Background: Pediatric epilepsy is one of the most common neurological disorders with low mortality and high morbidity, often requiring hospitalization. Weekend admissions have been shown to be associated with worse outcomes compared with their weekday counterparts. To date, no study has assessed the impact of weekend admission on clinical and quality outcomes in the pediatric epilepsy population.
Methods: Children with epilepsy were identified from the 2000, 2003, 2006, and 2009 Kids Inpatient Database. Quality outcomes were identified using the Centers of Medicare and Medicaid Services' hospital acquired conditions International Classification of Diseases, Ninth Edition; Clinical Modification (ICD-9CM) codes. Multivariable analyses were conducted to assess the association between weekend admission and inpatient mortality and hospital acquired condition occurrence.
Results: A total of 526,765 pediatric epilepsy discharges were identified, with 80% occurring on weekdays and 20% on weekends. Overall, the hospital acquired condition rate was 3.6% (3.2% vs 5.2% for weekday versus weekend) and inpatient mortality was 1.5% (1.2% vs 1.7%). Patients admitted on the weekend had 28% higher rates of hospital acquired conditions and 21% higher inpatient mortality rates compared with their weekday counterparts. Patients seen at nonpediatric centers had 10% to 28% lower rates of mortality, but 5% to 13% higher hospital acquired condition rates than those at pediatric centers.
Conclusions: Weekend admission is significantly associated with worse clinical and quality outcomes compared with weekday admissions among pediatric epilepsy inpatients. Weekend admissions likely represent unplanned, at risk admissions, coupled with less staffing. Further study is needed to isolate clinical and systemic factors to decrease this disparity in this highly comorbid pediatric subgroup.
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http://dx.doi.org/10.1016/j.pediatrneurol.2017.05.023 | DOI Listing |
Neurol Sci
January 2025
Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, 211 Eonju-ro, Gangnam-gu, Seoul, 135-720, Korea.
This study presents the first reported case of a Korean patient with Alpha-1,3-Mannosyltransferase-Congenital Disorder of Glycosylation (ALG3-CDG), characterized by a novel maternally inherited missense mutation and a previously reported paternally inherited nonsense mutation. The patient exhibited typical ALG3-CDG manifestations, including developmental delays, epilepsy, and multisystem involvement, alongside a diagnosis of Lennox-Gastaut Syndrome (LGS). Cannabidiol therapy, combined with dietary management, led to seizure freedom for over 13 months, significant EEG improvement, and enhanced developmental outcomes.
View Article and Find Full Text PDFMol Genet Genomic Med
January 2025
Department of Pediatrics, Taihe County People's Hospital, Fuyang, Anhui, China.
Background: Developmental and epileptic encephalopathies (DEEs) are a heterogeneous group of brain disorders. Variants in the Rho-related BTB domain-containing 2 gene (RHOBTB2) can lead to DEE64, which is characterized by early-onset epilepsy, varying degrees of motor developmental delay and intellectual disability, microcephaly, and movement disorders. More than half of the variants are located at Arg483 and Arg511 within the BTB domain; however, the underlying mechanism of action of these hotspot variants remains unexplored.
View Article and Find Full Text PDFMol Genet Genomic Med
January 2025
Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Background: Cornelia de Lange syndrome (CdLS) is a multisystem genetic disorder. Although individuals with variants in the SMC1A gene are less commonly seen in CdLS, they exhibit a high incidence of epilepsy and atypical phenotypic variability.
Methods: The clinical data of a patient with non-classic CdLS and epilepsy caused by an SMC1A variant were summarized.
Front Neurol
January 2025
Department of Neurosurgery and Neurotechnology, Eberhard Karls University, Tübingen, Germany.
Objective: Epilepsy is considered as a network disorder of interacting brain regions. The propagation of local epileptic activity from the seizure onset zone (SOZ) along neuronal networks determines the semiology of seizures. However, in highly interconnected brain regions such as the insula, the association between the SOZ and semiology is blurred necessitating invasive stereoelectroencephalography (SEEG).
View Article and Find Full Text PDFJIMD Rep
January 2025
Adult and Paediatric National Metabolic Service Starship Children's Hospital, Te Toka Tumai, Te Whatu Ora Health New Zealand Tāmaki Makaurau Auckland New Zealand.
Background: Deficiency of the Glut1 transporter due to mono-allelic variants in causes hypoglycorrhachia, resulting in a neurological spectrum from neonatal epilepsy to adult-onset paroxysmal movement disorders (PMD). The brain utilises ketone bodies as an alternative energy source to glucose. Thus, early initiation of the ketogenic diet (KD) is standard care for Glut1 deficiency syndrome (Glut1DS).
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