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COG7-CDG

ORPHA:79333DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

COG7
component of oligomeric golgi complex 7
Disease-causing germline mutation(s) in
OMIM: 606978

Фенотипы (33)

Очень частый (80–99%)9
HP:0001252Hypotonia
HP:0001508Failure to thrive
HP:0001954Recurrent fever
HP:0002719Recurrent infections
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0008897Postnatal growth retardation
HP:0011968Feeding difficulties
HP:0012301Type II transferrin isoform profile
HP:0012444Brain atrophy
Частый (30–79%)15
HP:0000077Abnormality of the kidney
HP:0000253Progressive microcephaly
HP:0000952Jaundice
HP:0001167Abnormality of finger
HP:0001250Seizure
HP:0001265Hyporeflexia
HP:0001284Areflexia
HP:0001518Small for gestational age
HP:0001627Abnormal heart morphology
HP:0001999Abnormal facial shape
HP:0002014Diarrhea
HP:0002240Hepatomegaly
HP:0003236Elevated circulating creatine kinase concentration
HP:0007392Excessive wrinkled skin
HP:0011451Congenital microcephaly
Периодический (5–29%)9
HP:0000160Narrow mouth
HP:0000278Retrognathia
HP:0000347Micrognathia
HP:0000470Short neck
HP:0001181Adducted thumb
HP:0001272Cerebellar atrophy
HP:0001433Hepatosplenomegaly
HP:0012157Subcortical cerebral atrophy
HP:0100807Long fingers

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