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Flashcards in CF clinical Deck (39)
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1

what are the characteristics of CF patients?

bronchiectasis under 40

upper lobe bronchiectasis

colonisation with staph aureus

infertility

low weight

2

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

3

case 1 - examination

BMI 19

crackles in both upper zones

otherwise normal

CXR shows signet ring

4

case 1 - what she doesnt mention?

younger sister has CF

Another sister died age 8 with “A Respiratory Illness

5

case 1 - tests

Del508
Confirms diagnosis of Cystic Fibrosis
Referred on from my normal clinic to the CF service

6

case 2

65 Year old woman
Asthma since childhood
Frequent chest infections

7

case 2 - examinations

Normal pulmonary function
No Allergies
No Wheeze
More frequent chest infections over past 6 months

8

case 2 - clinical course

HRCT
Sputum Culture
Staph Aureus
Flucloxacillin
Repeat Culture
Staph Aureus

9

what are the clinical features of CF for neonates?

Failure to thrive; meconium ileus; rectal prolapse.

10

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.

11

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.

12

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.

13

how is a diagnosis made?

sweat test

genetics - screening for known common mutations

faecal elastase is a simple and useful screening test for exocrine pancreatic dysfunction

14

what are the features of sweat test of CF?

sweat sodium and chloride >60mmol/L;

chloride usual- ly > sodium

15

what things are analysed in blood tests?

FBC, U&E, LFT; clotting; vitamin A, D, E levels; annual glucose tolerance test

16

how is bacteriology assessed?

cough swab, sputum culture.

17

what radiological tests are done and what would they show?

CXR; hyperinflation; bronchiectasis.

Abdominal ultrasound: fatty liver; cirrhosis; chronic pancreatitis

18

what would spirometry show?

obstructive defect

19

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.

20

what happens in exocrine failure ?

Sludged up ducts
Failure of secretion of lipases, amylase
Digestive failure

21

what is the treatment for this prophylactically

CREON

Patients hate taking it
Without it they can’t absorb energy

22

what happens in endocrine failure

destruction of pancreatic islet cells

fatty replacement of pancreatic tissue

23

what is the treatment for endocrine failure prophylactically

Annual OGTT
CGMS

Usually need insulin as they have insulin production failure

24

what happens to the bowels in CF?

Thick mucus blocks up the large and small intestine
Symptoms similar to constipation

25

what is used in the treatment and prevention of these bowel problems

Treatment:
Gastrograffin
Laxido
Fluids

Prevention:
Laxido
Hydration
Keep moving

26

what happens to the liver in CF?

sludging up of the hepatic ducts
EITHER INTRA HEPATIC OR EXTRA HEPATIC

portal hypertension

27

what does portal hypertension cause?

Porto-systemic anastamoses
Variceal Bleeding
Hepatic encephalopathy

28

what is the treatment for liver problems

TIPSS

29

what does TIPSS do?

Reduces anastamoses
Reduction in bleeding risk
Can increase encephalopathy risk

30

how are exacerbations managed?

antibiotics

physiotherapy

adequate hydration

increased dietary input