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Greig cephalopolysyndactyly syndrome

ORPHA:380Malformation syndromeAutosomal dominantAntenatal

Ассоциированные гены (1)

GLI3
GLI family zinc finger 3
Disease-causing germline mutation(s) (loss of function) in
OMIM: 165240

Фенотипы (22)

Очень частый (80–99%)3
HP:0000256Macrocephaly
HP:0001162Postaxial hand polydactyly
HP:0001841Preaxial foot polydactyly
Частый (30–79%)8
HP:0000316Hypertelorism
HP:0000348High forehead
HP:0000431Wide nasal bridge
HP:0000506Telecanthus
HP:0001770Toe syndactyly
HP:0002007Frontal bossing
HP:0005616Accelerated skeletal maturation
HP:0006101Finger syndactyly
Периодический (5–29%)11
HP:0000238Hydrocephalus
HP:0000776Congenital diaphragmatic hernia
HP:0001177Preaxial hand polydactyly
HP:0001250Seizure
HP:0001256Intellectual disability, mild
HP:0001274Agenesis of corpus callosum
HP:0001363Craniosynostosis
HP:0001537Umbilical hernia
HP:0001830Postaxial foot polydactyly
HP:0010059Broad hallux phalanx
HP:0011304Broad thumb

Эпидемиология (2)

Prevalence at birth
1-9 / 1 000 000
Europe
Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы