This is a PA chest x-ray is of a 19 year old female, showing extensive abnormalities of the lung parenchyma, most pronounced by the central tram-track opacities. Apart from respiratory symptoms, what else is she likely to present with?

A) Obesity.
B) Diarrhoea.
C) Hypertension.
D) Tremor.
E) Recurrent epistaxis. 



The answer is B. This is chest x-ray shows extensive abnormalities of the lunch parenchyma characterised by the presence of multiple ring shadows and tram-track opacities. These are more pronounced centrally and are symmetric. A PICC line is in situ.

Findings are consistent with bronchiectasis and the patient is known to have cystic fibrosis.

Cystic fibrosis is the most common autosomal recessive disease among Caucasians [1 in 2500] and is caused by defective epithelial chloride channel (CFTR) resulting in excessively concentrated epithelial secretions. With the lungs this presents as bronchiectasis, atelectasis and recurrent pneumonia. In the liver portal hypertension and gallstones develop. There is diarrhoea and weight loss to due pancreatic enzyme insufficiency. 30% of people with CF develop diabetes mellitus type 1. There is infertility in both males [hypogonadism, azoospermia, vas deferens maldevelopment] and females [cervical mucus thickening].

Image courtesy of Dr Jeremy Jones, Radiopaedia.org. (Original file here) Creative Commons BY-SA-NC