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SYNDROME OMENN [1 record]

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Record 1 2019-11-28

English

Subject field(s)
  • Human Diseases - Various
  • Genetics
  • Immunology
CONT

Omenn syndrome (OS) is a rare inherited disorder presenting with early-onset generalized erythrodermia, alopecia, chronic diarrhea, lymphoadenopathy, failure to thrive, and recurrent infections.

French

Domaine(s)
  • Maladies humaines diverses
  • Génétique
  • Immunologie
CONT

Le syndrome d'Omenn apparaît au cours de la première année de vie avec des manifestations caractéristiques du déficit immunitaire combiné sévère incluant des diarrhées chroniques, une pneumonite et un retard de croissance staturo-pondérale. Les patients présentent aussi des symptômes inflammatoires tels qu'une adénopathie, une hépatosplénomégalie et une érythrodermie généralisée, souvent à l'origine d'une alopécie avec chute des sourcils et des cils.

Spanish

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