Ch. 393 Flashcards Preview

Nelson - Exam 4 (Respiratory) > Ch. 393 > Flashcards

Flashcards in Ch. 393 Deck (10)
1

homozygous deficiency of α1-antitrypsin (α1-AT) is an important cause of ___ in children

Liver disease

2

Mode of inheritance of alpha 1 antitrypsin deficiency

Autosomal recessive

3

T/F Asians have little to no risk for α1-AT deficiency

T

4

Emphysema: Most patients who have the ___ defect have little or no detectable pulmonary disease during childhood

PiZZ

5

Smoking greatly increases the risk of emphysema in patients with ___ type

mutant Pi types

6

T/F PE of children with emphysema usually is normal

T

7

T/F Emphysema: Lung function testing is usually normal in children

T

8

Therapy for alpha 1 AT deficiency

IV replacement of enzyme derived from pooled HUMAN plasma

9

Alpha 1 AT deficiency: Target level for augmentation therapy is usually achieved with doses of ___

60 mg/kg IV weekly

10

Emphysema: Augmentation therapy is not indicated for persons with the ___ who have pulmonary disease, because their disease is not from enzyme deficiency.

PiMZ type