Weyers' syndrome IV

Alternative eponyms

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A congenital syndrome of abnormalities of the iris microdontia, oligodontia, enamel hypoplasia, and virilisation. Later, dwarfism and myotonic dystrophy become evident.

Description

A familial syndrome of unknown aetiology present from birth, characterized by an association of iris abnormalities, corneal disorders, and anomalous tooth development. Abnormalities include dysplasia and minute perforations of iris, pupillary synechiae, microphthalmia, corneal opacity, microdontia, oligodontia, enamel hypoplasia, and virilisation. Later, dwarfism and myotonic dystrophy become evident. Inheritance is autosomal dominant.

Bibliography

  • H. Weyers:
    Dysgenesis irido-dentalis. Ein neues Syndrom mit abweichendem chromosomalen Geschlecht bei weiblichen Merkmalträgern.
    Presented at the meeting of the deutsche Gesellschaft für Kinderheilkunde, Kassel, 1960.

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