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| http://dx.doi.org/10.6061/clinics/2016(04)01 | DOI Listing |
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Glycerol-3-Phosphate Dehydrogenase 1 Deficiency: A Rare Cause of Fatty Liver Disease in Children.
Indian Pediatr
January 2025
Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Prenatal diagnosis of mucopolysaccharidosis type I on hepatosplenomegaly and coarse features: a case-report.
BMC Pregnancy Childbirth
January 2025
Department of Clinical Genetics, Rennes University Hospital, Rennes, France.
Background: Mucopolysaccharidosis type I (MPS I - IDUA gene) is a rare autosomal recessive lysosomal storage disorder. Clinical symptoms, including visceral overload, are progressive and typically begin postnatally. Descriptions of hepatosplenomegaly associated with lysosomal pathology are uncommon during the prenatal period.
View Article and Find Full Text PDFIndocyanine Green Fluorescence-Guided Surgery in Pediatric Hepatobiliary Procedures: A Feasibility Study for Improved Intraoperative Visualization.
Eur J Pediatr Surg
January 2025
Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, JODHPUR, India.
Introduction Indocyanine Green (ICG) fluorescence guided surgery (FGS) is reported extensively in adult operations, but its safety and applications in Pediatric populations remain to be comprehensively understood. The dose, administration protocols and intraoperative imaging benefits in Pediatric hepatobiliary operations are not clear. Objectives To identify the feasibility and applications of ICG Fluorescence Guided Surgery (FGS) in hepatobiliary surgeries (for biliary atresia, choledochal cyst, and cholelithiasis) in children.
View Article and Find Full Text PDFHigh-alcohol-producing Klebsiella pneumoniae aggravates lung injury by affecting neutrophils and the airway epithelium.
Cell Rep Med
December 2024
Capital Institute of Pediatrics, Beijing 100020, China. Electronic address:
We have previously reported that high-alcohol-producing Klebsiella pneumoniae (HiAlc Kpn) in the gut can cause endo-alcoholic fatty liver disease. Here, we discover that 91.2% of Kpn isolates from pulmonary disease samples also produce excess ethanol, which may be associated with respiratory disease severity.
View Article and Find Full Text PDFGenetic factors shaping the plasma lipidome and the relations to cardiometabolic risk in children and adolescents.
EBioMedicine
January 2025
Novo Nordisk Foundation Center for Basic Metabolic Research, Faculty of Health and Medical Sciences, University of Copenhagen, Denmark. Electronic address:
Background: Lipid species are emerging as biomarkers for cardiometabolic risk in both adults and children. The genetic regulation of lipid species and their impact on cardiometabolic risk during early life remain unexplored.
Methods: Using mass spectrometry-based lipidomics, we measured 227 plasma lipid species in 1149 children and adolescents (44.
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