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PubMed2005

[Nontuberculous mycobacteria in cystic fibrosis].

Patients with cystic fibrosis are particularly at risk of infection with non-tuberculous mycobacteria (NTM). Prevalence of these infections increases with age to around 15 %. The main species involved are M. abscessus and M. avium, the latter not found in children under 15. Diagnosis relies on clinical, radiological and above all bacteriological criteria defined by the ATS. Identification of the causal species of NTM is essential and requires genetic techniques, some of which are currently evaluated. Treatment depends on the mycobacterial species. For M. avium, combined therapy with rifampicin, clarithromycin and ethambutol must be extended 12 months after negativation. M. abscessus infection is particularly resistant to therapy. Usual treatment is a one month course of intravenous imipenem or cefoxitin plus amikacin followed by oral clarithromycin plus ethambutol for at least 12 months after negativation. In case of local lesions, surgery is an option.

Anti-Bacterial Agents↗

PubMed1979

Cystic fibrosis and malnutrition.

Cystic fibrosis as a specific disease entity has been known to be associated with malnutrition for almost half a century. The importance of the malnutrition in the disease process remains unknown, as does much information about specific nutritional deficiencies in CF. Supplements for children with CF should include extra energy as fat or carbohydrate, a form of linoleic acid that can be absorbed, hydrolyzed protein, fat-soluble vitamins with vitamins A and E in a water emulsion, vitamin B12, probably B vitamins and vitamin C, and trace minerals. Routine measurements of nutritional status, particularly in children with growth failure, should be made at regular intervals and should include a three-day diet record and a simultaneous 72-hour stool fat determination. If fat malabsorption is not controlled by pancreatic enzymes, the use of antacids or cimetidine should be considered. The true role of nutrition in patients with CF will not be known until the appropriate studies are completed.

Cystic Fibrosis↗

PubMed1992

Short-chain fatty acid absorption in patients with cystic fibrosis.

Patients with cystic fibrosis (CF) often exhibit malabsorption despite the use of supplemental pancreatic enzymes. Unabsorbed carbohydrates and amino acids can serve as substrates for large intestine anaerobic fermentation, thus increasing excretion of short-chain fatty acids (SCFA) in the feces. Nine patients with CF on regular pancreatic enzyme supplementations in the age range of 5-11 years and one older patient were studied. Three-day stool samples were collected, as were 72-h food records. Stools were analyzed for gross energy, total nitrogen, fat content, and SCFA concentration. A significant difference was found between CF and normal controls in total caloric excretion due to fat malabsorption. No significant difference was found between CF and normal controls in protein or SCFA excretion. Fat excretion as percentage of fat intake was significantly increased in CF patients: 35.3 +/- 10.2% versus 8.0 +/- 3.0%, respectively. These data suggest that carbohydrate supplementation could be more widely used to increase caloric intake in CF patients without causing secondary osmotic diarrhea.

Adolescent↗

PubMed2001

Psychological interventions in cystic fibrosis and asthma.

Cystic fibrosis and asthma have many psychosocial implications for patients and their relatives. These can stem from parental adaptational problems following diagnosis, negative illness perceptions, feeling 'different' and other associated developmental intrusions. In asthma, psychological factors arising from a variety of sources are known to 'trigger' attacks. For adolescents, psychological problems are hallmarked by loss (e.g. of identity, independence and peers) which results in re-appraisals of self-worth, depression and anxiety. Early intervention strategies and screening are crucial in ameliorating unsuccessful emotional adaptation and preventing chronic psychological morbidity. Behavioural and cognitive-behavioural interventions are particularly effective for both treatment-related behavioural problems (e.g. procedural distress, and poor feeding behaviour) and some psychological disorders (e.g. anxiety and depression). However, outcome depends on patients' motivation to change and willingness to collaborate. Where this is absent, less directive, but nonetheless effective, psychotherapies are utilised. Family therapy is also considered useful in addressing family dysfunction when thought to be intrinsic to the emotional problems.

