CF Flashcards
Bugs airway colonists CF
Pseudomonas
Staph aureus
Burkholderia
Sweat test
Pilocarpine stimulates sweat gland
Cl- >60 diagnositic
False pos Eczema Ectodermal dysplasia Malnutrition/FTT AN G6PD Hypothyroidism Nephrogenic DI Klinefelter
CF pulmonary function
Obstructive with modest response to bronchodilator
Early: increased residual volume & FRC
Late: restrictive changes
Human recombinant DNase
2.5mg daily
Improves pulmonary function
Decreases exacerbations
Improves sense of well being
For moderate disease with purulent sputum
Indication for inhaled tobramycin
Longterm supressive therapy for those colonised with pseudomonas
Fewer hospitalisations and improves lung function
Ivacaftor
Small molecule potentiator of the CFTR mutation G551D (~5%)
A class 3 mutation which results in protein localised to plasma membrane, but loss of chloride channel fnx
Improves FEV1 and reduces frequency of exacerbations
Frequency of ABPA
Tests to dx
5-10% Deterioration Skin prick Sputum: rust coloured, culture aspergillus Peripheral eosinophilia
Nutrition in CF
90% have pancreatic exocrine fnx impairments
Higher calories
DEKA
Enzyme replacement
Correlation between BMI & lung function
Risk of pancreatic enzyme replacement
PPI indications
Colonic strictures
Not more than 2,500 lipase units/kg/meal
Mec ileus
Rx
NG
Gastrograffin
Surgery
Zinc deficiency
Acrodermatitis enteropathica
Distal intestinal ileus syndrome
2-5% patients w CF accumulate faecal material in terminal ileum & caecum
Increase pancreatic enzyme
Stool softener
Increased fluid intake
Ddx
Intususseption
Volvulus
Frequency of portal hypertension in CF
Acute mgmt
Definitive Treatment
8%
Nasogastric suction and cold saline lavage
Sclerotherapy
Nasal polyps CF
15-20%
2nd decade
Local steroids & decongestants
CF- surgery
Mod to severe pulmonary disease benefit from 1-2 w ivabx and intensive pt
