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Pfeiffer syndrome type 3

ORPHA:93260Clinical subtypeAutosomal dominant, Not applicableAntenatal, Neonatal

Ассоциированные гены (1)

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

Фенотипы (36)

Очень частый (80–99%)19
HP:0000218High palate
HP:0000244Brachyturricephaly
HP:0000316Hypertelorism
HP:0000348High forehead
HP:0000402Stenosis of the external auditory canal
HP:0000520Proptosis
HP:0001376Limitation of joint mobility
HP:0001601Laryngomalacia
HP:0002098Respiratory distress
HP:0002308Chiari malformation
HP:0002410Aqueductal stenosis
HP:0002779Tracheomalacia
HP:0003196Short nose
HP:0005280Depressed nasal bridge
HP:0008080Hallux varus
HP:0010059Broad hallux phalanx
HP:0010109Short hallux
HP:0011304Broad thumb
HP:0011800Midface retrusion
Частый (30–79%)8
HP:0000369Low-set ears
HP:0000453Choanal atresia
HP:0001249Intellectual disability
HP:0001250Seizure
HP:0001770Toe syndactyly
HP:0001773Short foot
HP:0006101Finger syndactyly
HP:0200055Small hand
Периодический (5–29%)9
HP:0000076Vesicoureteral reflux
HP:0000085Horseshoe kidney
HP:0000126Hydronephrosis
HP:0000175Cleft palate
HP:0000365Hearing impairment
HP:0000646Amblyopia
HP:0002023Anal atresia
HP:0002516Increased intracranial pressure
HP:0002566Intestinal malrotation

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