Cystic Fibrosis

Pulmonology

Overview

Definition

Autosomal recessive genetic disorder affecting the CFTR gene, causing thick mucus.

Epidemiology

Most common fatal genetic disease in Caucasians.

Etiology & Risk Factors

  • CFTR gene mutation (delta F508)

Clinical Symptoms

  • Chronic productive cough
  • Recurrent infections
  • Malabsorption
  • Salty skin

Clinical Approach

Diagnosis

  • Sweat chloride test
  • Genetic testing

Management

  • CFTR modulators (e.g., Trikafta)
  • Chest physiotherapy
  • Pancreatic enzymes

Complications

  • Bronchiectasis
  • Pancreatic insufficiency
  • Infertility