← Назад

X-linked intellectual disability-short stature-overweight syndrome

ORPHA:457240Malformation syndromeX-linked recessiveAdolescent, Childhood, Infancy

Ассоциированные гены (1)

THOC2
THO complex subunit 2
Disease-causing germline mutation(s) (loss of function) in
OMIM: 300395

Фенотипы (37)

Частый (30–79%)9
HP:0000218High palate
HP:0000708Atypical behavior
HP:0000750Delayed speech and language development
HP:0001252Hypotonia
HP:0001256Intellectual disability, mild
HP:0001337Tremor
HP:0002342Intellectual disability, moderate
HP:0004322Short stature
HP:0025502Overweight
Периодический (5–29%)28
HP:0000054Micropenis
HP:0000252Microcephaly
HP:0000316Hypertelorism
HP:0000337Broad forehead
HP:0000348High forehead
HP:0000400Macrotia
HP:0000486Strabismus
HP:0000639Nystagmus
HP:0000716Depression
HP:0000729Autistic behavior
HP:0000733Abnormal repetitive mannerisms
HP:0000739Anxiety
HP:0000742Self-mutilation
HP:0000824Decreased response to growth hormone stimulation test
HP:0000954Single transverse palmar crease
HP:0001250Seizure
HP:0001288Gait disturbance
HP:0001385Hip dysplasia
HP:0001658Myocardial infarction
HP:0002069Bilateral tonic-clonic seizure
HP:0002171Gliosis
HP:0002206Pulmonary fibrosis
HP:0002487Hyperkinetic movements
HP:0004437Cranial hyperostosis
HP:0006986Upper limb spasticity
HP:0007033Cerebellar dysplasia
HP:0008734Decreased testicular size
HP:0010864Intellectual disability, severe

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