← Назад

Central core disease

ORPHA:597DiseaseAutosomal dominantChildhood

Ассоциированные гены (1)

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

Фенотипы (25)

Частый (30–79%)16
HP:0001382Joint hypermobility
HP:0001252Hypotonia
HP:0001270Motor delay
HP:0001374Congenital hip dislocation
HP:0001634Mitral valve prolapse
HP:0001762Talipes equinovarus
HP:0001763Pes planus
HP:0002047Malignant hyperthermia
HP:0002751Kyphoscoliosis
HP:0002828Multiple joint contractures
HP:0003198Myopathy
HP:0003388Easy fatigability
HP:0003552Muscle stiffness
HP:0003749Pelvic girdle muscle weakness
HP:0003803Type 1 muscle fiber predominance
HP:0030230Central core regions in muscle fibers
Периодический (5–29%)4
HP:0000602Ophthalmoplegia
HP:0002483Bulbar signs
HP:0003798Nemaline bodies
HP:0040081Abnormal circulating creatine kinase concentration
Очень редкий (1–4%)4
HP:0001989Fetal akinesia sequence
HP:0002643Neonatal respiratory distress
HP:0002747Respiratory insufficiency due to muscle weakness
HP:0003236Elevated circulating creatine kinase concentration
Исключён (0%)1
HP:0040191Rectus femoris muscle atrophy

Эпидемиология (2)

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