| Literature DB >> 33625594 |
Yasuo M Tsutsumi1, Nami Kakuta2, Ryosuke Kawanishi2, Katsuya Tanaka2, Rieko Kanzaki3, Atsushi Morio3, Yuko Noda3, Hirotsugu Miyoshi3, Takashi Kondo3, Keiko Mukaida4.
Abstract
Malignant hyperthermia (MH) is a severe hypermetabolic disorder associated with dysregulation of calcium homeostasis and is triggered by inhalational anesthetics (isoflurane, sevoflurane, desflurane) and a depolarizing muscle relaxant (succinylcholine). We report the case of a 16-day-old infant undergoing laparoscopic surgery. The patient developed hyperthermia and hypercarbia with muscle rigidity. After the diagnosis of MH, dantrolene was administered with sufficient hydration. The patient was transferred to the pediatric intensive care unit for monitoring and treatment of acute renal injury due to myoglobinuria. Subsequently, two variants of the ryanodine receptor 1 (RYR1) gene were identified in the patient as the mutation point at c.1589G > A p.Arg530His and c.1841G > T p.Arg614Leu, which are known to be associated with MH. This was a rare case of MH in a 16-day-old infant that might be related to two RYR1 mutations inherited from the parents.Entities:
Keywords: Dantrolene; Malignant hyperthermia; RYR1; Sevoflurane
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Year: 2021 PMID： 33625594 DOI： 10.1007/s00540-021-02902-2
Source DB: PubMed Journal: J Anesth ISSN： 0913-8668 Impact factor: 2.078