skip to content
Alagille Syndrome : Pathogenesis and Clinical Management Preview this item
ClosePreview this item
Checking...

Alagille Syndrome : Pathogenesis and Clinical Management

Author: Binita M Kamath; Kathleen M Loomes
Publisher: Cham, Switzerland : Springer, [2018]
Edition/Format:   eBook : Document : EnglishView all editions and formats
Summary:
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 treatment options. Written by experts in the field, Alagille Syndrome: Pathogenesis and Clinical Management is a valuable  Read more...
Rating:

(not yet rated) 0 with reviews - Be the first.

Subjects
More like this

Find a copy online

Find a copy in the library

&AllPage.SpinnerRetrieving; Finding libraries that hold this item...

Details

Genre/Form: Electronic books
Additional Physical Format: Print version:
Alagille Syndrome.
Cham, Switzerland : Springer, [2018]
(OCoLC)1037809506
Material Type: Document, Internet resource
Document Type: Internet Resource, Computer File
All Authors / Contributors: Binita M Kamath; Kathleen M Loomes
ISBN: 9783319945712 3319945718 9783319945729 3319945726 9783030068783 3030068781
OCLC Number: 1055160511
Description: 1 online resource : illustrations
Contents: Intro; Preface; Acknowledgements; Contents; Contributors; Chapter 1: Alagille Syndrome: Overview and Introduction; Diagnosis of Alagille Syndrome and Jagged1 Mutation Status; Discovery of JAGGED1 Mutations as the Cause of ALGS: Implications for ALGS Patients; Family Considerations and Support; Summary; Suggested Reading; Chapter 2: Bile Duct Development and the Notch Signaling Pathway; Normal Intrahepatic Bile Duct Development; Cholangiocyte Specification; Cholangiocyte Morphogenesis; Refinement of Intrahepatic Bile Duct Architecture; The Notch Pathway. The Requirement of Notch during Intrahepatic Bile Duct DevelopmentNuances of Notch Signal Transduction that May Influence Intrahepatic Bile Duct Development; Threshold Sensitivity; Trans-Activation Versus Cis-Inhibition; Notch-Independent De Novo Intrahepatic Bile Duct Formation; Notch Pathway-Dependent Vascular Smooth Muscle Defects and the Role of RUMI; Key Points; References; Chapter 3: Genetics of Alagille Syndrome; JAG1 and NOTCH2; Haploinsufficiency of Notch Signaling in Disease Pathobiology; JAG1 and NOTCH2 Mutations; Clinical Testing for Alagille Syndrome. Variant Interpretation of JAG1 and NOTCH2 Missense MutationsVariable Expressivity of Alagille Syndrome; Genotype-Phenotype Correlations in Alagille Syndrome; Genetic Modifiers of Liver Disease Severity in Alagille Syndrome; Conclusions; References; Chapter 4: Liver Disease in Alagille Syndrome; Hepatic Disease in Alagille Syndrome; Clinical Features of Hepatic Disease in Alagille Syndrome; Biochemical Profile of Hepatic Disease in Alagille Syndrome; Diagnosis of Alagille Syndrome; Histopathologic Findings on Liver Biopsy in Alagille Syndrome. Imaging Studies in Patients with Hepatic Alagille SyndromeOutcomes of Hepatic Disease in Alagille Syndrome; Management of Hepatic Disease in Alagille Syndrome; Nutrition; Pruritus; Surgical Management of Cholestasis; Hypercholesterolemia Management; Portal Hypertension; Hepatocellular Carcinoma; Summary; References; Chapter 5: Transplant Considerations in Alagille Syndrome; Background/Introduction; Living-Related Liver Transplant in Alagille Syndrome; Multi-organ Transplant; Planning for Liver Transplantation; Abdominal Vascular Abnormalities; CNS Vascular Abnormalities; Cardiac Involvement. Previous Kasai OperationPrevious Biliary Diversion Operation; Post- liver Transplant Outcomes and Management; Conclusion; References; Chapter 6: Cardiac, Aortic, and Pulmonary Vascular Involvement in Alagille Syndrome; Spectrum and Frequency of Congenital Cardiovascular Disease Associated with Alagille Syndrome; Diagnostic Approach and Surveillance; Management; Outcomes; Conclusion; References; Chapter 7: Vascular Manifestations in Alagille Syndrome; Introduction; Normal Vascular Development; The Role of the Notch Signaling Pathway in Vascular Development.
Responsibility: Binita M. Kamath, Kathleen M. Loomes, editors.

