Flashcards in CF clinical Deck (39)
what are the characteristics of CF patients?
bronchiectasis under 40
upper lobe bronchiectasis
colonisation with staph aureus
1st patient case
has persistent cough
frequent chest infections
frequent courses of antibiotics
occasional chest pains
moves around a lot so doesn't keep the same GP for a very long time
case 1 - examination
crackles in both upper zones
CXR shows signet ring
case 1 - what she doesnt mention?
younger sister has CF
Another sister died age 8 with “A Respiratory Illness
case 1 - tests
Confirms diagnosis of Cystic Fibrosis
Referred on from my normal clinic to the CF service
65 Year old woman
Asthma since childhood
Frequent chest infections
case 2 - examinations
Normal pulmonary function
More frequent chest infections over past 6 months
case 2 - clinical course
what are the clinical features of CF for neonates?
Failure to thrive; meconium ileus; rectal prolapse.
what are the clinical respiratory features of CF for children and young adults ?
cough; wheeze; recurrent infections; bron- chiectasis; pneumothorax; haemoptysis; respiratory failure; cor pulmonale.
what are the clinical GI features of CF for children and young adults ?
pancreatic insufficiency (diabetes mellitus, steatorrhoea); distal intestinal obstruction syndrome (meconium ileus equivalent); gallstones; cirrhosis.
what are the other clinical features of CF for children and young adults ?
male infertility; osteoporosis; arthritis; vasculitis ; nasal polyps; sinusitis; and hypertrophic pulmonary osteoarthropathy (HPOA). Signs: cyanosis; finger clubbing; bilateral coarse crackles.
how is a diagnosis made?
genetics - screening for known common mutations
faecal elastase is a simple and useful screening test for exocrine pancreatic dysfunction
what are the features of sweat test of CF?
sweat sodium and chloride >60mmol/L;
chloride usual- ly > sodium
what things are analysed in blood tests?
FBC, U&E, LFT; clotting; vitamin A, D, E levels; annual glucose tolerance test
how is bacteriology assessed?
cough swab, sputum culture.
what radiological tests are done and what would they show?
CXR; hyperinflation; bronchiectasis.
Abdominal ultrasound: fatty liver; cirrhosis; chronic pancreatitis
what would spirometry show?
how are patients with CF best managed?
Patients with cystic fibrosis are best managed by a multidisciplinary team, eg physician, GP, physiotherapist, specialist nurse, and dietician, with attention to psy- chosocial as well as physical wellbeing. Gene therapy (transfer of CFTR gene using liposome or adenovirus vectors) is not yet possible.
what happens in exocrine failure ?
Sludged up ducts
Failure of secretion of lipases, amylase
what is the treatment for this prophylactically
Patients hate taking it
Without it they can’t absorb energy
what happens in endocrine failure
destruction of pancreatic islet cells
fatty replacement of pancreatic tissue
what is the treatment for endocrine failure prophylactically
Usually need insulin as they have insulin production failure
what happens to the bowels in CF?
Thick mucus blocks up the large and small intestine
Symptoms similar to constipation
what is used in the treatment and prevention of these bowel problems
what happens to the liver in CF?
sludging up of the hepatic ducts
EITHER INTRA HEPATIC OR EXTRA HEPATIC
what does portal hypertension cause?
what is the treatment for liver problems
what does TIPSS do?
Reduction in bleeding risk
Can increase encephalopathy risk