scholarly journals A retrospective review of Achromobacter species and antibiotic treatments in patients with primary ciliary dyskinesia

2021 ◽  
Vol 18 ◽  
pp. 147997312110616
Author(s):  
Mathias G Holgersen ◽  
June K Marthin ◽  
Helle K Johansen ◽  
Kim G Nielsen
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Mucociliary Clearance ◽  
Congenital Disease ◽  
Pulmonary Infections ◽  
Opportunistic Pathogens ◽  
Median Length ◽  
Health Records ◽  
Ciliary Dyskinesia

Objectives: Primary ciliary dyskinesia (PCD) is a rare congenital disease with defective mucociliary clearance causing frequent and often persistent pulmonary infections. Achromobacter species are opportunistic pathogens renowned for the difficulty of effective treatments and deteriorating effects on lung function. We aimed to describe the occurrence, treatment, and rate of successful eradication of Achromobacter species in patients with PCD. Methods: We retrospectively reviewed 18 years of historical microbiological samples and 10 years of electronic health records for PCD patients in Denmark. Results: We included 136 patients. Twenty-six patients had isolates of Achromobacter species. On average, 5% of the cohort had at least one annual isolate. Infections became persistent in 38% with a median length of 6.6 years leading to a significant number of antibiotic treatments. Resistance toward tobramycin and ciprofloxacin was prevalent. Overall, successful eradication was achieved in 62% of patients. We found the course of lung function significantly worse during persistent Achromobacter species infection than during the two preceding years, but not different to the course in unaffected age-matched controls. Conclusion The prevalence of Achromobacter species in patients with PCD is in line with what has been reported in cystic fibrosis and can occur transiently, intermittently, or develop into a serious persistent lung infection associated with long-term antibiotic treatment.

scholarly journals Clinical features and management of children with primary ciliary dyskinesia in England

2020 ◽  
Vol 105 (8) ◽  
pp. 724-729 ◽  
Author(s):  
Bruna Rubbo ◽  
Sunayna Best ◽  
Robert Anthony Hirst ◽  
Amelia Shoemark ◽  
Patricia Goggin ◽  
...  
Keyword(s):  
Lung Function ◽  
Hearing Impairment ◽  
Hearing Aids ◽  
Primary Ciliary Dyskinesia ◽  
Service Evaluation ◽  
Forced Expiratory Volume ◽  
Management Service ◽  
National Data ◽  
Randomised Controlled ◽  
Ciliary Dyskinesia

ObjectiveIn England, the National Health Service commissioned a National Management Service for children with primary ciliary dyskinesia (PCD). The aims of this study were to describe the health of children seen in this Service and compare lung function to children with cystic fibrosis (CF).DesignMulti-centre service evaluation of the English National Management PCD Service.SettingFour nationally commissioned PCD centres in England.Patients333 children with PCD reviewed in the Service in 2015; lung function data were also compared with 2970 children with CF.ResultsMedian age at diagnosis for PCD was 2.6 years, significantly lower in children with situs inversus (1.0 vs 6.0 years, p<0.001). Compared with national data from the CF Registry, mean (SD) %predicted forced expiratory volume in one second (FEV1) was 76.8% in PCD (n=240) and 85.0% in CF, and FEV1 was lower in children with PCD up to the age of 15 years. Approximately half of children had some hearing impairment, with 26% requiring hearing aids. Children with a lower body mass index (BMI) had lower FEV1 (p<0.001). One-third of children had positive respiratory cultures at review, 54% of these grew Haemophilus influenzae.ConclusionsWe provide evidence that children with PCD in England have worse lung function than those with CF. Nutritional status should be considered in PCD management, as those with a lower BMI have significantly lower FEV1. Hearing impairment is common but seems to improve with age. Well-designed and powered randomised controlled trials on management of PCD are needed to inform best clinical practice.

scholarly journals Emerging Genotype-Phenotype Relationships in Primary Ciliary Dyskinesia

2021 ◽  
Vol 22 (15) ◽  
pp. 8272
Author(s):  
Steven K Brennan ◽  
Thomas W Ferkol ◽  
Stephanie D Davis
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Pulmonary Infections ◽  
Disease Research ◽  
The Past ◽  
Clinical Presentations ◽  
Organ Laterality ◽  
Ciliary Dyskinesia ◽  
Laterality Defects ◽  
Severe Lung Disease ◽  
Severe Lung

Primary ciliary dyskinesia (PCD) is a rare inherited condition affecting motile cilia and leading to organ laterality defects, recurrent sino-pulmonary infections, bronchiectasis, and severe lung disease. Research over the past twenty years has revealed variability in clinical presentations, ranging from mild to more severe phenotypes. Genotype and phenotype relationships have emerged. The increasing availability of genetic panels for PCD continue to redefine these genotype-phenotype relationships and reveal milder forms of disease that had previously gone unrecognized.

