defective respiratory burst

Question 1

• Absence affect the HEXOSE MONOPHOSPHATE SHUNT
• Leukocytes are unable to produce a respiratory burst, resulting in a DEFECTIVE BACTERICIDAL ACTIVITY
• is needed for the production of NADPH oxidase

Answer 1

G-6-PD deficiency

Question 2

• A rare condition caused by the decreased ability of neutrophils to undergo a respiratory burst after phagocytosis of foreign organisms
• CanbeX-linked recessive (60%) or autosomal recessive (40%)
• Caused by mutations in genes responsible for proteins that make up the reduced form of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (NADPH oxidase deficiency)
• Patients experience life-threatening catalase-positive bacterial and fungal infections

Answer 2

chronic granulomatous disease

Question 3

detection of respiratory burst

uses dihydrorhodamine to measure intracellular production of reactive oxygen species

Answer 3

chemiluminescence

Question 4

• Autosomal recessive ; also known as ALIUS-GRIGNASHI ANOMALY
• low or absent in neutrophils and monocytes but not in eosinophils
• Absence slows down bactericidal killing

Answer 4

myeloperoxidase (MPO) deficiency

Question 5

• Autosomal recessive
• Heterozygous: Carriers have half the normal C3 activity (adequate for disease resistance)
• Homozygous: Repeated severe infections with encapsulated bacteria which are poorly recognized and inefficiently phagocytized because of failure of opsonisation by C3

Answer 5

congenital C3 deficiency

Question 6

detection of respiratory burst

• NBT is a yellow, water-soluble dye
• NORMAL PATIENTS: REDUCED NBT because of the generation of respiratory burst (+BLUE FORMAZAN)
• PATIENTS WITH CGD: UNREDUCED (COLORLESS-YELLOW)

Answer 6

nitroblue tetrazolium (NBT) test