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Genetic abnormalities, Cystic fibrosis - Coggle Diagram
Genetic abnormalities
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Pathophysiology
Cystic fibrosis transmembrane conduction regulatory protein malfunction
CFTR is expressed largely in epithelial cells of the respiratory tract, gastrointestinal tract Sweat glands and genitourinary system.
It acts as a chloride channel and has other regulatory functions Alterations in chloride transport are associated with the production of abnormally thick secretions in glandular tissues
The pulmonary bronchioles and pancreatic ducts are mainly affected, often leading to progressive destruction of organs
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Complications
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Digestive system
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Rectal prolapse. Frequent coughing or straining during constipation can cause internal rectal tissue to protrude outside the anus.
Reproductive system
Almost all men with cystic fibrosis are infertile because the tube that connects the testes and prostate gland (vas deferens) are either blocked with mucus or missing entirely.
Women with cystic fibrosis may be less fertile than other women, it's possible for them to conceive.
Cystic fibrosis
Medication
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Mucus-thinning drugs to help you cough up the mucus, which improves lung function
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Health education
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Teach patient clearing or bringing up mucus, secretions from the airway
Encourage client to avoid smoking, dust, dirt, fumes and household chemicals