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X-linked intellectual disability with isolated growth hormone deficiency

ORPHA:67045Clinical subtypeX-linked recessiveInfancy, Neonatal

Ассоциированные гены (1)

SOX3
SRY-box transcription factor 3
Disease-causing germline mutation(s) in
OMIM: 313430

Фенотипы (14)

Очень частый (80–99%)4
HP:0000824Decreased response to growth hormone stimulation test
HP:0001249Intellectual disability
HP:0004322Short stature
HP:0030353Decreased serum insulin-like growth factor 1
Периодический (5–29%)10
HP:0000570Abnormal saccadic eye movements
HP:0000657Oculomotor apraxia
HP:0002079Hypoplasia of the corpus callosum
HP:0002750Delayed skeletal maturation
HP:0007979Gaze-evoked horizontal nystagmus
HP:0010627Anterior pituitary hypoplasia
HP:0011342Mild global developmental delay
HP:0011755Ectopic posterior pituitary
HP:0012447Abnormal myelination
HP:0040010Small posterior fossa

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