Evaluation of otorhinolaryngological manifestations in patients with primary ciliary dyskinesia.

OBJECTIVES: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by congenital impairment of mucociliary clearance causing recurrent respiratory tract infections. Pulmonary manifestations of PCD are well-known whereas adequate data on otorhinolaryngological complications is lacking. The aim of this study was to investigate clinical features, course and related factors of otorhinolaryngologic domains in PCD patients. METHODS: Patients with a diagnosis of PCD who were on follow-up in the ear-nose-throat (ENT) department of our center between 2000 and 2021 were enrolled. Demographic and clinical data, frequency of sinonasal and otological complaints, examination findings and possible risk factors associated with otorhinolaryngological diseases were obtained via electronic medical charts retrospectively. RESULTS: Of the 121 patients, 53% were male, median age at PCD diagnosis was 7 years (1 month - 20 yrs). The most common ENT manifestation was otitis media with effusion (OME) (66.1%, n = 80), followed by acute otitis media (43.8%, n = 53), acute rhinosinusitis (ARS) (28.9%, n = 35), chronic rhinosinusitis (CRS) (27.3%, n = 33) and chronic otitis media (10.7%, n = 13). Patients with ARS and CRS were significantly older than patients who did not have ARS and CRS (p = 0.045 and p = 0.028, respectively). The annual number of ARS attacks also correlated with age of patients positively (r = 0.170, p = 0.06). Of the 45 patients with pure-tone audiometry, most common finding was conductive hearing loss (CHL) in 57,8% (n = 26). Presence of OME significantly increased tympanic membrane injury which was observed as sclerosis, perforation, retraction or changes due to ventilation tube insertion (VTI). (OR: 8.6, 95% CI: 3.6-20.3, p < 0.001). CONCLUSIONS: Otorhinolaryngologic diseases are common, variable and complicated in PCD patients, consequently ENT physicians' awareness should be improved through shared experiences. ARS and CRS seem to appear in older PCD patients. Presence of OME is the most important risk factor for tympanic membrane damage.

Reversible bronchiectasis caused by influenza virus mimicking Williams-Campbell syndrome.

The term bronchiectasis refers to permanent enlargement of the bronchi. It is increasingly diagnosed because of high-resolution computed investigations. It can be congenital or acquired, the latter mostly following infection. Williams-Campbell syndrome is a rare form of congenital non-cystic fibrosis bronchiectasis. Here we report a 5-month-old girl with reversible bronchiectasis treated with extracorporeal membrane oxygenation for acute respiratory distress syndrome (ARDS) caused by influenza virus following surgery for congenital heart disease. Chest CT showed an abnormally large bronchial tree mimicking Williams-Campbell syndrome. At 9 months later, chest CT showed regression of bronchiectasis and normalized caliber of previously collapsed segments in both lungs. This atypical course illustrates that influenza virus can cause reversible bronchiectasis in infants and mimic congenital disease such as Williams-Campbell syndrome.

Clinical implications of fungal isolation from sputum in adult patients with cystic fibrosis

Background/aim: Cystic fibrosis is an autosomal recessive disease with a defect in mucociliary activity that is characterized by recurrent pulmonary infections. Bacterial agents frequently implicated in airway colonization are Haemophilus influenzae, Staphylococcus spp., and Pseudomonas spp. Fungal isolation from sputum is common in adults. However, growth of fungal agent only in sputum culture in patients with cystic fibrosis is insufficient for the diagnosis of fungal diseases. There is limited data about the clinical significance of fungal isolation in sputum cultures. The aim of the study was to investigate the clinical outcomes andsignificance of fungal isolation from sputum samples in adult CF. Materials and methods: This retrospective study included patients who have been admitted between October 2017 and January 2019 in an adult cystic fibrosis unit. Patients were grouped according to fungal pathogenicity as; fungal disease group, colonization group, and nonisolated group. The data of the last one year, including demographics, clinical data, laboratory, treatment modalities, results of cultured bacteria and fungus from sputum samples, respiratory function parameters, frequency of exacerbation, and hospitalizationwere compared between groups. Results: A total of 330 sputum samples from 88 adult patients with CF were collected. Patients were divided into 3 groups, the fungal disease group (n = 10, 11.4%), colonization group (n = 49, 55.7%), and nonisolated group (n = 29, 32.9%). Presence of pulmonary exacerbation, number of admissions to emergency department, and the number of positive cultures for bacteria from sputum were higher in the fungal disease group (p = 0.03, p = 0.01 and p < 0.001). The fungal disease group had higher rate of antibiotics by parenteral routethan other groups (p = 0.001) whereas lung functions were similar. Use of nutritional supplementation and parenteral antibiotherapy were the factors associated with elevated risk of fungal isolation. Conclusion: Frequent use of parenteral antibiotics and use of nutritional supplementation were found to be independent risk factors for fungal isolation from sputum in adult CF.