Presentation

1. Cystic Fibrosis
is genetic disorder( autosomal recessive) is disorder of the
exocrine gland in the particular case these exocrine gland
of the bronchioles,small intestines ,pancreas and pile
ducts are effected secretion of abnormally thick mucus all
of these organ

2. Sings and Symptoms
Respiratory system
digestive system exocrine gland
wheezing,
dry and non productive cough
frequent respiratory infection.
in later stages :
the cough will turn wet with thick
sputum crackles ,ronchi
dyspnea or tachypnea,
emphysema and atelectasis
cor pulmonale (Right sided heart
failure), clubbing of the finger and
barrel chest
steatorrhoea or fatty stool
malnutrition and delayed growth
deficient in Vitamin A,D,E and K
A lead to eye tissue thickening
K
lead to problem with clotting or
bleeding disorders.
protuberant abdomen and thin
extremities
thin and underweight .
increase sodium and
chloride in the sweat
Risk for electrolyte
imbalances

3. Diagnostic
Routine newborn screeningPilocarpine
Pilocarpine iontophoresis
test (measure the sodium and chloride of
sweat )
72
_
hour fecal fat determination test
Liver function
X_ray
Sputum cultures
Pulmonary function

4. Treatment goal
Promote optimal
nutrition

5. Management
Respiratory system
Mobilize the secretion
Regular aerobic exercise
Antibiotics
Steroids
Need supplemental oxygen
Genetic counseling

6. Mobilize the secretion
Chest physiotherapy Postural drainage Percussion

7. Digestive system
High calorie Pancreatic enzymes
High protein diet Fat soluble vitamin supplements
Management