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Acrocallosal syndrome

ORPHA:36Malformation syndromeAutosomal recessiveAntenatal, Neonatal

Ассоциированные гены (2)

GLI3
GLI family zinc finger 3
Disease-causing germline mutation(s) in
OMIM: 165240
KIF7
kinesin family member 7
Disease-causing germline mutation(s) in
OMIM: 611254

Фенотипы (18)

Очень частый (80–99%)5
HP:0000256Macrocephaly
HP:0000316Hypertelorism
HP:0001162Postaxial hand polydactyly
HP:0007370Aplasia/Hypoplasia of the corpus callosum
HP:0010864Intellectual disability, severe
Частый (30–79%)4
HP:0000269Prominent occiput
HP:0000340Sloping forehead
HP:0001199Triphalangeal thumb
HP:0001305Dandy-Walker malformation
Периодический (5–29%)9
HP:0000023Inguinal hernia
HP:0000028Cryptorchidism
HP:0000047Hypospadias
HP:0000098Tall stature
HP:0000260Wide anterior fontanel
HP:0000407Sensorineural hearing impairment
HP:0000776Congenital diaphragmatic hernia
HP:0000889Abnormality of the clavicle
HP:0007360Aplasia/Hypoplasia of the cerebellum

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