scholarly journals Follow-Up and Management of Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia: Review and Experience of Our Reference Centers

2019 ◽  
Vol 8 (9) ◽  
pp. 1495 ◽  
Author(s):  
Emilie Bequignon ◽  
Laurence Dupuy ◽  
Virginie Escabasse ◽  
Francoise Zerah-Lancner ◽  
Laurence Bassinet ◽  
...  
Keyword(s):  
Chronic Rhinosinusitis ◽  
Primary Ciliary Dyskinesia ◽  
Adult Patients ◽  
Middle Meatus ◽  
Endoscopic View ◽  
Therapy Strategy ◽  
Significant Difference ◽  
Diagnostic Work ◽  
Surgical Treatments ◽  
Ciliary Dyskinesia

Chronic rhinosinusitis is the foremost manifestation in adult patients with primary ciliary dyskinesia (PCD). We present a retrospective series of 41 adult patients with a confirmed diagnosis of PCD followed in our reference centers. As part of the diagnostic work up in our centers, sinus computed tomography scans (CTs) are systematically performed. All patients also undergo a sampling of purulent secretions sampled from the middle meatus under endoscopic view for bacteriological analysis. In our series, CT opacities were consistent in all the patients, as well as mainly partial and located in ethmoid cells (100% of patients) and in maxillary sinuses (85.4% of patients), and stayed stable over time. In the 31 patients who had purulent secretions, bacteriological culture showed at least one bacterium in 83.9% (n = 26). There was no significant difference in positive cultures for Pseudomonas aeruginosa in patients >40 years old versus those <40 (p = 0.17; Fisher). Surgical management was performed in only 19% of patients in order to improve sinonasal mechanical drainage. Our data support the hypothesis that the sinuses can be considered as a bacterial reservoir. From this retrospective study, we have introduced several changes into our routine clinical practice in our reference centers. Based on our analyses, medical and surgical treatments benefit from incorporating bacteriological information and sinonasal symptoms much more than CT scan evaluation alone. All patients now undergo systematically an annual simultaneous bacteriological sampling of the middle meatus and sputum to follow the relationship between ENT and lung disease and to help to antibiotic therapy strategy.

scholarly journals Etiological Work-Up for Adults with Bronchiectasis: A Predictive Diagnostic Score for Primary Ciliary Dyskinesia and Cystic Fibrosis

2021 ◽  
Vol 10 (16) ◽  
pp. 3478
Author(s):  
Frederic Schlemmer ◽  
Agnes Hamzaoui ◽  
Sonia Zebachi ◽  
Aurelie Le Thuaut ◽  
Gilles Mangiapan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Clinical Score ◽  
Adult Patients ◽  
Operating Characteristics ◽  
Diagnostic Score ◽  
Receiver Operating Characteristics Curve ◽  
Diagnostic Work ◽  
Work Up ◽  
Ciliary Dyskinesia

Background: etiological investigations are not done for all adult patients with bronchiectasis because of the availability and interpretation of tests. The aim of the study was to elaborate a score to identify patients at high risk of having cystic fibrosis or primary ciliary dyskinesia (CF/PCD), which require appropriate management. Methods: diagnostic work-ups were carried out on a French monocenter cohort, and results were subjected to logistic-regression analyses to identify the independent factors associated with CF/PCD diagnosis and, thereby, elaborate a score to validate in a second cohort. Results: among 188 patients, 158 had no obvious diagnosis and were enrolled in the algorithm-construction group. In multivariate analyses, age at symptom onset (8.69 (2.10–35.99); p = 0.003), chronic ENT symptoms or diagnosed sinusitis (10.53 (1.26–87.57); p = 0.03), digestive symptoms or situs inversus (5.10 (1.23–21.14); p = 0.025), and Pseudomonas. aeruginosa and/or Staphylococcus aureus isolated from sputum (11.13 (1.34–92.21); p = 0.02) are associated with CF or PCD. Receiver operating characteristics curve analysis, using a validation group of 167 patients with bronchiectasis, confirmed the score’s performance with AUC 0.92 (95% CI: 0.84–0.98). Conclusions: a clinical score may help identify adult patients with bronchiectasis at higher risk of having CF or PCD.

scholarly journals Computed Tomography Description of the Uncinate Process Angulation in Patients With Cystic Fibrosis and Comparison With Primary Ciliary Dyskinesia, Nasal Polyposis, and Controls

2019 ◽  
Vol 98 (2) ◽  
pp. 89-93 ◽  
Author(s):  
Rémi Hervochon ◽  
Natacha Teissier ◽  
Jean-Robert Blondeau ◽  
Natacha Remus ◽  
Laurence Bassinet ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Nasal Polyposis ◽  
Uncinate Process ◽  
Ct Scans ◽  
Lateral Nasal Wall ◽  
Average Value ◽  
Significant Difference ◽  
Ciliary Dyskinesia