Adolescent↗

PubMed1992

Mucosal immunity to Pseudomonas aeruginosa alginate in cystic fibrosis.

Patients with cystic fibrosis commonly acquire chronic pulmonary infection with alginate-producing Pseudomonas aeruginosa. The infection remains localized at the mucosal surfaces of the airways. Using enzyme-linked immunosorbent assays immunoglobulin concentrations and titers of specific antibodies to purified P. aeruginosa alginate and to P. aeruginosa sonicated antigens were measured in tears, saliva, sputum and serum. CF patients had significantly higher concentrations of IgG, IgA and SIgA in serum and saliva than controls. They also had significantly higher levels of specific antibodies to alginate and sonicated antigen in secretions and serum. Local production of IgA, IgG and IgM antibodies to P. aeruginosa was demonstrated. Only a minor proportion of specific IgA antibodies were present as secretory IgA in tears, saliva and sputum. The ratio of alginate-specific SIgA to specific monomeric IgA in sputum was significantly lower than the similar ratio in saliva, whereas the same ratio for specific P. aeruginosa sonicate antigens was found in saliva and sputum.

Adolescent↗

PubMed1988

Physical exercise for patients with cystic fibrosis: a review.

Cystic fibrosis (CF) is an inherited, chronic disease which mainly affects the lungs and the nutrition. Intensive treatment has gradually improved the outcome for the patients. Exercise therapy has been increasingly advocated to be included in the CF regimen. Our own studies indicate that both short-term and long-term training ameliorate the physical conditions for CF patients, that there is no danger for well-trained CF patients--both boys and girls--to take part even in vigorous, prolonged exercises, and that CF patients might have a positive attitude toward physical activities. Of course, all physical activities have to be individually designed. A review of the literature is also given: The specific ventilatory factors in CF, the limitations of exercise, the problem with exercise-induced asthma, the training effect on lung drainage, on lung function, on infections, on biochemical and hormonal variables, on nutrition and the gastrointestinal tract, on the musculoskeletal apparatus, and on the mind. Nearly all these reports are favorable for a carefully monitored, high activity in CF patients. Practical considerations for activities are given.

Biomechanical Phenomena↗

PubMed1997

Altered erythrocyte sodium-lithium counter-transport and Na+/K(+)-ATPase activity in cystic fibrosis.

Patients with cystic fibrosis (CF) exhibit normal concentrations of sodium and chloride in spite of the disturbance of Cl- and Na+ transport in epithelial cells. To characterize compensatory mechanisms in the regulation of sodium homeostasis, erythrocytes of 13 CF patients were analysed for sodium-lithium counter-transport (SLC), Na+/K(+)-ATPase activity and intracellular sodium content. Values were compared to those of healthy controls. Patients with CF had normal serum sodium and chloride concentrations and renal excretions of these ions were within the physiological range. Intracellular sodium concentration was similar in the CF and the control group (6.8 +/- 2.2 vs 5.7 +/- 1.0 mmol/l RBCs). Red blood cells' SLC and Na+/ K(+)-ATPase activity were elevated in CF patients (381 +/- 106 mumol/h/l RBCs vs 281 +/- 64; p < 0.01) and (445 +/- 129 mumol ATP mg prot/h vs 322 +/- 84, p < 0.01). Our study demonstrates that transmembrane cation transport systems are highly activated in CF. The increased sodium transport may be part of a compensatory mechanism of sodium homeostasis in children with CF.

Adolescent↗

PubMed1989

DNA marker haplotype association with pancreatic sufficiency in cystic fibrosis.

Patients with cystic fibrosis (CF) generally suffer from chronic obstructive lung disease, pancreatic insufficiency (PI), and a number of other exocrine malfunctions. Approximately 15% of CF patients are, however, pancreatic sufficient. To investigate whether the two clinical subgroups, PI and pancreatic sufficiency (PS), are caused by different CF mutant alleles, we have performed linkage disequilibrium and haplotype association analysis with three DNA markers that are tightly linked to the CF locus. The study showed that the allelic and haplotype distributions for these RFLPs are significantly different between the two groups. The data suggest that most of the CF-PI patients are probably descendants of a single mutational event at the CF locus and that the CF-PS patients resulted from multiple, different mutations. While final interpretation of these data awaits molecular cloning of the CF gene, the information on haplotype association in CF may be useful in genetic counseling and disease prognosis, in identifying the gene itself, and in defining the mutations.

