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Vascular Manifestations in Alagille Syndrome

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Alagille Syndrome

Abstract

Non-cardiac vascular anomalies and events are now widely recognized in Alagille syndrome (ALGS) and are a significant cause of morbidity and mortality. Cerebrovascular disorders in ALGS includes cerebral artery aneurysms, moyamoya syndrome, and anomalies of the internal carotid artery. Systemic vascular abnormalities have also been documented. These structural anomalies contribute to but do not fully explain the high incidence of intracranial and systemic bleeding episodes in ALGS. The presence of vascular anomalies in ALGS is consistent with the intrinsic role of the Notch signaling pathway in vascular morphogenesis, angiogenesis, and vessel homeostasis. Given the relatively high prevalence of silent cerebrovascular disease in ALGS, it is becoming 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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Author information

Authors and Affiliations

  1. Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada

    Shannon M. Vandriel & Binita M. Kamath

  2. Division of Neurology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Rebecca N. Ichord

  3. Department of Pediatrics, University of Toronto, Toronto, ON, Canada

    Binita M. Kamath

Authors
  1. Shannon M. Vandriel
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  2. Rebecca N. Ichord
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  3. Binita M. Kamath
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Corresponding author

Correspondence to Binita M. Kamath .

Editor information

Editors and Affiliations

  1. Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada

    Binita M. Kamath

  2. Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Kathleen M. Loomes

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Vandriel, S.M., Ichord, R.N., Kamath, B.M. (2018). Vascular Manifestations in Alagille Syndrome. In: Kamath, B., Loomes, K. (eds) Alagille Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-319-94571-2_7

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  • DOI: https://doi.org/10.1007/978-3-319-94571-2_7

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-94570-5

  • Online ISBN: 978-3-319-94571-2

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