Malignant Hyperthermia Pathophysiology

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Questions and Answers

What is the primary characteristic of Malignant Hyperthermia?

  • Reduced heart rate and normal body temperature.
  • Hypotension and reduced oxygen consumption.
  • Excessive skeletal muscle contraction, heat production, and excessive CO2 production. (correct)
  • Decreased muscle contraction and reduced CO2 production.

Which of the following ions is primarily responsible for initiating the release of Calcium (Ca++) during muscle contraction?

  • Potassium (K+)
  • Chloride (Cl-)
  • Sodium (Na+) (correct)
  • Magnesium (Mg++)

Where is the Calcium (Ca++) primarily stored in muscle cells?

  • Cytoplasm
  • Golgi Apparatus
  • Mitochondria
  • Sarcoplasmic Reticulum (SR) (correct)

What is the role of acetylcholine (ACh) in skeletal muscle contraction?

<p>It binds to receptors on the muscle and depolarizes it. (C)</p> Signup and view all the answers

What structure is responsible for releasing Calcium (Ca++) from the sarcoplasmic reticulum (SR)?

<p>Ryanodine receptors (RyRs) (A)</p> Signup and view all the answers

What is required for muscle contraction, besides the release of calcium?

<p>Muscle depolarization is also required (A)</p> Signup and view all the answers

What does ATP do in relation to ions in muscle contraction?

<p>Consumes ions for energy (B)</p> Signup and view all the answers

Considering the process of muscle contraction, what happens immediately after ACh binds to receptors on the muscle cell?

<p>The muscle cell is depolarized (B)</p> Signup and view all the answers

What is the primary action that occurs when actin and myosin filaments interact during muscle contraction?

<p>The filaments slide past each other, shortening the muscle fiber (B)</p> Signup and view all the answers

Which molecule or ion is essential for initiating the interaction between actin and myosin during muscle contraction?

<p>Calcium (Ca++) (B)</p> Signup and view all the answers

What is the role of troponin in resting muscle fibers?

<p>To block the interaction between actin and myosin (A)</p> Signup and view all the answers

In what two ways is ATP used during skeletal muscle contraction?

<p>Energy use in blocking actin-myosin interaction and during calcium reuptake into the SR. (D)</p> Signup and view all the answers

How does exercise influence the levels of oxygen, ATP, and carbon dioxide (CO2) in the body?

<p>Oxygen and ATP levels decrease, while CO2 levels increase. (C)</p> Signup and view all the answers

What is a key characteristic of the ryanodine (RyR1) receptor in individuals with the malignant hyperthermia (MH) trait?

<p>It is genetically altered to release too much calcium (A)</p> Signup and view all the answers

What is the direct consequence of excessive muscle contraction in malignant hyperthermia?

<p>A sustained hypermetabolic state (A)</p> Signup and view all the answers

Which of the following is not mentioned as a consequence of the hypermetabolic state in malignant hyperthermia?

<p>Decreased myoglobin levels (B)</p> Signup and view all the answers

An increased production of which gas is often observed in the early stages of malignant hyperthermia?

<p>Carbon dioxide (C)</p> Signup and view all the answers

What is the primary treatment for the increased CO2 production and oxygen utilization seen in malignant hyperthermia?

<p>Administering 100% oxygen and eliminating volatile agents (B)</p> Signup and view all the answers

Tachycardia in malignant hyperthermia is a direct result of which state?

<p>Hypermetabolic state (B)</p> Signup and view all the answers

What electrolyte imbalance contributes to ventricular extrasystoles in malignant hyperthermia?

<p>Hyperkalemia (C)</p> Signup and view all the answers

What is the primary action of the masseter muscle?

<p>Closing the mouth (D)</p> Signup and view all the answers

An unregulated increase of which ion triggers the hypercatabolic state in malignant hyperthermia?

<p>Calcium (B)</p> Signup and view all the answers

Which muscle relaxant is associated with causing fasciculations?

<p>Succinylcholine (C)</p> Signup and view all the answers

Which of the following is a common symptom of malignant hyperthermia?

<p>Muscle Rigidity (B)</p> Signup and view all the answers

What is the initial acid-base imbalance that occurs during malignant hyperthermia?

<p>Respiratory acidosis (D)</p> Signup and view all the answers

How long does a normal masseter spasm typically resolve?

<p>Within 5 minutes (C)</p> Signup and view all the answers

What is the chance of Malignant Hyperthermia susceptibility if a masseter spasm lasts more than 2 minutes and hinders intubation?

<p>24-50% (D)</p> Signup and view all the answers

Besides increased Hydrogen ions, which other substance's increased production is associated with Malignant Hyperthermia?

<p>Lactate (D)</p> Signup and view all the answers

What is generally required for surgical procedures within body cavities?

<p>Profound muscle relaxation (D)</p> Signup and view all the answers

Which type of drugs are used to cause muscle paralysis?

<p>Neuromuscular blocking drugs (C)</p> Signup and view all the answers

What physiological change is an early sign preceding generalized muscle rigidity?

<p>Hypercapnia (A)</p> Signup and view all the answers

What is the primary mechanism for the increase in heart rate in the context of Malignant Hyperthermia?

<p>Decreased oxygen (A)</p> Signup and view all the answers

What is the role of Mr Ca++ in muscle contraction?

<p>To bind with Troponin allowing interaction between Actin and Myosin (C)</p> Signup and view all the answers

What consequence occurs when the Ryanodine receptors are damaged?

<p>Excessive muscle contraction occurs (B)</p> Signup and view all the answers

What effect does the excessive muscle contraction have on muscle tissue?

<p>It leads to muscle damage and myoglobin release (B)</p> Signup and view all the answers

What is the treatment used to block the Ryanodine receptors?

<p>Dantrolene (A)</p> Signup and view all the answers

What symptom might indicate a problem due to excessive muscle contraction?

<p>Hypercapnia (C)</p> Signup and view all the answers

What effect do beta-blockers have on heart rate in patients?

<p>They may prevent a rise in heart rate. (D)</p> Signup and view all the answers

Which condition is often related to tachycardia during the perioperative period?

<p>Sympathetic stimulation. (A)</p> Signup and view all the answers

What is the intracellular potassium concentration in skeletal muscle cells?

<p>150 mmol/L (B)</p> Signup and view all the answers

What is the main action of dantrolene in treating malignant hyperthermia?

<p>Decrease intracellular calcium concentration. (C)</p> Signup and view all the answers

What is the consequence of muscle cell death in malignant hyperthermia?

<p>Hyperkalaemia. (D)</p> Signup and view all the answers

What does the term 'excitation-contraction coupling' refer to?

<p>The process of muscle contraction following nerve signal. (A)</p> Signup and view all the answers

What is the primary use of dantrolene in a clinical setting?

<p>To treat malignant hyperthermia. (A)</p> Signup and view all the answers

During a sympathetic response, what typically happens to heart rate?

<p>It increases. (B)</p> Signup and view all the answers

Flashcards

Sodium (Na+)

The ion responsible for initiating skeletal muscle contraction.

Calcium (Ca++)

The primary ion involved in muscle contraction, stored within the sarcoplasmic reticulum.

Adenosine Triphosphate (ATP)

The energy source for muscle contraction, providing the power for muscle fibers to move.

Sarcoplasmic Reticulum (SR)

This structure within muscle cells stores calcium ions, releasing them when stimulated.

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Acetylcholine (ACh)

The chemical messenger released at the neuromuscular junction, triggering muscle contraction.

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Depolarization

The process of increasing the positive charge inside a cell, like when ACh binds to muscle receptors.

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Ryanodine Receptors (RyRs)

Large ion channels responsible for releasing calcium from the SR, leading to muscle contraction.

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Neuromuscular Junction

The point where a nerve communicates with a muscle fiber, releasing neurotransmitters like ACh.

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Troponin

A protein that binds to calcium ions, initiating muscle contraction by allowing the interaction of actin and myosin.

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Malignant Hyperthermia (MH)

A genetic disorder causing an excessive release of calcium ions from the sarcoplasmic reticulum (SR) due to a mutated ryanodine receptor (RyR1).

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Sliding Filament Theory

The process by which the actin and myosin filaments slide past each other, causing muscle shortening.

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Hypermetabolic state

A state of excessive metabolism, often associated with increased heat production due to rapid muscle activity.

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Anaerobic Metabolism

The breakdown of glucose to produce energy in the absence of oxygen.

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Hypoxemia

A condition characterized by low oxygen levels in the blood.

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Lactic Acidosis

A buildup of lactic acid in the body, often associated with strenuous exercise or oxygen deprivation.

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What is Malignant Hyperthermia (MH)?

A life-threatening condition characterized by a rapid increase in body temperature, muscle rigidity, and metabolic changes, caused by a genetic predisposition and triggered by certain anesthetic agents.

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What is succinylcholine?

The primary trigger for MH, a muscle relaxant commonly used in surgery.

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What is the Ryanodine Receptor (RyR1)?

The primary protein responsible for the Ca++ release from the SR in MH, becoming hyperactive and releasing excessive calcium.

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What is hyperthermia in MH?

The primary symptom of MH, an abnormally high body temperature due to increased metabolic activity.

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What is muscle rigidity in MH?

The characteristic muscle stiffness and rigidity associated with MH, caused by sustained muscle contraction.

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What is respiratory acidosis in MH?

The initial acid-base abnormality in MH, caused by increased CO2 production due to rapid metabolism.

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What is metabolic acidosis in MH?

The subsequent acid-base disturbance in MH, characterized by a decrease in blood pH due to excess lactate production.

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What is the treatment for MH?

The treatment for MH, involving halting the anesthetic agents, administering 100% oxygen, and using a specific medication to control the hyperactive RyR1.

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Muscle Contraction

The process by which muscle fibers contract, involving the sliding of actin and myosin filaments past each other, powered by ATP.

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Dantrolene

A drug used to treat malignant hyperthermia by blocking the release of calcium from the SR, ultimately reducing muscle contraction and heat production.

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Ryanodine Receptor

The gateway in the sarcoplasmic reticulum (SR) that allows calcium ions (Ca++) to flood into the muscle cell, triggering muscle contraction.

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Masseter Spasm

A strong muscle contraction that occurs when the masseter muscle, responsible for chewing, is affected by a condition like Malignant Hyperthermia (MH).

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Succinylcholine

A drug used to relax muscles during surgery. It acts by stimulating then blocking the receptors involved in muscle contraction.

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Depolarizing Muscle Relaxant

The sustained activation of muscle receptors caused by succinylcholine. It leads to strong muscle contractions (fasciculations)

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Generalized Muscle Rigidity

A sign of MH. It occurs later in the progression of the condition and signifies a more severe stage.

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Tachycardia in MH

The rapid heartbeat that occurs during MH. It's a response to the body trying to compensate for reduced oxygen levels and increased stress hormones.

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Hypercapnia in MH

An increase in carbon dioxide levels in the blood, a consequence of the increased metabolic rate and oxygen consumption during MH.

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Increased Oxygen Consumption

The increase in oxygen consumption caused by the body's response to the increased stress hormones and metabolic changes during MH. It contributes to the reduced oxygen availability and the development of tachycardia.

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Rhabdomyolysis

The breakdown of skeletal muscle tissue, leading to the release of myoglobin and potassium into the bloodstream.

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Hypermetabolic State (MH)

The state of increased metabolism that occurs during MH, causing a rapid rise in body temperature and other complications.

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Tachycardia (MH)

A rapid increase in heart rate, often a symptom of a sympathetic nervous system response to stress or pain. The tachycardia in MH is related to the hypermetabolic state.

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Study Notes

Malignant Hyperthermia

  • A life-threatening hyperthermic reaction to general anesthetic gases and succinylcholine
  • Characterized by excessive skeletal muscle contraction, heat production, and tachycardia (rapid heart rate)

Normal Skeletal Muscle Contraction

  • Initiated by sodium ions (Na+)
  • Followed by calcium ions (Ca++) triggering muscle contraction
  • Requires adenosine triphosphate (ATP) for energy
  • Calcium is stored in the sarcoplasmic reticulum (SR) within muscle cells
  • Calcium release is mediated by ryanodine receptors (RyRs)
  • Muscle contraction involves actin and myosin filaments sliding past each other
  • Troponin prevents this interaction at rest, and calcium binding to troponin shifts it out of the way to allow for contraction

Malignant Hyperthermia Pathophysiology

  • Altered ryanodine receptors (RyRs) are the primary genetic cause
  • The altered RyRs cause excessive calcium release from the SR, leading to sustained muscle contraction
  • This uncontrolled muscle contraction leads to hypermetabolism, increased heat production, and accumulation of metabolic byproducts
  • Elevated CO2 (carbon dioxide) and lactic acid levels further contribute to the physiological picture
  • These conditions collectively result in a potentially life-threatening hypermetabolic state and acidosis
  • Rhabdomyolysis, a breakdown of skeletal muscle, can occur, releasing myoglobin and potassium into the blood
  • Myoglobin can damage the kidneys

Treatment

  • Dantrolene is the primary treatment for malignant hyperthermia
  • Dantrolene binds to the RyR1 receptors, decreasing intracellular calcium concentration and inhibiting muscle contraction.

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