LEADER 03068nam 22005175 450 001 9910300262603321 005 20200630101907.0 010 $a3-319-94571-8 024 7 $a10.1007/978-3-319-94571-2 035 $a(CKB)4100000006674610 035 $a(MiAaPQ)EBC5528140 035 $a(DE-He213)978-3-319-94571-2 035 $a(PPN)230542042 035 $a(EXLCZ)994100000006674610 100 $a20180926d2018 u| 0 101 0 $aeng 135 $aurcnu|||||||| 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 10$aAlagille Syndrome $ePathogenesis and Clinical Management /$fedited by Binita M. Kamath, Kathleen M. Loomes 205 $a1st ed. 2018. 210 1$aCham :$cSpringer International Publishing :$cImprint: Springer,$d2018. 215 $a1 online resource (205 pages) 311 $a3-319-94570-X 327 $aThe 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. 330 $aThis 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 treatment 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. . 606 $aGastroenterology  606 $aHepatology 606 $aCardiology 606 $aGastroenterology$3https://scigraph.springernature.com/ontologies/product-market-codes/H33061 606 $aHepatology$3https://scigraph.springernature.com/ontologies/product-market-codes/H33088 606 $aCardiology$3https://scigraph.springernature.com/ontologies/product-market-codes/H33037 615 0$aGastroenterology . 615 0$aHepatology. 615 0$aCardiology. 615 14$aGastroenterology. 615 24$aHepatology. 615 24$aCardiology. 676 $a616.042 702 $aKamath$b Binita M$4edt$4http://id.loc.gov/vocabulary/relators/edt 702 $aLoomes$b Kathleen M$4edt$4http://id.loc.gov/vocabulary/relators/edt 906 $aBOOK 912 $a9910300262603321 996 $aAlagille Syndrome$91742375 997 $aUNINA