Zunich-Kaye syndrome
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| ICD9 = | ICDO = | OMIM = 280000 | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = Zunich-Kaye syndrome, also known as Zunich neuroectodermal syndrome, is a rare condition first described in 1983. It is also referred to as CHIME syndrome, after its main symptoms (colobomas, heart defects, ichthyosiform dermatosis, mental retardation, and either ear defects or epilepsy).OrphaNet entry It is a congenitalhttp://www.bdid.com/defectz.htm syndrome with only a few cases studied and published. Associated symptoms range from things such as colobomas of the eyes, heart defects, ichthyosiform dermatosis, mental retardation, and ear abnormalities. Further symptoms that may be suggested include characteristic facies, hearing loss, and cleft palate. It is assumed the syndrome follows an autosomal recessive pattern of inheritance. Treatment with isotretinoin may induce substantial resolution of skin lesions, but the risk of secondary infection remains.
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