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Moderate multiminicore disease with hand involvement

ORPHA:178145Clinical subtypeAutosomal dominantChildhood, Infancy, Neonatal

Ассоциированные гены (1)

RYR1
ryanodine receptor 1
Disease-causing germline mutation(s) in
OMIM: 180901

Фенотипы (14)

Частый (30–79%)11
HP:0001319Neonatal hypotonia
HP:0001382Joint hypermobility
HP:0001762Talipes equinovarus
HP:0002194Delayed gross motor development
HP:0003324Generalized muscle weakness
HP:0003327Axial muscle weakness
HP:0008954Intrinsic hand muscle atrophy
HP:0008959Distal upper limb muscle weakness
HP:0009046Difficulty running
HP:0010628Facial palsy
HP:0012391Hyporeflexia of upper limbs
Периодический (5–29%)3
HP:0003803Type 1 muscle fiber predominance
HP:0004976Knee dislocation
HP:0005001Recurrent patellar dislocation

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы