Impotent Neutrophil Syndrome

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

• Bone and joint infections
• 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