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Trichorhinophalangeal syndrome type 1

ORPHA:77258Malformation syndromeAutosomal dominantInfancy, Neonatal

Ассоциированные гены (1)

TRPS1
transcriptional repressor GATA binding 1
Disease-causing germline mutation(s) in
OMIM: 604386

Фенотипы (29)

Очень частый (80–99%)18
HP:0000325Triangular face
HP:0000343Long philtrum
HP:0000347Micrognathia
HP:0000400Macrotia
HP:0000411Protruding ear
HP:0000414Bulbous nose
HP:0000653Sparse eyelashes
HP:0002007Frontal bossing
HP:0004209Clinodactyly of the 5th finger
HP:0004322Short stature
HP:0008070Sparse hair
HP:0009882Short distal phalanx of finger
HP:0010049Short metacarpal
HP:0010579Cone-shaped epiphysis
HP:0010743Short metatarsal
HP:0011341Long upper lip
HP:0011910Shortening of all phalanges of fingers
HP:0045075Sparse eyebrow
Частый (30–79%)11
HP:0000164Abnormality of the dentition
HP:0000218High palate
HP:0000768Pectus carinatum
HP:0001252Hypotonia
HP:0001808Fragile nails
HP:0001820Leukonychia
HP:0002650Scoliosis
HP:0003307Hyperlordosis
HP:0005743Avascular necrosis of the capital femoral epiphysis
HP:0011069Supernumerary tooth
HP:0100490Camptodactyly of finger

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