Management der Primären Ciliären Dyskinesie

Raidt, Johanna; Brillault, Julien; Brinkmann, Folke; Jung, Anna; Koerner-Rettberg, Cordula; Koitschev, Assen; Linz-Keul, H.; Nüßlein, Thomas; Ringshausen, Felix C.; Röhmel, Jobst; Rosewich, Martin; Werner, Claudius; Omran, Heymut

doi:10.1055/a-1235-1520

Cited by 8 publications

(4 citation statements)

References 112 publications

(199 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…All patients at our PCD center received a standardized therapeutic regime according to published recommendations. [16][17][18] All patients are encouraged to perform inhalation with sodium chloride twice a day and perform physiotherapy and sports.…”

Section: Methodsmentioning

confidence: 99%

Risk factors for the deterioration of pulmonary function in primary ciliary dyskinesia

Fein

Maier

Schlegtendal

et al. 2023

Pediatric Pulmonology

View full text Add to dashboard Cite

Background: PCD is a genetic disease leading to a decline in pulmonary function.There is only little knowledge of factors determining the long-term pulmonary outcome. Especially adherence has not been addressed yet although being an independent risk factor for an increased loss of lung capacity in other chronic respiratory diseases.Objective: Assessing the impact of bacterial airway colonization and adherence on long-term lung function in patients with PCD.Methods: Data on colonization and lung function parameters like forced expiratory volume in the first second (FEV 1, Z-score) and lung clearance index (LCI 2,5% ) were collected for 7.01 ± 2.2 years (893 quarters) in 44 PCD patients. Adherence was classified as good, moderate or poor. The impact of both adherence and colonization was assessed for the long-term course of FEV1, the association of colonization with lung function also quarterly.Statistics: Kruskall-Wallis test, T test, ANOVA, linear regression, linear mixed model.Results: Chronic colonization did not show any impact on the for long-term course of FEV 1 , but adherence was a significant factor: patients with good adherence showed better FEV 1 at the end of the observation period than children with poor adherence (-0.15 ± 0.88 vs. -2.63 ± 1.79, p < 0.01). Conclusion: Adherencehas not yet been investigated for PCD. However, we found it to be a major significant factor affecting long-term FEV 1 in PCD. Thus, it should be taken into consideration in the treatment protocols for PCD.

show abstract

Section: Methodsmentioning

confidence: 99%

Risk factors for the deterioration of pulmonary function in primary ciliary dyskinesia

Fein

Maier

Schlegtendal

et al. 2023

Pediatric Pulmonology

View full text Add to dashboard Cite

show abstract

“…Рекомендовано рассмотреть возможность длительного применения ингаляций дорназы альфа с муколитической целью у пациентов с ПЦД при увеличении ФВД на фоне данного препарата [23,[80][81][82].…”

Section: принципы леченияunclassified

“…Назначение ацетилцистеина показано пациентам с ПЦД только при использовании аминогликозидов с целью профилактики снижения слуха. Ацетилцистеин не рекомендован пациентам с ПЦД для применения с муколитической целью в связи с отсутствием доказательств эффективности [80][81][82].…”

Section: принципы леченияunclassified

“…Не рекомендуется применение противовоспалительной терапии преднизолоном или глюкокортикостероидами (для ингаляционного применения) ввиду отсутствия эффективности [81,82].…”

Section: таблица 6 режим дозирования и особенности применения системн...unclassified

See 1 more Smart Citation

Primary ciliary dyskinesia: review of the draft clinical guidelines, 2022

Kondratyeva

Avdeev

Mizernitsky

et al. 2022

Pulʹmonologiâ (Mosk.)

View full text Add to dashboard Cite

Primary ciliary dyskinesia (PCD) is a rare hereditary disease from the group of ciliopathies, which is based on a defect in the cilia ultrastructure of the respiratory epithelium and similar structures (sperm flagella, villi of the fallopian tubes, ventricular ependyma, etc.), leading to motor function impairment. The prevalence of the disease varies significantly around the world and is not known reliably in the Russian Federation.The aim of the review was to analyze literature data on modern approaches to the diagnosis and treatment of PCD.Methodology. The data of 90 articles and the opinions of experts providing care to patients with PCD were used.Results. The classic manifestations of PCD depend on age. The leading manifestations of the disease in patients with PCD are recurrent inflammatory diseases of the upper and lower respiratory tract (bronchitis, pneumonia), with the formation of bronchiectasis, damage to the ENT organs (chronic rhinitis, rhinosinusitis, nasal polyposis, repeated otitis media, progressive hearing loss). Currently, there is no single method which could serve as a “gold” standard for diagnosing PCD. The diagnosis of PCD is based on the characteristic clinical picture in combination with the results of special tests (nitric oxide in exhaled air, DNA diagnostics, high-speed video microscopy, transmission electron microscopy). The genetic diagnostics has not been developed sufficiently in the global practice yet and is unavailable in our country. The approach to the treatment of a patient with PCD should be multidisciplinary due to multiple organ lesions. According to the European consensus, the goal of PCD therapy is to restore or maintain normal lung function. There have been no randomized trials of treatment for PCD, and therefore all treatment recommendations are based on very low-level evidence or extrapolated from cystic fibrosis guidelines. Recommendations on mucolytic, antibacterial and anti-inflammatory therapy of PCD are given with consideration for the international and domestic experience.Conclusion. The development of a new version of clinical guidelines containing up-to-date relevant information will improve the diagnosis and treatment of PCD in the Russian Federation.

show abstract

Primäre ziliäre Dyskinesie

Werner

Omran

2022

Seltene Lungenerkrankungen

View full text Add to dashboard Cite

Management der Primären Ciliären Dyskinesie

Cited by 8 publications

References 112 publications

Risk factors for the deterioration of pulmonary function in primary ciliary dyskinesia

Risk factors for the deterioration of pulmonary function in primary ciliary dyskinesia

Primary ciliary dyskinesia: review of the draft clinical guidelines, 2022

Primäre ziliäre Dyskinesie

Contact Info

Product

Resources

About