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Syndactyly-telecanthus-anogenital and renal malformations syndrome

ORPHA:140952Malformation syndromeX-linked dominantInfancy, Neonatal

Ассоциированные гены (1)

CCNQ
cyclin Q
Disease-causing germline mutation(s) in
OMIM: 300708

Фенотипы (28)

Очень частый (80–99%)5
HP:0000394Lop ear
HP:0001770Toe syndactyly
HP:0002023Anal atresia
HP:0004209Clinodactyly of the 5th finger
HP:0004322Short stature
Частый (30–79%)13
HP:0000066Labial hypoplasia
HP:0000076Vesicoureteral reflux
HP:0000083Renal insufficiency
HP:0000085Horseshoe kidney
HP:0000086Ectopic kidney
HP:0000104Renal agenesis
HP:0000219Thin upper lip vermilion
HP:0000414Bulbous nose
HP:0000431Wide nasal bridge
HP:0000506Telecanthus
HP:0000813Bicornuate uterus
HP:0001671Abnormal cardiac septum morphology
HP:0008665Clitoral hypertrophy
Периодический (5–29%)10
HP:0000545Myopia
HP:0000556Retinal dystrophy
HP:0000625Eyelid coloboma
HP:0001250Seizure
HP:0001659Aortic regurgitation
HP:0002984Hypoplasia of the radius
HP:0003396Syringomyelia
HP:0004415Pulmonary artery stenosis
HP:0007754Macular dystrophy
HP:0011560Mitral atresia

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