The chronic granulomatous disease is-
Chronic granulomatous disease (CGD)
o It is a group of disorders of granulocyte and monocyte oxidative metabolism,
o Inheritance

X-linked (Most common)
Autosomal recessive

o Phagocytosis is normal but leucocytes do not undergo degranulation following phagocytosis,
o The diminished H.CL production appears to be the major reason for the defective bactericidal defect.
o Mutation in the genes for the four proteins that assemble at the plasma membrane account for all patients with CGD:

Two proteins from the heterodimer cytochrome b - 558 in the plasma membrane.
Two other proteins interact with cytochrome after cel! activation to form NADPH oxidase required for hydrogen peroxide production.

o The bacteria involved in recurrent infections are catalase positive pyogenic pathogens such as staphylococci and coliforms. Catalase negative pathogens such as streptococci and pneumococci are handled normally.
o There is excessive inflammation with granulomas formation,
o C/Fs □ Suppurative lymphadenopathy

Gingivitis
Aphthous ulceration
Seborrheic dermatitis
Obstruction of the gastrointestinal or genitourinary tract by granulomas.
Autoimmune complications:
Immune thrombocytopenic purpura (ITP)
Juvenile rheumatoid arthritis (JRA).

o Test for the diagnosis
4. Nitroblue tetrazolium (P4B T) test
5. Dihydrorhodamine, DHR (oxidation) test
6. Immunoblot for NADPH oxidase components
A leukocyte function defect
Associated with formation of multiple granulomas
A benign neoplastic process
A parasitic disease