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[Pneumonectomy in cystic fibrosis]

P Ferrer González1, A Pereda Pérez, J Pastor Guillén

  • 1Gastroenterología Infantil. Hospital Universitario La Fe. Valencia. España.

Anales De Pediatria (Barcelona, Spain : 2003)
|March 12, 2003
PubMed

Insights

A 13-year-old with cystic fibrosis (CF) experienced severe lung infection. Surgical removal of the affected lung led to significant, long-term improvement in quality of life.

Area of Science:

  • Pulmonology
  • Nephrology
  • Surgical Oncology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder primarily affecting the lungs, leading to chronic infections and progressive lung damage.
  • Lung disease is the leading cause of mortality in adult CF patients, with lung transplantation being the standard treatment for severe bilateral disease.
  • Comorbidities like nephrotic syndrome and secondary amyloidosis can complicate CF management.

Observation:

  • A 13-year-old male with CF presented with a severe right-sided lung infection, including abscess formation and localized bronchiectasis.
  • The patient's condition was further complicated by nephrotic syndrome and secondary amyloidosis.
  • Unilateral pneumonectomy (surgical removal of one lung) was performed to address the severe, localized lung disease.

Findings:

  • The pneumonectomy resulted in significant clinical improvement for the patient.
  • A remarkable increase in the patient's quality of life was observed post-surgery.
  • These positive effects have been sustained for 15 years following the procedure.

Implications:

  • Surgical resection of severely affected unilateral lung segments offers a viable rescue therapy option for CF patients.
  • This approach is particularly relevant for patients with localized bronchiectasis or those at high risk of mortality while awaiting lung transplantation.
  • Pneumonectomy can significantly improve long-term outcomes and quality of life in select CF cases with unilateral lung disease.

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