Adolescent↗

PubMed2006

Possible dysregulation of chaperon and metabolic proteins in cystic fibrosis bronchial tissue.

Cystic fibrosis (CF) is an autosomal recessive disease due to mutations of the CF transmembrane conductance regulator gene. A systematic approach to generate a protein expressional pattern in CF bronchial tissue has not been performed so far. It was the aim of this hypothesis-generating study to construct differential proteomes of bronchial biopsies in controls (n = 8) and CF patients (n = 9). Biopsies (pools of three per patient) were taken; proteins were extracted and run on 2-DE with subsequent in-gel digestion and mass spectrometrical identification and quantification of proteins using specific software. Three hundred sixty-six protein spots were identified and compared between groups. Following an approach for multiple testing correction, the chaperone 75 kDa glucose-regulated protein and ubiquinol-cytochrome c reductase complex core protein I and one form of nidogen, a pseudogene of aconitase 2, were increased in CF (p < 0.005). Aberrant protein levels may reflect molecular changes of CF as well as CF-linked inflammation, infection and cellular stress response.

Aconitate Hydratase↗

PubMed2004

MUC5AC and MUC5B Mucins Are Decreased in Cystic Fibrosis Airway Secretions.

Cystic fibrosis (CF) is characterized by progressive airway obstruction. Although it has been postulated that this is due in part to mucus hypersecretion, there are no published data showing an increase in the gel-forming mucins MUC5AC or MUC5B in CF secretions. We used confocal microscopy to assess the amount of mucin-like glycoprotein and DNA in CF sputum and found more mucin in bronchitis sputum and a much greater amount of DNA in CF sputum. We then used antibodies to MUC5AC and MUC5B with Western gels and dot-blot to quantify mucin in sputum from 12 patients with CF and 11 subjects without lung disease. There was a 70% decrease in MUC5B and a 93% decrease in MUC5AC in CF sputum (P < 0.005 for both). We conclude that the vol/vol concentration of MUC5AC and MUC5B are decreased in the CF airways relative to normal mucus. This may be due to a relative increase in other components of sputum in the CF airway or to a primary defect in mucin secretion in CF.

Adolescent↗

PubMed1981

The relationship between flow transients and bronchial lability in cystic fibrosis.

Patients with cystic fibrosis (CF), in contrast to asthmatic subjects, often show a larger increase than decrease (bronchial lability) in peak flows following exercise. Children with CF also often have large supramaximal expiratory flow transients, produced by rapid expulsion of air from conducting airways that are being dynamically compressed. We studied the relationship between bronchial lability and flow transients to explain the peculiar form of bronchial lability found in certain CF patients. At baseline, six of seven CF patients had flow transients, suggestive of decreased resting tone of the conducting airways. Following exercise, there was a strong positive correlation (r = 0.76, P less than .01) between changes in peak flow and changes in the volume of the flow transients. Four subjects increased both, two did not change either, and in one subject both peak flow and the volume of the flow transient decreased. This study suggests that the unique, previously unexplained form of postexercise bronchial lability observed in some CF patients is probably due mainly to flow transients that contribute to peak flows. These transients are probably related to increased compliance of the conducting airways in this disease.

Adolescent↗

PubMed1985

Pseudomonas aeruginosa in the oral cavity and sputum of patients with cystic fibrosis.

