Alpha one antitrypsin deficiency,diffuse interstitial pneumonitis,amiodarone (MRCP Part 1 MCQS)

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01)  alpha one antitrypsin deficiency is most likely to be manifested by the following phenotype

a) PiZZ
b) PiMM
c) PiZM
d) PiCZ
e) PiCC

02)  diffuse interstitial pneumonitis with interstitial edema, varying degrees of fibrosis, lymphoid cell infiltration, and alveolar-cell hyperplasia are pathological features typical of :

a) CMV
b) Influenza virus
c) RSV
d) Parainfluenza virus
e) Paramyxovirus

03)  Which of the following medications have the highest risk of producing the arrythmia known as torsades de pointes when the patient is also taking amiodarone?

a) Flecainide
b) Phenytoin
c) Propafenone
d) Chlorpromazine
e) Lidocaine


01) The correct answer is A 

Explanation

A1AD is hereditary; it is passed onto children by their parents. Everyone receives one alpha-1 antitrypsin gene from each parent. A person who inherits an M gene (normal gene) from each parent has normal A1AD levels. People with no abnormality are designated PiMM. If a person inherits one normal gene and one abnormal gene (Z), they are carriers of the disease and have a phenotype of PiMZ. This person may not have normal alpha-1 antitrypsin levels, but there is usually enough to protect the lungs. PiZZ is the most common phenotype that can cause disease. This occurs when a person inherits the abnormal Z gene from both parents. It usually results in a severe deficiency which is associated with an increased risk of liver disease in childhood, and emphysema later in life.
Combination. 4747 Question number 2 
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02) The correct answer is A 

Explanation

The two patterns of CMV involvement include (1) a multifocal or miliary pattern characterized by discrete spherical lesions as large as 4 mm in diameter, with alveolar hemorrhage, fibrin deposition, and a moderate neutrophilic response, and (2) a diffuse interstitial pneumonitis with interstitial edema, varying degrees of fibrosis, lymphoid cell infiltration, and alveolar-cell hyperplasia
Combination. 4857 Question number 3 
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03) The correct answer is D 

Explanation

Common clinical features in drug-induced torsade de pointes include hypokalemia and cycle-length prolongation just prior to initiation of the arrhythmia
Among these agents sotalol, which is a class III agent, has the highest risk of producing torsades, which is a polymorphic, pause-dependent ventricular tachycardia causing syncope and sudden cardiac death.


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