Alagille syndrome

E Mitchell, M Gilbert, KM Loomes - Clinics in liver disease, 2018 - liver.theclinics.com
Alagille syndrome (ALGS) is an autosomal dominant, multisystem disorder with variable
phenotypic penetrance that was first described in 1969 by Daniel Alagille. Initial diagnosis …

Liver biopsy in children: position paper of the ESPGHAN Hepatology Committee

A Dezsofi, U Baumann, A Dhawan… - Journal of pediatric …, 2015 - journals.lww.com
Liver biopsy (LB) is still the criterion standard procedure for obtaining liver tissue for
histopathological examination and a valuable tool in the diagnosis, prognosis, and …

Systematic review: the epidemiology, natural history, and burden of Alagille syndrome

BM Kamath, A Baker, R Houwen… - Journal of Pediatric …, 2018 - journals.lww.com
Methods: Electronic databases and proceedings from key congresses were searched in
accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses …

[HTML][HTML] Alagille syndrome: a focused review on clinical features, genetics, and treatment

TJ Kohut, MA Gilbert, KM Loomes - Seminars in Liver Disease, 2021 - thieme-connect.com
Alagille syndrome (ALGS) is an autosomal dominant disorder caused by pathogenic
variants in JAG1 or NOTCH2, which encode fundamental components of the Notch signaling …

Alagille syndrome: clinical perspectives

M Saleh, BM Kamath, D Chitayat - The Application of Clinical …, 2016 - Taylor & Francis
Alagille syndrome is an autosomal dominant, complex multisystem disorder characterized
by the presence of three out of five major clinical criteria: cholestasis with bile duct paucity …

Alagille syndrome: diagnostic challenges and advances in management

MD Ayoub, BM Kamath - Diagnostics, 2020 - mdpi.com
Alagille syndrome (ALGS) is a multisystem disease characterized by cholestasis and bile
duct paucity on liver biopsy in addition to variable involvement of the heart, eyes, skeleton …

[PDF][PDF] Outcomes of childhood cholestasis in Alagille syndrome: results of a multicenter observational study

BM Kamath, W Ye, NP Goodrich… - Hepatology …, 2020 - Wiley Online Library
Alagille syndrome (ALGS) is an autosomal dominant multisystem disorder with cholestasis
as a defining clinical feature. We sought to characterize hepatic outcomes in a molecularly …

Regenerative failure of intrahepatic biliary cells in Alagille syndrome rescued by elevated Jagged/Notch/Sox9 signaling

C Zhao, J Matalonga, JJ Lancman… - Proceedings of the …, 2022 - National Acad Sciences
Despite the robust healing capacity of the liver, regenerative failure underlies numerous
hepatic diseases, including the JAG1 haploinsufficient disorder, Alagille syndrome (ALGS) …

[PDF][PDF] Outcomes of liver transplantation for patients with Alagille syndrome: the studies of pediatric liver transplantation experience

BM Kamath, W Yin, H Miller, R Anand… - Liver …, 2012 - Wiley Online Library
Alagille syndrome (ALGS) is a multisystem disorder that manifests as childhood cholestasis.
Reports of liver transplantation (LT) for patients with ALGS have come largely from single …

Alagille syndrome: current understanding of pathogenesis, and challenges in diagnosis and management

MD Ayoub, BM Kamath - Clinics in Liver Disease, 2022 - liver.theclinics.com
Alagille syndrome (ALGS) is the most common inherited cholestatic liver disease
encountered by pediatric hepatologists. Unlike most other causes of cholestasis, the …