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[Controversies in cystic fibrosis--from pediatrician to specialist]

Jose Dirceu Ribeiro1, Maria Angela G de O Ribeiro, Antonio Fernando Ribeiro

  • 1Departamento de Pediatria, Universidade Estadual de Campinas, Campinas, SP. dirceu@head.fcm.unicamp.br

Jornal De Pediatria
|December 17, 2003
PubMed

Insights

Cystic fibrosis (CF) is a serious inherited disease affecting multiple organs. Recent discoveries in CF etiology and pathophysiology have led to improved treatments and patient survival, despite no definitive cure.

Area of Science:

  • Genetics
  • Pulmonology
  • Gastroenterology

Context:

  • Cystic fibrosis (CF) is a significant inherited disorder impacting various organs, primarily the lungs and gastrointestinal tract.
  • Patient clinical responses to CF vary widely, despite its genetic basis.
  • The global prevalence of CF necessitates updated medical understanding and treatment strategies.

Purpose:

  • To provide pediatricians with a comprehensive review of current controversies and recent advancements in cystic fibrosis (CF) treatment.
  • To synthesize current knowledge on CF incidence, pathophysiology, diagnosis, and therapeutic interventions.

Summary:

  • A systematic review of 79 articles from international journals on cystic fibrosis (CF) was conducted.
  • The review critically examines key developments in CF, including its incidence, underlying mechanisms, diagnostic methods, and treatment approaches.
  • Key findings highlight progress in understanding CF's origins and disease processes over the past two decades.

Impact:

  • Advances in understanding cystic fibrosis (CF) etiology and pathophysiology have significantly improved patient management and survival rates.
  • The review offers valuable insights for pediatricians managing CF patients, guiding them on current treatment controversies and emerging therapies.
  • Ongoing research continues to enhance therapeutic options and long-term outcomes for individuals with cystic fibrosis (CF).
Abstract

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