF) exacerbations. A secondary endpoint was length of stay (LOS). Logistic regression analysis was used for MACE and all-cause mortality. LOS was analyzed using multiple linear regression analysis. Results were considered statistically significant for analyses showing p <0.05. All outcomes were adjusted for demographic variables and comorbidities.ResultsLogistic regression, after adjustment for comorbidities, demonstrated that SCN patients had significantly higher odds of all-cause mortality (odds ratio [OR] 2.343, p = 0.035, 95% confidence interval [CI] 1.063-5.166) compared to patients without SCN. Compared to those without SCN, those with SCN did not have a higher odds of MACE (OR 1.281, p = 0.265, 95% CI 0.828-1.982). Linear regression for LOS did not reveal a significant association with SCN (p = 0.169, 95% CI 0.157-0.899).ConclusionBased on the analysis of 6,693 patients with SCD, SCN was associated with significantly higher odds of all-cause mortality. SCN was not associated with significantly higher odds of MACE or prolonged LOS.

Highlights

  • Sickle cell disease (SCD) is a hematologic disorder caused by a single base-pair mutation in the gene for the beta-globin chain of adult hemoglobin (HbS) [1]

  • Compared to those without sickle cell nephropathy (SCN), those with SCN did not have a higher odds of major adverse cardiac events (MACE)

  • Linear regression for length of stay (LOS) did not reveal a significant association with SCN (p = 0.169, 95% CI 0.157-0.899)

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Summary

Introduction

Sickle cell disease (SCD) is a hematologic disorder caused by a single base-pair mutation in the gene for the beta-globin chain of adult hemoglobin (HbS) [1]. Left ventricular diastolic dysfunction, elevated serum levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP), hemoglobinuria, proteinuria, macro-albuminuria, chronic kidney disease (CKD), and coronary disease are all “biomarkers” that have been shown to help identify adult patients with SCD who are at a higher risk of morbidity and death [5]. How to cite this article Udani K, Parisio-Poldiak N, Campbell J, et al (May 16, 2021) All-Cause Mortality and Incidence of Major Adverse Cardiac Events in Sickle Cell Nephropathy: A Comparative Study. Sickle cell disease (SCD) is an autosomal recessive disease resulting in hemolytic anemia and recurrent vaso-occlusive events. It can result in a broad range of functional and structural renal and cardiac alterations. Our primary aim was to compare the incidence of major adverse cardiac events (MACE) and all-cause mortality in sickle cell nephropathy (SCN)

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