Diseases with Genetic Complement Deficiencies

Question 1

Defect in the classical complement pathway leads to…

Answer 1

SLE

Question 2

SLE is associated with defects in what complement proteins?

Answer 2

C1q,r,s
C4
C2

Question 3

Lectin Pathway

Answer 3

Defect in MBL and MASP

Recurrent childhood infection with pyogenic bacteria

Question 4

Complement defects are associated with what type of infection?

Answer 4

Neisseria speciesAZ

Question 5

C1-inhibitor defect

Answer 5

Recurrent angioedema

Question 6

C1-inhibitor is in what pathway?

Answer 6

Classical

Question 7

CD46 (MCP), CD55 (DAF), CD59, and Factor H defects are related to a defect in what process?

Answer 7

Membrane Attack Complex (MAC)

Question 8

Atypical hemolytic uremic syndrome is related to defect in

Answer 8

MAC

Question 9

What disease is associated with CD55 (DAF) and CD59 defects in the MAC process?

Answer 9

Paroxysmal nocturnal hemoglobinuria

Question 10

What disease is associated with a deficiency in C1-inhibitor?

Answer 10

Hereditary Angioedema

Defect in Classical Pathway of complement activation

C4 and C2 decreased

Question 11

Bacterial infections in early childhood are associated with a defect in which complement pathway?

Answer 11

Lectin Pathway