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Flashcards in PID Deck (8)
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1

Chronic Granulomatous Disease

-Childhood disease (can be diagnosed as late as 3rd decade)

-deficiency in NADPH oxidase
-->reduction in leukocyte oxidative burst
-recurrent infections of S. aureus, Burkholderia, Serratia, Nocardia, Aspergillus

Diagnosed-abnormal nitroblue tetrazolium test or flow cytometry DHR assay

TX- antimicrobial prophylaxis

2

Serum Protein ElectroPhoresis

CVID

3

CH50

Terminal complement components

4

Nitrozoleum blue test

CGD

5

CVID

-5 years or 25-30 years

-recurrent bacterial infections- respiratory with encapsulated bacteria and GI-Giardia

Diagnosis-low Ig levels or one of its subsets

Poor response to vaccines- check pneumococcal and tetanus titers

Increased risk of autoimmunity and malignancy and interstitial lung disease

Tx-regular Ig infusions

6

Terminal complement component deficiency C5-9

Recurrent neisserial infections

CH50

Treatment-Early initiation of antibiotics
Meningococcal vaccine

7

IgA deficiency

Common
Usually asymptomatic

Low IgA levels alone do not cause recurrent bacterial infections
-prone to GI infection-GIardia
-increased risk of autoimmune disorders

Transfusion
-washed
-ffp form iga deficient donor

8

Hereditary Angioedema

no hives

angioedema (respiratory distress)

GI (symptoms similar to obstruction)

Low C4-C1 esterase inhibitor levels

Treatment
-C1 inhibitor concentrate, kallikrein inhibitor- ecallantide, or icatibant-bradykinin receptor antagonist

Prophylaxis Rx
-attenuated androgens
increase C1-inhibitor via increased hepatic synthesis
-fibrinolytic agents help in fibrinolysis and spare C1 inhibitor this work

Decks in MHD Class (81):