A novel, noninvasive assay shows that distal airway oxygen tension is low in cystic fibrosis, but not in primary ciliary dyskinesia

2018 ◽  
Vol 54 (1) ◽  
pp. 27-32 ◽  
Author(s):  
Lori Mendelsohn ◽  
Christiaan Wijers ◽  
Ritika Gupta ◽  
Nadzeya Marozkina ◽  
Chun Li ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Oxygen Tension ◽  
Primary Ciliary Dyskinesia ◽  
Distal Airway ◽  
Noninvasive Assay ◽  
Ciliary Dyskinesia

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.

Caregiver burden in children with cystic fibrosis and primary ciliary dyskinesia

2019 ◽  
Vol 54 (12) ◽  
pp. 1936-1940 ◽  
Author(s):  
Özge Keniş Coşkun ◽  
Kardelen Gençer Atalay ◽  
Ela Erdem ◽  
Evrim Karadag‐Saygi ◽  
Yasemin Gökdemir ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Caregiver Burden ◽  
Primary Ciliary Dyskinesia ◽  
Ciliary Dyskinesia

Exhaled Molecular Profiles In The Diagnosis Of Cystic Fibrosis And Primary Ciliary Dyskinesia

2012 ◽  
Author(s):  
Tamara Paff ◽  
Marc P. van der Schee ◽  
Johannes M.A. Daniels ◽  
Gerard Pals ◽  
Pieter E. Postmus ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Molecular Profiles ◽  
Ciliary Dyskinesia

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.

scholarly journals Extended Interval Tobramycin Pharmacokinetics in a Pediatric Patient With Primary Ciliary Dyskinesia Presenting With an Acute Respiratory Exacerbation

2018 ◽  
Vol 23 (2) ◽  
pp. 159-163
Author(s):  
Kristi L. Higgins ◽  
Cady Noda ◽  
Jeremy S. Stultz
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Elimination Rate ◽  
Area Under The Curve ◽  
Volume Of Distribution ◽  
Treatment Recommendation ◽  
Pharmacokinetic Parameters ◽  
Maximum Serum ◽  
First Case ◽  
Ciliary Dyskinesia

The pharmacokinetics of tobramycin in patients with ciliary dyskinesia have not been previously reported. A 10-year-old female patient with primary ciliary dyskinesia was admitted to the general pediatrics floor with an acute respiratory exacerbation after several months of worsening lung function that was unresponsive to oral antibiotics. Extrapolating from cystic fibrosis dosing regimens, she was given intravenous tobramycin 320 mg (10.3 mg/kg/day) on admission as a result of concern for a Pseudomonas aeruginosa infection. Two-point pharmacokinetic monitoring revealed a maximum serum concentration (Cmax) of 18.9 mg/L and a 24-hour area under the curve (AUC0–24hr) of 58.8 (mg × hr)/L, as well as a volume of distribution (Vd) of 0.5 L/kg and an elimination rate (Ke) of 0.34 hr−1. After a dosage increase to tobramycin 400 mg (12.8 mg/kg/day), pharmacokinetic parameters on 2 assessments were as follows: Vd 0.37 to 0.39 L/kg, Ke 0.33 to 0.39 hr−1, Cmax 27.8 to 28.7 mg/L, and AUC0–24h 78.4 to 89.4 (mg × hr)/L. This was the first case report of aminoglycoside pharmacokinetics in a patient with ciliary dyskinesia. The administration of larger doses (up to 12.8 mg/kg/day) of extended-interval tobramycin, similar to the treatment recommendation of at least 10 mg/kg/day for cystic fibrosis patients, was necessary in this patient to achieve serum concentrations that were appropriate for treatment.

scholarly journals Current opportunities for the treatment of respiratory diseases in paediatric practice

2018 ◽  
pp. 132-136 ◽  
Author(s):  
V. N. Drozdov ◽  
S. Yu. Serebrova ◽  
O. А. Vorobyeva ◽  
A. K. Starodubtsev ◽  
O. V. Dobrovolsky
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Bacterial Pneumonia ◽  
Respiratory Diseases ◽  
Clinical Effect ◽  
Fixed Dose ◽  
Low Doses ◽  
Dose Combination ◽  
Paediatric Practice ◽  
Ciliary Dyskinesia

Immediacy of the problem of combination therapy of respiratory diseases in paediatric practice is caused by their multifactorial pathogenesis in children and the need to achieve a high clinical effect in the use of drugs at relatively low doses and with minimum risks of serious adverse effects and drug interactions. The fixed-dose combination of salbutamol, bromhexine and guaifenesin produced in the form of tablets and syrup fully meets these requirements, which makes it possible to use it in children and adults with acute, chronic infectious (ARVI, bacterial pneumonia, respiratory tuberculosis, etc.) and non-infectious (bronchial asthma, pulmonary cystic fibrosis, primary ciliary dyskinesia, etc.) diseases.

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