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Questions and Answers
What is the primary genetic cause of malignant hyperthermia?
What is the primary genetic cause of malignant hyperthermia?
Which laboratory finding is often elevated in cases of malignant hyperthermia?
Which laboratory finding is often elevated in cases of malignant hyperthermia?
Which symptom is considered an early sign of malignant hyperthermia during anesthesia?
Which symptom is considered an early sign of malignant hyperthermia during anesthesia?
What is a contraindication during an episode of malignant hyperthermia?
What is a contraindication during an episode of malignant hyperthermia?
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Which of the following are potential complications of malignant hyperthermia?
Which of the following are potential complications of malignant hyperthermia?
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In the management of hyperkalemia during malignant hyperthermia, which treatment method is recommended?
In the management of hyperkalemia during malignant hyperthermia, which treatment method is recommended?
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What is the primary reason for mortality in malignant hyperthermia despite treatment with dantrolene?
What is the primary reason for mortality in malignant hyperthermia despite treatment with dantrolene?
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What does the mutation in the RYR1 gene cause when exposed to volatile anesthetics and depolarizing neuromuscular blocking agents?
What does the mutation in the RYR1 gene cause when exposed to volatile anesthetics and depolarizing neuromuscular blocking agents?
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What causes patients with malignant hyperthermia to become hyperthermic?
What causes patients with malignant hyperthermia to become hyperthermic?
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How does malignant hyperthermia (MH) lead to myoglobinuria?
How does malignant hyperthermia (MH) lead to myoglobinuria?
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Why are patients with Malignant Hyperthermia (MH) typically acidotic?
Why are patients with Malignant Hyperthermia (MH) typically acidotic?
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What patient conditions are associated with Malignant Hyperthermia (MH)? (Select all that apply)
What patient conditions are associated with Malignant Hyperthermia (MH)? (Select all that apply)
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What are the anesthetic triggers for Malignant Hyperthermia (MH)?
What are the anesthetic triggers for Malignant Hyperthermia (MH)?
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What cardiac sign is typical of a patient with malignant hyperthermia?
What cardiac sign is typical of a patient with malignant hyperthermia?
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Rhabdomyolysis and coagulopathies are a late sign of MH
Rhabdomyolysis and coagulopathies are a late sign of MH
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What is the gold standard for diagnosing Malignant Hyperthermia (MH)?
What is the gold standard for diagnosing Malignant Hyperthermia (MH)?
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What solution is used to reconstitute dantrolene?
What solution is used to reconstitute dantrolene?
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Which of the following are non-anesthetic or environmental triggers for Malignant Hyperthermia? (Select all that apply)
Which of the following are non-anesthetic or environmental triggers for Malignant Hyperthermia? (Select all that apply)
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MH can occur at any point during anesthesia and emergence
MH can occur at any point during anesthesia and emergence
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Dantrolene therapy must continue for days after MH onset to prevent recurrence
Dantrolene therapy must continue for days after MH onset to prevent recurrence
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What are the expected lab findings for a patient with Malignant Hyperthermia?
What are the expected lab findings for a patient with Malignant Hyperthermia?
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You suspect your patient is experiencing a malignant hyperthermia crisis. Which of the following actions do you take first?
You suspect your patient is experiencing a malignant hyperthermia crisis. Which of the following actions do you take first?
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What type of gas flows should be used when you suspect a patient has malignant hyperthermia?
What type of gas flows should be used when you suspect a patient has malignant hyperthermia?
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What is the initial dose of dantrolene used in Malignant Hyperthermia?
What is the initial dose of dantrolene used in Malignant Hyperthermia?
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Dysrhythmias in malignant hyperthermia (MH) are secondary to which of the following conditions? (Select all that apply)
Dysrhythmias in malignant hyperthermia (MH) are secondary to which of the following conditions? (Select all that apply)
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What are the preferred treatments for hyperkalemia during malignant hyperthermia? (Select all that apply)
What are the preferred treatments for hyperkalemia during malignant hyperthermia? (Select all that apply)
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How does calcium help treat hyperkalemia?
How does calcium help treat hyperkalemia?
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Calcium does not directly lower serum potassium levels; it helps stabilize cellular membranes
Calcium does not directly lower serum potassium levels; it helps stabilize cellular membranes
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Once the malignant hyperthermia patient has stabilized, which interventions can be done to maintain urinary output? (Select all that apply)
Once the malignant hyperthermia patient has stabilized, which interventions can be done to maintain urinary output? (Select all that apply)
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What is the recurrence rate for Malignant Hyperthermia (MH)?
What is the recurrence rate for Malignant Hyperthermia (MH)?
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What is the maintenance dose for dantrolene?
What is the maintenance dose for dantrolene?
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What does it mean that Malignant Hyperthermia (MH) is an autosomal dominant disorder?
What does it mean that Malignant Hyperthermia (MH) is an autosomal dominant disorder?
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Which of the following conditions can mimic Malignant Hyperthermia? (Select all that apply)
Which of the following conditions can mimic Malignant Hyperthermia? (Select all that apply)
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What is the purpose of a vapor clean anesthesia filter?
What is the purpose of a vapor clean anesthesia filter?
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Should patients at risk for Malignant Hyperthermia (MH) be pretreated with dantrolene?
Should patients at risk for Malignant Hyperthermia (MH) be pretreated with dantrolene?
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What are the recommended PACU holding times for patients with Malignant Hyperthermia?
What are the recommended PACU holding times for patients with Malignant Hyperthermia?
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Which of the following medications should be available in a malignant hyperthermia (MH) kit? (Select all that apply)
Which of the following medications should be available in a malignant hyperthermia (MH) kit? (Select all that apply)
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Which group is most likely to experience intraoperative malignant hyperthermia?
Which group is most likely to experience intraoperative malignant hyperthermia?
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What is the appearance of the skin of a patient with Malignant Hyperthermia?
What is the appearance of the skin of a patient with Malignant Hyperthermia?
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What is an abnormal length of masseter muscle rigidity post-succinylcholine administration?
What is an abnormal length of masseter muscle rigidity post-succinylcholine administration?
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What is the mechanism of action of dantrolene?
What is the mechanism of action of dantrolene?
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How is dantrolene reconstituted?
How is dantrolene reconstituted?
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Match the sign with its timing
Match the sign with its timing
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Early signs of MH = tachycardia, tachypnea, warm soda lime, increased ETCO2, masseter spasm
Early signs of MH = tachycardia, tachypnea, warm soda lime, increased ETCO2, masseter spasm
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Late signs of MH = hyperthermia, rhabdomyolysis, coagulopathy, muscle rigidity
Late signs of MH = hyperthermia, rhabdomyolysis, coagulopathy, muscle rigidity
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Study Notes
Malignant Hyperthermia Overview
- Malignant hyperthermia (MH) is a genetic disorder linked to mutations in the ryanodine receptor type 1 (RYR1 gene).
- Triggered by volatile anesthetics and depolarizing neuromuscular blockers.
- Results in uncontrolled calcium release leading to sustained muscle contractions.
Hypermetabolic Response
- Increased oxygen consumption and ATP usage lead to heightened heat production.
- Enhanced production of carbon dioxide (CO2) and lactate.
- Cellular breakdown releases myoglobin into the bloodstream.
- Increased sympathetic nervous activity results in stress responses.
Signs and Symptoms
- Early indication of MH includes increased end-tidal carbon dioxide (ETCO2).
- Increased body temperature is a late sign of MH.
- Tachycardia is almost universally present during an episode.
- Muscle rigidity may or may not be observed.
- Symptoms may include tachypnea (increased breathing rate) and signs of exhausted CO2 absorbent.
- Myoglobinuria (presence of myoglobin in urine), disseminated intravascular coagulation (DIC), and dysrhythmias may occur.
Onset and Triggers
- Onset can occur at any stage during anesthesia, on emergence, or during the post-anesthesia care unit (PACU) stay.
- Fulminant MH can manifest within minutes of induction of general anesthesia.
- Environmental factors such as hyperthermia, stress, and exercise-induced rhabdomyolysis can trigger episodes.
Associated Conditions
- MH is linked with several myopathies including central core disease, multi-minicore disease, and King Denborough syndrome.
Electrolyte Abnormalities
- Patients may exhibit mixed respiratory and metabolic acidosis.
- Elevated creatinine kinase levels indicate muscle damage.
- Electrolyte disturbances include hyperkalemia, hyperphosphatemia, and hypercalcemia.
- Abnormal coagulation profiles may be present.
Immediate Treatment Protocol
- Obtain the red MH bag and vapor-clean filters for emergency management.
- Discontinue the use of volatile anesthetic agents or succinylcholine.
- Hyperventilate the patient with 100% fraction of inspired oxygen (FiO2).
- Administer dantrolene at a dosage of 2.5 mg/kg mixed with sterile water; reconstitute 250 mg/vial with 5 mL of sterile water.
- Utilize large bore IV access to minimize phlebitis risks.
- Cool the patient using cold normal saline fluids, ice packs, and by lowering room temperature.
- Address acidosis and hyperkalemia with bicarbonate, glucose/insulin, and calcium for severe cases.
Contraindications and Follow-up Care
- Calcium channel blockers are contraindicated in MH management.
- Dantrolene therapy must be continued for days following an episode to prevent recurrence.
- Maintenance dosing of dantrolene is 1 mg/kg every 4-6 hours for 24-48 hours.
Prognosis
- Acute mortality in MH patients often results from complications like hyperkalemia and hyperthermia, underscoring the urgency of treatment.
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Description
This quiz covers the genetic disorder of malignant hyperthermia, focusing on the role of the ryanodine receptor type 1 and its triggers such as volatile anesthetics. Learn about the hypermetabolic response, including increased oxygen consumption, ATP usage, and the consequences of muscle contraction. Test your understanding of this critical condition in anesthesiology.