To: AARC Participant:
“Standards for the Diagnosis and Management of Individuals with
Alpha-1 Antitrypsin Deficiency”
This learning activity has been approved by the American Association for Respiratory
Care (AARC) for 3 hours of Continuing Respiratory Care Education (CRCE).
To assist us in evaluating the effectiveness of this CRCE/CEU presentation and make
recommendations for future educational activities, please take a few minutes to answer
the following questions.
Please print this test and evaluation, or use the enclosed test/evaluation and mail or fax the test
and the evaluation to us at the following address (Please do not send to the AARC; they will not
process the documents)
Alpha-1 Foundation
3300 Ponce de Leon Blvd.
Coral Gables, Florida 33134
FAX: 305-675-0299
ATTN: Adriana De Arce
NAME: ________________________________________
DATE: _________
ADDRESS: ______________________________________________
______________________________________________
AARC Member #/LICENSE # (for AARC Records): _____________________________
CRCE/CEU Self Assessment Test:
Three hours of CRCE Credit.
To obtain CRCE credit, please review the test and possible responses, and circle
the best correct answer for each question.
After finalizing the test, please complete the evaluation and contact information and mail
or fax these pages to the above address.
1. All of the following apply to AAT except: (one only)
a. Hepatocytes are the main source
b. It circulates in the blood and permeates tissues
c. It inhibits many enzymes, but is most effective against neutrophil elastase
d. The blood level falls during acute febrile illnesses
2. Severe AAT deficiency can be detected definitively by which of the
following? (one or more)
a. Routine plasma protein electrophoresis
b. Determination of the plasma AAT level
c. Determination of the AAT phenotype
d. Determination of the AAT genotype
3. What is the estimated number of individuals with severe AAT deficiency in
the US?
a. 10,000
b. 25,000
c. 100,000
d. 1,000,000
4. Testing for AAT deficiency is recommended for which of the following?
(one or more)
a. All adults with COPD or emphysema
b. Adults with asthma that is completely reversible
c. Siblings of an individual with severe AAT deficiency
d. Adults with unexplained bronchiectasis
5. Which of the following apply to individuals with severe AAT deficiency?
(one or more)
a. Asthma-like symptoms occur more often among those with COPD than among
individuals with COPD and normal AAT
b. There are no physical findings that distinguish COPD associated with AAT
Deficiency from COPD with normal AAT
c. Cigarette smoking markedly accelerates the progression of COPD
d. In never-smokers, symptomatic COPD is uncommon prior to age 50
6. The typical lung pathology of COPD associated with severe AAT
Deficiency is which one of the following?
a. Panacinar emphysema with basal predominance
b. Panacinar emphysema with apical predominance
c. Centrilobular emphysema with basal predominance
d. Centrilobular emphysema with apical predominance
7. Which of the following apply to individuals with MZ type AAT? (one or more)
a. The risk of COPD is increased by cigarette smoking compared to individuals with
normal (MM) AAT
b. Among non-smokers the risk of COPD is no different than for non-smokers with
normal (MM) AAT
c. The risk of COPD is increased among those with first-degree relatives with severe
AAT deficiency and COPD
d. They are over-represented among individuals hospitalized for COPD exacerbations
8. As currently practiced, augmentation therapy for severe AAT deficiency: (one
or more)
a. Refers to infusions of recombinant AAT
b. Refers to infusions of AAT isolated from pooled human plasma
c. Aims to keep the bronchoalveolar lavage AAT level above 60-70% of the normal,
MM, level
d. Includes aerosol nebulization of AAT
9. In adults with severe AAT deficiency, liver disease is: (one or more)
a. Due to the low levels of circulating AAT
b. Due to accumulation of Z type AAT in hepatocytes
c. Prevented in most individuals by efficient hepatocellular degradation of the Z
type AAT
d. Associated with an increased incidence of hepatitis B and C
10. Augmentation therapy is effective for the liver disease related to AAT
deficiency.
True ________
False________
11. Which of the following apply to clinical liver disease associated with
severe AAT Deficiency? (one or more)
a. Is promoted by smoking
b. Typically occurs in non-smokers in middle age or older
c. Has the pathology of cirrhosis
d. Includes a significant risk of primary liver cancer that is usually associated with
cirrhosis
12. Regarding childhood liver disease associated with severe AAT
deficiency, which of the following is correct? (one or more)
a. Liver dysfunction is often first evident as hepatomegaly or prolonged jaundice
b. In most individuals, liver function abnormalities in early life resolve by age 1218
c. Siblings with the deficiency often have similar types of liver problems
d. AAT deficiency is rarely the underlying cause of liver disease requiring liver
transplantation in children
13. A diagnosis of AAT deficiency-associated liver disease is made
definitively by which one of the following?
a. Liver biopsy demonstrating PAS positive, diastase resistant intraheptocyte
inclusions
b. Liver biopsy demonstrating PAS positive, diastase resistant intraheptocyte
inclusions and abnormal liver function tests
c. Liver biopsy and absent alpha1 globulin on plasma protein electrophoresis
d. Abdominal ultrasound and plasma Z type AAT by phenotyping
14. Extrapulmonary conditions associated with severe AAT deficiency
include: (one or more)
a. Celiac disease
b. Necrotizing panniculitis
c. Systemic necrotizing vasculitis (cANCA positive)
d. Intracranial aneurysm
15. Which of the following applies to asymptomatic individuals identified with
severe AAT deficiency? (one or more)
a. Is associated with high rates of smoking prevention among adolescents identified as
neonates
b. Leads to high rates of smoking cessation among adult smokers
c. Has been shown to create psychologic stress in most individuals
d. Should lead the physician to encourage testing close family members for the
deficiency
Participant Evaluation
“Standards for the Diagnosis and Management of Individuals with
Alpha-1 Antitrypsin Deficiency”
To assist us in evaluating the effectiveness of this course and make recommendations for
the future educational activities, please take a few minutes to answer the following
questions and mail/fax to:
Adriana De Arce
Alpha-1 Foundation
3300 Ponce de Leon Blvd.
Coral Gables, Florida 33134
FAX: 305-675-0299
NAME: _____________________________________
DATE: _____________
ADDRESS: __________________________________
__________________________________
Objectives:
At the conclusion of this presentation the participant will be able to:
• Become proficient on the standard of care for Alpha-1.
• Increase their knowledge and awareness of Alpha-1 as presented in the ATS/ERS
Statement: “Standards for the Diagnosis and Management of Alpha-1 Antitrypsin
Deficiency.”
• Have the knowledge of when to perform diagnostic testing based upon the classification
of recommendations for genetic testing (types A-D).
Please Circle the appropriate rating for each of the following questions:
4=Excellent 3=Good 2=Fair 1=Poor
1. Overall organization of this presentation 4 3 2 1
2. Ease of use of this presentation 4 3 2 1
3. The subject was of interest to me 4 3 2 1
4. Subject was relevant to my practice 4 3 2 1
5. Subject was of medical value for improving patient care 4 3 2 1
6. Extent to which it met the objectives 4 3 2 1
7. Subject was covered in adequate detail 4 3 2 1
8. Extent to which it was scientifically rigorous and balanced 4 3 2 1
9. Extent to which it met my expectations 4 3 2 1
10. Extent to which the content will cause me to make changes in my practice 4 3 2 1
Additional comments about this course/presentation?
Needs Assessment: I would like more information on the following subjects: