Rhinosinusitis, symptomatology & absence of polyposis in children with primary ciliary dyskinesia

M Rollin1, K Seymour, M Hariri

  • 1Department of Otolaryngology/Head & Neck Surgery, Charing Cross Hospital, London, United Kingdom.

Rhinology
|April 23, 2009
PubMed

Insights

Nasal polyps are not found in children with Primary Ciliary Dyskinesia (PCD), even though they experience chronic rhinosinusitis. Further research is needed to understand this unexpected finding in PCD patients.

Area of Science:

  • Pediatric Otolaryngology
  • Respiratory Medicine
  • Genetics

Background:

  • Primary Ciliary Dyskinesia (PCD) is an inherited disorder characterized by defective cilia leading to mucus buildup.
  • Common symptoms include recurrent ear infections and chronic sinusitis.
  • Nasal polyposis was previously hypothesized to be associated with PCD due to chronic inflammation.

Purpose of the Study:

  • To investigate the prevalence of nasal polyps in children diagnosed with PCD.
  • To evaluate the association between PCD, chronic rhinosinusitis, and nasal polyposis.

Main Methods:

  • A cross-sectional observational study was conducted over 3 months.
  • Included children attending a national tertiary-referral PCD clinic.
  • Utilized clinical examination and the SNOT-20 questionnaire.

Main Results:

  • Thirty children with PCD were included in the study.
  • No cases of nasal polyps were observed, despite significant rhinosinusitis symptoms.
  • The SNOT-20 questionnaire indicated high symptom burden, particularly in rhinological aspects.

Conclusions:

  • Nasal polyps are absent in children with PCD, contradicting previous hypotheses.
  • The lack of polyps in the context of chronic mucous stasis warrants further investigation into polyp pathogenesis.
  • This finding suggests unique inflammatory pathways in PCD patients.
Abstract

Related Concept Videos

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
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, but...
Respiratory Syncytial Virus Disease01:29

Respiratory Syncytial Virus Disease

Human respiratory syncytial virus (RSV) is a widespread pathogen that primarily targets infants and young children but also poses a serious health risk to elderly and immunocompromised individuals. Belonging to the Pneumoviridae family, RSV is a negative-sense, single-stranded RNA virus within the Pneumovirus genus. Its global health burden is significant, with millions of cases annually resulting in hospitalizations and mortality, particularly in resource-limited settings. Although most...
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 sinusitis...
Chronic Obstructive Pulmonary Disease IV: Clinical Manifestations01:19

Chronic Obstructive Pulmonary Disease IV: Clinical Manifestations

Chronic Obstructive Pulmonary Disease, or COPD, is a long-term condition marked by persistent and only partially reversible airflow limitation. It involves two overlapping conditions—chronic bronchitis and emphysema—which often co-appear but differ in dominant symptoms and underlying mechanisms.Chronic Bronchitis FeaturesChronic bronchitis presents with a persistent productive cough and thick, sometimes purulent mucus due to airway inflammation, enlarged mucus glands, and goblet cell...
Poliomyelitis01:17

Poliomyelitis

Poliomyelitis is caused by poliovirus, a small, non-enveloped, positive-sense RNA virus of the Picornaviridae family and Enterovirus genus. Transmission occurs primarily via the fecal-oral route, often through ingestion of contaminated water or food. The virus initially replicates in the oropharynx and intestinal mucosa, particularly in lymphoid tissues such as the tonsils, Peyer’s patches, and regional lymph nodes. Primary viremia follows, allowing dissemination throughout the body.In most...