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Malignant hyperthermia

A case study.

Learn how positioning injuries occur and the best methods for preventing these injuries for optimum patient care.

YOU'RE CARING FOR Don McMann, 42, in the postanesthesia care unit (PACU) when you notice muscle rigidity and see that he's becoming tachypneic and tachycardic. His blood pressure is 154/90 and his temperature, which was 98° F (37° C) preoperatively and 100° F (38° C) at PACU admission, has jumped to 104° F (40° C).

Mr. McMann underwent arthroscopic repair of a torn anterior cruciate ligament and arrived in the PACU about 5 minutes ago. He has no significant medical or surgical history. Reviewing his medical record, you see he received isoflurane for general anesthesia. He's receiving supplemental oxygen by nasal cannula.

Mr. McMann's signs and symptoms make you suspect malignant hyperthermia (MH), a rare but potentially deadly disorder that can be triggered by certain anesthetics (isoflurane, halothane, enflurane, sevoflurane, and desflurane) and the skeletal muscle relaxant succinylcholine. Other signs and symptoms of MH include generalized erythematous flush followed by mottled skin and cyanosis, increased end-tidal carbon dioxide production, myoglobinuria, elevated serum creatine kinase, and hyperkalemia.

An inherited hypermetabolic syndrome, MH, once triggered, causes sustained skeletal muscle contraction that can lead to severe hyperthermia, left ventricular failure, brain damage, organ failure, disseminated intravascular coagulation, cardiac arrest, and death.

Recognizing symptoms early and discontinuing anesthesia promptly are imperative. Goals of treatment are to decrease metabolism, reverse metabolic and respiratory acidosis, correct dysrhythmias, decrease body temperature, provide oxygen and nutrition to tissues, and correct electrolyte imbalance.

Early diagnosis and treatment of MH are crucial. Administer 100% oxygen via non-rebreather mask and stay with the patient to continue assessing his cardiac rhythm and vital signs. Notify the anesthesia provider and call for the MH cart.

Make sure Mr. McMann's intravenous (I.V.) line is patent, remove the sheets and blankets covering him, and lower the room temperature. Other measures to reduce core body temperature include administering chilled 0.9% sodium chloride solution I.V.; placing ice packs at his groin, axillae, and neck; and applying a temperature-regulating blanket. Monitor his core body temperature. To prevent hypothermia, discontinue cooling measures when his temperature reaches 100° F.

The anesthesia provider arrives and orders I.V. dantrolene, a direct-acting skeletal muscle relaxant and the drug of choice for treating MH. Reconstitute the dantrolene as directed. Administer an initial dose of 2.5 mg/kg by rapid I.V. push. Repeat this dose, up to a maximum of 10 mg/kg, until signs and symptoms of MH resolve. Monitor Mr. McMann closely for respiratory depression.

Your colleague inserts an indwelling urinary catheter to monitor for urine output and signs of myoglobinuria, such as brown urine. Obtain blood samples for testing, including arterial blood gas analysis. Monitor for elevated potassium, calcium, magnesium, and creatine kinase levels and assess for signs of respiratory or metabolic acidosis. If necessary, give sodium bicarbonate to correct metabolic acidosis and I.V. glucose and regular insulin to treat hyperkalemia.

If Mr. McMann develops cardiac arrhythmias, treat them according to advanced cardiac life support guidelines. However, don't give calcium channel blockers, which may interact with dantrolene and cause severe hyperkalemia, ventricular fibrillation, and cardiac arrest.

Once Mr. McMann is stabilized, he's transferred to the intensive care unit. He'll receive 1 mg/kg of I.V. dantrolene every 4 to 6 hours for 24 to 48 hours to prevent MH recurrence. He'll also be closely monitored for complications such as renal failure and disseminated intravascular coagulation.

