Paeds & CF

Question 1

What is a common complication of ventilating newborns?

Answer 1

Pneumothorax?

Question 2

What should you remember as the other cause of difficulty breathing in newborns aside from respiratory distress syndrome?

Answer 2

Diaphragmatic hernia

Question 3

Buzzword - barking cough

Answer 3

Croup

Question 4

Briefly describe how CF is caused.

Answer 4

CF is caused by the CF transmembrane conductance regulator – chloride is pumped out of the cell, using ATP as energy. As well as this, it stops sodium coming in. If this doesn’t work, chloride can’t be pumped out and sodium is pumped in in great quantities – water follows sodium, leaving the lumen. This dries things out.

Question 5

Briefly describe the five classes of mutation in the CFTR gene.
What mutation causes 70% of all CF cases?

Answer 5

Class I- no synthesis whatsoever of CFTR – dies in utero
Class II – protein is made but is abnormal shape – can’t get through Golgi apparatus. This is most severe kind.
Class III – makes in to epithelial surface and embeds, but channel doesn’t open properly.
Class IV – channel only open partially – reduced amount of transport of fluoride through channel
Class V - channel is fine but number on epithelial surface if reduced.
F508 del

Question 6

Where in the lung is bronchiectasis if it is caused by CF?

Answer 6

Upper lobe