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Dysequilibrium syndrome

ORPHA:1766DiseaseAutosomal recessiveNeonatal

Ассоциированные гены (5)

VLDLR
very low density lipoprotein receptor
Disease-causing germline mutation(s) in
OMIM: 192977
WDR81
WD repeat domain 81
Disease-causing germline mutation(s) in
OMIM: 614218
CA8
carbonic anhydrase 8
Disease-causing germline mutation(s) in
OMIM: 114815
ATP8A2
ATPase phospholipid transporting 8A2
Disease-causing germline mutation(s) in
OMIM: 605870
TUBB2B
tubulin beta 2B class IIb
Disease-causing germline mutation(s) in
OMIM: 612850

Фенотипы (14)

Очень частый (80–99%)5
HP:0001249Intellectual disability
HP:0001251Ataxia
HP:0001252Hypotonia
HP:0001288Gait disturbance
HP:0001347Hyperreflexia
Частый (30–79%)6
HP:0000486Strabismus
HP:0001250Seizure
HP:0003202Skeletal muscle atrophy
HP:0004322Short stature
HP:0100021Cerebral palsy
HP:0100022Abnormality of movement
Периодический (5–29%)3
HP:0000478Abnormality of the eye
HP:0000504Abnormality of vision
HP:0000518Cataract

Эпидемиология (2)

Point prevalence
<1 / 1 000 000
Worldwide
Cases/families
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы