Bronchoalveolar lavage (BAL) performed in specialist centres has improved the understanding of infant cystic fibrosis (CF) lung disease. As most researchers sample from a single lobe, it was determined whether BAL results could be generalized to other lung segments. Thirty-three CF children, aged 1.5-57 months, underwent in random order sequential BAL of their right middle and lingula lobes. Sp...

AIM To compare the prevalence of dental caries, dental calculus, and enamel defects in children with cystic fibrosis (CF) and children with other chronic respiratory disorders. METHODS A cross sectional observational survey. One examiner (AN) undertook oral examinations to assess dental caries, periodontal health, and enamel defects in children attending respiratory outpatient clinics. RESU...

OBJECTIVE To evaluate the association between nutritional status, lung function and morbidity in a 36-month cohort in children and adolescents with cystic fibrosis. METHODS Prospective cohort of children and adolescents with cystic fibrosis aged 1-15 years. At the baseline, the nutritional status was determined by weight-for-height and body mass index-for-age for children <2 years and ≥2 year...

Many children with cystic fibrosis (CF), receiving modern, aggressive CF care, have normal spirometry results. This study aimed to see if homogeneity of ventilation distribution is impaired early in the course of CF lung disease, and if ventilation inhomogeneity is a more frequent finding than abnormal spirometry in children benefiting from modern CF care. The study compared spirometry findings...

Identifying noninvasive markers of pulmonary inflammation would be useful in assessing new therapies in children. Breath condensate is a simple and potentially acceptable sample medium even in small children. The technique has previously been used in adults, but not children with cystic fibrosis. The technique was assessed in 36 children with cystic fibrosis (mean age 10.4 yrs) and 17 control s...

BACKGROUND People with cystic fibrosis (CF) are managed differently in the USA and UK providing an opportunity to learn from differences in practice patterns. OBJECTIVES To compare cross-sectional demographics, practice patterns and clinical outcomes between US and UK CF patients. METHODS This was a cross-sectional study using 2010 data from patients in the US Cystic Fibrosis Foundation and...

Proximal small intestinal mucosal biopsies were carried out in children with cystic fibrosis who had diarrhoea and failed to thrive in spite of adequate treatment, including pancreatic supplements. Histological examination of eight of the 17 biopsies taken over a period of 12 years showed evidence of enteropathy, and accounted for one in 13 (8%) children with cystic fibrosis under 3 years of ag...

To identify the incidence and evaluate the causes of high mortality among Bahraini children with cystic fibrosis, we studied, retrospectively, 25 patients diagnosed as proven cases of cystic fibrosis at Sulmaniya Medical Center, the main referral center on the island, from January 1978 to December 1995. With extrapolation of the study data to the general population, the incidence of cystic fibr...

A four year regional screening programme to detect cystic fibrosis using measurement of immunoreactive trypsinogen is described. During this period 60 infants were diagnosed; 34 by screening, 12 born with meconium ileus, and 14 not identified by the screening assay but who presented with clinical symptoms at a later age, giving an incidence of cyst fibrosis in the region during this time of 1/1...

BACKGROUND Published articles have described a lack of willingness to allow preventative measures, as well as other types of modern therapies, as an obstacle to providing medical care for Amish and Mennonite populations. METHODS We present data regarding the 12 Amish and Mennonite patients at the SUNY Upstate Medical University Pediatric Cystic Fibrosis Center and three representative case re...