Before discharge, teach Mr. McMann about MH and refer him to the Malignant Hyperthermia Association of the United States for more information (1-800-644-9737 or http://www.mhaus.org ). Encourage him to wear a medical-alert bracelet and to let his healthcare providers know about his MH episode.

Adapted from Malignant hyperthermia, Nursing2005, H Carter-Templeton, 35(6):88, June 2005.

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Malignant Hyperthermia Syndrome: A Clinical Case Report

Affiliations.

1 Hospital Comarcal de Laredo, Laredo, Cantabria, Spain.
2 Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain.
PMID: 34421497
PMCID: PMC8343040

Malignant hyperthermia is a pharmacogenetic disorder. It manifests as a hypercatabolic skeletal muscle syndrome linked to inhaled volatile anesthetics or depolarizing muscle relaxants. Its clinical signs and symptoms are tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, rhabdomyolysis, hyperkalemia, arrhythmia and renal failure. Mortality without specific treatment is 80% and decreases to 5% with the use of dantrolene sodium. This article presents the case of a 39-year-old patient admitted to the Intensive Care Unit for malignant hyperthermia after surgery for septoplasty plus turbinoplasty.

Keywords: dantrolene; desflurane; inhaled anesthetics; malignant hyperthermia; ryanodine; succinylcholine.

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Saudi J Anaesth
v.11(2); Apr-Jun 2017

A case report of suspected malignant hyperthermia where patient survived the episode

Department of Anaesthesiology and Critical Care, SMHS Hospital, GMC, Srinagar, Jammu and Kashmir, India

Shoaib Badoo

Ruqsana naqeeb.

Malignant hyperthermia is rare inherited disorder in our part of the world; there are only few cases reported in literature in India who were suspected of having this condition. The overall incidence of malignant hyperthermia during general anesthesia is estimated to range from 1: 5000 to 1: 50,000–100,000 and mortality rate is estimated to be <5% in the presence of standard care. In India, there is no center where in vitro halothane caffeine contraction test is performed to confirm diagnosis in suspected cases. Second, dantrolene drug of choice for this condition is not freely available in market in India and is stored only in some hospitals in few major cities. Among the cases reported of suspected of malignant hyperthermia in India almost 50% have survived the condition despite nonavailability of dantrolene emphasizing role of early detection and aggressive management in these cases.

Introduction

Malignant hyperthermia is a relatively rare disease in India with very few case reports present in the literature in this regard. Malignant hyperthermia was brought into attention for anesthetic world by deaths attributable to general anesthetics in a family living in Melbourne, Australia.[ 1 ] Incidence of malignant hyperthermia during general anesthesia is estimated to range from 1: 5000 to 1: 50,000–100,000.[ 2 , 3 ] The mortality rate is estimated to be <5%, with early detection of malignant hyperthermia episode, using capnography, prompt use of the drug dantrolene, and the introduction of diagnostic testing.[ 2 ]

Case Report

We present a case report of a 45-year-old female operated for parotid tumor presenting with features suspected of malignant hyperthermia. On preanesthetic evaluation, patient had no comorbid condition was not on any drugs had no previous anesthetic exposure. She was suspected to have difficult airway in view of swelling due to tumor and mallampati Grade 3. On the day of surgery, venous access was established minimum basic monitoring was attached to the patient. Her blood pressure was 130/80 mmHg, heart rate 78 beats/min and oxygen saturation were 98% on the operation table on the day of surgery.

Anesthesia was induced on propofol and suxamethonium and ventilated with oxygen and halothane. Laryngoscopy was difficult due to masseter rigidity attributed to suxamethonium; patient was intubated with size 7.5 ID cuffed polyvinyl endotrachial tube and connected to ventillator. The patient was given morphine for analgesia; anesthesia was maintained with 50% oxygen: 50% nitrous oxide and isoflurane with boluses of atracurium.

