Skeletal Muscle Metabolism in Cystic Fibrosis and Primary Ciliary Dyskinesia
نویسندگان
چکیده
منابع مشابه
Cystic fibrosis, primary ciliary dyskinesia and non-cystic fibrosis bronchiectasis: update 2008-11.
A review is presented of key clinical papers published in Thorax and elsewhere between 2008 and April 2011 which have advanced our understanding of cystic fibrosis (CF), primary ciliary dyskinesia and non-CF bronchiectasis. Studies were identified through searches of the Thorax archive and the Medline database. Within the field of CF, the following key themes were studied: diagnosis in equivoca...
متن کاملChanges in airway inflammation during pulmonary exacerbations in patients with cystic fibrosis and primary ciliary dyskinesia.
Lung disease in patients with both primary ciliary dyskinesia (PCD) or cystic fibrosis (CF) is associated with impaired mucociliary clearance; however, clinical outcomes are typically worse in CF patients. We assessed whether CF and PCD patients differ in inflammatory response in the airways during pulmonary exacerbation.We first studied clinically stable PCD patients with a spectrum of bacteri...
متن کاملEffect of L-arginine infusion on airway NO in cystic fibrosis and primary ciliary dyskinesia syndrome.
Airway nitric oxide concentrations in patients with cystic fibrosis or primary ciliary dyskinesia syndrome have been shown to be lower than in healthy subjects. Decreased NO concentrations may contribute to impaired ciliary clearance, respiratory tract infections, or obstructive lung disease in these conditions. Nasal and exhaled NO concentrations were compared before and after infusion of 500 ...
متن کاملBacterial infections in patients with primary ciliary dyskinesia: Comparison with cystic fibrosis
Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder associated with severely impaired mucociliary clearance caused by defects in ciliary structure and function. Although recurrent bacterial infection of the respiratory tract is one of the major clinical features of this disease, PCD airway microbiology is understudied. Despite the differences in pathophysiology, assumptions abou...
متن کاملLung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study
BACKGROUND Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There are no chest magnetic resonance imaging (MRI) comparative studies of PCD and CF. We assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease. METHODS Twenty PCD (15.1 years) and 20 CF subjects with mi...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 2011
ISSN: 0031-3998,1530-0447
DOI: 10.1203/pdr.0b013e3181fff35f