Titlebook: Alagille Syndrome; Pathogenesis and Cli Binita M. Kamath,Kathleen M. Loomes Book 2018 Springer International Publishing AG, part of Springe

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,The Renal Sequelae of Alagille Syndrome as a?Product of Altered Notch Signaling During Kidney Develthway plays a role in the evolution of chronic renal injury. How that response to renal injury is altered in ALGS and its impact on the clinical sequelae in ALGS is not known. Given the frequency of renal findings in ALGS, kidney specialists should form part of the multidisciplinary care team for these patients.

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,Immune Dysregulation in Alagille Syndrome: A Feature of the?Evolving Phenotype,ALGS having had recurrent infections have been described with an immune profile compatible with a persistent pro-inflammatory immune state related to failure of the Jagged1-Notch-CD46 system. Studies to elucidate the prevalence, cohort clinical significance, and optimal management have still to be undertaken.

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Assimilation of Operational Databecoming accepted that all patients undergo screening imaging with computed tomography scan or magnetic resonance angiography. This is particularly important in those undergoing liver transplantation or major surgery.

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,Bile Duct Development and the?Notch Signaling Pathway,re complete insight into the complexities of intrahepatic bile duct development and the signaling pathways involved (Notch and others) is a requisite preamble to defining cellular and molecular therapies with the potential of attenuating, or reversing, biliary injury in Alagille syndrome and other cholangiopathies.

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Book 2018eatment options..Written by experts in the field,?.Alagille Syndrome: Pathogenesis and Clinical Management.?is a valuable resource for physicians and researchers dealing with this disorder, one that will help guide patient management and stimulate investigative efforts.??.