Ten minutes into surgery a rising trend in endtidal CO 2 was noticed, initially, adjustment in minute ventilation, circuit check, sodalime replacement was done, but endtidal CO 2 continued to rise with maximum rise of endtidal to 80 mmHg. There was associated rise in temperature with maximum reading of 104 F recorded by nasopharyngeal probe. The patient also had risen in heart rate and blood pressure with maximum reading of 150 beats/min and 180 mmHg, respectively. Suspecting malignant hyperthermia isoflurane was stopped propofol infusion was started, and patient was ventilated with 100% oxygen through a fresh banes circuit using higher gas flows and higher minute ventilation.

Surgeon was informed and asked to expedite surgery. Active cooling was started with ice cold saline intravenously and irrigation through Ryles tube and bladder catheter. Ice packs and cold towels were used for surface cooling to control the rising temperature. Blood gas sample at this time showed the following result: pH-7.12, pCO 2 -96 mmHg, pO 2 -224 mmHg, base excess-6, HCO 3 -20 mEq/L, Na-142 mEq/L, and K-5.5 mEq/L suggestive mixed respiratory and metabolic acidosis. Patient's other tests done intraoperatively were creatinine kinase −1300 IU LDH-120 IU, blood urea-20 mg%, s. creatinine-1.2 mg%, blood sugar 138.9 mg%. Patient also showed features of rigidity in limbs along with mottling. Dantrolene drug of choice for malignant hyperthermia could not be used due nonavailability of the drug in our hospital.

With active cooling patients temperature stabilized and then started to drop toward normal. With high minute ventilation and higher flows end tidal and PaCO 2 were also controlled and then started to drop. Surgery was completed within 2 h and patient was shifted to Intensive Care Unit for postoperatively management. Blood samples including thyroid function test and urine for myoglobin sent from Intensive Care Unit were within normal limits. The patient was extubated after 2 h of ventilation once endtidal CO 2 temperature and acid-base status returned to normal and patient achieved criteria for extubation.

Patient and her attendants were made aware of suspected diagnosis of malignant hyperthermia in her and risks of recurrence in the patient and other family members on future exposure to anesthesia. The episode was also mentioned in anesthesia record of the patient for future reference.

Malignant hyperthermia is a myopathy associated with abnormal skeletal muscle calcium homeostasis in response to triggering agents such as succinylcholine and halothane. Sustained high levels of calcium in sarcoplasmic reticulum lead to increased aerobic and glycolytic metabolism leading to acidosis, rigidity, altered permeability, and hyperkalemia.[ 4 ] Diagnosis of malignant hyperthermia is based on clinical parameters at the time of crisis which is later confirmed by muscle biopsy test.

Larach et al .[ 5 ] described a scoring system to label a patient of hypermetabolic crisis as malignant hyperthermia using different patient parameters during this crisis [Tables [Tables1 1 and and2]. 2 ]. According to this grading, a patient with a score >50 points is definitely a case of malignant hyperthermia. Our patient had a score of 68 points [ Table 3 ] which was highly suggestive of malignant hyperthermia in this patient. Furthermore, other causes of hypermetabolic crisis such as thyroid storm, neuroleptic malignant syndrome, and pheochromocytoma were ruled out by normal thyroid function test, patient not being on any antipsychotic drugs and having no history suggestive of pheochromocytoma.

Malignant hyperthermia clinical grading scale[ 5 ]

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Clinical significance of malignant hyperthermia raw score and its rank[ 5 ]

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Malignant hyperthermia score in patient

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For definitive diagnosis of malignant hyperthermia in vitro halothane caffeine contraction test is used.[ 6 ] This test has to be done after 3 months of hypermetabolic crisis[ 7 ] genetic research into the condition implicate the ryanodine receptor gene (RYR1) located on chromosome 19[ 8 , 9 ] as cause of malignant hyperthermia. DNA testing is now used routinely for diagnosis before muscle biopsy when a familial RYR1 mutation is known.[ 10 ]

First case of malignant hyperthermia in India was reported in 2001 by Punj et al .[ 11 ] patient developed a gradual increase in heart rate, PaCO 2 , temperature 44°C, pH 7.17, bicarbonate concentration 19.7 mmol/L, potassium concentration 6 mmol/L, and creatine kinase concentration 29,900 IU/L. Followed by disseminated intravascular coagulation with hematuria and patient died 12 h after the initial episode. Similar cases were reported by Gupta et al .[ 12 ] and Pillai et al .[ 13 ] who succumbed in spite of aggressive supportive measures.

