Cystic fibrosis

Elborn, J.S.; McCloskey, M.

doi:10.1097/00001432-199704000-00011

Cited by 9 publications

(14 citation statements)

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“…However, there are also some retrospective studies showing a reduction in the rate of FEV 1 decline with the use of ICSs in CF patients (27,28). In CF, the dominant pathology in the lung is inflammation generated primarily by failure to clear microorganisms and the generation of a toxic proinflammatory local microenvironment (29). There is chronic neutrophilic airway inflammation, typified by increased membrane-bound and free neutrophil elastase activity.…”

Section: Discussionmentioning

confidence: 99%

Effect of inhaled steroids on clinical and inflammatoryparameters in children with cystic fibrosis

Uyan¹,

Ustun²,

Haklar³

et al. 2017

Turk J Med Sci

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Background/aim: The effectiveness of inhaled corticosteroids (ICSs) in cystic fibrosis (CF) is controversial. The aim of this study was to investigate the effect of an ICS on bronchial hyperreactivity (BHR), oxidative status, and clinical and inflammatory parameters in CF patients.Materials and methods: CF patients were randomized to receive either 2 mg/day nebulized budesonide or 0.9% normal saline as placebo for 8 weeks.Results: Twenty-nine CF patients (mean age: 10.5 ± 2.9 years) were enrolled in the study. There was no statistically significant difference between the two groups at the end of 8 weeks in terms of symptoms, pulmonary function, BHR, oxidative burst, hs-CRP, or ESR. Although there was a significant decrease in malondialdehyde levels in both groups, there was no difference between the two groups. Percentage of neutrophils in the sputum of patients decreased in the budesonide group (P = 0.006). Although sputum IL-8 levels significantly increased in both groups, there was no statistically significant difference between the two groups. Conclusion:Although there was a significant decrease in the percentage of neutrophils in sputum with budesonide, 8 weeks of 2 mg/ day nebulized budesonide was not effective in terms of BHR, oxidative status, or clinical and other inflammatory parameters in children with CF.

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Section: Discussionmentioning

confidence: 99%

Effect of inhaled steroids on clinical and inflammatoryparameters in children with cystic fibrosis

Uyan¹,

Ustun²,

Haklar³

et al. 2017

Turk J Med Sci

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“…Eine Störung in diesem stark regulierten Mechanismus zur Alkalisierung der Galle, das heißt ein Defekt des biliären Bicarbonatschirms, kann die Anfälligkeit von Cholangiozyten gegenüber toxischen Gallensäuren erhöhen [11]. CFTR interagiert aber auch direkt mit dem Inflammasom, sodass eine Störung seiner Funktion unmittelbar Auslöser der Inflammation sein könnte [40]. Therapie.…”

Section: Cftrunclassified

Chronische cholestatische Leberkrankheiten

Hohenester

Beuers²

2017

Internist

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“…[14] KBVDA hastalarında, CFTR geninin en yaygın mutasyonu T5 varyantı, bunu takiben F508 del ve R117H mutasyonları olur.…”

Section: Bulgularunclassified

Kistik fibrozis transmembran regülatör (CFTR) genindeki M470V polimorfizmi ve erkek infertilitesi

Gökçe¹,

Gül²

2017

Androl Bul

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GİRİŞErkek infertilitesi; korunmasız cinsel ilişkiye rağmen bir yı-lın sonunda konsepsiyon meydana gelmemesi veya çocuk sahibi olunamaması olarak tanımlanmaktadır.[1] Evli çiftlerin yaklaşık %10-15'i bebek sahibi olabilmek için tıbbi yardım ABSTRACT OBJECTIVE: Cystic fibrosis (CF) is one of the conditions that cause male infertility. CF is caused infertility in men by usually resulting congenital bilateral vas deferens agenesis (CBVDA). However, studies on how mutations and polymorphisms in CF gene affect spermatogenesis are limited. In this study, we aimed to investigate the association of severe oligozoospermia or nonobstructive azoospermia (NOA) with M470V polymorphism in the CF gene in non-CBVDA males. MATERIAL and METHODS:Thirty-three patients with severe oligozoospermia or NAO who were admitted to our andrology clinic because of infertility were enrolled in the study. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene analysis was performed to evaluate the presence of M470V polymorphism. RESULTS:The mean age was 34.3 years. 13 of 33 patients (39.4%) had NOA, and 20 (60.4%) had severe oligozoospermia. M470V polymorphism was present in 19 (58%) patients, 8 of them were in the NOA group (62%) and 11 (55%) were in the oligozoospermia group. The frequency of M470V polymorphism in our patient group was found higher than expected in the population when compared with the rates reported in previous studies (28-47%) with healthy population. CONCLUSION:The results of this study suggest that increased M470V polymorphism in individuals with severe oligozoospermia or NOA may also have effects on spermatogenesis other than CBVDA. However, there is a need for more extensive studies on this topic.

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Cystic fibrosis

Cited by 9 publications

References 0 publications

Effect of inhaled steroids on clinical and inflammatoryparameters in children with cystic fibrosis

Effect of inhaled steroids on clinical and inflammatoryparameters in children with cystic fibrosis

Chronische cholestatische Leberkrankheiten

Kistik fibrozis transmembran regülatör (CFTR) genindeki M470V polimorfizmi ve erkek infertilitesi

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