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PubMed1997

Pulmonary oxidative stress response in young children with cystic fibrosis.

BACKGROUND: It has been suggested that oxidative stress contributes to lung injury in cystic fibrosis. There is, however, no direct evidence of increased pulmonary oxidative stress in cystic fibrosis nor of the effects of inflammation on the major pulmonary antioxidant, glutathione. A study was undertaken to measure these parameters in infants and young children in the presence or absence of pulmonary inflammation. METHODS: Thirty two infants and young children with cystic fibrosis of mean (SD) age 21.4 (15.3) months (range 2-54) and seven non-cystic fibrosis control subjects of mean (SD) age 21.0 (21.2) months (range 2-54) were studied using bronchoalveolar lavage (BAL). On the basis of the BAL findings the cystic fibrosis group was divided into those with (CF-I) and those without pulmonary inflammation (CF-NI). Levels of lipid hydroperoxide, total glutathione, and gamma-glutamyl transpeptidase (gamma-GT) were then measured in the BAL fluid. RESULTS: The concentrations of lipid hydroperoxide and gamma-GT in the epithelial lining fluid were significantly increased in the CF-I group compared with the control and CF-NI groups, each of which had similar values for these parameters (ratio of geometric means for CF-I group versus control for lipid hydroperoxide 5.4 (95% confidence interval (CI) 1.8 to 15.8) and for gamma-GT 5.2 (95% CI 1.4 to 19.4)). The glutathione concentration tended to be lower in the CF-I subjects but the difference did not reach statistical significance. CONCLUSIONS: These results demonstrate that the airways in patients with cystic fibrosis are exposed to increased oxidative stress which appears to be a consequence of pulmonary inflammation rather than part of the primary cystic fibrosis defect. The increase in gamma-GT in the CF-I group suggests a mechanism by which extracellular glutathione could be utilised by airway epithelial cells.

Bronchoalveolar Lavage Fluid↗

PubMed1999

Fatal pulmonary haemorrhage during anaesthesia for bronchial artery embolization in cystic fibrosis.

Three children with cystic fibrosis (CF) had significant pulmonary haemorrhage during anaesthetic induction prior to bronchial artery embolization (BAE). Haemorrhage was associated with rapid clinical deterioration and subsequent early death. We believe that the stresses associated with intermittent positive pressure ventilation (IPPV) were the most likely precipitant to rebleeding and that the inability to clear blood through coughing was also an important factor leading to deterioration. Intermittent positive pressure ventilation should be avoided when possible in children with CF with recent significant pulmonary haemorrhage.

Adolescent↗

PubMed2000

Head and neck manifestations of cystic fibrosis and ciliary dyskinesia.

Cystic fibrosis and the various conditions associated with ciliary dysmotility are well known to the otolaryngologist. Although the underlying pathophysiology and clinical courses of these diseases are quite different, they both involve chronic and acute infection in the paranasal sinuses. Some patients may suffer from persistent ear disease beyond early childhood. A high index of suspicion and early diagnosis are key to managing these conditions. The otolaryngologist often is the first to suspect and confirm the diagnosis and may have an important role in the overall management of these patients.

Ciliary Motility Disorders↗

PubMed2003

[Digestive symptoms in cystic fibrosis].

The prognosis of cystic fibrosis (CF) is strongly linked to the pulmonary status, nevertheless digestive disorders should be taken into account in order to prevent malnutrition which may increase pulmonary surinfections and speed up the deterioration of the respiratory function. Digestion, gut motility and absorption are the three major functions of the gastrointestinal tract. Functional disorders may involve the whole gut; in addition to pancreatic insufficiency we describe the failure of chloride secretion with dehydration of bowel content. Insufficient knowledge of the pathophysiology stresses the need for further studies to allow development of more rational and effective therapy.

Cystic Fibrosis↗

PubMedDate not supplied

Influenza immunization in children with cystic fibrosis.

Nineteen children with cystic fibrosis and aged between 5 and 13 years were randomized to receive two doses at monthly intervals of either a split-virion influenza vaccine (MFV-Ject, Institut Merieux) or a sub-unit vaccine (Fluvirin, Evans). In those completing the study, there was a satisfactory serological response. There was no statistically significant difference between the immunogenicity of the two vaccines as evaluated by haemagglutination inhibition or single radial haemolysis tests. The incidence of local side-effects was similar in the two groups.

Adolescent↗

PubMed2006

Catheter-related thrombosis associated with elevated factor VIII levels in cystic fibrosis.

We report a cystic fibrosis (CF) subject with extensive, central venous catheter-associated thrombosis and sustained elevation of factor VIII to levels normally associated with significantly increased risks of deep venous thrombosis. To determine the potential significance of this finding, the prevalence of elevated factor VIII levels in 22 adults with CF was investigated. Mean (S.D.) factor VIII level was 177 (43) U/dl, with 77% of subjects having levels >150 U/dl. The high prevalence of elevated factor VIII levels questions the significance of this finding in CF subjects with catheter-related thrombosis.

Adolescent↗

PubMed1976

A therapeutic trial of fatty acid supplementation in cystic fibrosis.

Seven children with cystic fibrosis (CF) have been treated for at least one year with intravenously administered soya oil emulsion. In all, an improvement of at least one biochemical abnormality in character with the disease appeared. The children's clinical course remains benign. This course is remarkably better than that of other children with CF treated without Intralipid in Auckland in the same period, though a placebo effect cannot be discounted. It is postulated that intravenous supplementation with essential fatty acid in CF may in turn partially correct an error of metabolism of prostaglandins present in the disease.

Body Weight↗

PubMed1977

Influence of lyophilized total pancreas on plasma lipids and on fat absorption in cystic fibrosis.

31 children with cystic fibrosis (CF) subdivided into three groups of age were studied before and after treatment with lyophilized pancreas and compared to 27 controls. CF was characterized by lower phospholipid, cholesterol and polyunsaturated fatty acid plasma concentrations and reduced coefficient of fat absorption. Treatment tended towards normalization of these values. With age polyunsaturated fatty acids increased in normals but not in CF. The significant correlations existing between fat absorption coefficient and polyunsaturated fatty acid percentage in neutral lipids of CF children suggests the use of the latter percentage as index of the stage of malabsorption.

Adolescent↗

PubMed1999

Specific lipopolysaccharide found in cystic fibrosis airway Pseudomonas aeruginosa.

Cystic fibrosis (CF) patients develop chronic airway infections with Pseudomonas aeruginosa (PA). Pseudomonas aeruginosa synthesized lipopolysaccharide (LPS) with a variety of penta- and hexa-acylated lipid A structures under different environmental conditions. CF patient PA synthesized LPS with specific lipid A structures indicating unique recognition of the CF airway environment. CF-specific lipid A forms containing palmitate and aminoarabinose were associated with resistance to cationic antimicrobial peptides and increased inflammatory responses, indicating that they are likely to be involved in airway disease.

Acylation↗

PubMed1998

Prospects for gene therapy for cystic fibrosis.

Despite advances in conventional treatments for cystic fibrosis (CF), the disease is still associated with significant morbidity and mortality. The cloning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene and the understanding of the functions of the CFTR protein have led to the development of novel treatment strategies, including gene therapy. Here, we review the underlying molecular defect in CF cells, and the progress in gene-transfer studies from in vitro work through to clinical trials. We discuss the problems encountered, the end-points used to assess efficacy, and the likely future directions of the field.

Cystic Fibrosis↗

PubMed1985

Sodium cromoglycate-induced changes in the dose-response curve of inhaled methacholine in cystic fibrosis.

Fifteen patients with cystic fibrosis (CF) (aged 7 to 20 years, mean 12.3 years), all known to respond to a methacholine (MCh) challenge, had two MCh challenges preceded by an inhalation of normal saline on one occasion and sodium cromoglycate (SCG) on another. The variation in baseline FEV1 between the two days of the study was 10% or less in all patients. Four patients (27%) gained significant protection with SCG against this non-allergenic challenge and, in two patients, SCG completely blocked the effects of MCh. These results would support clinical studies of SCG in some patients with CF but not the widespread use of SCG in CF.

Adolescent↗

PubMed1978

Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis.

12 children with cystic fibrosis (C.F.) had lower levels of the essential fatty acid, linoleic acid, in plasma and in red blood cells, than did control children, and production of prostaglandin F2alpha (P.G.F2alpha) was higher than in controls. After 10 months of oral linoleic-acid supplementation in 6 of the children with C.F., the linoleic-acid levels in plasma, red cells, and platelets were higher and P.G.F2alpha production was lower than in the 6 children with C.F. who received a placebo lipid. Prostaglandin F2alpha is associated with bronchoconstriction and its increased production in children with C.F. might be causally related to their chronic pulmonary disease.

Child↗

PubMed1993

Intestinal bile acid malabsorption in cystic fibrosis.