Abstract:

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  Read more...

Reviews

User-contributed reviews
Retrieving GoodReads reviews...
Retrieving DOGObooks reviews...

Tags

Be the first.
Confirm this request

You may have already requested this item. Please select Ok if you would like to proceed with this request anyway.

Linked Data


Primary Entity

<http://www.worldcat.org/oclc/1055160511> # Alagille Syndrome : Pathogenesis and Clinical Management
    a schema:Book, schema:CreativeWork, schema:MediaObject ;
    library:oclcnum "1055160511" ;
    library:placeOfPublication <http://id.loc.gov/vocabulary/countries/sz> ;
    schema:about <http://experiment.worldcat.org/entity/work/data/5463536983#Topic/medical_genetics> ; # MEDICAL--Genetics
    schema:about <http://experiment.worldcat.org/entity/work/data/5463536983#Topic/liver_diseases_genetic_aspects> ; # Liver--Diseases--Genetic aspects
    schema:about <http://experiment.worldcat.org/entity/work/data/5463536983#Topic/genetic_disorders> ; # Genetic disorders
    schema:about <http://dewey.info/class/616.042/e23/> ;
    schema:about <http://experiment.worldcat.org/entity/work/data/5463536983#Topic/cardiovascular_medicine> ; # Cardiovascular medicine
    schema:about <http://experiment.worldcat.org/entity/work/data/5463536983#Topic/gastroenterology> ; # Gastroenterology
    schema:about <http://experiment.worldcat.org/entity/work/data/5463536983#Topic/hepatology> ; # Hepatology
    schema:about <http://experiment.worldcat.org/entity/work/data/5463536983#Topic/heart_diseases_genetic_aspects> ; # Heart--Diseases--Genetic aspects
    schema:bookFormat schema:EBook ;
    schema:datePublished "2018" ;
    schema:description "Intro; Preface; Acknowledgements; Contents; Contributors; Chapter 1: Alagille Syndrome: Overview and Introduction; Diagnosis of Alagille Syndrome and Jagged1 Mutation Status; Discovery of JAGGED1 Mutations as the Cause of ALGS: Implications for ALGS Patients; Family Considerations and Support; Summary; Suggested Reading; Chapter 2: Bile Duct Development and the Notch Signaling Pathway; Normal Intrahepatic Bile Duct Development; Cholangiocyte Specification; Cholangiocyte Morphogenesis; Refinement of Intrahepatic Bile Duct Architecture; The Notch Pathway."@en ;
    schema:description "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 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."@en ;
    schema:editor <http://experiment.worldcat.org/entity/work/data/5463536983#Person/kamath_binita_m> ; # Binita M. Kamath
    schema:editor <http://experiment.worldcat.org/entity/work/data/5463536983#Person/loomes_kathleen_m> ; # Kathleen M. Loomes
    schema:exampleOfWork <http://worldcat.org/entity/work/id/5463536983> ;
    schema:genre "Electronic books"@en ;
    schema:inLanguage "en" ;
    schema:isSimilarTo <http://www.worldcat.org/oclc/1037809506> ;
    schema:name "Alagille Syndrome : Pathogenesis and Clinical Management"@en ;
    schema:productID "1055160511" ;
    schema:url <http://public.eblib.com/choice/publicfullrecord.aspx?p=5528140> ;
    schema:url <http://www.vlebooks.com/vleweb/product/openreader?id=none&isbn=9783319945712> ;
    schema:url <https://search.ebscohost.com/login.aspx?direct=true&scope=site&db=nlebk&db=nlabk&AN=1905316> ;
    schema:url <https://0-link-springer-com.pugwash.lib.warwick.ac.uk/book/10.1007/978-3-319-94571-2> ;
    schema:url <https://doi.org/10.1007/978-3-319-94571-2> ;
    schema:url <https://link.springer.com/10.1007/978-3-319-94571-2> ;
    schema:url <http://link.springer.com/10.1007/978-3-319-94571-2> ;
    schema:workExample <http://worldcat.org/isbn/9783319945712> ;
    schema:workExample <http://worldcat.org/isbn/9783319945729> ;
    schema:workExample <http://dx.doi.org/10.1007/978-3-319-94571-2> ;
    schema:workExample <http://worldcat.org/isbn/9783030068783> ;
    wdrs:describedby <http://www.worldcat.org/title/-/oclc/1055160511> ;
    .


