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Introduction: Despite being a rare cause of end-stage kidney disease (ESKD), sickle cell disease has broad kidney implications. Patients with both sickle cell disease and ESKD are at a greater risk of adverse events including mortality; while these patients can be managed with hemodialysis (HD), the use of nocturnal HD in sickle cell disease has not been previously described.

Methods: Here, we report a patient with sickle cell disease and ESKD who transitioned from conventional HD to nocturnal home HD.

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Background: In this planned ancillary analysis of our completed clinical trial, we hypothesized that among older children with sickle cell anemia (SCA) and severe acute malnutrition, those with higher levels of food insecurity would have lower end-of-trial body mass index (BMI) z-scores compared to their peers with SCA and lower levels of food insecurity.

Procedure: Data from 108 children who completed the feasibility trial for managing severe acute malnutrition in older children with SCA in Nigeria were analyzed. Children aged 5-12 years old with severe acute malnutrition (BMI z-score of <-3.

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While asthma is a known risk factor for Acute Chest Syndrome (ACS) and may increase overall mortality in SCD patients, this study specifically focuses on the rate of inpatient mortality, hospital stay, and costs in SCD patients who develop ACS. Our study was conducted using a retrospective cohort from the National Inpatient Sample (NIS), spanning 5 years from 2016 to 2020. Patients were carefully divided into two cohorts for comparison: those admitted with ACS and a history of asthma and those with ACS without a history of asthma.

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Screening and Diagnosis of Type 2 Diabetes in Sickle Cell Disease.

J Am Board Fam Med

February 2025

From the Department of Internal Medicine, University of Alabama Birmingham School of Medicine, Birmingham, AL (BC); Division of Academic General Pediatrics, University of Alabama Birmingham School of Medicine, Birmingham, AL (RK); Division of Endocrinology, Diabetes, and Metabolism, University of Alabama Birmingham School of Medicine, Birmingham, AL (FO); Division of Hematology and Oncology, University of Alabama Birmingham School of Medicine, Birmingham, AL (RK, JK).

Background: Diagnosing type 2 diabetes mellitus (T2DM) in people with sickle cell disease (SCD) is complicated due to hemoglobin A variance in affected individuals. Accurate screening assessments are needed to prevent excessive morbidity and mortality. Primary care physicians need recommendations for screening and diagnosis in this unique population.

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Background: The objective of our case-control study was to evaluate the determinants of childhood cardio-vagal baroreflex failure and exaggerated orthostatic pressor response, which are risk factors for subsequent hypertension.

Methods: Four groups of children were matched for sex and age: 12 with congenital central hypoventilation syndrome (autonomic nervous system dysfunction), 12 with chronic kidney disease (frequently abnormal blood pressure [BP]), 12 with sickle cell disease (frequently abnormal orthostatic BP), and 24 control children (preterm birth with normal BP). The children underwent tonometry evaluation (aortic systolic BP) and continuous BP and ECG measurements in supine and standing positions, allowing ambulatory BP monitoring and the computation of heart rate variability indices, baroreflex sensitivity (BRS), and orthostatic systolic BP response.

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