Cystic Fibrosis Flashcards
What is the complication of CF in males?
Infertility due to obstructive azoospermia
Why is CF associated with pancreatic insufficiency?
In 85% of cases, the pancreatic exocrine ducts can become sufficiently blocked, resulting in intestinal malabsorption.
Patients with CF are often infected with?
Pseudomonas aeruginosa and Staphylococcus Aureus
What are the complications of CF?
Male (and female) infertility Pancreatic insufficiency Recurrent lower respiratory tract infections CF-related diabetes Chronic liver disease CF arthropathy Upper airway complications such as nasal polyps and sinusitis Fat-soluble vitamin insufficiency Osteoporosis
What test is done to check for exocrine pancreatic insufficiency?
A non-invasive technique: Faecal elastase
* Elastase 1 is a pancreatic enzyme that can be detected in the serum, urine and faeces. It is a useful marker for pancreatic activity.
What is the management for those with pancreatic insufficiency?
Offer pancreatic enzyme replacement therapy for those with pancreatic insufficiency.
Consider an acid suppression agent, such as a PPI or H2 antagonist, if patient still presents with signs of malabsorption after pancreatic enzyme replacement therapy.
What test is done to check for CF in children?
Sweat test
What are the common signs and symptoms of children with CF?
In neonates, some will present with meconium ileus.
In older toddlers, they may present with slow growth, recurrent pneumonia (with clubbing of fingers) and steatorrhoea
What can a Chest X-ray show in a child with CF?
There will be shadowing suggestive of bronchiectasis, especially in the upper lobes.
What are the common causes of death in CF patients?
Death is often from pneumonia or cor pulmonale
What is meconium ileus?
Meconium is the first black sticky stool/poo that is passed by the neonate, usually happening within the first 24 hours after birth.
However, in Meconium ileus, there is a delay in passing the meconium due to the poo being too sticky and getting stuck in the intestines
What is the management of meconium ileus?
Baby will first have to stop being fed milk.
A tube will be passed through the nose into the baby’s stomach to drain the bile.
Contrast enema can help unblock the intestines, but sometimes, surgery is needed.
What are the newer treatment options for CF?
Recombinant human deoxyribonuclease (rhDNAse) which has been shown to improve lung function and reduce number of pulmonary exacerbations.
Lung transplantation
Gene therapy which aims to deliver cystic fibrosis gene into patients so they can make the CFTR protein.
