a good question

[match2011]

20-month-old girl, fever and cough for 2 days. She has had several similar episodes since the age of 4 months. Three months ago, she and her family visited her grandmother in Finland for 2 weeks. She is at the 25th percentile for length and 5th percentile for weight. She appears thin and pale. Her temperature is 38 C (100.4 F), pulse is 150/min, and respirations are 40/min. Examination shows mild clubbing. Wheezing and bilateral crackles are heard at the lung bases. An x-ray film of the chest shows streaky densities bilaterally with mild hyperinflation.

How can u tell the difference of chest X-ray between α1-Antitrypsin deficiency and Cystic fibrosis ?

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[Surf Rx]

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[OveractiveBrain]

20-month-old girl, fever and cough for 2 days. She has had several similar episodes since the age of 4 months. Three months ago, she and her family visited her grandmother in Finland for 2 weeks. She is at the 25th percentile for length and 5th percentile for weight. She appears thin and pale. Her temperature is 38 C (100.4 F), pulse is 150/min, and respirations are 40/min. Examination shows mild clubbing. Wheezing and bilateral crackles are heard at the lung bases. An x-ray film of the chest shows streaky densities bilaterally with mild hyperinflation.

How can u tell the difference of chest X-ray between α1-Antitrypsin deficiency and Cystic fibrosis ?

The fact that one is a 20month old girl and the other is a 55 year old man? A1-AT doesn't present with emphysema until late in life. Its an adult disease. CF is a kids disease. Kids with CF don't really enter adulthood, and certainly not old age.

The question doesn't have anything to do with the CXR. Its that she has a failure to thrive (small stature and low weight) with pulmonary disease and repeated pulmonary infections. They can't very well say "mom thinks her baby tastes salty" or "genetic screening at birth showed CF" now can they?

 

[match2011]

The fact that one is a 20month old girl and the other is a 55 year old man? A1-AT doesn't present with emphysema until late in life. Its an adult disease. CF is a kids disease. Kids with CF don't really enter adulthood, and certainly not old age.

The question doesn't have anything to do with the CXR. Its that she has a failure to thrive (small stature and low weight) with pulmonary disease. They can't very well say "mom thinks her baby tastes salty" or "genetic screening at birth showed CF" now can they?

thanks

 

[PokerDoc]

Well the chest x-ray is just supporting evidence of the acute pulmonary exacerbation the patient is experiencing.. but yes, obviously unneeded for this question.

 

[ThePursuit]

As Overactivebrain did a great job of pointing out, age is the only thing you really need here. alpha1 def won't present in a young kid. Additionally, if a patient did actually have A1, the CXR would show diffuse emphysema (in a question) vs. CF that would show other stuff like bronchiectasis, fibrosis...