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Related Concept Videos

Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
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Inflammatory Bowel Disease I: Ulcerative Colitis

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Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
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The human gut microbiome includes a diverse array of microbial species, including beneficial commensals and opportunistic pathogens, which interact to support host health. These microbes contribute to essential functions such as nutrient metabolism, immune system modulation, and maintenance of intestinal barrier integrity. However, disruptions to this equilibrium—referred to as dysbiosis—can have widespread physiological consequences.Dysbiosis is often characterized by reduced microbial...
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Inflammatory Bowel Disease I: Introduction

Inflammatory bowel disease is a group of chronic disorders marked by recurrent inflammation of the gastrointestinal tract due to an abnormal immune response against gut microflora. This leads to tissue damage. The two main forms are Crohn’s disease and ulcerative colitis.Crohn’s DiseaseCrohn’s disease is a relapsing inflammatory disorder that can affect any part of the GI tract, from the mouth to the anus. It involves all layers of the bowel wall (transmural) and shows “skip lesions” in which...
Inflammatory Bowel Disease II: Ulcerative Colitis01:20

Inflammatory Bowel Disease II: Ulcerative Colitis

Ulcerative colitis is a chronic inflammatory disorder of the colon characterized by continuous mucosal inflammation that typically begins in the rectum and extends proximally in a uniform pattern. Its pathogenesis involves a complex interplay of genetic predisposition, immune dysregulation, and environmental influences. These factors converge to impair the colon’s epithelial defenses and promote an exaggerated inflammatory response against luminal contents.Breakdown of the Mucosal BarrierA...
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Inflammatory Bowel Disease III: Crohn's Disease

Crohn’s disease is a chronic, relapsing form of inflammatory bowel disease characterized by segmental, transmural inflammation that can affect any part of the gastrointestinal tract. Its pathogenesis arises from a combination of genetic susceptibility, environmental exposures, epithelial barrier dysfunction, and immune dysregulation. Together, these factors lead to an exaggerated immune response against components of the gut microbiome.Genetic and Environmental InfluencesMultiple genetic...

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Related Experiment Video

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Analyzing Beneficial Effects of Nutritional Supplements on Intestinal Epithelial Barrier Functions During Experimental Colitis
08:58

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Dysfunctional LAD-1 neutrophils and colitis.

G Uzel1, D E Kleiner, D B Kuhns

  • 1Immunology Services, Department of Laboratory Medicine, National Cancer Institute NIH, Bethesda, Maryland, USA.

Gastroenterology
|October 19, 2001
PubMed
Summary

Leukocyte adhesion deficiency 1 (LAD-1) can cause chronic colitis, a condition not typically associated with this immunodeficiency. This case highlights a distinct inflammatory bowel disease (IBD) pathology linked to neutrophil dysfunction in LAD-1.

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Area of Science:

  • Immunology
  • Gastroenterology
  • Genetics

Background:

  • Leukocyte adhesion deficiency 1 (LAD-1) is a primary immunodeficiency caused by absent or dysfunctional beta2 integrin (CD18).
  • Defective CD18 impairs neutrophil function, including chemotaxis, adherence, and bacterial killing, leading to recurrent infections.
  • Colitis is an uncommon manifestation of LAD-1, with only rare reports of necrotizing intestinal events.

Observation:

  • This report details a case of moderate LAD-1 in an adult patient presenting with chronic colitis.
  • The colitis was characterized by extensive inflammation and ulceration in the right colon and terminal ileum.
  • Clinical and pathological findings led to adhesions and strictures, mimicking features of Crohn's disease.

Findings:

  • The chronic colitis associated with dysfunctional neutrophils in LAD-1 represents a distinct pathology compared to typical forms of inflammatory bowel disease (IBD).
  • This case demonstrates active IBD in the context of impaired CD18/CD11(a-b) intercellular adhesion molecules (ICAM-1) interaction.
  • Neutrophil dysfunction in LAD-1 contributes to significant gastrointestinal inflammation and structural damage.

Implications:

  • Understanding LAD-1-associated colitis is crucial for accurate diagnosis and management of IBD-like conditions in patients with primary immunodeficiencies.
  • The findings suggest that neutrophil adhesion molecules play a critical role in the pathogenesis of gastrointestinal inflammation.
  • Targeting ICAM-1 interactions, a strategy being explored for Crohn's disease, may need careful consideration in patients with underlying integrin deficiencies.