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Alpers-Huttenlocher syndrome

ORPHA:726DiseaseAutosomal recessiveAdolescent, Adult, Childhood, Infancy

Ассоциированные гены (1)

POLG
DNA polymerase gamma, catalytic subunit
Disease-causing germline mutation(s) in
OMIM: 174763

Фенотипы (19)

Частый (30–79%)18
HP:0000252Microcephaly
HP:0000478Abnormality of the eye
HP:0000504Abnormality of vision
HP:0001251Ataxia
HP:0001252Hypotonia
HP:0001257Spasticity
HP:0001259Coma
HP:0001263Global developmental delay
HP:0001266Choreoathetosis
HP:0001284Areflexia
HP:0001336Myoclonus
HP:0002069Bilateral tonic-clonic seizure
HP:0002191Progressive spasticity
HP:0002313Spastic paraparesis
HP:0002376Developmental regression
HP:0002385Paraparesis
HP:0007359Focal-onset seizure
HP:0100022Abnormality of movement
Периодический (5–29%)1
HP:0000618Blindness

Эпидемиология (2)

Prevalence at birth
1-9 / 1 000 000
Europe
Point prevalence
<1 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы