Objective: The objective of this study is to retrospectively determine the co-occurrence, associated characteristics, and risk factors for neurodevelopmental disorders (NDD) in a pediatric sickle cell disease (SCD) clinic population.
Method: We investigated the co-occurrence and features of NDD in pediatric SCD through a retrospective cohort study conducted between July 2017 and January 2019. The participants were patients with SCD younger than 18 years of age identified from our institutions' clinic rosters and medical records databases.
Results: A total of 276 participants were eligible for study inclusion, and 65 participants were found to have various NDD. Children with SCD and NDD were more likely to have a history of multiple SCD-related complications in comparison to children with SCD without NDD. Children with SCD and NDD were more likely to use disease-modifying therapies in comparison to children with SCD without NDD (χ2 27.2, p < 0.001).
Conclusion: Children with SCD and NDD have higher odds of having certain disease-related complications and higher use of disease-modifying treatments than children with SCD who do not have NDD. Screening and diagnoses of NDD may be relevant to clinical management of pediatric SCD.
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http://dx.doi.org/10.1097/DBP.0000000000000914 | DOI Listing |
Pediatr Blood Cancer
January 2025
Division of Pediatric Hematology & Oncology, University Hospitals Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA.
Background: The clinical manifestations of sickle cell disease (SCD) result in significant morbidity and healthcare costs. The effect of weather as a risk factor for the development of vaso-occlusive episodes (VOEs) has been previously studied, although with variable results. The aim of our retrospective, nationwide study was to determine the association between weather patterns and pediatric VOE and acute chest syndrome (ACS).
View Article and Find Full Text PDFCase Rep Genet
December 2024
Cardiovascular Research Department, Lankenau Institute for Medical Research, Lankenau Hearth Institute, Wynnewood, Pennsylvania 19096, USA.
Structural or electrophysiologic cardiac anomalies may compromise cardiac function, leading to sudden cardiac death (SCD). Genetic screening of families with severe cardiomyopathies underlines the role of genetic variations in cardiac-specific genes. The present study details the clinical and genetic characterization of a malignant dilated cardiomyopathy (DCM) case in a 1-year-old Mexican child who presented a severe left ventricular dilation and dysfunction that led to SCD.
View Article and Find Full Text PDFSpec Care Dentist
January 2025
Cerebral Palsy Alliance Research Institute, Specialty of Child & Adolescent Health, Sydney Medical School, Faculty of Medicine & Health, The University of Sydney, Sydney, Australia.
Aims: To investigate caregiver-reported dental care experiences and oral health-related quality of life (OHRQoL) of children and young people with cerebral palsy (CP).
Methods: Between May and August 2023, caregivers of children and young people from three Australian states were invited to complete questionnaires, including the Child Oral Health Impact Profile (COHIP-SF 19).
Results: Sixty-eight caregivers participated in the survey.
Indian Pediatr
January 2025
Department of Pediatrics, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India. Correspondence to: Dr Anil Kumar Goel, Department of Pediatrics, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India.
Objectives: To compare the efficacy and safety of intravenous (IV) ketorolac and IV morphine in the management of severe vaso-occlusive crisis (VOC) in children with sickle cell disease (SCD).
Method: An open-label, randomized controlled trial was conducted from January 2021 to July 2022 wherein children with SCD aged 3 to 15 years, presenting with severe VOC (score > 6 on the Wong-Baker Faces Pain scale) were included. Block randomization with minimization was done and participants received either IV ketorolac (intervention) or IV morphine infusion (standard).
Front Mol Biosci
December 2024
Department of Microbiology, Biochemistry and Immunology, Morehouse School of Medicine, Atlanta, GA, United States.
Introduction: Sickle cell disease (SCD) is a genetic blood disorder caused by a mutation in the HBB gene, which encodes the beta-globin subunit of hemoglobin. This mutation leads to the production of abnormal hemoglobin S (HbS), causing red blood cells to deform into a sickle shape. These deformed cells can block blood flow, leading to complications like chronic hemolysis, anemia, severe pain episodes, and organ damage.
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