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Metadata
ID DOID:0070746
PURL http://purl.obolibrary.org/obo/DOID_0070746 Copy
Name HELIX syndrome
Definition A syndrome characterized by hypohidrosis, electrolyte imbalance, lacrimal gland dysfunction, ichthyosis, and xerostomia that has_material_basis_in homozygous mutation in the CLDN10 gene on chromosome 13q32.
https://pubmed.ncbi.nlm.nih.gov/28771254/
Xrefs

GARD:0017967

ICD10CM:N25.8

MIM:617671

ORDO:528105

SNOMEDCT_US_2026_03_01:1217380005

UMLS_CUI:C4522164

SKOS

exactMatch UMLS_CUI:C4522164

exactMatch GARD:0017967

exactMatch MIM:617671

exactMatch ORDO:528105

broadMatch ICD10CM:N25.8

Subsets

DO_rare_slim

Synonyms

hypohidrosis, electrolyte imbalance, lacrimal gland dysfunction, ichthyosis, and xerostomia [EXACT]

Parent Relationships

is_a syndrome

is_a autosomal recessive disease

Subclass Logical Relationships

has material basis in some autosomal recessive inheritance

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