Zunich-kaye syndromeQ82.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

CHIME; CHIME Syndrome; Neuroectodermal syndrome; Neuroectodermal Syndrome Zunich

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DefinitionThis section has been translated automatically.

Zunich-Kaye syndrome, also known as "Neuroectodermal Syndrome Zunich", is a very rare, autosomal recessive, congenital ichthyosiform disease. The disease is also known as CHIME syndrome, an acronym for the main symptoms of the syndrome: "colobomas, heart defects, ichthyosiform dermatosis, mental retardation and either ear defect or epilepsia".

Clinical featuresThis section has been translated automatically.

Ichthyosiform skin changes, colobomas of the eyes, heart defects, hearing loss, cerebral seizures, mental retardation. The teeth of those affected may be widely spaced and have square shapes.

TherapyThis section has been translated automatically.

Isotretinoin improves the ichthyosis of the skin.

LiteratureThis section has been translated automatically.

  1. Zunich J, Kaye CI (1983) New syndrome of congenital ichthyosis with neurologic abnormalities. At J Med Genet 15: 331-335

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer