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MEDNIK syndrome

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Disease definition

A rare disorder of copper metabolism characterized by intellectual deficit, enteropathy, sensorineural hearing loss, peripheral neuropathy, lamellar and erythrodermic ichthyosis, and keratodermia.

ORPHA:171851

Classification level: Disorder

Synonym(s):
  • Intellectual disability-enteropathy-deafness-peripheral neuropathy-ichthyosis-keratodermia syndrome
  • Intellectual disability-enteropathy-hearing loss-peripheral neuropathy-ichthyosis-keratodermia syndrome

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Antenatal, Infancy, Neonatal

ICD-10: E83.0

ICD-11: LD2H.Y

OMIM: 609313

UMLS: C1836330

A summary on this disease is available in Français, Español, Deutsch, Português, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2018.pdf) - Orphanet Urgences
Clinical practice guidelines
Disease review articles
Clinical genetics review
English (2024) - GeneReviews
English (2013.pdf) - Eur J Hum Genet
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.