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Glycerol-3-Phosphate Dehydrogenase 1 Deficiency and Steatotic Liver Disease in Children: Our Cases and Review of Literature.
J Clin Exp Hepatol
December 2024
Departments of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Introduction: Glycerol-3-phosphate dehydrogenase 1 (GPD1) deficiency is an autosomal recessive disorder causing hypertriglyceridemia, hepatomegaly, fatty liver, and hepatic fibrosis in infancy. It is an under-recognized cause of pediatric steatotic liver disease (SLD) with only 36 cases reported worldwide.
Method: We analyzed the clinical profile of our five cases diagnosed by exome sequencing (ES) and reviewed the published cases till December 2023 using PubMed search.
Hepatic and Pancreatic Cellular Response to Early Life Nutritional Mismatch.
Endocrinology
January 2025
Department of Pediatrics, Divisions of Neonatology & Developmental Biology and Endocrinology, Neonatal Research Center of the UCLA Children's Discovery & Innovation Institute at the David Geffen School of Medicine at UCLA, Los Angeles, California 90095-1752.
To determine the basis for perinatal nutritional mismatch causing metabolic dysfunction associated steatotic liver disease (MASLD) and diabetes mellitus, we examined adult phenotype, hepatic transcriptome, and pancreatic β-islet function. In prenatal caloric restricted rat with intrauterine growth restriction (IUGR) and postnatal exposure to high fat with fructose (HFhf) or high carbohydrate (RC), we investigated male and female IUGR-Hfhf and IUGR-RC, versus HFhf and CON offspring. Males more than females displayed adiposity, glucose intolerance, insulin resistance, hyperlipidemia, hepatomegaly with hepatic steatosis.
View Article and Find Full Text PDFGuava polysaccharides attenuate high fat and STZ-induced hyperglycemia by regulating gut microbiota and arachidonic acid metabolism.
Int J Biol Macromol
September 2024
Guangdong Provincial Key Lab of Food Safety and Quality, South China Agricultural University, Guangzhou, Guangdong 510642, PR China; College of Food Science, South China Agricultural University, Guangzhou 510642, PR China. Electronic address:
This study investigated the hypoglycemic mechanism of guava polysaccharides (GP) through the gut microbiota (GM) and related metabolites. Our findings demonstrated that GP significantly mitigated high-fat diet- and streptozotocin-induced hyperglycemia, insulin resistance, hyperlipidemia, elevated alanine aminotransferase, high hepatic inflammation levels, and prevented pancreatic atrophy and hepatomegaly. Interestingly, the benefits of GP were attributed to alterations in the GM.
View Article and Find Full Text PDFAetiological Spectrum of Pancreaticobiliary Diseases in Adult Nigerians on Magnetic Resonance Cholangiopancreatography.
Niger J Clin Pract
May 2024
Department of Radiology, Oshakati Intermediate Hospital, Oshakati, Oshana Region, Namibia.
Background: Imaging is vital for assessing pancreaticobiliary diseases.
Aim: The aim of the study was to investigate the spectrum and pattern of pancreaticobiliary diseases in adult Nigerians using magnetic resonance cholangiopancreatography (MRCP).
Methods: This was a retrospective, descriptive cross-sectional study.
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