Background: The prevalence of obstructive sleep apnea syndrome (OSAS) is higher in children with sickle cell disease (SCD) as compared with the general pediatric population. It has been speculated that overgrowth of the adenoid and tonsils is an important contributor.
Methods: The current study used MRI to evaluate such an association. We studied 36 subjects with SCD (aged 6.9 ± 4.3 years) and 36 control subjects (aged 6.6 ± 3.4 years).
Results: Compared with control subjects, children with SCD had a significantly smaller upper airway (2.8 ± 1.2 cm(3) vs 3.7 ± 1.6 cm(3), P < .01), and significantly larger adenoid (8.4 ± 4.1 cm(3) vs 6.0 ± 2.2 cm(3), P < .01), tonsils (7.0 ± 4.3 cm(3) vs 5.1 ± 1.9 cm(3), P < .01), retropharyngeal nodes (3.0 ± 1.9 cm(3) vs 2.2 ± 0.9 cm(3), P < .05), and deep cervical nodes (15.7 ± 5.7 cm(3) vs 12.7 ± 4.0 cm(3), P < .05). Polysomnography showed that 19.4% (seven of 36) of children with SCD had OSAS compared with 0% (zero of 20) of control subjects (P < .05) and that in children with SCD the apnea-hypopnea index correlated positively with upper airway lymphoid tissues size (r = 0.57, P < 001). In addition, children with SCD had lower arterial oxygen saturation nadir (84.3% ± 12.3% vs 91.2% ± 4.2%, P < .05), increased peak end-tidal CO(2) (53.4 ± 8.5 mm Hg vs 42.3 ± 5.3 mm Hg, P < .001), and increased arousals (13.7 ± 4.7 events/h vs 10.8 ± 3.8 events/h, P < .05).
Conclusions: Children with SCD have reduced upper airway size due to overgrowth of the surrounding lymphoid tissues, which may explain their predisposition to OSAS.
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http://dx.doi.org/10.1378/chest.11-2013 | 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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