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Related Concept Videos

Gastrointestinal Motility Disorders01:20

Gastrointestinal Motility Disorders

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Gastrointestinal or GI motility disorders are characterized by irregular gastrointestinal tract movements, disrupting food transit from the mouth to the anus. They are caused by damage or dysfunction in gut muscles or nerves. These disorders can cause symptoms such as severe constipation, diarrhea, abdominal pain, and swallowing difficulties. Disorders can affect any segment of the GI tract and range widely in severity, from common conditions like GERD to life-threatening conditions like...
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Drugs Affecting GI Tract Motility: Dopamine Receptor Antagonists01:28

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Prokinetic agents are specialized medications that stimulate gastrointestinal (GI) motility, promoting food movement through the GI tract. Dopamine, an inhibitory neurotransmitter, plays a significant role in this process, reducing GI motility and indirectly controlling the speed of digestion. Dopamine receptor antagonists, such as metoclopramide and domperidone, offer a unique advantage as prokinetic agents. By blocking the dopamine receptors, these drugs increase GI motility, improving food...
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Enteric Nervous System: Regulation of GI Motor Activity01:11

Enteric Nervous System: Regulation of GI Motor Activity

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The Enteric Nervous System (ENS) plays a pivotal role in regulating gastrointestinal or GI motor activity. This complex network of nerves, deeply embedded within the gut wall, responds to changes in the gut environment and receives input from both the autonomic nervous system and the central nervous system. By doing so, the ENS operates various programs tailored to the body's nutritional status and needs.
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Gastric Motility01:16

Gastric Motility

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Gastric motility is the coordinated contraction and relaxation of stomach muscles that convert ingested food into chyme, a semi-liquid substance ready for further digestion in the intestines. The process begins with the vagus nerve inducing the relaxation of the smooth muscles in the fundus and body of the stomach, allowing these regions to expand and accommodate up to approximately 1.5 liters of food and liquid.
Peristaltic Waves and Chyme Formation
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Drugs Affecting GI Tract Motility: Serotonin Receptor Agonists01:23

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Serotonin, a crucial neurotransmitter synthesized by enterochromaffin cells, plays a cardinal role in regulating gastrointestinal (GI) motility. With over 90% of the body's total serotonin in the GI tract, its influence on digestive processes is profound. Serotonin is swiftly released upon various stimuli, such as food boluses or certain drugs, triggering intrinsic sensory neurons in the myenteric plexus and extrinsic vagal and spinal sensory neurons. This leads to the activation of the...
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Enteral Nutrition II: Nasointestinal and Gastrostomy Feeding01:15

Enteral Nutrition II: Nasointestinal and Gastrostomy Feeding

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Enteral nutrition encompasses various methods of delivering nutrition directly to the gastrointestinal (GI) tract, bypassing traditional oral intake. It is particularly beneficial for patients who cannot eat by mouth but have a functioning digestive system. Key methods include nasointestinal feeding, gastrostomy, and jejunostomy, each suited to different clinical scenarios based on the patient's needs and condition.
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Gastrointestinal Motility Monitor GIMM
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Distinctive gastrointestinal motor dysfunction in patients with MNGIE.

Luis G Alcalá-González1,2,3, Anna Accarino1,2,3, Ramon Martí4,5

  • 1Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain.

Neurogastroenterology and Motility
|July 14, 2023
PubMed
Summary

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) patients show distinct small bowel motor dysfunction, even with mild symptoms. Early investigation is crucial as symptoms don't predict objective findings in this rare disease.

Keywords:
MNGIEintestinal manometryintestinal pseudo-obstructionsmall bowel motility

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Area of Science:

  • Gastroenterology
  • Neurology
  • Genetics
  • Rare Diseases

Background:

  • Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare genetic disorder caused by TYMP gene mutations.
  • It leads to severe gastrointestinal dysmotility, cachexia, and neuropathy.
  • Gastrointestinal motor dysfunction in MNGIE requires systematic evaluation.

Purpose of the Study:

  • To characterize gastrointestinal motor dysfunction in MNGIE patients.
  • To correlate motor abnormalities with clinical symptoms using advanced techniques.

Main Methods:

  • Prospective study of MNGIE patients at a Spanish national referral center (Jan 2018 - July 2022).
  • Evaluation included high-resolution manometry (esophageal and small bowel) and gastric emptying scintigraphy.
  • Assessment of digestive symptoms alongside objective motor function tests.

Main Results:

  • Five MNGIE patients (16-46 years) were evaluated.
  • Esophageal motility was abnormal in 4/5 patients.
  • Small bowel manometry revealed a unique dysmotility pattern (spasmodic contractions) in all patients.
  • Gastric emptying was delayed in 4/5 patients.
  • Objective motor dysfunction was present even with mild or absent severe digestive symptoms.

Conclusions:

  • MNGIE patients exhibit characteristic motor dysfunction, especially in the small bowel.
  • This dysfunction occurs irrespective of symptom severity or morphological signs of intestinal failure.
  • Early investigation is recommended as symptoms do not reliably predict objective motor findings.