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Malignant hyperthermia of anesthesia

ORPHA:423DiseaseAutosomal dominantAll ages

Ассоциированные гены (2)

CACNA1S
calcium voltage-gated channel subunit alpha1 S
Major susceptibility factor in
OMIM: 114208
RYR1
ryanodine receptor 1
Disease-causing germline mutation(s) in
OMIM: 180901

Фенотипы (23)

Частый (30–79%)11
HP:0001942Metabolic acidosis
HP:0001945Fever
HP:0002047Malignant hyperthermia
HP:0002789Tachypnea
HP:0002905Hyperphosphatemia
HP:0003552Muscle stiffness
HP:0004755Supraventricular tachycardia
HP:0004756Ventricular tachycardia
HP:0011964Intermittent painful muscle spasms
HP:0012416Hypercapnia
HP:0031320Cardiomyocyte mitochondrial proliferation
Периодический (5–29%)12
HP:0001722High-output congestive heart failure
HP:0001919Acute kidney injury
HP:0002153Hyperkalemia
HP:0002913Myoglobinuria
HP:0003256Abnormality of the coagulation cascade
HP:0006554Acute hepatic failure
HP:0006682Ventricular extrasystoles
HP:0008331Elevated creatine kinase after exercise
HP:0008942Acute rhabdomyolysis
HP:0008978Necrotizing myopathy
HP:0009045Exercise-induced rhabdomyolysis
HP:3000005Abnormality of masseter muscle

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы