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Related Concept Videos

Depolarizing Blockers: Pharmocokinetics01:19

Depolarizing Blockers: Pharmocokinetics

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Depolarizing blockers are administered through intravenous injection. Succinylcholine is the most common choice of depolarizing blockers in emergency clinical practices. Although they have a rapid onset, they readily diffuse away from the motor end plate into the extracellular fluid. They are metabolized by enzymes such as liver butyrylcholinesterase and plasma pseudocholinesterases. This produces a short duration of action, typically 5-10 minutes long, unlike nondepolarizing blockers, which...
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Nondepolarizing (Competitive) Neuromuscular Blockers: Pharmacological Actions01:27

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Nondepolarizing neuromuscular blockers prevent the membrane depolarization of muscle cells and inhibit muscle contraction. These are usually administered with anesthetics to achieve complete muscle relaxation. Upon administration, these drugs first block the small, rapidly contracting muscles of the face and hands, followed by the larger muscles of the trunk and the intercostal muscles. The diaphragm is the last muscle to be affected.
Although all competitive neuromuscular blockers are designed...
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Parenteral Anesthetics: Overview01:24

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Intravenous anesthetics are drugs administered parenterally to induce anesthesia or sedation. Propofol is a widely used agent formulated as a 1% emulsion in soybean oil, glycerol, and egg phosphatide. It induces rapid anesthesia primarily due to its rapid distribution from the bloodstream to target tissues and is metabolized in the liver. However, it can cause significant pain on injection and hypertriglyceridemia. Fospropofol, a water-based prodrug of propofol, lacks these adverse effects.
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Skeletal Muscle Relaxants: Adverse Effects01:21

Skeletal Muscle Relaxants: Adverse Effects

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Skeletal muscle relaxants are widely used for muscle paralysis and relieving pain following any muscle injury or stiffness. However, depending on the drug type, they can have adverse effects that range from mild to severe. Usually, nondepolarizing neuromuscular blockers have minimal side effects. For example, drugs like d-tubocurarine, cisatracurium, and rocuronium cause hypotension, whereas drugs like baclofen, when stopped abruptly, can lead to the recurrence of spastic conditions.
Unlike...
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Anticholinesterase Agents: Poisoning and Treatment01:26

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Anticholinesterases, also known as cholinesterase inhibitors, work by blocking the breakdown of acetylcholine, leading to its accumulation in the synaptic cleft. This accumulation indirectly enhances both muscarinic and nicotinic actions. These agents are classified as reversible or irreversible based on their mechanism of action.     
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Skeletal Muscle Relaxants: Therapeutic Uses01:31

Skeletal Muscle Relaxants: Therapeutic Uses

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Skeletal muscle relaxants are used to relax muscle tone and alleviate painful muscle contractions. However, the choice of skeletal muscle relaxants depends on the duration of the surgical procedure in order to minimize potential side effects. Skeletal muscle relaxants like neuromuscular blocking agents [NMBAs] are commonly employed as adjuvants alongside general anesthetics in clinical settings. NMBAs are also used to maintain controlled ventilation during surgery of the larynx or pharynx...
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Anaesthetic implications for Pompe disease. A case description.

M Ruano Santiago1, E Soto Garrucho1, Y González Marín1

  • 1Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitario de Valme, Sevilla, Spain.

Revista Espanola De Anestesiologia Y Reanimacion
|February 22, 2023
PubMed
Summary

Pompe disease, a rare metabolic disorder, presents significant anesthesia risks, especially airway management challenges. Comprehensive preoperative assessment is crucial for managing patients with Pompe disease undergoing surgery.

Keywords:
AnaesthesiaAnestesiaDifficult airwayEnfermedad de PompeGlucogenosis tipo IIHipertermia malignaHypertrophic cardiomyopathyMalignant hyperthermiaMiocardiopatía hipertróficaMiopatíaMyopathyPompe diseaseType II glycogenosisVía aérea difícil

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Area of Science:

  • Metabolic Myopathies
  • Autosomal Recessive Disorders
  • Anesthesiology

Background:

  • Pompe disease (glycogenosis type II) is a rare, autosomal recessive metabolic myopathy causing progressive muscle weakness and multisystem issues.
  • It is associated with a high risk of anesthesia complications, notably cardiac, respiratory, and difficult airway management.
  • Effective perioperative risk reduction necessitates thorough preoperative evaluation.

Observation:

  • This article details the anesthetic management of an adult Pompe disease patient undergoing osteosynthesis of the proximal left humerus.
  • The patient's history of adult-onset Pompe disease required specialized anesthetic considerations.

Findings:

  • Difficult airway management is identified as the primary anesthesia-related complication in Pompe disease patients.
  • Comprehensive preoperative assessment is vital for mitigating perioperative morbidity and mortality.

Implications:

  • This case highlights the critical need for meticulous preoperative assessment and planning for anesthesia in Pompe disease patients.
  • Understanding the specific anesthetic risks associated with Pompe disease is essential for surgical teams to ensure patient safety.