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Wilson-Turner syndrome

ORPHA:3459Malformation syndromeX-linked dominant, X-linked recessiveChildhood

Ассоциированные гены (2)

HDAC8
histone deacetylase 8
Disease-causing germline mutation(s) (loss of function) in
OMIM: 300269
LAS1L
LAS1 like ribosome biogenesis factor
Disease-causing germline mutation(s) in
OMIM: 300964

Фенотипы (28)

Очень частый (80–99%)22
HP:0000028Cryptorchidism
HP:0000219Thin upper lip vermilion
HP:0000336Prominent supraorbital ridges
HP:0000347Micrognathia
HP:0000455Broad nasal tip
HP:0000490Deeply set eye
HP:0000574Thick eyebrow
HP:0000712Emotional lability
HP:0000771Gynecomastia
HP:0001182Tapered finger
HP:0001249Intellectual disability
HP:0001263Global developmental delay
HP:0001761Pes cavus
HP:0001763Pes planus
HP:0001773Short foot
HP:0001956Truncal obesity
HP:0001999Abnormal facial shape
HP:0002465Poor speech
HP:0004322Short stature
HP:0008551Microtia
HP:0010620Malar prominence
HP:0200055Small hand
Частый (30–79%)1
HP:0000044Hypogonadotropic hypogonadism
Периодический (5–29%)2
HP:0001250Seizure
HP:0001328Specific learning disability
Исключён (0%)3
HP:0000518Cataract
HP:0000540Hypermetropia
HP:0009909Uplifted earlobe

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