Alagille Syndrome Pathogenesis and Clinical Management /

• Corporate Author: SpringerLink (Online service)
• Other Authors: Kamath, Binita M. (Editor, http://id.loc.gov/vocabulary/relators/edt), Loomes, Kathleen M. (Editor, http://id.loc.gov/vocabulary/relators/edt)
• Format: Electronic eBook
• Language: English
• Published: Cham : Springer International Publishing : Imprint: Springer, 2018.
• Edition: 1st ed. 2018.
• Subjects: Gastroenterology .; Hepatology.; Cardiology.; Gastroenterology.

This text provides a concise yet comprehensive overview of Alagille syndrome. The book reviews the pathophysiology and genetics of the disorder, discusses recent molecular advances and its impact on diagnostics, and describes management challenges and strategies. The text also touches upon future tr...

Table of Contents:

• The Alagille Syndrome: Overview and Introduction
• Bile Duct Development and the Notch Signaling Pathway
• Genetics of Alagille Syndrome
• Liver Disease in Alagille Syndrome
• Transplant Considerations in Alagille Syndrome
• Cardiac, Aortic, and Pulmonary Vascular Involvement in Alagille Syndrome
• Vascular Manifestations in Alagille Syndrome
• The Renal Sequelae of Alagille Syndrome as a Product of Altered Notch Signaling During Kidney Development
• Skeletal Involvement in Alagille Syndrome
• Immune Dysregulation in Alagille Syndrome: A Feature of the Evolving Phenotype
• Health-Related Quality of Life and Neurocognition in Alagille Syndrome
• Future Therapeutic Approaches for Alagille Syndrome.