Assessment of the Kidney Size in the African American Population of Children and Adolescents With Sickle Cell Hemoglobinopathy: A Matched Pair Analysis

BACKGROUND: Sickle cell disease (SCD) is caused by a variant of the beta-globin gene that produces sickling of the red blood cells. A parallel increase in cardiac output from the ensuing hemolytic anemia leads to glomerular hyperfiltration and progressive nephromegaly. Our goal is to examine the impact of SCD morbidity on the kidney size using kidney ultrasonography. MATERIALS AND METHODS: We studied 31 African Americans with SCD with ages between 3 and 20 years, along with 57 age, sex, weight, height and body mass index-matched controls who were seen in an outpatient clinic from January 2018 to December 2022. We examined the impact of genotype variants and the morbidity score of hemoglobinopathy on kidney size. RESULTS: The comparison of the kidney size of matched SCD children with healthy controls showed a larger volume (P<0.0001). A unit increase in SCD morbidity score was associated with a 5.7 cm3 and 5.1 cm3 increase in the volume of the right and left kidneys, respectively. CONCLUSION: The impact of hyperfiltration on nephromegaly in SCD, compared with healthy controls, was suggested by a higher estimated glomerular filtration rate value among subjects with more severe hemoglobinopathy. The larger volume amongst subset with HBSS compared with those with the HBSC genotype confirmed the is observation.