Evolution of sinonasal clinical features in children with cystic fibrosis

P Suy1, A Coudert2, S Vrielynck3

  • 1Service d'ORL pédiatrique, Hôpital femme Mère Enfants, Centre Hospitalier et Universitaire Lyon, France; Service d'ORL, Hôpital Edouard Herriot, Centre Hospitalier et Universitaire Lyon, France.

Insights

Sinonasal symptoms in children with cystic fibrosis (CF) improve significantly during childhood. This improvement in nasal obstruction and rhinorrhea suggests better management and natural nasal cavity growth in CF patients.

Area of Science:

  • Pediatric Otolaryngology
  • Respiratory Medicine
  • Genetics and Inherited Diseases

Background:

  • Cystic Fibrosis (CF) prognosis has improved, necessitating evaluation of associated sinonasal manifestations.
  • Sinonasal issues are common in CF patients, impacting quality of life.

Purpose of the Study:

  • To assess the evolution of sinonasal manifestations in children with cystic fibrosis.
  • To correlate sinonasal symptom changes with CF disease severity and management.

Main Methods:

  • Observational, retrospective cohort study of 173 children (4-18 years) with CF.
  • Sinonasal symptoms, physical examination, and CF data collected at symptom onset, peak severity, and end of follow-up.

Main Results:

  • Sinonasal symptoms appeared early (5.4 yrs), peaked (6.9 yrs), and significantly improved by end of follow-up.
  • Nasal obstruction, rhinorrhea, and snoring showed significant improvement.
  • Lildholdt score, turbinate hypertrophy, and maxillary sinus wall bulging also improved.

Conclusions:

  • Sinusitis in children with CF improves during childhood, with better outcomes than at initial management.
  • Improvement may stem from effective ENT management and nasal cavity growth, independent of other CF-related issues.
Abstract

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
743
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
485
COPD: Pathogenesis and Clinical Features01:20

COPD: Pathogenesis and Clinical Features

Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
1.8K
Convergent Evolution01:54

Convergent Evolution

Evolution shapes the features of organisms over time, ensuring that they are suited for the environments in which they live. Sometimes, selection pressure leads to the rise of similar but unrelated adaptations in organisms with no recent common ancestors, a process known as convergent evolution.
31.6K
Esophageal Strictures-II: Clinical Features and Management01:26

Esophageal Strictures-II: Clinical Features and Management

Patients with esophageal strictures often experience a range of symptoms. Initially, they may have difficulty swallowing solid foods, which can progress to include liquids. Additional symptoms may involve chest pain or discomfort, regurgitating food and fluids, heartburn, unintentional weight loss, coughing or choking during meals, and hoarseness.
Healthcare providers should gather a comprehensive medical history and conduct a physical examination for diagnosis. If esophageal stricture is...
470
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
454