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Fried syndrome

ORPHA:85335Malformation syndromeX-linked recessiveInfancy, Neonatal

Ассоциированные гены (1)

AP1S2
adaptor related protein complex 1 subunit sigma 2
Disease-causing germline mutation(s) in
OMIM: 300629

Фенотипы (21)

Очень частый (80–99%)7
HP:0000238Hydrocephalus
HP:0001252Hypotonia
HP:0001263Global developmental delay
HP:0001264Spastic diplegia
HP:0002342Intellectual disability, moderate
HP:0002465Poor speech
HP:0002514Cerebral calcification
Частый (30–79%)9
HP:0000218High palate
HP:0000276Long face
HP:0000280Coarse facial features
HP:0000322Short philtrum
HP:0000400Macrotia
HP:0000718Aggressive behavior
HP:0000729Autistic behavior
HP:0001288Gait disturbance
HP:0002650Scoliosis
Периодический (5–29%)5
HP:0000365Hearing impairment
HP:0000587Abnormal optic nerve morphology
HP:0001317Abnormal cerebellum morphology
HP:0002684Thickened calvaria
HP:0003202Skeletal muscle atrophy

Эпидемиология (2)

Point prevalence
<1 / 1 000 000
Worldwide
Cases/families
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы