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Moebius syndrome

ORPHA:570DiseaseAutosomal dominantAntenatal, Neonatal

Ассоциированные гены (2)

PLXND1
plexin D1
Disease-causing germline mutation(s) in
OMIM: 604282
REV3L
REV3 like, DNA directed polymerase zeta catalytic subunit
Disease-causing germline mutation(s) in
OMIM: 602776

Фенотипы (42)

Очень частый (80–99%)9
HP:0000194Open mouth
HP:0000298Mask-like facies
HP:0000486Strabismus
HP:0000508Ptosis
HP:0000602Ophthalmoplegia
HP:0001608Abnormality of the voice
HP:0006824Cranial nerve paralysis
HP:0008872Feeding difficulties in infancy
HP:0010628Facial palsy
Частый (30–79%)8
HP:0000232Everted lower lip vermilion
HP:0001156Brachydactyly
HP:0001252Hypotonia
HP:0001270Motor delay
HP:0001762Talipes equinovarus
HP:0002015Dysphagia
HP:0007957Corneal opacity
HP:0009751Aplasia of the pectoralis major muscle
Периодический (5–29%)25
HP:0000044Hypogonadotropic hypogonadism
HP:0000175Cleft palate
HP:0000218High palate
HP:0000286Epicanthus
HP:0000347Micrognathia
HP:0000365Hearing impairment
HP:0000498Blepharitis
HP:0000505Visual impairment
HP:0000691Microdontia
HP:0000717Autism
HP:0001522Death in infancy
HP:0002804Arthrogryposis multiplex congenita
HP:0003202Skeletal muscle atrophy
HP:0004050Absent hand
HP:0004209Clinodactyly of the 5th finger
HP:0004408Abnormality of the sense of smell
HP:0005914Aplasia/Hypoplasia involving the metacarpal bones
HP:0006101Finger syndactyly
HP:0006501Aplasia/Hypoplasia of the radius
HP:0007565Multiple cafe-au-lait spots
HP:0009601Aplasia/Hypoplasia of the thumb
HP:0009804Tooth agenesis
HP:0010295Aplasia/Hypoplasia of the tongue
HP:0040071Abnormal morphology of ulna
HP:0100783Breast aplasia

Эпидемиология (5)

Cases/families
Worldwide
Point prevalence
1-9 / 100 000
Worldwide
Prevalence at birth
1-9 / 100 000
Netherlands
Point prevalence
1-9 / 1 000 000
Italy
Prevalence at birth
<1 / 1 000 000
Italy

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы