The aim of this study was to investigate the selection of plasma exchange (PE) parameters and the safety of children with severe ricinism. The PE parameters and heparin dosage in 7 children with severe ricinism were recorded, and changes in the patients' vital signs and coagulation function were monitored before and after PE. All patients successfully completed PE. The speed of blood flow was 50-80 mL/min, speed of exchange flow was 600-800 mL/h, and isolating rate of blood plasma was 12.5-19.05%. Transmembrane pressure was stable at approximately 100 mmHg, and venous pressure was stable at approximately 95 mmHg. The first dose of heparin was 0.39 ± 0.04 mg/kg, and the maintaining heparin dose was 0.40 ± 0.05 to 0.22 ± 0.03 mg·kg(-1)·h(-1). During the PE process, mean arterial pressure, heart rate, respiratory rate, and pulse oxygen saturation were steady. After PE, the activated partial thromboplastin time and thrombin time prolonged to 2-3 times greater than that before PE. However, no bleeding tendency was seen. For children with severe ricinism, the choice of PE to eliminate the toxin from blood, tissues, and organs was safe and effective.
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http://dx.doi.org/10.4238/2015.April.27.32 | DOI Listing |
Mol Genet Genomic Med
February 2025
Department of Pediatric Neurology, Hospital Universitario Quirónsalud, Madrid, Spain.
Background: Biallelic pathogenic variants in the FUCA1 gene are associated with fucosidosis. This report describes a 4-year-old boy presenting with psychomotor regression, spasticity, and dystonic postures.
Methods And Results: Trio-based whole exome sequencing revealed two previously unreported loss-of-function variants in the FUCA1 gene.
Pediatr Blood Cancer
January 2025
Lifespan Cancer Institute and Legorreta Cancer Center at Brown University, Providence, Rhode Island, USA.
Purpose: To explore the potential relationship between social media (SoMe) and burnout or overall wellbeing within the field of oncology.
Design: A cross-sectional study of adult and pediatric oncology professionals conducted using an anonymous electronic survey. The survey was disseminated through the Children's Oncology Group (COG) and the SWOG Cancer Research Network (SWOG) member listservs.
Ital J Pediatr
January 2025
Department of developmental and behavior pediatrics, Children's Medical Center, The First Hospital of Jilin University, Jilin University, Changchun, China.
Background: Recent studies have emphasized the association between prolonged screen exposure and neurodevelopmental disorders, though its correlation with tic disorders (TDs) remains ambiguous. We thus conducted this study to investigate the association between screen time (ST) and the severity of tic symptoms in children diagnosed with TDs.
Methods: We conducted a retrospective case-control study with 342 cases of TDs and 270 controls, collecting data from March 2021 to December 2023.
BMC Med
January 2025
Department of Gynaecology and Obstetrics, Women and Children's Hospital of Chongqing Medical University (Chongqing Health Center for Women and Children), Chongqing, China.
Background: Prospective trial evidence is lacking regarding the application of enhanced recovery after surgery (ERAS) in transvaginal pelvic floor reconstruction surgery among older patients. Our study aimed to investigate whether implementing the ERAS protocol could enhance post-operative recovery in this patient population.
Methods: Older patients undergoing elective transvaginal pelvic floor reconstruction surgery were randomly assigned to either the ERAS group or the conventional group.
Mol Psychiatry
January 2025
Telethon Institute of Genetics and Medicine, Via Campi Flegrei 34, Pozzuoli, 80078, Naples, Italy.
Lysosomal storage disorders characterized by defective heparan sulfate (HS) degradation, such as Mucopolysaccharidosis type IIIA-D (MPS-IIIA-D), result in neurodegeneration and dementia in children. However, dementia is preceded by severe autistic-like behaviours (ALBs), presenting as hyperactivity, stereotypies, social interaction deficits, and sleep disturbances. The absence of experimental studies on ALBs' mechanisms in MPS-III has led clinicians to adopt symptomatic treatments, such as antipsychotics, which are used for non-genetic neuropsychiatric disorders.
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