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Dr. Andrew Rynne

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Article Home Children's Health Impotent Neutrophil Syndrome

Impotent Neutrophil Syndrome

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Also called as chronic granulomatous disease. Chronic granulomatous disease is a primary immunodeficiency that affects phagocytes of the immune system and leads to recurrent or persistent intracellular bacterial and fungal infections and to granuloma formation.

 

Chronic granulomatous disease is a genetically heterogeneous group of immunodefeciencies resulting from the inability of phagocytes to kill microbes they have ingested.

Clinical types

  • Chronic X-linked disease
  • Chronic b-negative disease
  • X-linked cytochrome b-positive disease
  • X-linked variant disease
  • Atypical granulomatous disease

Signs and symptoms

  • Skin infections- Abscesses, chronic infection inside the nose, furuncles, Impetiginized eczema, impetigo, perianal abscesses (abscesses around the anus)
  • Persistent diarrhea
  • Pneumonia
  • Swollen lymph nodes in the neck- suppurative lymphadenitis
  • Otitis media- ear infection
  • Adenitis

Causes

  • Leukocytes ingest bacteria but do not kill them because of a defect in the production of the superoxide anion.
  • Most infections in chronic granulomatous disease are caused by Staphylococcus aureus.
  • BCG vaccination may cause chronic granulomatous disease.

The most common infecting organisms

  • Pneumonia -Aspergillus, Staphylococcus, Nocardia, and Burkholderia cepacia (formerly Pseudomonas cepacia)
  • Subcutaneous, perirectal abscess- Staphylococcus, Serratia, and Aspergillus species
  • Lung abscess- Aspergillus fungus
  • Brain abscess- Aspergillus fungus
  • Suppurative adenitis- Staphylococcus and Serratia species
  • Osteomyelitis- Serratia and Aspergillus species
  • Bacteremia or fungemia -Salmonella and Candida species

Tests and diagnosis

  • Complete blood count
  • Blood culture, antibiotic culture and sensitivity
  • Nitroblue tetrazolium (NBT) dye test
  • Flow cytometric reduction tests
  • Chest X-ray and CT chest

Treatment

  • Conventional treatment consists of lifelong anti-infectious prophylaxis with antibiotics such as trimethoprim-sulfamethoxazole (TMP-SMZ)
  • Lifelong prophylaxis of antifungals such as Itraconazole
  • Biological response modifiers- INF-gamma
  • INF-gamma possesses antiviral, immunomodulatory, and antiproliferative activity.
  • INF-gamma has potent phagocyte-activating effects not seen with other interferon preparations