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Miyoshi myopathy

ORPHA:45448DiseaseAutosomal recessiveAdult

Ассоциированные гены (1)

DYSF
dysferlin
Disease-causing germline mutation(s) in
OMIM: 603009

Фенотипы (25)

Частый (30–79%)14
HP:0001288Gait disturbance
HP:0003547Shoulder girdle muscle weakness
HP:0003551Difficulty climbing stairs
HP:0003698Difficulty standing
HP:0003731Quadriceps muscle weakness
HP:0003738Exercise-induced myalgia
HP:0003749Pelvic girdle muscle weakness
HP:0007126Proximal amyotrophy
HP:0007149Distal upper limb amyotrophy
HP:0008944Distal lower limb amyotrophy
HP:0008963Tibialis muscle weakness
HP:0008994Proximal muscle weakness in lower limbs
HP:0009053Distal lower limb muscle weakness
HP:0011399Tibialis atrophy
Периодический (5–29%)7
HP:0002505Loss of ambulation
HP:0003326Myalgia
HP:0008981Calf muscle hypertrophy
HP:0009027Foot dorsiflexor weakness
HP:0030051Tip-toe gait
HP:0031108Triceps weakness
HP:0200101Decreased/absent ankle reflexes
Исключён (0%)4
HP:0001626Abnormality of the cardiovascular system
HP:0002747Respiratory insufficiency due to muscle weakness
HP:0003552Muscle stiffness
HP:0008954Intrinsic hand muscle atrophy

Эпидемиология (3)

Annual incidence
1-9 / 1 000 000
Japan
Point prevalence
1-9 / 1 000 000
United Kingdom
Point prevalence
1-9 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы