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Pfeiffer syndrome type 1

ORPHA:93258Clinical subtypeAutosomal dominant, Not applicableAntenatal, Neonatal

Ассоциированные гены (2)

FGFR1
fibroblast growth factor receptor 1
Disease-causing germline mutation(s) in
OMIM: 136350
FGFR2
fibroblast growth factor receptor 2
Disease-causing germline mutation(s) (gain of function) in
OMIM: 176943

Фенотипы (21)

Очень частый (80–99%)13
HP:0000218High palate
HP:0000248Brachycephaly
HP:0000316Hypertelorism
HP:0000348High forehead
HP:0003196Short nose
HP:0005280Depressed nasal bridge
HP:0008080Hallux varus
HP:0009601Aplasia/Hypoplasia of the thumb
HP:0010059Broad hallux phalanx
HP:0010109Short hallux
HP:0011304Broad thumb
HP:0011318Bicoronal synostosis
HP:0011800Midface retrusion
Частый (30–79%)6
HP:0000369Low-set ears
HP:0000520Proptosis
HP:0001770Toe syndactyly
HP:0001773Short foot
HP:0004279Short palm
HP:0006101Finger syndactyly
Периодический (5–29%)2
HP:0000365Hearing impairment
HP:0002410Aqueductal stenosis

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы