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Laryngo-onycho-cutaneous syndrome

ORPHA:2407DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

LAMA3
laminin subunit alpha 3
Disease-causing germline mutation(s) in
OMIM: 600805

Фенотипы (22)

Очень частый (80–99%)2
HP:0001607Subglottic stenosis
HP:0200042Skin ulcer
Частый (30–79%)16
HP:0000670Carious teeth
HP:0000684Delayed eruption of teeth
HP:0000691Microdontia
HP:0001030Fragile skin
HP:0001609Hoarse voice
HP:0005348Inspiratory stridor
HP:0006297Enamel hypoplasia
HP:0008066Abnormal blistering of the skin
HP:0008404Nail dystrophy
HP:0012804Corneal ulceration
HP:0100803Abnormality of the periungual region
HP:0200020Corneal erosion
HP:0200041Skin erosion
HP:0200097Oral mucosal blisters
HP:0430007Symblepharon
HP:6000956Exuberant granulation tissue
Периодический (5–29%)4
HP:0000421Epistaxis
HP:0000505Visual impairment
HP:0004840Hypochromic microcytic anemia
HP:0008777Abnormal vocal cord morphology

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы