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Brain Age Modeling and Cognitive Outcomes in Young Adults With and Without Sickle Cell Anemia.
JAMA Netw Open
January 2025
Department of Neurology, Washington University in St Louis School of Medicine, St Louis, Missouri.
Importance: Both sickle cell anemia (SCA) and socioeconomic status have been associated with altered brain structure and cognitive disability, yet precise mechanisms underlying these associations are unclear.
Objective: To determine whether brains of individuals with and without SCA appear older than chronological age and if brain age modeling using brain age gap (BAG) can estimate cognitive outcomes and mediate the association of socioeconomic status and disease with these outcomes.
Design, Setting, And Participants: In this cross-sectional study of 230 adults with and without SCA, individuals underwent brain magnetic resonance imaging (MRI) and cognitive assessment.
Introduction: CLN8-Batten disease is a rare neurodegenerative disorder characterized phenotypically by progressive deterioration of motor and cognitive abilities, visual symptoms, epileptic seizures, and premature death. Mutations in CLN8 result in characteristic Batten disease symptoms and brain-wide pathology including accumulation of lysosomal storage material, gliosis, and neurodegeneration. Recent investigations of other subtypes of Batten disease (CLN1, CLN3, CLN6) have emphasized the influence of biological sex on disease and treatment outcomes; however, little is known about sex differences in the CLN8 subtype.
View Article and Find Full Text PDFBackground: Pulmonary vein stenosis in children is associated with a poor prognosis. However, the cause and risk factors for mortality remain uncertain.
Methods: This retrospective, single-center study identified children with primary and secondary pulmonary vein stenosis through a cardiac catheterization database.
Lateralization of Neural Speech Discrimination at Birth Is a Predictor for Later Language Development.
Dev Sci
March 2025
Department of Pediatrics and Adolescent Medicine, Comprehensive Center for Pediatrics, Medical University of Vienna, Vienna, Austria.
Newborns are able to neurally discriminate between speech and nonspeech right after birth. To date it remains unknown whether this early speech discrimination and the underlying neural language network is associated with later language development. Preterm-born children are an interesting cohort to investigate this relationship, as previous studies have shown that preterm-born neonates exhibit alterations of speech processing and have a greater risk of later language deficits.
View Article and Find Full Text PDFDefining Successful Radiographic Physeal Arrest: A Comparison Between Ulnar Epiphysiodesis With and Without a Sliding Bone Autograft.
J Pediatr Orthop
January 2025
Division of Orthopaedic Surgery, Rady Children's Hospital-San Diego, San Diego, CA.
Background: Distal radius physeal injuries can result in growth arrest and progressive deformity in children. Ulnar epiphysiodesis may be used to prevent deformity in the skeletally immature child; however, predicting success may be challenging. The purpose of this study was to (1) develop a method to predict successful ulnar epiphysiodesis, and (2) determine the utility of adding a sliding bone autograft as an adjunct to achieving successful epiphysiodesis.
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