Related Entities

<http://experiment.worldcat.org/entity/work/data/5463536983#Person/kamath_binita_m> # Binita M. Kamath
    a schema:Person ;
    schema:familyName "Kamath" ;
    schema:givenName "Binita M." ;
    schema:name "Binita M. Kamath" ;
    .

<http://experiment.worldcat.org/entity/work/data/5463536983#Person/loomes_kathleen_m> # Kathleen M. Loomes
    a schema:Person ;
    schema:familyName "Loomes" ;
    schema:givenName "Kathleen M." ;
    schema:name "Kathleen M. Loomes" ;
    .

<http://experiment.worldcat.org/entity/work/data/5463536983#Topic/cardiovascular_medicine> # Cardiovascular medicine
    a schema:Intangible ;
    schema:name "Cardiovascular medicine"@en ;
    .

<http://experiment.worldcat.org/entity/work/data/5463536983#Topic/genetic_disorders> # Genetic disorders
    a schema:Intangible ;
    schema:name "Genetic disorders"@en ;
    .

<http://experiment.worldcat.org/entity/work/data/5463536983#Topic/heart_diseases_genetic_aspects> # Heart--Diseases--Genetic aspects
    a schema:Intangible ;
    schema:name "Heart--Diseases--Genetic aspects"@en ;
    .

<http://experiment.worldcat.org/entity/work/data/5463536983#Topic/liver_diseases_genetic_aspects> # Liver--Diseases--Genetic aspects
    a schema:Intangible ;
    schema:name "Liver--Diseases--Genetic aspects"@en ;
    .

<http://experiment.worldcat.org/entity/work/data/5463536983#Topic/medical_genetics> # MEDICAL--Genetics
    a schema:Intangible ;
    schema:name "MEDICAL--Genetics"@en ;
    .

<http://worldcat.org/isbn/9783030068783>
    a schema:ProductModel ;
    schema:isbn "3030068781" ;
    schema:isbn "9783030068783" ;
    .

<http://worldcat.org/isbn/9783319945712>
    a schema:ProductModel ;
    schema:isbn "3319945718" ;
    schema:isbn "9783319945712" ;
    .

<http://worldcat.org/isbn/9783319945729>
    a schema:ProductModel ;
    schema:isbn "3319945726" ;
    schema:isbn "9783319945729" ;
    .

<http://www.worldcat.org/oclc/1037809506>
    a schema:CreativeWork ;
    rdfs:label "Alagille Syndrome." ;
    schema:description "Print version:" ;
    schema:isSimilarTo <http://www.worldcat.org/oclc/1055160511> ; # Alagille Syndrome : Pathogenesis and Clinical Management
    .


Content-negotiable representations

Close Window

Please sign in to WorldCat 

Don't have an account? You can easily create a free account.