Saxena and Dua[ 4 ] and Gopalakrishnan et al .[ 14 ] also reported cases who survived the episode of malignant hyperthermia without use dantrolene as was the case in our patient.

Currently, there is no center in India which performs IVHCT, so we were not able to offer it to the patient in order to confirm the diagnosis of malignant hyperthermia. Dantrolene, the drug of choice for this disease, is not freely available in market is stocked in only few hospitals in our country. Hence, could not be used in this patient as it was not available in our hospital. Although license for import of dantrolene can be obtained within few days dantrolene is not available in market due to its limited use, its cost, and storage facility needed for the drug.

Since more cases of malignant hyperthermia have been recorded in people of Indian subcontinent descent in the United Kingdom than in India, this discrepancy may suggest lack of essential monitoring, as may be the case in some peripheral centers and nonavailability of accredited diagnostic center for diagnosis.[ 5 ]

Time has come for more awareness about possibility of malignant hyperthermia in our patient as early awareness and proper management even in the absence of dantrolene can improve survival in these patients. Furthermore, diagnostic center for diagnosis of malignant hyperthermia must be made available, and dantrolene must be kept available at many more hospitals so that these patients could have best chance of survival.

Financial support and sponsorship

Conflicts of interest.

There are no conflicts of interest.


	Case Study: Hyperthermia

Hyperthermia

Case Presentation

It was July 20 in Houston and the fourth straight day that would have a high temperature above 100°F. Janice was running some errands and decided to stop by her mother's house. Janice's mother, Marian, was eighty-four and in pretty good health. She was able to keep up with her housekeeping and still tended a small garden in her backyard. Just that morning, Janice had told her mother not to spend too much time working in the garden today. Janice knew that the heat could be dangerous, especially to the elderly, and her mother's place didn't have an air conditioner, but Janice felt that her mother was alert enough to know her own limits.

When Janice reached her mother's house, she found her mother unconscious on the couch in the living room. All of the windows in the house were closed. Janice immediately tried to rouse her mother and was able to get her to say a few words, but Marian seemed delirious. Janice grabbed the telephone and called for help. The emergency services operator instructed Janice to apply cold wash cloths to her mother's forehead and face and if possible to position her mother in front of a fan while using a spray bottle to spray tepid water on her skin.

When the paramedics arrived Marian was conscious but confused and feeling nauseous. At the hospital the doctor told Janice just how lucky she was to have visited Marian at that moment. He informed Janice that Marian had suffered heat stroke, a form of hyperthermia and that Janice's quick action at the house had saved her mother's life. Marian was making rapid progress to recovery but was being given fluids and electrolytes intravenously and was going to stay in the hospital overnight for observation.

Case Background

Hyperthermia occurs when the body temperature increases without an increase in the set point of the thermoregulatory center in the hypothalamus. Heat exhaustion and heatstroke are two common forms of hyperthermia. Symptoms of heat exhaustion include thirst, fatigue, profuse sweat, and giddiness or delirium. Individuals with heat exhaustion generally have a normal or only slightly elevated body temperature and the symptoms are the result of the loss of water and electrolytes. Symptoms of heatstroke include a temperature of 104°F, absence of sweating, and loss of consciousness. If untreated, heat exhaustion precedes heatstroke, and heat stroke is often fatal. Treatment for hyperthermia consists of reducing the body temperature to normal. Special attention is placed on reducing the temperature of the brain as tissue damage can result if the body temperature rises above 109°F.

