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GNB5-related intellectual disability-cardiac arrhythmia syndrome

ORPHA:542306DiseaseAutosomal recessiveChildhood, Infancy

Ассоциированные гены (1)

GNB5
G protein subunit beta 5
Disease-causing germline mutation(s) in
OMIM: 604447

Фенотипы (22)

Очень частый (80–99%)2
HP:0001263Global developmental delay
HP:0001626Abnormality of the cardiovascular system
Частый (30–79%)12
HP:0000512Abnormal electroretinogram
HP:0000639Nystagmus
HP:0000750Delayed speech and language development
HP:0001250Seizure
HP:0001252Hypotonia
HP:0001344Absent speech
HP:0001662Bradycardia
HP:0002020Gastroesophageal reflux
HP:0005155Ventricular escape rhythm
HP:0010864Intellectual disability, severe
HP:0011675Arrhythmia
HP:0011704Sick sinus syndrome
Периодический (5–29%)8
HP:0000546Retinal degeneration
HP:0000563Keratoconus
HP:0000817Reduced eye contact
HP:0001655Patent foramen ovale
HP:0002421Poor head control
HP:0002521Hypsarrhythmia
HP:0007010Poor fine motor coordination
HP:0012248Prolonged PR interval

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