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Huntington disease

ORPHA:399DiseaseAutosomal dominantAdolescent, Adult, Childhood, Elderly

Ассоциированные гены (2)

HTT
huntingtin
Disease-causing germline mutation(s) (gain of function) in
OMIM: 613004
SLC2A3
solute carrier family 2 member 3
Modifying germline mutation in
OMIM: 138170

Фенотипы (53)

Очень частый (80–99%)3
HP:0001268Mental deterioration
HP:0001347Hyperreflexia
HP:0002072Chorea
Частый (30–79%)31
HP:0001288Gait disturbance
HP:0000496Abnormality of eye movement
HP:0000713Agitation
HP:0000716Depression
HP:0000718Aggressive behavior
HP:0000722Compulsive behaviors
HP:0000734Disinhibition
HP:0000737Irritability
HP:0000738Hallucinations
HP:0000739Anxiety
HP:0000741Apathy
HP:0000746Delusion
HP:0001250Seizure
HP:0001288Gait disturbance
HP:0001332Dystonia
HP:0001336Myoclonus
HP:0001824Weight loss
HP:0002067Bradykinesia
HP:0002141Gait imbalance
HP:0002312Clumsiness
HP:0002354Memory impairment
HP:0002375Hypokinesia
HP:0003324Generalized muscle weakness
HP:0004305Involuntary movements
HP:0004408Abnormality of the sense of smell
HP:0007010Poor fine motor coordination
HP:0009088Speech articulation difficulties
HP:0025401Staring gaze
HP:0031473Hostility
HP:0031843Bradyphrenia
HP:0031845Abnormal libido
Периодический (5–29%)19
HP:0001262Excessive daytime somnolence
HP:0002059Cerebral atrophy
HP:0002063Rigidity
HP:0002169Clonus
HP:0002300Mutism
HP:0002340Caudate atrophy
HP:0002500Abnormal cerebral white matter morphology
HP:0002540Inability to walk
HP:0002591Polyphagia
HP:0003107Abnormality of cholesterol metabolism
HP:0003487Babinski sign
HP:0010794Impaired visuospatial constructive cognition
HP:0030842Choking episodes
HP:0030955Alcoholism
HP:0031589Suicidal ideation
HP:0040140Degeneration of the striatum
HP:0045082Decreased body mass index
HP:0100785Insomnia
HP:0200136Oral-pharyngeal dysphagia

Эпидемиология (32)

Point prevalence
1-9 / 100 000
Greece
Annual incidence
1-9 / 1 000 000
Worldwide
Point prevalence
1-9 / 100 000
Worldwide
Point prevalence
1-5 / 10 000
Europe
Point prevalence
1-5 / 10 000
Ireland
Annual incidence
1-9 / 1 000 000
United Kingdom
Point prevalence
1-9 / 100 000
United Kingdom
Annual incidence
1-9 / 1 000 000
Italy
Point prevalence
1-9 / 100 000
Italy
Annual incidence
1-9 / 1 000 000
Canada
Annual incidence
1-9 / 1 000 000
United States
Point prevalence
1-9 / 100 000
United States
Point prevalence
1-9 / 1 000 000
Japan
Annual incidence
1-9 / 1 000 000
Australia
Point prevalence
1-9 / 100 000
Australia
Annual incidence
<1 / 1 000 000
China
Point prevalence
1-9 / 1 000 000
China
Point prevalence
1-9 / 100 000
Slovenia
Annual incidence
<1 / 1 000 000
Taiwan, Province of China
Point prevalence
1-9 / 1 000 000
Taiwan, Province of China
Annual incidence
1-9 / 1 000 000
Iceland
Point prevalence
1-9 / 100 000
Iceland
Point prevalence
1-9 / 100 000
Norway
Point prevalence
1-9 / 100 000
Sweden
Annual incidence
1-9 / 1 000 000
Greece
Point prevalence
1-9 / 1 000 000
Finland
Point prevalence
1-5 / 10 000
Egypt
Point prevalence
1-9 / 100 000
Cyprus
Annual incidence
1-9 / 1 000 000
Cyprus
Point prevalence
1-9 / 100 000
Denmark
Point prevalence
1-9 / 100 000
Spain
Annual incidence
1-9 / 1 000 000
Spain

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы