Discussion in 'Spot Diagnosis' started by J.P.C. Peper, Jun 2, 2012.
What's your diagnosis?
I'll post the correct answer in a day or three!
yes,it's cystic fibrosis
Cystic fibrosis.. nodular densities of mucoid impaction and diffuse interstitial disease with bronchiectasis
That's right, it's cystic fibrosis.
It's an autosomal recessive genetic condition caused by a mutation coding for the CFTR (chloride transporter) channel, which results in hardening of mucus throughout the body. The picture shows mucus impaction, bronchiectasises and possibly signs of cor pulmonale, all fitting the diagnosis.
Other related consequences or signs include bad growth (caused by reduced absorption in the digestive tract, because of the affected mucus) and a salty skin.
Some Differentials ( based on what I have seen ), since Cystic Fibrosis is not common in my part of the world
- atypical pattern of Pneumocystis jirovecii ( formerly called carinii ) pneumonia
- Aspergillosis ( mucus impaction in this case, is called "finger in glove" pattern )
- Acute on Chronic bronchitis or chronic bronchitis by itself
- 2 cases of suspected bronchopneumonia that i investigated had underlying leukemia ( CLL and AML ) and had a very similar Xray picture
So mucus impaction, nodular densities, bronchiectasis and cor-pulmonale are not 'tell-tale signs' of Cystic fibrosis ( even in places where the disease is common ), unless there is support lab evidence or other features of Cystic Fibrosis.
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