CBL: Respiratory Flashcards
PC: Recurrent chest infection
What further questions to ask to explore it further?
Recurrent infection in a child - > 12 in a year
- Tell the parents to define how many
- How sick (severity) e.g. attendance to school, GP, hospital, did they require Abx/X ray/ admissions etc.
- Was recovery normal length or longer
What is the red flag for a child presenting with ‘recurrent infection’?
Red flags: if a child had an uncommon infection e.g. TB, Pseudomonas -> is the immune system ok?
Case: 18 month old girl has recurrent chest infections and failure to thrive, chronic diarrhoea but had a normal newborn screening test
What investigations would you perform?
-
Investigations:
* Urinalysis
* Respiratory exam -> for signs of an acute illness and chronic signs (e.g. clubbing)
* chest x ray -> possibly not when sick if you are worried about chronic; acute if required during acute infection
* test the responses for antibodies -> by vaccines (e.g. response to HiB, pneumococcal)
* sputum culture -> by cough swaps/ induce cough swaps, sputum (if able to obtain)
* FBCs -> look at all components
* LFTs à raised enzymes with viral infections, U&Es
* Sweat test à repeat
* Stool sample
What (on the resp exam inspection) would you see as a sign of a long-term difficulty of breathing in a child?
- Excavatum or carinatum -> rib cage would deform as a child would try hard to breath -> deformities form as a cartilage develops
- Use of accessory muscles etc
Would crackles be usually audible in a child presenting with a chest infection? Why?
In children:
when there is a chest infection -> decreased air entry (before crackles develop as paediatric pts tend to be seen earlier - worried parents - so crackles have not developed yet)
Newborn screen for CF - what does it include?
Newborn screening for CF:
- Blood spot immunoreactive trypsinogen test (IRT) -> released from pancreas damaged by potentially blocked ducts (*it is a first stage of testing)
- Gene testing (if positive at IRT)
- Sweat test -> to analyze Na+ in sweat (30 is normal value; below 30 is not likely to be CF)
Other testing (e.g. to confirm, when symptoms are manifested):
- Genetic testing
- Sweat test
Simple genetics and pathophysiology of CF
The genetics and pathophysiology of CF
- Pathophysiology: genetic mutation -> defect in Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) -> defects in Cl- channel
- Autosomal recessive (AR) type
- In the UK, 80% mutations on chromosome 7 -> delta 508 type
Give names of few organisms that may colonise a patient with CF
Organisms which may colonise CF patients
- Staphylococcus aureus
- Pseudomonas aeruginosa
- Aspergillus
- Haemophilis influenza
- Burkholderia ce pacia
- Non-TB Mycobacterium
What diet is recommended for CF patients?
- high calorie diet, including high fat intake, normal protein
- Creon before all meals containing fat (excluding vegetables and fruits) is usually taken -> to replace pancreatic enzymes
(Creon granules for baby, capsules) -> delta 508 causes pancreatic insufficiency
What medication is used to help with mucous?
(to make it thinner and easier to cough out)
mucolytic agent e.g. Dornase alpha (dnAse)
What med is used to treat Staph Aureus infection in CF patient?
- Staph Aureus -> Flucloxacillin prophylaxis / anti-Staphylococcus Aureus agent (if allergic to Penicillin)
How to treat CF patient with Pseudomona Auerginosa infection?
1st line
2nd line (if 1st line does not work)
- Pseudomona Auerginosa -> oral (if well) or / and IV antibiotics and inhaled (if culture is positive)
*if those treatments against Pseudomona do not work -> use nebulised Colistimethate Sodium (polymixin class of antibiotic against gram negative bacilli)
Name of a medication that is used in (suitable mutation) CF patient to improve work of CFTR channel
Meds to improve how CFTR channel work -> Ivacaftor (Kalydeco)
Name the medication that is used to improve liver function (e.g. in CF patient)
To improve liver function:
Ursodeoxycholic acid -> to improve liver function if needed
