BACKGROUND To assess psychosocial functioning and distress of children and adolescents with cystic fibrosis compared to healthy controls. METHODS Thirty-six patients with cystic fibrosis aged 8-18 years (24 boys, mean age ± SD: 11.5 ± 2.6 years) and 31 sex- and age-matched healthy control subjects (18 boys, mean age ± SD: 12 ± 2.5 years) were enrolled in the study. In order to assess the self...

Chronic bacterial colonization of the lungs, with an excessive inflammatory response, is the major cause of morbidity and mortality in cystic fibrosis. Lung surfactant exhibits a spectrum of potential immunomodulatory properties: phospholipid components inhibit cellular inflammatory responses, whereas the hydrophilic surfactant proteins A (SP-A) and D (SP-D) are integral components of the innat...

Abdominal ultrasound imaging was performed in 50 children. Thirty nine were proved to have cystic fibrosis while 11 had respiratory infections and thus acted as controls. The pancreas was abnormal in 75% of cystic fibrosis patients aged under 5 years and in 95% over 5 years. In cystic fibrosis patients over 5 years old who had malabsorption, the pancreas was abnormal in 100%. Abnormalities of t...

OBJECTIVE To describe clinical profile, etiology and outcome in children with non-cystic fibrosis bronchiectasis. METHODS A chart review of children diagnosed with non-cystic fibrosis bronchiectasis, attending pediatric chest clinic of tertiary care hospital. RESULTS The underlying cause was identified in 51 (63.8%) out of 80 children (mean age, 9.6 y). Common causes were post-infectious in...

Background: Cystic fibrosis (CF) is the most common lethal genetic disorder of Cystic Fibrosis Trans-membrane Conductance (CFTR) Regulator gene mutations. We aimed to investigate common mutations in CF patients and to assess its possible relationship with clinical presentations. Materials and Methods: This cross sectional study was conducted on 36 CF patients who were referred to a tertiary ped...

The secretory response to cholinergic stimulation was investigated in rectal biopsy specimens from children with cystic fibrosis and a control group using a modified Ussing chamber technique. Acetylcholine (10(-3) mol/l) increased the short circuit current in 12 control specimens by mean (SEM) 83.0 (16.4) microA/cm2, but samples from five children with cystic fibrosis failed to exhibit such a r...

Cellobiose and mannitol were used as probe molecules to measure intestinal permeability in 36 children with cystic fibrosis, and 25 age matched controls. Orocaecal transit was also evaluated for each subject using the lactulose/hydrogen breath test. There was a fourfold increase in permeability to disaccharide (cellobiose) in patients with cystic fibrosis, but permeability to the monosaccharide...

s: 1. Urquhart DS, Laverty A, Stanojevic S, Odendaal D, Derrick G, Scrase E, Bryon M, Montgomery H, Narang I , Jaffé A. Association of nocturnal hypoxia and clinical status in childhood cystic fibrosis. Thorax 2009; 64 (Supplement 4): A114. 2. Urquhart DS, Stanojevic S, Laverty A, Montgomery H, Narang I , Jaffé A. Selection of an optimal definition of clinically-significant hypoxia in children ...

In adults, cough sensitivity is influenced by gender and is heightened in those with non-productive cough. This study examined if cough sensitivity is (i) altered in children with asthma, recurrent cough, and cystic fibrosis and (ii) influenced by age, gender, or forced expiratory volume in one second (FEV1). Cough sensitivity to capsaicin and spirometry were performed on 209 children grouped b...

A t the basic level, we know the genetic cause of cystic fibrosis: it is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR).1,2 At the clinical level, we know that chronic bacterial airway infection, prominent neutrophilic inflammation and mucus in airways, and progressive bronchiectasis characterize advanced cy...