Alagille Syndrome: Pathogenesis and Clinical Management

ISBN-13: 9783319945705 / Angielski / Twarda / 2018 / 198 str.

Kamath, Binita M.

Opis

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.

Spis treści

1 The Alagille Syndrome: Overview and Introduction

David Piccoli

2 Bile Duct Development and the Notch Signaling Pathway

Stacey S. Huppert, Kathleen M. Campbell

3 Genetics of Alagille Syndrome

Melissa A. Gilbert, Nancy B. Spinner

4 Liver Disease in Alagille Syndrome

Alyssa Kriegermeier, Andrew Wehrman, Binita M. Kamath, Kathleen M. Loomes

5 Transplant Considerations in Alagille Syndrome

Evelyn Hsu, Elizabeth Rand

6 Cardiac, Aortic, and Pulmonary Vascular Involvement in Alagille Syndrome

Justin T. Tretter, Doff B. McElhinney

7 Vascular Manifestations in Alagille Syndrome

Shannon M. Vandriel, Rebecca N. Ichord, Binita M. Kamath

8 The Renal Sequelae of Alagille Syndrome as a Product of Altered Notch Signaling During Kidney Development

René Romero

9 Skeletal Involvement in Alagille Syndrome

Yadav Wagley, Troy Mitchell, Jason Ashley, Kathleen M. Loomes, Kurt Hankenson

10 Immune Dysregulation in Alagille Syndrome: A Feature of the Evolving Phenotype

Alastair Baker

11 Health-Related Quality of Life and Neurocognition in Alagille Syndrome

Saeed Mohammad, Estella M Alonso

12 Future Therapeutic Approaches for Alagille Syndrome 

Emma R. Andersson

O autorze

Binita M. Kamath, MBBChir MRCP MTR

The Hospital for Sick Children

Division of Gastroenterology, Hepatology and Nutrition 

Toronto, ON

Canada

Kathleen M. Loomes, MD

Children’s Hospital of Philadelphia

Division of Gastroenterology, Hepatology and Nutrition

Department of Pediatrics

Philadelphia, PA

USA