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Related Concept Videos

Direct-Acting Cholinergic Agonists: Therapeutic Uses01:11

Direct-Acting Cholinergic Agonists: Therapeutic Uses

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Direct-acting cholinergic agonists have many therapeutic uses in various medical fields. Choline esters, including acetylcholine, have limited clinical utility due to their non-selectivity and short duration of action. Still, acetylcholine and carbachol are applied topically during ophthalmologic surgery to induce miosis. Pilocarpine, a muscarinic and ganglionic stimulator, effectively treats open-angle glaucoma and alleviates xerostomia and dry mouth caused by radiotherapy or Sjögren...
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Cholinergic Antagonists: Therapeutic Uses01:26

Cholinergic Antagonists: Therapeutic Uses

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Antimuscarinic drugs have various therapeutic applications by inhibiting parasympathetic stimulation in different systems. Here are the key therapeutic uses of antimuscarinics:    
Respiratory Tract: Ipratropium, aclidinium, and tiotropium treat asthma, chronic bronchitis, and chronic obstructive pulmonary disease (COPD). They protect against bronchoconstriction caused by irritants like cigarette smoke, sulfur dioxide, and ozone. They also help reduce nasopharyngeal...
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Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

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Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
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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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Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin01:26

Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin

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Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...
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Direct-Acting Cholinergic Agonists: Pharmacological Actions00:59

Direct-Acting Cholinergic Agonists: Pharmacological Actions

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Direct-acting cholinergic agonists exert their pharmacological actions by mimicking the effects of acetylcholine on postsynaptic muscarinic receptors to generate parasympathetic responses. These agents elicit a range of physiological responses, including cardiovascular effects. For example, activation of muscarinic receptors induces bradycardia, decreased cardiac output, reduced peripheral resistance, and consequent hypotension. In the eye, stimulation of M3 receptors leads to smooth muscle...
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Related Experiment Video

Updated: Sep 5, 2025

A Whole Body Dosimetry Protocol for Peptide-Receptor Radionuclide Therapy PRRT: 2D Planar Image and Hybrid 2D+3D SPECT/CT Image Methods
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A Whole Body Dosimetry Protocol for Peptide-Receptor Radionuclide Therapy PRRT: 2D Planar Image and Hybrid 2D+3D SPECT/CT Image Methods

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Denosumab Therapy in Cherubism.

Steele I Liles1, Ian C Hoppe2, Laura Arnold3

  • 1School of Medicine, University of Mississippi Medical Center, Jackson, MS, USA.

The Cleft Palate-Craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association
|July 13, 2022
PubMed
Summary
This summary is machine-generated.

Denosumab effectively managed severe cherubism in a child, suppressing fibro-osseous lesions and reducing the need for surgery. This treatment offers a promising option for refractory cases of this rare genetic disorder.

Keywords:
bone regenerationcraniofacial growthcraniofacial morphologyfacial growthmandiblemaxillapediatrics

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Area of Science:

  • Genetics and Developmental Biology
  • Oncology
  • Pharmacology

Background:

  • Cherubism is a rare genetic disorder causing facial bone hyperplasia due to proliferative fibro-osseous lesions.
  • Current management involves observation, medication, or surgery, with varying success rates.
  • Recurrence after surgical intervention is common, necessitating long-term management strategies.

Observation:

  • A pediatric case of severe, refractory cherubism presented with significant facial bony overgrowth.
  • The patient's condition was resistant to conventional therapeutic approaches.
  • Longitudinal monitoring of electrolyte levels was crucial throughout the treatment period.

Findings:

  • Off-label use of denosumab, a RANKL inhibitor, demonstrated significant symptomatic control.
  • Denosumab suppressed fibro-osseous lesion proliferation, minimizing the need for surgical debulking.
  • Effective control of postoperative reproliferation was achieved, indicating sustained therapeutic benefit.

Implications:

  • Denosumab represents a viable therapeutic option for managing severe and refractory cherubism.
  • This approach may reduce surgical interventions and improve patient outcomes.
  • Further research into denosumab's long-term efficacy and safety in cherubism is warranted.