This study aimed at examining the mechanisms participating in excessive faecal bile acid loss in cystic fibrosis. The study was designed to define the relation between faecal fat and faecal bile acid loss in patients with and without cystic fibrosis related liver disease; to assess terminal ileal bile acid absorption by a seven day whole body retention of selenium labelled homotaurocholic acid (SeHCAT); and to determine if small intestinal bacterial overgrowth contributes to faecal bile acid loss. The study population comprised 40 patients (27 men; median age 18 years) with cystic fibrosis (n = 8) and without (n = 32) liver disease and eight control subjects. Faecal bile acid excretion was significantly higher in cystic fibrosis patients without liver disease compared with control subjects (mean (SEM) 21.5 (2.4) and 7.3 (1.2) micromoles/kg/24 hours respectively; p < 0.01) and patients with liver disease (7.9 (1.3) micromoles/kg/24 hours; p < 0.01). No correlation was found between faecal fat (g fat/24 hours) and faecal bile acid (micromoles 24 hours) excretion. Eight (33%) of cystic fibrosis patients had seven day SeHCAT retention < 10% (normal retention > 20%). SeHCAT retention in cystic fibrosis patients with liver disease was comparable with control subjects (30.0 (SEM) 8.3% v 36.8 (5.9)%; p = NS) while SeHCAT retention in cystic fibrosis patients who did not have liver disease was significantly reduced (19.9 (3.8); p < 0.05). Although evidence of small bowel bacterial overgrowth was present in 40% of patients no relation was found between breath hydrogen excretion, faecal fat, and faecal bile acid loss. The results are consistent with the presence of an abnormality in terminal ideal function in patients with cystic fibrosis who do not have liver disease and that a defect in the ileal absorption of bile acids may be a contributory factor to excessive faecal bile acid loss. Faecal bile acid loss in cystic fibrosis is unrelated to the presence of intraluminal fat or intestinal bacterial overgrowth.

Adolescent↗

PubMed2005

Airway gene therapy and cystic fibrosis.

Airway disease in cystic fibrosis (CF) is the major cause of death and is presently inadequately treatable, but genetic therapies offer the hope that such life-long disease will be curable, or at least satisfactorily treated. Normal pathogen defences that have evolved on airway surfaces also prevent the various gene vectors now available from producing effective gene transfer. Nevertheless, findings from basic research and human clinical trials are revealing how these barriers might be overcome or circumvented, with benefits to therapeutic efficacy and patient safety. Though progress is slower than expected or desired, the therapeutic rewards will be great when safe and effective gene therapy for CF airway disease becomes a clinical reality.

Airway Obstruction↗

PubMed1985

Imipenem/cilastatin treatment of multiresistant Pseudomonas aeruginosa lung infection in cystic fibrosis.

Ten patients with cystic fibrosis and chronic broncho-pulmonary Pseudomonas aeruginosa infection received 45 mg imipenem/cilastatin per kg body weight/day, intravenously for two weeks. The treatment was safe with only minor side effects and clinical parameters improved considerably during therapy. In all patients resistance of Ps. aeruginosa to imipenem developed in the second week of treatment; in seven patients the therapy selected for a resistant strain and in three resistance developed in the original strain. The resistance persisted after cessation of treatment and thus the clinical usefulness of imipenem/cilastatin as monotherapy in CF-patients with Ps. aeruginosa seems to be limited.

Adolescent↗

PubMed1982

Complications of gastroesophageal reflux in patients with cystic fibrosis.

Seven patients with cystic fibrosis who had complications of gastroesophageal reflux including abdominal pain, peptic esophagitis, upper gastrointestinal hemorrhage, and esophageal stricture are described. We believe that these are gastrointestinal complications of CF and that they may be responsible for significant morbidity. The mechanical influence of a depressed diaphragm caused by hyperinflation, along with increased abdominal pressure with chronic coughing, may contribute to GER in CF. Early detection and treatment are important not only to prevent esophageal complications but also to increase the quality of life by relief of pain and by avoiding the resultant decrease in appetite, which can contribute to malnutrition.

Adolescent↗

PubMed1992

Microbiology of lung infection in cystic fibrosis.

Bronchopulmonary infection in cystic fibrosis (CF) patients is associated with chronic progressive lung disease and episodes of acute exacerbation. Infection is predominantly caused by bacteria, although infections with viruses, mycoplasma and fungi may play undervalued roles. Bacteria commonly isolated from CF sputum include Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa. Colonisation of the airways by mucoid, alginate-producing variants of P. aeruginosa is recognised as a major cause of pulmonary deterioration. In addition, there is now considerable concern relating to the clinical consequences of colonisation and cross-infection with P. cepacia. This review discusses the microbiology of CF focussing on the pathogenesis and epidemiology of P. aeruginosa and P. cepacia.

Anti-Bacterial Agents↗

PubMed1987

Insulin sensitivity and beta-cell function assessed by C-peptide in young adults with cystic fibrosis.

Eight patients with cystic fibrosis (CF) aged 18-34 who had normal random plasma glucose levels were studied with a continuous infusion of glucose with model assessment (CIGMA) and plasma C-peptide to assess beta-cell function, and plasma insulin to assess insulin sensitivity. Two had impaired glucose tolerance, two had impaired beta-cell function and all had normal insulin sensitivity. The results of previous studies suggesting that glucose intolerance is common in CF may reflect the inability of an impaired liver to handle a large oral glucose load. The few CF patients who become diabetic may be those who have a preexisting diabetic trait.

Adolescent↗