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Developmental Aspects of the Lymphatic Vascular System pp 187–204Cite as

Clinical Disorders of Primary Malfunctioning of the Lymphatic System

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Part of the book series: Advances in Anatomy, Embryology and Cell Biology ((ADVSANAT,volume 214))

Abstract

Primary lymphedema is defined as lymphedema caused by dysplasia of the lymph vessels. This complex group of diseases is discussed in detail from a clinical perspective. A review of the epidemiology and classification of lymphedema on the backdrop of its clinical presentation reveals weaknesses of the present classification system, which, however, is the basis for the choice of optimal patient care. Non-syndrome and syndrome types of primary lymphedema are presented in detail and related molecular findings are summarized.

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Authors and Affiliations

  1. Neonatal Intensive Care Unit, Emergency Department, Gaslini Institute, Genoa, Italy

    Carlo Bellini

  2. Department of Pediatrics and Translational Genetics, Academic Medical Center, Amsterdam, The Netherlands

    Raoul CM Hennekam

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  1. Carlo Bellini
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  2. Raoul CM Hennekam
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Correspondence to Raoul CM Hennekam .

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Editors and Affiliations

  1. MPI for Molecular Biomedicine, Münster, Germany

    Friedemann Kiefer

  2. Hubrecht Institute, Utrecht, The Netherlands

    Stefan Schulte-Merker

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Bellini, C., Hennekam, R.C. (2014). Clinical Disorders of Primary Malfunctioning of the Lymphatic System. In: Kiefer, F., Schulte-Merker, S. (eds) Developmental Aspects of the Lymphatic Vascular System. Advances in Anatomy, Embryology and Cell Biology, vol 214. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1646-3_14

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  • DOI: https://doi.org/10.1007/978-3-7091-1646-3_14

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  • Print ISBN: 978-3-7091-1645-6

  • Online ISBN: 978-3-7091-1646-3

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