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Hypoparathyroidism-sensorineural deafness-renal disease syndrome

ORPHA:2237Malformation syndromeAutosomal dominantAll ages

Ассоциированные гены (1)

GATA3
GATA binding protein 3
Disease-causing germline mutation(s) (loss of function) in
OMIM: 131320

Фенотипы (22)

Облигатный (100%)3
HP:0000110Renal dysplasia
HP:0000408Progressive sensorineural hearing impairment
HP:0000829Hypoparathyroidism
Частый (30–79%)8
HP:0000076Vesicoureteral reflux
HP:0000083Renal insufficiency
HP:0000113Polycystic kidney dysplasia
HP:0000122Unilateral renal agenesis
HP:0000126Hydronephrosis
HP:0000860Parathyroid hypoplasia
HP:0002199Hypocalcemic seizures
HP:0002901Hypocalcemia
Периодический (5–29%)3
HP:0000819Diabetes mellitus
HP:0001153Septate vagina
HP:0003762Uterus didelphys
Очень редкий (1–4%)8
HP:0000148Vaginal atresia
HP:0000151Aplasia of the uterus
HP:0000175Cleft palate
HP:0000510Rod-cone dystrophy
HP:0001627Abnormal heart morphology
HP:0003765Psoriasiform dermatitis
HP:0008850Severe postnatal growth retardation
HP:0011840Abnormality of T cell physiology

Эпидемиология (2)

Point prevalence
<1 / 1 000 000
Worldwide
Cases/families
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы