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Frank-Ter Haar syndrome

ORPHA:137834DiseaseAutosomal recessiveAntenatal, Neonatal

Ассоциированные гены (1)

SH3PXD2B
SH3 and PX domains 2B
Disease-causing germline mutation(s) (loss of function) in
OMIM: 613293

Фенотипы (33)

Очень частый (80–99%)14
HP:0000154Wide mouth
HP:0000280Coarse facial features
HP:0000316Hypertelorism
HP:0000322Short philtrum
HP:0000431Wide nasal bridge
HP:0000490Deeply set eye
HP:0001061Acne
HP:0001072Thickened skin
HP:0001156Brachydactyly
HP:0001634Mitral valve prolapse
HP:0002797Osteolysis
HP:0005280Depressed nasal bridge
HP:0010885Avascular necrosis
HP:0012471Thick vermilion border
Частый (30–79%)16
HP:0000212Gingival overgrowth
HP:0000303Mandibular prognathia
HP:0000337Broad forehead
HP:0000348High forehead
HP:0000411Protruding ear
HP:0000494Downslanted palpebral fissures
HP:0000684Delayed eruption of teeth
HP:0001387Joint stiffness
HP:0002650Scoliosis
HP:0002808Kyphosis
HP:0002816Genu recurvatum
HP:0004209Clinodactyly of the 5th finger
HP:0004568Beaking of vertebral bodies
HP:0005916Abnormal metacarpal morphology
HP:0006480Premature loss of teeth
HP:0100490Camptodactyly of finger
Периодический (5–29%)3
HP:0000023Inguinal hernia
HP:0000771Gynecomastia
HP:0001537Umbilical hernia

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