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