Abstract

Malignant hyperthermia is a pharmacogenetic disorder in the regulation of calcium in skeletal muscles which is related to an uninhibited muscle hypermetabolic reaction to potent inhalation agents, the depolarizing muscle relaxant succinylcholine, and to stressors such as vigorous exercise and heat. MH is diagnosed by the clinical presentation of the disease and laboratory testing. There are a few previous studies working on if there is an association between the occurrence of malignant hyperthermia and the existence of glucose 6-phosphate dehydrogenase (G6PD) deficiency, and there was no report on growth hormone doping in the literature. So, our main goal was to show this rare case of malignant hyperthermia seen in a G6PD patient with growth hormone abuse who underwent surgery and to find if there is an association between G6PD deficiency, growth hormone abuse, and malignant hyperthermia. Our patient was a 17-year-old boy with right lower quadrant abdominal pain and tenderness who underwent appendectomy. At the end of the operation, the patient developed with an increased heart rate (sinus tachycardia), increased body temperature and end-tidal carbon dioxide (ETCO2) level, masseter muscle rigidity, and then, generalized body rigidity, so the malignant hyperthermia susceptibility was considered. The patient was managed by cooling down the patient and the administration of dantrolene. We could hypothesize that malignant hyperthermia might be associated with G6PD deficiency as a triggering factor, but has no association with recombinant human growth hormone (rhGH) abuse. Another main lesson which this study tells us is to make a careful and proper history taking before going on an operation for preoperative evaluation and identification of patients with any form of suspicious drug abuse in order not to receive volatile inhalational agents and, also, performing some preventive measures including avoidance of heat extremes and restricting athletic activity in a patient with a history of malignant hyperthermia, and if the malignant hyperthermia susceptibility is suspected, urgent management should be carried out. As the association between G6PD deficiency, human growth hormone abuse, and malignant hyperthermia has remained unclear up-to-date, further potent studies are seriously needed in the future.

Highlights

  • Malignant hyperthermia (MH) is a pharmacogenetic disorder in the regulation of calcium in skeletal muscles which is related to an uninhibited muscle hypermetabolic reaction to potent inhalation agents, the depolarizing muscle relaxant succinylcholine, and to stressors such as vigorous exercise and heat

  • E patient had been in the hospital for twelve days; the first three nights were in the intensive care unit (ICU) for stabilization and, till the discharge day in the surgical ward. e patient was under close observation, and continuous monitoring of heart, blood pressure, temperature, and urine output was performed while checking for arterial blood gases (PH, partial pressure of carbon dioxide (PCO2), HCO3− and base excess in the extracellular fluid compartment (BEecf )), white blood cell count (WBC), Hb, platelet count, liver enzymes as aspartate aminotransferase (AST), alanine aminotransferase (ALT), total and direct bilirubin, alkaline phosphatase (AlK–P), albumin (Alb), blood urea nitrogen (BUN), creatinine (Cr), blood sugar (BS), electrolytes (Na+ and K+), calcium (Ca2+), creatine phosphokinase (CPK), creatine kinase-muscle/brain (CKMB), and lactate dehydrogenase (LDH) (Tables 1 and 2)

  • In the pathophysiology of the MH, the triggering elements may cause the calcium stores to be released from the sarcoplasmic reticulum, uncontrolled entry of calcium from the myoplasm, activation of biochemical routes related to muscle activation causing the contracture of skeletal muscles, glycogenolysis, and increased cellular metabolism, all of which resulting in the production of heat and increased lactate level and acidosis [1, 2]. e mechanism of dantrolene is to act as an antagonist, and it binds to a specific region in the ryanodine receptor 1 (RYR-1) channel

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Summary

Background

Malignant hyperthermia (MH) is a pharmacogenetic disorder in the regulation of calcium in skeletal muscles which is related to an uninhibited muscle hypermetabolic reaction to potent inhalation agents (such as halothane, isoflurane, sevoflurane, and desflurane), the depolarizing muscle relaxant succinylcholine, and to stressors such as vigorous exercise and heat (environmental temperature). Another reason related to the development of MH is the genetic background and family history [1,2,3]. Our main goal was to show this rare case of malignant hyperthermia seen in a G6PD deficient patient with growth hormone doping who underwent surgery and to find if there is an association between G6PD deficiency, growth hormone doping or abuse, and MH

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