Patients with cystic fibrosis (CF) are often hosts to colonies of both mucoid and nonmucoid strains of Pseudomonas aeruginosa. The major pathogens for chronic and recurrent pulmonary infection in these patients are the mucoid variants of P. aeruginosa. Of the 31 CF patients studied, 14 patients yielded both mucoid and nonmucoid strains of P. aeruginosa from the various oral ecologic sites and saliva. Of the sites tested, the dorsum of the tongue gave the highest yield of P. aeruginosa (27 strains), followed by the buccal mucosa (17 strains), saliva (15 strains), and dental plaques (6 strains). Eleven patients had P. aeruginosa in the oral cavity and sputum simultaneously. Antibiotic susceptibility tests on these multiple isolates suggest that CF patients may be cocolonized or coinfected by two or more strains of P. aeruginosa. Therefore, it may be important to identify multiple isolates of P. aeruginosa, not only from sputum cultures but also from oral cultures, for antibiotic-susceptibility testing. Oral colonization by the mucoid variants of P. aeruginosa may lead to further colonization in the lower respiratory tract and subsequent pulmonary infection in CF patients.

Adolescent↗

PubMed1998

Cystic fibrosis and reproduction.

Cystic fibrosis (CF) is the most common autosomal recessive disease. CF and congenital bilateral absence of the vas deferens (CBAVD) share a genetic and embryological background. Since the 1960s, medical therapy to reduce the progressive obstructive lung disease and nutritional deficiencies has resulted in most CF patients reaching adulthood. With the improved life expectancy of CF patients, new issues in reproductive health and pregnancy management have arisen. Puberty is delayed, with menarche often occurring eighteen months later than the average. Almost all men with CF are azoospermic. In both CF and CBAVD, the vas deferens is absent and the seminal vesicles are often hypoplastic. Many women with CF are subfertile, and if pregnancy is achieved there is an observed increase in maternal morbidity and mortality. The understanding of the molecular basis of CF and CBAVD has evolved, with the identification of hundreds of CF gene mutations and discovery of an associated intron polymorphism of the CF gene. The concept of severe and mild mutations has been introduced to explain the severe and mild phenotype variations such as the pancreatic insufficient and pancreatic sufficient patient. This paper reviews the above issues to assist with the management of infertile couples with CF or CBAVD.

Cystic Fibrosis↗

PubMed1985

Pancreatic polypeptide in cystic fibrosis.

Patients with cystic fibrosis (CF) and their normal siblings and parents were studied for protein meal-stimulated pancreatic polypeptide (PP) secretion. Patients with CF who had exocrine pancreatic insufficiency did not respond to a protein meal, whereas patients with CF and normal pancreatic function presented relatively well-preserved basal and elevated postmeal PP levels. The siblings responded with relatively lower PP levels compared with the control subjects. Plasma insulin levels were also investigated, and showed sluggish initial-phase but relatively well-preserved insulin responses in the patients with CF. Immunocytochemical and morphometric studies were made of pancreata obtained at autopsy from patients with CF. In the patients younger than 7 years of age, well-preserved islet tissue was disclosed by islet-area morphometry with normal and/or below-normal PP cell counts, whereas patients older than 9 years had relatively less insulin-containing islet tissue and scanty PP cells. Absent PP secretory response in patients with CF who had exocrine pancreatic insufficiency may suggest defects in the PP secretory mechanism. Abnormal PP secretion may be used as an indirect index of pancreatic damage in CF.

Adolescent↗

PubMed1990

Volume and emptying of the gallbladder in patients with cystic fibrosis.

As in cystic fibrosis (CF), gallbladder (GB) hypokinesia might play a role in the high incidence of either cholelithiasis or atrophic and hypoplastic GBs. We studied the contractility of the GB by ultrasonography (US) in 57 CF patients and in 25 age- and sex-matched healthy controls. We evaluated the status of the GB, the GB volume after an overnight fasting and 30 min after the ingestion of a standardized milk meal, the GB contractility (the percentage of reduction, (%R) of the GB volume after a meal), the presence of gallstones, and finally, the span of the liver. In addition, liver function test results were evaluated in the CF group. We concluded that the mean values of GB contractility in CF patients do not differ from those of healthy controls, the most hypokinetic GBs (%R less than 40%) being observed, however, only in the CF group. Gallstones occur in CF patients with a greater frequency than seen in the normal population, even in early childhood. The CF patients' GB mean volumes in fasting conditions are significantly greater than in healthy controls (13.5 +/- 2 versus 8.5 +/- 1.5 cc, p less than 0.0001).