Background: There is a medial bulging of the lateral nasal wall in patients with cystic fibrosis (CF). Aims: Uncinate process (UP) angulation measurements in patients and controls to objectify this bulging. Materials and Methods: Thirty CF, 17 primary ciliary dyskinesia (PCD), 13 chronic rhinosinusitis with polyps (CRSwp), and 30 controls were included. Angles were measured bilaterally on computed tomography (CT) scans: A, B, C on coronal sections, D and E on axial sections. Angle A was between the UP and the orbit inner wall, whereas the others were between UP and midline. Results: There was no significant difference between controls, PCD, and CRSwp. However, CF had 3 statistically different angles with controls, 5 with CRSwp, and 4 with PCD. Angle A average value was 126° (±16°) in patients with CF, 138° (±19°) in controls ( P = .007), 145° (±15°) in PCD ( P = .001), and 138° (±14°) in CRSwp ( P = .001). Angle E average value was 35° (±10°) in patients with CF, 20° (±6°) in controls ( P < .001), 21° (±4°) in PCD ( P < .001), and 22° (±6°) in CRSwp ( P < .001). Conclusion: Uncinate process’s anatomy is only modified in CF: Angle between UP and inner wall of orbit is closed, and angles between UP and midline are opened. Significance: These measures quantify the medial bulging of lateral nasal wall and support nasofibroscopic observations.

A novel mutation of DNAH5 in chronic rhinosinusitis and primary ciliary dyskinesia in a Chinese family

2013 ◽  
Vol 271 (6) ◽  
pp. 1589-1594 ◽  
Author(s):  
Jing Zhang ◽  
Liping Guan ◽  
Weiping Wen ◽  
Yu Lu ◽  
Qianyan Zhu ◽  
...  
Keyword(s):  
Chronic Rhinosinusitis ◽  
Primary Ciliary Dyskinesia ◽  
Novel Mutation ◽  
Chinese Family ◽  
Ciliary Dyskinesia

Primary ciliary dyskinesia diagnosis in adult patients with bronchiectasis in Sao Paulo, Brazil.

2018 ◽  
Author(s):  
Mary Anne Kowal Olm ◽  
Rodrigo Abensur Athanazio ◽  
Samia Rached ◽  
Niki Tomas Loges ◽  
Heymut Omran ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Sao Paulo ◽  
Adult Patients ◽  
São Paulo ◽  
Ciliary Dyskinesia

Bacterial Infection in Chronic Rhinosinusitis: A Controlled Paired Analysis

2005 ◽  
Vol 19 (6) ◽  
pp. 544-548 ◽  
Author(s):  
Neil Bhattacharyya
Keyword(s):  
Chronic Rhinosinusitis ◽  
Pathogenic Bacteria ◽  
Bacterial Species ◽  
Adult Patients ◽  
Sinus Surgery ◽  
Consecutive Series ◽  
Coagulase Negative Staphylococci ◽  
Positive Bacterial Culture ◽  
Significant Difference ◽  
Aerobic And Anaerobic

Background The aim of this study was to determine if pathogenic bacteria are involved in the pathogenesis of chronic rhinosinusitis (CRS). Methods A consecutive series of adult patients with unilateral sinus disease determined by unilateral radiographic involvement or unilateral purulent secretions was microbiologically studied. Aerobic and anaerobic bacterial and fungal cultures were obtained during endoscopic sinus surgery from purulent secretions or tissue culture. Positive culture rates were compared between the diseased sinus and the contralateral nondiseased (control) sinus to determine if pathogenic bacteria were more commonly recovered from the diseased sinuses. Results Forty-nine adult patients completed the study with appropriate microbiological data. Coagulase-negative staphylococci were the most commonly recovered bacteria followed by Staphylococcus aureus from the diseased side of the sinuses with similar findings for the control sinus. Bacterial species were recovered from 87.8% of the diseased side of the sinuses versus 85.7% from the control sinuses (p = 0.50). Reanalysis with coagulase-negative staphylococci considered as non-pathogen showed a 46.9 and 49.0% positive bacterial culture rate in diseased and control groups, respectively (p = 0.50). No significant difference in positive anaerobic culture rates were identified between groups (59.1% diseased versus 55.1% control, respectively, p = 0.61). Antibiotic resistance rates were no different between bacteria cultured from diseased sinuses versus control (p = 0.115). Conclusion Both aerobic and anaerobic bacterial species may be recovered from both diseased and nondiseased sinuses in patients with CRS. These findings cast some doubt on the exact etiologic role of bacteria in CRS, suggesting other factors or other agents also may be responsible in CRS pathogenesis.

A Case of Primary Ciliary Dyskinesia with Refractory Chronic Rhinosinusitis

2017 ◽  
Vol 110 (8) ◽  
pp. 531-538
Author(s):  
Sayuri Yamamoto ◽  
Ichiro Tojima ◽  
Kazuhiko Takeuchi ◽  
Takeshi Shimizu
Keyword(s):  
Chronic Rhinosinusitis ◽  
Primary Ciliary Dyskinesia ◽  
Ciliary Dyskinesia
Sign in / Sign up

Export Citation Format

Share Document