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Gastrointestinal complications of hepatic glycogen storage disease: a national survey questionnaire study in China.
Orphanet J Rare Dis
January 2025
Department of Pediatrics, Guangdong Provincial People's Hospital, The Second School of Clinical Medicine, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, 510080, China.
Background: Hepatic glycogen storage diseases (GSD) are inborn errors of metabolism with abnormal storage or utilization of glycogen, a complex disease with significant genetic heterogeneity and similar clinical manifestations. This study aimed to describe the gastrointestinal symptoms and endoscopic features of hepatic GSD, including types Ia, Ib, III, VI, and IX, to provide evidence for etiology and treatment.
Methods: A national cohort survey questionnaire was distributed to patients diagnosed with GSD type Ia, Ib, III, VI, and IX through genetic testing or their parents in mainland China in May 2022.
Neuromuscular monitoring and incidence of postoperative residual neuromuscular blockade: a prospective observational study.
J Anesth Analg Crit Care
January 2025
Department of Anesthesia and Intensive Care, Agostino Gemelli IRCCS University Polyclinic Foundation, Rome, Italy.
Background: Neuromuscular blocking agents (NMBAs) are routinely used in anesthesia practice. An undetected, incomplete recovery of neuromuscular function at the end of surgery potentially exposes patients to clinical deterioration in the postoperative period. The aim of this study was to investigate the incidence of postoperative residual neuromuscular blockade (RNMB) in a cohort of patients receiving NMBAs.
View Article and Find Full Text PDFEffect of early administration of fibrinogen replacement therapy in traumatic haemorrhage: a systematic review and meta-analysis of randomised controlled trials with narrative synthesis of observational studies.
Crit Care
January 2025
Centre for Trauma Sciences, Blizard Institute, Queen Mary University of London, London, England.
Background: In severely injured trauma patients, hypofibrinoginaemia is associated with increased mortality. There is no evidence-based consensus for what constitutes optimal fibrinogen therapy, treatment dose or timing of administration. The aim of this systematic review was to evaluate the effects of early fibrinogen replacement, either cryoprecipitate or fibrinogen concentrate (FgC) on mortality, transfusion requirements and deep venous thrombosis (DVT).
View Article and Find Full Text PDFWhite noise's effect on premature infants' physiological parameters during peripheral intravenous catheter insertion.
BMC Pediatr
January 2025
Faculty of Nursing, Yasouj University of Medical Sciences, Kohkiloyeh and Boyer-Ahmad, Yasuj, Iran.
Background: Early and continuous exposure to painful stimuli in premature infants leads to short-and long-term complications. Listening to white noise is an accessible and inexpensive non-invasive method that can be used as a safe nursing intervention in hospitals. This study aimed to assess white noise's effect on premature Infants' physiological parameters during peripheral intravenous catheter insertion.
View Article and Find Full Text PDFObjective: This study aims to investigate the relationship between preoperative cervical intervertebral foramen width and area and the persistence of postoperative pain in patients diagnosed with cervical spondylotic radiculopathy (CSR).
Methods: Patients were divided into two groups, based on their pain relief at the 6-month postoperative follow-up: the pain relief group and the persistent pain group. We compared various parameters, including age, sex, body mass index (BMI), duration of symptoms, preoperative Japanese Orthopedic Association (JOA) score, Neck Disability Index (NDI) score, postoperative ratio of disc space distraction, preoperative width of the intervertebral foramen (WIVF), and area of the intervertebral foramen (AIVF) between the two groups.
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