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DDOST-CDG

ORPHA:300536DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

DDOST
dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit
Disease-causing germline mutation(s) in
OMIM: 602202

Фенотипы (23)

Очень частый (80–99%)19
HP:0000565Esotropia
HP:0000938Osteopenia
HP:0001250Seizure
HP:0001290Generalized hypotonia
HP:0001337Tremor
HP:0001397Hepatic steatosis
HP:0001508Failure to thrive
HP:0002019Constipation
HP:0002020Gastroesophageal reflux
HP:0002167Abnormality of speech or vocalization
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0003256Abnormality of the coagulation cascade
HP:0003429CNS hypomyelination
HP:0003642Type I transferrin isoform profile
HP:0004322Short stature
HP:0005616Accelerated skeletal maturation
HP:0007301Oromotor apraxia
HP:0012758Neurodevelopmental delay
HP:0410018Recurrent ear infections
Периодический (5–29%)2
HP:0000958Dry skin
HP:0009125Lipodystrophy
Очень редкий (1–4%)2
HP:0000832Primary hypothyroidism
HP:0012593Nephrotic range proteinuria

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