Metabolic Rhabdomyolysis: update
REVISTA MEDICA CLINICA LAS CONDES(2018)
摘要
Rhabdomyolysis results from the destruction of muscle tissue and the release of its components into the systemic circulation, which can be potentially serious. Causes can be acquired or hereditary (genetic-metabolic), and according to the pathophysiological mechanisms have been classified into metabolic causes (failure in the production of energy), structural (structural myopathies or dystrophies), by abnormalities in the calcium pump and inflammatory causes. Regardless of the cause of rhabdomyolysis, the common final mechanism is the sarcolemma injury, either by increased intracellular calcium or by failure of energy production, which leads to fiber necrosis and, consequently, the release to the circulation of electrolytes and intracellular proteins. Metabolic abnormalities most frequently reported with rhabdomyolysis are Carnitine palmitoyltransferase II deficiency (CPT II), glycolysis defects, and mutations in the Lipin 1 gene (LPIN1), which have been described recently. Under an episode of rhabdomyolysis, potentially treatable acquired disorders should be ruled out initially, and then studies of structural or metabolic myopathies should be carried out. Regarding management, in acute phase aims to preserve renal function and restore metabolic abnormalities, through an early and adequate volume, ensuring adequate diuresis, and in situations of extreme acidosis or hyperkalemia, should be considered hemodialysis or therapies of continuous renal replacement. Consideration should be given to serious complications in this clinical picture, such as disseminated intravascular coagulation and compartment syndromes that may require early multiple fasciotomies. Specifically, in mutations in the gene of LPIN1, which cause up to 50% of episodes of rhabdomyolysis in the pediatric age, therapeutic strategies should be directed to the prevention and early treatment of catabolism, through the use of intravenous solutions with high concentrations of glucose.
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关键词
Metabolic myopathies, rhabdomyolysis
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