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(Referência obtida automaticamente do Web of Science, por meio da informação sobre o financiamento pela FAPESP e o número do processo correspondente, incluída na publicação pelos autores.)

pinal cord injury-related thermoregulatory impairment masks a fatal malignant hyperthermia crisis: a case repor

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Autor(es):
Andrade, Pamela V. [1] ; Souza, Lucas S. [2] ; Santos, Joilson M. [1] ; Alves, Jose F. F. [3] ; Lutke, Claudia [1] ; Amaral, Jose L. G. [1] ; Vainzof, Mariz [2] ; Silva, Helga C. A. [1]
Número total de Autores: 8
Afiliação do(s) autor(es):
[1] Univ Fed Sao Paulo, Discipline Anesthesiol Pain & Intens Care, Brazilian Malignant Hyperthermia Unit, Rua Pedro Toledo, 781, BR-04039032 Sao Paulo, SP - Brazil
[2] Univ Sao Paulo, Human Genome & Stem Cells Res Ctr, Inst Biosci, Sao Paulo, SP - Brazil
[3] Hosp Minist Costa Cavalcante, Av. Gramado, 580, Foz Iguacu, BR-85860460 Parana - Brazil
Número total de Afiliações: 3
Tipo de documento: Artigo Científico
Fonte: Canadian Journal of Anesthesia : Journal canadien d'anesthésie; v. 69, n. 3 DEC 2021.
Citações Web of Science: 0
Resumo

Purpose Malignant hyperthermia (MH) is a hypermetabolic disorder that can occur in genetically susceptible individuals exposed to halogenated anesthetics and succinylcholine. Spinal cord injury (SCI) above the sixth thoracic vertebra is associated with dysfunction of the sympathetic/parasympathetic nervous pathways, including thermoregulatory dysfunction, presenting as hypothermia in cold environments because of vasodilation and heat loss. This effect could mitigate or obscure an MH episode. Here, we describe development of a fatal MH crisis in a patient with SCI. Clinical features A 27-yr-old male patient with an SCI after fracture of the sixth cervical vertebra was admitted for spinal arthrodesis. Anesthetic medications included remifentanil, propofol, succinylcholine, rocuronium, and isoflurane. After the start of the surgery, muscular contractures resembling myoclonus were noted, which resolved with pancuronium administration. Four hours after the start of anesthesia, the patient presented with hyperthermia, hypercarbia, hypotension, muscle rigidity, arrhythmia, and cardiogenic shock, with metabolic/respiratory acidosis. Malignant hyperthermia was suspected and the treatment was started, but he developed cardiopulmonary arrest and died an hour and a half after the first cardiac arrest. Both parents were investigated and were found to have normal creatine kinase levels and positive in vitro contracture tests. His mother carried a variant in the ryanodine receptor type 1 (RYR1) gene (c.14918C>T), which is associated with MH. Conclusion Spinal cord injury-induced thermoregulatory dysfunction may obscure the early diagnosis of MH and lead to fatal outcome. (AU)

Processo FAPESP: 96/02222-3 - Padronização e implantação do teste da contratura muscular para estudo do espectro de doenças associadas à hipertermia maligna
Beneficiário:Helga Cristina Almeida da Silva
Modalidade de apoio: Bolsas no Brasil - Doutorado