Lymphangiectasia-lymphedema syndrome I89.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Hennekam Syndrome

Definition
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Rare syndrome with mental retardation, severe congenital and progressive lymphedema of the extremities and face, genitals and lymphangiectatic malformations in various organs (e.g. gastrointestinal) with consecutive hypoproteinemia. Furthermore, anomalies of the face (flat face, epicanthus, hypertelorism) of the extremities (syndactyly, pes equinovarus) and epileptic seizures are described. Multiple hemangiomas may be associated.

Etiopathogenesis
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Autosomal recessive malformations.

Literature
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  1. Kröll A et al (2006) Etiology and epidemiology of primary lymphedema. Phlebology 35: 3-7

Incoming links (1)

Lymphatic malformations;

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Last updated on: 29.10.2020