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CODAS syndrome

ORPHA:1458Malformation syndromeAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

LONP1
lon peptidase 1, mitochondrial
Disease-causing germline mutation(s) in
OMIM: 605490

Фенотипы (33)

Очень частый (80–99%)20
HP:0000286Epicanthus
HP:0000396Overfolded helix
HP:0000463Anteverted nares
HP:0000518Cataract
HP:0000682Abnormality of dental enamel
HP:0000684Delayed eruption of teeth
HP:0001156Brachydactyly
HP:0001263Global developmental delay
HP:0002750Delayed skeletal maturation
HP:0003196Short nose
HP:0003312Abnormal form of the vertebral bodies
HP:0003417Coronal cleft vertebrae
HP:0004122Midline defect of the nose
HP:0004322Short stature
HP:0005280Depressed nasal bridge
HP:0005930Abnormality of epiphysis morphology
HP:0006482Abnormal dental morphology
HP:0009901Crumpled ear
HP:0010049Short metacarpal
HP:0012368Flat face
Частый (30–79%)7
HP:0000407Sensorineural hearing impairment
HP:0000508Ptosis
HP:0001252Hypotonia
HP:0001374Congenital hip dislocation
HP:0002644Abnormality of pelvic girdle bone morphology
HP:0002650Scoliosis
HP:0001382Joint hypermobility
Периодический (5–29%)6
HP:0000072Hydroureter
HP:0000486Strabismus
HP:0000639Nystagmus
HP:0001600Abnormality of the larynx
HP:0001629Ventricular septal defect
HP:0005242Extrahepatic biliary duct atresia

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