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Pooled prevalence and subgroup variations of Tetralogy of Fallot among children and adolescents with congenital heart defect in Sub-Saharan Africa: A systematic review and meta-analysis.
PLoS One
January 2025
Department of Anatomy, School of Medicine, Addis Ababa University, Addis Ababa, Ethiopia.
Background: Tetralogy of Fallot is one of the critical congenital heart defects needing intervention within the first year of life.
Objective: This review aims to systematically assess the prevalence of Tetralogy of Fallot among children and adolescents with congenital heart defects in Sub-Saharan Africa from January 2000 to January 2024.
Methods: All original observational studies focused on children and adolescent population diagnosed with congenital heart defects within Sub-Saharan Africa; reported the primary outcome of interest were included.
Amniotic fluid stem cell extracellular vesicles as a novel fetal therapy for pulmonary hypoplasia: a review on mechanisms and translational potential.
Stem Cells Transl Med
January 2025
Developmental and Stem Cell Biology Program, Peter Gilgan Centre for Research and Learning, The Hospital for Sick Children, Toronto, ON, Canada M5G 0A4.
Disruption of developmental processes affecting the fetal lung leads to pulmonary hypoplasia. Pulmonary hypoplasia results from several conditions including congenital diaphragmatic hernia (CDH) and oligohydramnios. Both entities have high morbidity and mortality, and no effective therapy that fully restores normal lung development.
View Article and Find Full Text PDFMaralixibat Reduces Serum Bile Acids and Improves Cholestatic Pruritus in Adolescents With Alagille Syndrome.
Liver Int
February 2025
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada.
Background: Alagille syndrome (ALGS) is a multisystem cholestatic disorder. Maralixibat is approved for the treatment of cholestatic pruritus in ALGS with limited data in adults.
Methods: Participants were included if they received ≥ 2 doses of maralixibat at age ≥ 16 years in one of the three previously published maralixibat ALGS clinical trials.
Reduction of elevated Gli3 does not alter the progression of autosomal recessive polycystic kidney disease.
Physiol Rep
January 2025
Developmental Biology and Cancer Research and Teaching Department, University College London, Great Ormond Street Institute of Child Health, London, UK.
Polycystic kidney diseases (PKD) are genetic disorders which disrupt kidney architecture and function. Autosomal recessive PKD (ARPKD) is a rare form of PKD, caused by mutations in PKHD1, and clinically more severe than the more common autosomal dominant PKD (ADPKD). Prior studies have implicated Hedgehog (Hh) signaling in ADPKD, with increased levels of Hh components in experimental ADPKD and reduced cystogenesis following pharmacological Hh inhibition.
View Article and Find Full Text PDFA case of simultaneous bilateral pulmonary resection for arteriovenous pulmonary malformation.
Indian J Thorac Cardiovasc Surg
February 2025
Sechenov First Moscow State Medical University, Moscow, Russia.
Pulmonary arteriovenous malformation (PAVM) is a congenital vascular pathology, which is caused by the presence of a direct connection between the branches of the artery and the veins of the lungs, and the discharge of unoxygenated blood into the arterial bed. Arteriovenous malformations are characterized by a wide variety of clinical manifestations and, in some cases, may be accompanied with severe circulatory disorders.
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