scholarly journals Growth and nutritional status, and their association with lung function: a study from the international Primary Ciliary Dyskinesia Cohort

2017 ◽  
Vol 50 (6) ◽  
pp. 1701659 ◽  
Author(s):  
Myrofora Goutaki ◽  
Florian S. Halbeisen ◽  
Ben D. Spycher ◽  
Elisabeth Maurer ◽  
Fabiën Belle ◽  
...  
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Repeated Measurements ◽  
World Health ◽  
Z Score ◽  
Multilevel Regression ◽  
Growth And Nutrition ◽  
Diagnostic Certainty ◽  
Z Scores ◽  
Ciliary Dyskinesia

Chronic respiratory disease can affect growth and nutrition, which can influence lung function. We investigated height, body mass index (BMI), and lung function in patients with primary ciliary dyskinesia (PCD).In this study, based on the international PCD (iPCD) Cohort, we calculated z-scores for height and BMI using World Health Organization (WHO) and national growth references, and assessed associations with age, sex, country, diagnostic certainty, age at diagnosis, organ laterality and lung function in multilevel regression models that accounted for repeated measurements.We analysed 6402 measurements from 1609 iPCD Cohort patients. Height was reduced compared to WHO (z-score −0.12, 95% CI −0.17 to −0.06) and national references (z-score −0.27, 95% CI −0.33 to −0.21) in male and female patients in all age groups, with variation between countries. Height and BMI were higher in patients diagnosed earlier in life (p=0.026 and p<0.001, respectively) and closely associated with forced expiratory volume in 1 s and forced vital capacity z-scores (p<0.001).Our study indicates that both growth and nutrition are affected adversely in PCD patients from early life and are both strongly associated with lung function. If supported by longitudinal studies, these findings suggest that early diagnosis with multidisciplinary management and nutritional advice could improve growth and delay disease progression and lung function impairment in PCD.

Sinus surgery can improve quality of life, lung infections, and lung function in patients with primary ciliary dyskinesia

2016 ◽  
Vol 7 (3) ◽  
pp. 240-247 ◽  
Author(s):  
Mikkel Christian Alanin ◽  
Kasper Aanaes ◽  
Niels Høiby ◽  
Tania Pressler ◽  
Marianne Skov ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Sinus Surgery ◽  
Improve Quality ◽  
Lung Infections ◽  
Ciliary Dyskinesia

scholarly journals Evaluation of the pulmonary radioaerosol mucociliary clearance scan as an adjunctive test for the diagnosis of primary ciliary dyskinesia in children

2019 ◽  
Vol 54 (12) ◽  
pp. 2021-2027
Author(s):  
Reza Vali ◽  
Hasan Ghandourah ◽  
Martin Charron ◽  
Kimiya V. Nezhad ◽  
Yusuaf Omarkhail ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Mucociliary Clearance ◽  
Ciliary Dyskinesia

scholarly journals Pulmonary radioaerosol mucociliary clearance in primary ciliary dyskinesia

2014 ◽  
Vol 44 (2) ◽  
pp. 533-535 ◽  
Author(s):  
W. T. Walker ◽  
A. Young ◽  
M. Bennett ◽  
M. Guy ◽  
M. Carroll ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Mucociliary Clearance ◽  
Ciliary Dyskinesia

scholarly journals Management of chronic Pseudomonas aeruginosa infection with inhaled levofloxacin in people with cystic fibrosis

2021 ◽  
Author(s):  
J Stuart Elborn ◽  
Patrick A Flume ◽  
Donald R Van Devanter ◽  
Claudio Procaccianti
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Function ◽  
Standard Care ◽  
Bacterial Infections ◽  
Pulmonary Infections ◽  
Pseudomonas Aeruginosa Infection

People with cystic fibrosis (CF) are highly susceptible to bacterial infections of the airways. By adulthood, chronic Pseudomonas aeruginosa ( Pa) is the most prevalent infective organism and is difficult to eradicate owing to its adaptation to the CF lung microenvironment. Long-term suppressive treatment with inhaled antimicrobials is the standard care for reducing exacerbation frequency, improving quality of life and increasing measures of lung function. Levofloxacin (a fluoroquinolone antimicrobial) has been approved as an inhaled solution in Europe and Canada, for the treatment of adults with CF with chronic P. aeruginosa pulmonary infections. Here, we review the clinical principles relating to the use of inhaled antimicrobials and inhaled levofloxacin for the management of P. aeruginosa infections in patients with CF.

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