pulmonology simonsez please look at this Q too - cris

[ArchivalUser]

20 mounth old baby with fever and cough for 2 days. She has similar episodes since the age of 3 m. She and her family visited her grandmother in Finland for 2 weeks She is at 25 precentile for weight and 5 th percentile for high She appears thin and pale Her tem 38 C pulse is 150/min resp.44/min
examination show clubbing wheezing and bilateral cracklesbeest heard at the lung basesx ray film of the chest shouws streacky densites bilateraly with hyperinflation Diagnosis is
A alpha trypsin deficency
B CF
C Pneumothorax
d pulmonary alveolar proteinosis
D Aspergillosis
F TB

Please help with any suggestions because I thinking about option A since the hyperinflation are mostly on the bases of lung and the child has grandmother in Finland, CF has more predilection in upper zones but I am not sure Anyone can help

[ArchivalUser]

hi cris,
I will choose B with a 50-50 probability. Though alpha1- antitrypsin def is the MCC of emphysema in a young adult/middle aged patient, I doubt if destructive changes are seen so early in the course. But this Q has buzzwords for AAT def like "hyperinflation" and lower lobe crackles(these changes can be seen in early Xray findings of CF as well)
The symptoms which favor CF being recurrent infections and bronchiectasis like features.
I would have chosen AAT def. if the liver involvement was predominant as I remember reading that AAT predom present with liver abnormalities in childhood with emphysema being a problem in young adults and middle age people.
chill

[ArchivalUser]

Thank you Simonsez

[ArchivalUser]

hi,
Is this an NBME q? I remember this q from somewhere. I think I had answered AAT. But now when I think about it CF seems so right. Like I said Cris, its a 50-50 deal. I hope u get a better answer from someone.
best of luck
chill

[ArchivalUser]

Simonsez it is NBME form 1