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COMMENTS

Malignant Hyperthermia Syndrome: A Clinical Case Report
Malignant hyperthermia is a pharmacogenetic disorder. It manifests as a hypercatabolic skeletal muscle syndrome linked to inhaled volatile anesthetics or depolarizing muscle relaxants. Its clinical signs and symptoms are tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, rhabdomyolysis, hyperkalemia, arrhythmia and renal failure ...
Case Study: Hyperthermia
Case Study: Hyperthermia. It was July 20 in Houston and the fourth straight day that would have a high temperature above 100°F. Janice was running some errands and decided to stop by her mother's house. Janice's mother, Marian, was eighty-four and in pretty good health. She was able to keep up with her housekeeping and still tended a small ...
Hole's Human Anatomy & Physiology
Hyperthermia occurs when the body temperature increases without an increase in the set point of the thermoregulatory center in the hypothalamus. Heat exhaustion and heatstroke are two common forms of hyperthermia. Symptoms of heat exhaustion include thirst, fatigue, profuse sweat, and giddiness or delirium.
Malignant hyperthermia: a case report with good outcome
Malignant hyperthermia is a rare, life-threatening and hypermetabolic response, occurring most often during general anesthesia, with an incidence estimated to be 1:100,000. 1 It is a pharmacogenetic condition affecting Ca 2+ homeostasis in skeletal muscle, which requires an autosomal dominant genetic defect and a trigger agent like volatile ...
A case report of malignant hyperthermia in a patient with
1 Introduction. Malignant hyperthermia (MH) is a fatal pharmacogenetic disorder of skeletal muscle resulting from general anesthesia upon exposure to potent halogenated volatile anesthetics or succinylcholine. It manifests various clinical signs of hypermetabolism including hypercapnia, tachycardia, and generalized muscle rigidity, followed by ...
Hyperthermia: From Diagnostic and Treatments to New Discoveries
INTRODUCTION. Hyperthermia is an important approach for the treatment of several diseases [1, 2].Hyperthermia is also thought to induce hypertrophy of skeletal muscles in vitro and in vivo [], but molecular mechanisms for these effects remain elusive.. Much of the current knowledge about the effects of heat shock proteins in striated muscles is the result of pioneering studies by Mestril ...
Malignant hyperthermia: A case study : Nursing Management
Mr. McMann's signs and symptoms make you suspect malignant hyperthermia (MH), a rare but potentially deadly disorder that can be triggered by certain anesthetics (isoflurane, halothane, enflurane, sevoflurane, and desflurane) and the skeletal muscle relaxant succinylcholine. Other signs and symptoms of MH include generalized erythematous flush ...
Malignant hyperthermia in a young man: A case report
Malignant hyperthermia is a life-threatening reaction to halogenated anesthetics. Symptoms of malignant hyperthermia are nonspecific, making its diagnosis challenging. Diagnostic tests are relatively expensive and have low sensitivity. Management requires knowledge, staff education, and logistical preparedness.
Hyperthermia Case Presentation
hyperthermia consists of reducing the body temperature to normal. Special attention is placed on reducing the temperature of the brain as tissue damage can result if the body temperature rises above 109°F. 1. Define homeostasis and describe how it relates to hyperthermia. 2.
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Define homeostasis and describe how it relates to hyperthermia. Explain why elderly individuals with poor circulation would have a greater risk of suffering heat exhaustion or heatstroke. ... Clinical Case Study Hyperthermia Author: weavedan Last modified by: weavedan Created Date: 9/13/2005 11:19:00 AM
Malignant hyperthermia: a case report with good outcome
Malignant hyperthermia is a rare, life-threatening and hypermetabolic response, occurring most often during general anesthesia, with an incidence estimated to be 1:100,000. 1 It is a pharmacogenetic condition affecting Ca 2+ homeostasis in skeletal muscle, which requires an autosomal dominant genetic defect and a trigger agent like volatile ...
Malignant hyperthermia: A case report
In this case study, we have announced the successful management of a patient who underwent emergency surgery to repair small bowel perforation suspected of MH. The frequency of the first unique symptoms reported in cases of malignant hyperthermia is as follows: hypercarbia, 30.7 %; Master's spasm, 24.8 %; and sinus tachycardia, 21.1 % [14] .
Case Report of Malignant Hyperthermia in the Emergency Department
Malignant hyperthermia (MH) describes a condition of a hypermetabolic response to anesthetic gases (eg, halothane and sevoflurane) or the depolarizing muscle relaxant succinylcholine. 1 Although rare clinically (estimated incidence of 1 in 10,000 to 1 in 250,000 anesthesia procedures), the genetic abnormality may occur as frequently as one in ...
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Physiology 335 Laboratory Workshop: Case Study Malignant Hyperthermia Purpose: Why is this case study important to review? This case is an excellent example of how protein structure influences the functionality of the protein. Malignant Hyperthermia results from a genetic mutation to the ryanodine receptor located on skeletal muscle sarcoplasmic reticulum.
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Case Study encompassing Hyperthermia case study it was july 20 in houston and the fourth straight day that would have high temperature above janice was running. Skip to document. University; High School; Books; Discovery. ... Homeostasis, in a physiological sense, refers to the persistent monitoring and regulation done by the body in order to ...
Malignant Hyperthermia Syndrome: A Clinical Case Report
Case Reports. Malignant hyperthermia is a pharmacogenetic disorder. It manifests as a hypercatabolic skeletal muscle syndrome linked to inhaled volatile anesthetics or depolarizing muscle relaxants. Its clinical signs and symptoms are tachycardia, hyperthermia, hypercapnia, acidosis, muscle rigidity, rhabdomyolys ….
Frontiers
An increasing number of preclinical and clinical studies are focusing on heating tumors with nanoparticles: magnetic ... Ahmed K, Zaidi SF, Mati Ur R, Rehman R, Kondo T. Hyperthermia and protein homeostasis: Cytoprotection and cell death. J Therm Biol. (2020) 91:102615. doi: 10.1016/j.jtherbio.2020.102615. PubMed Abstract | CrossRef Full ...
Human Anatomy
Hyperthermia occurs when the body temperature increases without an increase in the set point of the thermoregulatory center in the hypothalamus. Heat exhaustion and heatstroke are two common forms of hyperthermia. Symptoms of heat exhaustion include thirst, fatigue, profuse sweat, and giddiness or delirium.
A case report of suspected malignant hyperthermia where patient
The overall incidence of malignant hyperthermia during general anesthesia is estimated to range from 1: 5000 to 1: 50,000-100,000 and mortality rate is estimated to be <5% in the presence of standard care. In India, there is no center where in vitro halothane caffeine contraction test is performed to confirm diagnosis in suspected cases.
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Case Study: Hyperthermia
Case Background. Hyperthermia occurs when the body temperature increases without an increase in the set point of the thermoregulatory center in the hypothalamus. Heat exhaustion and heatstroke are two common forms of hyperthermia. Symptoms of heat exhaustion include thirst, fatigue, profuse sweat, and giddiness or delirium.
Frontiers
In the case of dysbiosis, such as in the Salmonella infection model, CD103 + DCs were found to accumulate in the enteric epithelium layer and phagocytosed pathogenic bacteria (Farache et al., 2013). Lactic acid bacteria (LAB) were also discovered to stimulate immature DCs within the gut, inducing them to produce cytokines like IL-12 and IL-15 ...
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Clinical Case Study - Homeostasis Hyperthermia Case Presentation It was July 20 in Houston and the fourth straight day that would have a high temperature above 100°F. Janice was running some errands and decided to stop by her mother's house. Janice's mother, Marian, was eighty-four and in pretty good health.