Hepatology, EarlyView., 2022 Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study. Abstract Background and Aims Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real‐world data are lacking.
Shannon M. Vandriel +93 more
wiley +1 more source
Interleukin‐18 signaling promotes activation of hepatic stellate cells in mouse liver fibrosis
Hepatology, EarlyView., 2022 Interleukin‐18 signaling promotes activation of hepatic stellate cells in mouse liver fibrosis. Abstract Background and Aims Nucleotide‐binding oligomerization domain‐like receptor‐family pyrin domain‐containing 3 (NLRP3) inflammasome activation has been shown to result in liver fibrosis.
Jana Knorr +19 more
wiley +1 more source
Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy
Hepatology, EarlyView., 2022 Serum bile acid levels predict outcomes in patients with biliary atresia who achieve normalized bilirubin levels after Kasai portoenterostomy. Abstract Background and Aims In biliary atresia, serum bilirubin is commonly used to predict outcomes after Kasai portoenterostomy (KP).
Sanjiv Harpavat +22 more
wiley +1 more source
Dysmotility in Esophageal Atresia: Pathophysiology, Characterization, and Treatment
Frontiers in Pediatrics, 2017 Esophageal dysmotility is almost universal after esophageal atresia (EA) repair and is mainly related to the developmental anomaly of the esophagus.
Christophe Faure +1 more
doaj +1 more source
Journal of Pediatric Surgery Case Reports, 2021 We present a rare case of simultaneous esophageal and tracheal atresia with trachea-esophageal fistula in a premature infant with VACTERL association. Although multiple surgical interventions were attempted in the management of this patient, the patient ...
Lauren S. McTaggart +7 more
doaj +1 more source
Live transplantation in children with biliary atresia and vascular anomalies [PDF]
, 1974 Eight of 29 infants and children undergoing orthotopic liver transplantation for extrahepatic biliary atresia had associated major vascular anomalies.
Lilly, JR, Starzl, TE
core +1 more source
Dysphagia in children with esophageal atresia: current diagnostic options [PDF]
, 2015 © 2017 Georg Thieme Verlag KGDysphagia or swallowing disorder is very common (range, 15–52%) in patients with esophageal atresia. Children present with a wide range of symptoms.
Allegaert, K +3 more
core +1 more source
Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence:two case reports and a review of the literature [PDF]
, 2016 Background: The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions.
Bjørsum-Meyer, T. +3 more
core +1 more source
Case report: Upper neck pouch sign in the antenatal diagnosis of esophageal atresia
Indian Journal of Radiology and Imaging, 2009 Prenatal diagnosis of esophageal atresia remains a challenge for the imaging consultant. On antenatal USG, the finding of an absent or small stomach in the setting of polyhydramnios used to be considered suspicious of esophageal atresia.
Mukesh Kumar Garg
doaj +1 more source
Atrioventricular canal defect and genetic syndromes: the unifying role of sonic hedgehog [PDF]
, 2019 The atrioventricular canal defect (AVCD) is a congenital heart defect (CHD) frequently associated with extracardiac anomalies (75%). Previous observations from a personal series of patients with AVCD and "polydactyly syndromes" showed that the distinct ...
Baban, A +9 more
core +1 more source