Upper respiratory tract in cystic fibrosis: ear-nose-throat survey of 50 children. An ear-nose-throat survey was carried out on 50 children aged 4 to 10 years with cystic fibrosis. 10% were shown to be transiently deaf, associated with eustachian tube dysfunction. There were no confirmed cases of secretory otitis media. 10 children (20%) were found to have nasal polyps or gave a history of poly...

The clinical and diagnostic aspects of cystic fibrosis have been extensively reviewed, with an emphasis on neonatal screening. This systematic literature review involved a search for relevant contributions in the PubMed and SciELO databases. The first references to cystic fibrosis date to the Middle Ages. Cystic fibrosis is the most frequent autosomal recessive hereditary disease among Caucasia...

BACKGROUND Allergic bronchopulmonary aspergillosis is a serious complication of cystic fibrosis and may be difficult to diagnose. The aim of this study was to define the usefulness of measuring total IgE compared with other major criteria in the diagnosis of allergic bronchopulmonary aspergillosis in children with cystic fibrosis. METHODS A retrospective analysis was carried out of the case r...

1 Morrell D, Cromartie E, Swift M. Mortality and cancer incidence in 263 patients with ataxia-telangiectasia. J Natl Cancer Inst 1986; 77: 89–92. 2 McGrath-Morrow SA, Gower WA, Rothblum-Oviatt C, et al. Evaluation and management of pulmonary disease in ataxia-telangiectasia. Pediatr Pulmonol 2010; 45: 847–859. 3 Bott L, Lebreton J, Thumerelle C, et al. Lung disease in ataxia-telangiectasia. Act...

objective(s):  more than 1500 registered mutations in cystic fibrosis transmembrane regulator (cftr) gene are responsible for dysfunction of an ion channel protein and a wide spectrum of clinical manifestations in patients with cystic fibrosis (cf). this study was performed to investigate the frequency of a number of well-known cftr mutations in north eastern iranian cf patients. material and m...

cystic fibrosis (cf) is the most common severe autosomal recessive disorder caused by a wide spectrum of mutations in the gene encoding for the cystic fibrosis transmembrane conductance regulator (cftr) protein. the frequencies, types and distributions of mutations vary widely between different populations and ethnic groups. the aim of this study was to perform a comprehensive analysis of the c...

In children with cystic fibrosis, objective parameters of exercise tolerance are needed which are easy to measure in nonspecialized centres. We investigated maximal workload (W'max) in children with cystic fibrosis in relation to body weight and fat-free mass, and compared this with results for maximal oxygen consumption (V'O2,max). Fourteen patients with cystic fibrosis performed an incrementa...

Vitamin E is an antioxidant and may have a role in the protection of lung tissue against oxidative damage in cystic fibrosis. Previous studies of vitamin E status in cystic fibrosis have used plasma or serum concentrations, which vary with levels of carrier lipoproteins and hence may not reflect the concentration of vitamin E in tissues, where it is found in highest concentration in membranes. ...

OBJECTIVE To describe the unusual presentation among patients with confirmed cystic fibrosis. METHODS A retrospective review was carried out on all children (n=90) with the diagnosis of classical cystic fibrosis who attended the Respiratory Pediatric Clinic at King Hussein Medical Center, Amman, Jordan from January 2002--December 2008. All children from one day old to 14 years of age were inc...