Neuromuscular disorders : NMD · 2025
276th ENMC International Workshop: recommendations on optimal diagnostic pathway and management strategy for patients with acute rhabdomyolysis worldwide. 15th-17th March 2024, Hoofddorp, The Netherlands.
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Kruijt Nick, Laforet Pascal, Vissing John, Bhai Salman, Stemmerik Mads Godtfeldt, Kleefeld Felix, Voermans Nicol C, 276th ENMC workshop participants
Аннотация
The 276th ENMC International Workshop on rhabdomyolysis brought together 21 experts to address the compelling need for standardized guidelines on the clinical approach of rhabdomyolysis. There was a general agreement that a diagnosis of rhabdomyolysis require that 1) clinical symptoms include severe muscle swelling, weakness and/or myalgia; 2) serum CK-levels exceed 10,000 IU/L in case of exertional, and >5000 IU/L in non-exertional rhabdomyolysis; 3) CK-levels reaching a maximum 1-4 days after the event and normalizing to baseline within 1-2 weeks of rest. In case of an underlying neuromuscular condition, CK-levels should exceed 5-10 times the patient's baseline level. Treatment should be initiated only in case of high risk on acute kidney injury, which can be predicted by the McMahon score. Furthermore, recommendations on performing genetic testing were formulated and the use of the 'RHABDO'- acronym was generally agreed upon as a tool to aid clinicians in deciding which patients require genetic testing. Moreover, recommendations on follow-up were made, with a particular emphasis on evaluation of physical and psychological sequelae. Patient representatives present during the workshop emphasized the importance of the current recommendations for future clinical guidelines on rhabdomyolysis.