Cystic Fibrosis

Question 1

● aka Mucoviscidosis
● An inherited disorder of the exocrine gland (Ex: sweat gland, pancreas)

Answer 1

Cystic Fibrosis

Question 2

Cystic Fibrosis Manifestation

Answer 2

○ Glands produced abnormally thick secretion
○ Sweat electrolytes are elevated
○ Saliva- there is increased organic and enzymatic constituents
○ Muco-secreting organ - respiratory system
○ Overactivity of autonomic nervous system

Question 3

Defective gene- Cystic fibrosis transmembranous conductance regulator gene; located on

Answer 3

chromosome 7

Question 4

Cystic fibrosis transmembranous conductance regulator protein

Answer 4

Defective protein

Question 5

Usually recognized in infancy or early childhood

Answer 5

Cystic fibrosis

Question 6

Cystic fibrosis Affected organs:

Answer 6

pancreas, respiratory system, sweat glands, salivary glands

Question 7

Cystic fibrosis Signs:

Answer 7

Chronic Cough
Frequent foul smelling stool
Persistent Upper Respiratory Tract (URT) Infection

Question 8

overproduction of mucus

Answer 8

Chronic Cough

Question 9

due to high organic and enzymatic constituents of saliva, change in the activity of pancreas

Answer 9

Frequent foul smelling stool

Question 10

mucus traps bacteria → multiplication

Answer 10

Persistent Upper Respiratory Tract (URT) Infection

Question 11

Cystic fibrosis Test requirements:

Answer 11

48 hrs of age (newborns), without rashes, cuts, or skin inflammation where sweat can be collected

Question 12

Diagnostic test: Sweat test
○ Measurement of sweat electrolytes (sodium and chloride) using

Answer 12

coulometric principle

Question 13

Diagnostic test: Sweat test
○ Sweat sample must be collected within

Answer 13

30 minutes

Question 14

Diagnostic test: Sweat test
○ Sweat must be more than

Answer 14

50 mg of sample

Question 15

Reference method

Answer 15

○ Gibson and Cooke Pilocarpine Iontophoresis
○ (+) Result: >65 mmol/L of sweat
electrolytes (5-40 mmol/L *Normal range)