Adolescent↗

PubMed2000

Breath isoprene during acute respiratory exacerbation in cystic fibrosis.

Patients with cystic fibrosis (CF) experience a combination of chronic systemic oxidative stress, generation of free radicals in the lungs due to a hyperimmune response and a diminished ability to scavenge free radicals secondary to malabsorption and increased consumption. The authors asked the question, "Does breath isoprene content reflect systemic oxidative stress?" The study involved 12 CF patients and 12 matched healthy controls. The patients were sampled during acute respiratory exacerbation (increased respiratory symptoms, reduction in forced expiratory volume (FEV1) of >10%, and a decision to treat with intravenous antibiotics) and after two weeks of antibiotic treatment. Blood samples were examined for markers of oxidative stress. Breath samples were analysed for isoprene content. Malondialdehyde (MDA), erythrocyte membrane polyunsaturated fatty acids, protein sulphydryls and protein carbonyls all showed evidence of increased oxidative stress which was moderated by antibiotic treatment. Breath isoprene production rate was significantly lower in patients during exacerbation than in controls with a mean difference of-39 (95% confidence interval (CI) -11-57) pmol.min.kg(-1) and increased to normal values following treatment (mean change 63 (95% CI 42-84) pmol.min.kg(-1)). In conclusion, breath isoprene cannot be considered a reliable marker of oxidative stress.

Adult↗

PubMed1989

Clinical and pharmacokinetic aspects of ciprofloxacin in the treatment of acute exacerbations of pseudomonas infection in cystic fibrosis patients.

Twelve cystic fibrosis patients, aged over 18, who had developed an acute respiratory exacerbation and who had Pseudomonas species isolated from their sputum, were entered into a clinical trial involving ciprofloxacin. The dosage regimen was 100 mg iv followed by 500 mg twice daily orally if less than 40 kg in weight and 200 mg iv followed by 750 mg twice daily orally if greater than 40 kg. Ciprofloxacin was well tolerated with no major side effects, except in one patient who withdrew after onset of headaches and generalized aches and pains. Eleven of the 12 patients showed clinical improvement at the end of the treatment period as determined by weight gain, Shwachman Score, Chrispin Norman Score and pulmonary function tests. MICs of Pseudomonas species isolated from the sputum at the start of the trial were in the range 0.25-4 mg/l. During therapy, sensitivity of isolates decreased and did not return to starting levels at the end of a four week follow-up period. Pharmacokinetic parameters were similar to those reported for fasting healthy volunteers by other workers except for bioavailability which was reduced in the non-fasting patients.

Administration, Oral↗

PubMed1995

Results of heart-lung transplantation in children with cystic fibrosis.

Children with cystic fibrosis represent the largest group referred for, and undergoing, heart-lung transplantation at our institute. Between June 1988 and July 1993, 76 patients were accepted for transplantation, of whom 25 were transplanted, while a further 36 died waiting. Those transplanted ranged from 5-18 years of age and included 13 males and 12 females. Organs were used from donors matched by ABO blood group, size and cytomegalovirus (CMV) status. Post-transplant maintenance immunosuppression comprised cyclosporin A, azathioprine and prednisolone. Anti-thymocyte globulin and high dose methylprednisolone were given peri-operatively and for acute rejection episodes. Actuarial survival was 67% at 1 year, 61% at 2 years and 54% at 3 years. Obliterative bronchiolitis (OB) has occurred in 13 patients (52%) and was the major cause of mortality and morbidity. In three patients, OB was associated with the development of tracheal anastomotic stenosis. Other complications included diabetes mellitus (n = 9), pancreatitis (n = 1) and hypertension (n = 8). Despite these problems, those surviving the first year post-transplant showed a mean FEV1 of 71% (compared to 29% pre-transplant) and enjoyed an overall improved quality of life.

Actuarial Analysis↗