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Tibial muscular dystrophy

ORPHA:609DiseaseAutosomal dominant, Autosomal recessiveAdult

Ассоциированные гены (1)

TTN
titin
Disease-causing germline mutation(s) in
OMIM: 188840

Фенотипы (19)

Частый (30–79%)12
HP:0003198Myopathy
HP:0003376Steppage gait
HP:0003458EMG: myopathic abnormalities
HP:0003557Increased variability in muscle fiber diameter
HP:0003687Centrally nucleated skeletal muscle fibers
HP:0003805Rimmed vacuoles
HP:0008180Mildly elevated creatine kinase
HP:0009027Foot dorsiflexor weakness
HP:0009049Peroneal muscle atrophy
HP:0009058Increased muscle lipid content
HP:0031374Ankle weakness
HP:0001288Gait disturbance
Периодический (5–29%)3
HP:0002312Clumsiness
HP:0003731Quadriceps muscle weakness
HP:0008994Proximal muscle weakness in lower limbs
Очень редкий (1–4%)1
HP:0008959Distal upper limb muscle weakness
Исключён (0%)3
HP:0001638Cardiomyopathy
HP:0002878Respiratory failure
HP:0009077Weakness of long finger extensor muscles

Эпидемиология (2)

Point prevalence
1-9 / 100 000
Europe
Point prevalence
1-5 / 10 000
Finland

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы