Chronic Granulomatous Disease (CGD)

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Chronic Granulomatous Disease (CGD) is an inherited disease. It usually occurs when WBC (White Blood Cells) capacity to form oxygen-activated compounds decreases and weakens phagocytic cells; as a result, the infection spreads. Read More

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Chronic Granulomatous Disease (CGD)

Commonly known as primary immunodeficiency. Chronic Granulomatous Disease (CGD) is an inherited disease. It usually occurs when WBC (White Blood Cells) capacity to form oxygen-activated compounds decreases and weakens phagocytic cells; as a result, the infection spreads.
This infection starts spreading from the skin and passes through the internal organs such as the lungs, liver, stomach, spleen and may happen in other body parts. In the early stage of life, Chronic Granulomatous Disease doesn’t affect the body, but when the child grows, the chances to develop infection increase. The severity of CGD infection can lead to prolonged hospital care. The good thing is Chronic Granulomatous Disease is not common; only 5-7 people in a million get affected by this. Men get affected more than women, and the reason is CGD is inherited in an X-linked pattern.
Chronic Granulomatous Disease (CGD)
Picture Courtesy: Hindawi

Chronic Granulomatous Disease Treatment

To nullify the chances of infection, preventive antibiotics and some precautions can come to the rescue: 
  • Corticosteroids can be effective in curing inflamed tissues.
  • The major thing which one should include is antifungal medicines.
  • Perform a bone marrow transplant to avoid unnecessary risks.
  • Interferon-gamma injections can protect against infections.
  • Stem cell transplant is a useful and efficient method, but it requires a matchable donor.
  • Granulocyte transfusion also holds a great promise as a future cure.
  • Surgical care.

Symptoms

CGD shows many kinds of symptoms starting from minor ones to the ones which are long-lasting:

  • Swollen Lymph glands
  • Bone Infection
  • Skin rashes
  • Pneumonia
  • Diarrhea
  • Fever
  • Chest Pain

Causes

As it is a genetic disorder, when parents pass the gene mutation to the upcoming generation, mutations like NCF1, NCF2 and NCF4 generally cause CGD and at some point CYBA and CYBB can also be responsible for such conditions

FAQ

1. How is Chronic Granulomatous Disease diagnosed?

Doctors diagnose based on its intensity level and symptoms. Some tests like nitro blue tetrazolium test, some common genetic tests, neutrophil function test, prenatal testing [2], and respiratory burst assay are popular.

2. Which type of doctor should be preferred for CGD treatment?

General surgeon or Pathologist for biopsy and check-ups general physician and hematologist should be preferred.

3. Is it possible to vanish CGD?

Yes, if the WBCs become more active and immune cells, it is possible.

4. Does CGD cause blockage?

Yes, in some areas, it does, such as in the urinary tract, in the brain, and even in the intestinal tract.

5. What is the Chronic Granulomatous Disease’s life expectancy?

Nowadays, due to several test facilities and with the options of numerous antibiotics and therapies, people can live up to 45-50 years.

6. What to avoid in Chronic Granulomatous Disease?

  • Swimming
  • Stop smoking Marijuana
  • Avoid sitting in park’s hayrides
  • Sanitizing cellars

7. what are the complications of CGD?

A few of the complications are listed below:

  • Difficulty in digesting food due to abscess or inflammation of the intestine.
  • Growth retardation
  • Inflammatory bowel disease
  • Rheumatic disorders such as SLE, DLE, Raynaud’s disease, Juvenile rheumatoid arthritis, etc. [3].
  • Chorioretinitis, obstructive urinary tract, granulomatous cheilitis, etc.

 

References:

  1. https://my.clevelandclinic.org/health/diseases/21157-chronic-granulomatous-disease-cgd
  2. https://www.mayoclinic.org/diseases-conditions/chronic-granulomatous-disease/diagnosis-treatment/drc-20355818
  3. emedicine