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Crouzon syndrome

ORPHA:207Malformation syndromeAutosomal dominantAntenatal, Infancy, Neonatal

Ассоциированные гены (2)

FGFR2
fibroblast growth factor receptor 2
Disease-causing germline mutation(s) (gain of function) in
OMIM: 176943
ERF
ETS2 repressor factor
Disease-causing germline mutation(s) (loss of function) in
OMIM: 611888

Фенотипы (33)

Очень частый (80–99%)5
HP:0000348High forehead
HP:0000929Abnormal skull morphology
HP:0001999Abnormal facial shape
HP:0002007Frontal bossing
HP:0011324Multiple suture craniosynostosis
Частый (30–79%)13
HP:0000248Brachycephaly
HP:0000262Turricephaly
HP:0000316Hypertelorism
HP:0000327Hypoplasia of the maxilla
HP:0000405Conductive hearing impairment
HP:0000486Strabismus
HP:0000508Ptosis
HP:0000509Conjunctivitis
HP:0000520Proptosis
HP:0001321Cerebellar hypoplasia
HP:0002308Chiari malformation
HP:0002516Increased intracranial pressure
HP:0011800Midface retrusion
Периодический (5–29%)15
HP:0000189Narrow palate
HP:0000238Hydrocephalus
HP:0000365Hearing impairment
HP:0000444Convex nasal ridge
HP:0000453Choanal atresia
HP:0000612Iris coloboma
HP:0000646Amblyopia
HP:0000648Optic atrophy
HP:0000956Acanthosis nigricans
HP:0000995Melanocytic nevus
HP:0001053Hypopigmented skin patches
HP:0002093Respiratory insufficiency
HP:0002315Headache
HP:0005107Abnormal sacrum morphology
HP:0011386Narrow internal auditory canal

Эпидемиология (4)

Prevalence at birth
1-9 / 1 000 000
Europe
Point prevalence
1-9 / 1 000 000
Europe
Point prevalence
1-9 / 100 000
Canada
Prevalence at birth
1-9 / 100 000
Australia

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы