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Hartnup disease

ORPHA:2116DiseaseAutosomal recessiveAll ages

Ассоциированные гены (2)

CLTRN
collectrin, amino acid transport regulator
Disease-causing germline mutation(s) in
OMIM: 300631
SLC6A19
solute carrier family 6 member 19
Disease-causing germline mutation(s) in
OMIM: 608893

Фенотипы (30)

Очень частый (80–99%)11
HP:0000712Emotional lability
HP:0000738Hallucinations
HP:0000739Anxiety
HP:0000992Cutaneous photosensitivity
HP:0001251Ataxia
HP:0001252Hypotonia
HP:0001347Hyperreflexia
HP:0002076Migraine
HP:0002353EEG abnormality
HP:0008353Neutral hyperaminoaciduria
HP:0012086Abnormal urinary color
Частый (30–79%)8
HP:0000486Strabismus
HP:0000504Abnormality of vision
HP:0000613Photophobia
HP:0000639Nystagmus
HP:0000988Skin rash
HP:0001337Tremor
HP:0002024Malabsorption
HP:6000130Elevated urinary indican level
Периодический (5–29%)11
HP:0000206Glossitis
HP:0000230Gingivitis
HP:0000709Psychosis
HP:0001053Hypopigmented skin patches
HP:0001249Intellectual disability
HP:0001250Seizure
HP:0001263Global developmental delay
HP:0002383Infectious encephalitis
HP:0004322Short stature
HP:0007400Irregular hyperpigmentation
HP:0008066Abnormal blistering of the skin

Эпидемиология (5)

Point prevalence
1-9 / 100 000
Worldwide
Prevalence at birth
1-9 / 100 000
Australia
Prevalence at birth
1-9 / 100 000
United States
Prevalence at birth
1-9 / 100 000
Specific population
Point prevalence
1-9 / 100 000
United Kingdom

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы