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PubMed1982

Intestinal bile acid malabsorption in cystic fibrosis: a primary mucosal cell defect.

Bile acid malabsorption in cystic fibrosis reduces intraluminal bile acid concentration and may impair fat absorption. The cause of this malabsorption is unknown but it is believed due to intraluminal inhibition of uptake by undigested dietary nutrients. The purpose of this study was to determine the bile acid absorptive capability of cystic fibrosis intestine in a physiologic environment. Direct ileal mucosal taurocholic acid uptake was examined in vitro in seven patients with cystic fibrosis, and seven children and adolescents with ileostomies as controls. Jejunal uptake was studied in five normal individuals. A Crosby-Kugler biopsy capsule was used to obtain all tissues. Tissue was incubated in Krebs buffer, 10 mM glucose, and taurocholic acid at 0.1, 1.0 and 10.0 mM with shaking at 37 degrees C. Significant reduction of taurocholic acid uptake was present in every cystic fibrosis patient with mean uptake rates being 24%, 38%, and 29% of control ileum, respectively, at the three concentrations. Values paralleled those for passive jejunal taurocholic acid uptake in controls. These data illustrate a marked reduction in taurocholic acid uptake capability of cystic fibrosis ileal mucosa and may indicate a cellular defect suggestive of a primary lesion in this disease.

Adolescent↗

PubMed1995

IgG subclass responses to Pseudomonas aeruginosa a- and b-type flagellins in patients with cystic fibrosis: a prospective study.

Sera from 20 cystic fibrosis patients, whose lungs were colonised by Pseudomonas aeruginosa, were examined in a 3-5-year prospective study for any relationship between IgG subclass antibody levels to P. aeruginosa a- and b-type flagellins and pulmonary function (FEV1 and radiological score). Patients were divided into two groups according to their pulmonary status: group 1 comprised 11 patients with poor pulmonary status; group 2 comprised nine patients with relatively good pulmonary status. High concentrations of IgG1, IgG2 and IgG3 antibodies to flagellins, particularly to the b-type, were found in most patients. IgG4 reactivity was observed in only a few patients. Comparison of the two groups of patients showed that those with poor pulmonary status (group 1) had a significantly higher concentration (p < 0.05) of IgG3 for two of the three periods studied and of IgG2 for the last period studied. Moreover, IgG3 and IgG1 reactivities to b-type flagellin and IgG3 to a-type flagellin were also increased significantly (p < 0.05) in group 1 patients between the first and the last period studied. These patients also showed a significant (p < 0.05) time-dependent increase in IgG3 and IgG1 antibody concentrations. These data demonstrate that cystic fibrosis patients with poorer pulmonary status have higher IgG3 levels to flagellin than other cystic fibrosis patients. High concentrations of strong opsonic IgG3 and, to a lesser degree, of IgG1 antibodies may increase pulmonary inflammation and induce heightened pulmonary deterioration.

Adolescent↗

PubMed2002

Sonographic findings of the hepatobiliary-pancreatic system in adult patients with cystic fibrosis.

OBJECTIVE: Sonography of the liver, biliary system, and pancreas in adult patients with cystic fibrosis is by far less systematically documented than in pediatric patients with cystic fibrosis. In this prospective study, duplex sonographic findings of the liver, biliary system, and pancreas in adult patients with cystic fibrosis were compared with those of healthy control subjects. METHODS: Seventy-two consecutive patients with cystic fibrosis and 60 healthy control subjects were examined by high-resolution sonography. The incidence of perihepatic lymphadenopathy, the hepatic echo pattern, the detection rate of liver tumors, the flow patterns in the hepatic and portal veins, and pathologic gallbladder and pancreas findings were recorded. Additionally, cholestasis-indicating enzyme levels (gamma-glutamyl transpeptidase and alkaline phosphatase), liver function test results (alanine aminotransferase and aspartate aminotransferase levels), and amylase and lipase levels were recorded as well. RESULTS: Patients with cystic fibrosis, when compared with healthy subjects on sonographic examination, had a higher incidence of microgallbladder (25% versus 0%) and cystic lesions of the pancreas (18% versus 0%). The number of abnormal echo patterns of the liver was increased (46% versus 15%), with a higher incidence of a nontriphasic flow pattern in the right hepatic vein. The differences proved to be statistically significant (P < .05). CONCLUSIONS: Typical sonographic findings in adult patients with cystic fibrosis are a microgallbladder and small cystic lesions of the pancreas. Pathologic findings of the liver can be shown by B-mode and duplex sonography, but the resulting patterns are less characteristic.

Adolescent↗

PubMed2003

Mycobacterium abscessus and children with cystic fibrosis.

We prospectively studied 298 patients with cystic fibrosis (mean age 11.3 years; range 2 months to 32 years; sex ratio, 0.47) for nontuberculous mycobacteria in respiratory samples from January 1, 1996, to December 31, 1999. Mycobacterium abscessus was by far the most prevalent nontuberculous mycobacterium: 15 patients (6 male, 9 female; mean age 11.9 years; range 2.5-22 years) had at least one positive sample for this microorganism (versus 6 patients positive for M. avium complex), including 10 with >3 positive samples (versus 3 patients for M. avium complex). The M. abscessus isolates from 14 patients were typed by pulsed-field gel electrophoresis: each of the 14 patients harbored a unique strain, ruling out a common environmental reservoir or person-to-person transmission. Water samples collected in the cystic fibrosis center were negative for M. abscessus. This major mycobacterial pathogen in children and teenagers with cystic fibrosis does not appear to be acquired nosocomially.

Adolescent↗

PubMed2003

Totally implantable vascular access devices for cystic fibrosis.

BACKGROUND: Totally implantable vascular access devices are widely used in people with cystic fibrosis to provide intermittent venous access for therapeutic infusions. Their use is associated with some complications such as thrombosis, embolism and infection. OBJECTIVES: To assess if totally implantable venous access devices are a safe and effective route for providing venous access for intermittent administration of intravenous antibiotics in people with cystic fibrosis, also to assess strategies to reduce possible complications of totally implantable venous access devices (e.g. anticoagulants to reduce the risk of thrombosis). SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. Date of the most recent search: May 2003. SELECTION CRITERIA: Randomised and quasi-randomised controlled trials which compared the use of totally implantable venous access devices in people with cystic fibrosis to other means of vascular access, trials which compared the different types of these devices against each other and trials which assessed strategies to reduce complications of these devices. DATA COLLECTION AND ANALYSIS: No relevant trials were identified. MAIN RESULTS: No trials were included in this review. REVIEWER'S CONCLUSIONS: Totally implantable vascular access devices are widely used in people with cystic fibrosis to provide intermittent venous access for therapeutic infusions. Reports of their use in people with cystic fibrosis suggest that they are safe and effective. These reports also suggest that certain interventions might reduce the risk of complications; however, it is disappointing that these reports have not been assessed by randomised controlled trials. This systematic review identifies the need for a multicentre randomised controlled trial assessing both efficacy and possible adverse effects of totally implantable venous access devices in cystic fibrosis.

Catheters, Indwelling↗

PubMed2000

[Cystic fibrosis in the Western health region].

78 patients with cystic fibrosis have been diagnosed in western Norway (Health Region 3) since 1960. 31 were born before 1971 (40%). 44 patients were born in the county of Hordaland (62%), including the city of Bergen where the western Cystic Fibrosis Centre is located. 24 (31%) patients were born in Rogaland, and five (7%) in Sogn & Fjordane. 12 patients have died, 9 of them born before 1971 (survival in this cohort of patients is 71%). During two decades from 1971 to 1990, 3 out of 33 patients have died (survival 91%). All patients born after 1990 are alive. The median age at death was 18.5 years. Among patients alive in 1999, 32 (41.5%) are more than 20 years and 19 (24.7%) are more than 30 years old. Median age among all patients is 19.5 years (range 3-51). Chronic lung infection with Pseudomonas aeruginosa has been diagnosed in 20.8% of all patients, which is lower than the average of Norwegian cystic fibrosis patients (35%). Survival among cystic fibrosis patients in Western Norway is good compared to data from other countries. It seems realistic to expect that our patients should achieve a median survival to more than 30 years of age.

Adolescent↗

PubMed1995

Fluorescence-based oligonucleotide ligation assay for analysis of cystic fibrosis transmembrane conductance regulator gene mutations.

Isolation of the gene for cystic fibrosis (CF), the cystic fibrosis transmembrane conductance regulator (CFTR), provided a basis for analyzing its molecular pathology and resulted in the identification of > 400 mutations associated with disease. Except for the delta F508 mutation, no other single mutation accounts for > 5% of CF chromosomes in most populations, and most mutation frequencies are < 1%. A strategy based on multiplex PCR followed by multiplex allele-specific oligonucleotide probe ligation was used to detect 30 mutations, distributed throughout ten exons and seven introns of the CFTR gene, that together account for > 96% of CF mutant chromosomes worldwide. Mutations were detected by competitive oligonucleotide probe ligation to detect normal and/or mutant genotypes in one reaction. Three probes (one common and two allelic probes) were needed for analysis of each mutation. Probes hybridized to target DNA were joined by a thermostable ligase if there were no mismatches at their junctions; temperature cycling resulted in a linear increase in product. Common probes were labeled with fluorochromes, and allelic probes each had different lengths. Ligation products were analyzed electrophoretically on a fluorescent DNA sequencer. The results show that combined PCR and probe ligation amplification rapidly and reliably screen for CF homozygotes and carriers.

Alleles↗

PubMed1997

[Tuberculous pneumopathy in the course of cystic fibrosis].

BACKGROUND: Tuberculosis is rarely seen in patients with cystic fibrosis. CASE REPORT: A 14-year old female adolescent, regularly followed for a well-tolerated form of cystic fibrosis, developed an acute respiratory infection with consolidation of the left inferior lobe, and no response to the usual antibiotic treatment of cystic fibrosis. Mycobacterium tuberculosis was found in aspirate by fibroscopy, on Loewenstein medium. No familial or social infection contact were identified. Antituberculous chemotherapy with three drugs brought about a prompt improvement of sytemic signs, weight gain, resolution of pulmonary foci and sedation of biological findings referable to inflammation. CONCLUSION: This case report reminds us that tuberculosis may occur in cystic fibrosis patients. Loewenstein cultures should routinely be made when faced with an unexplainable worsening of the condition.

Adolescent↗

PubMed2002

Case-mix adjustment for evaluation of mortality in cystic fibrosis.

Comparison of patient mortality rates in cystic fibrosis (CF) obtained from different institutions requires the use of case-mix adjustment methods to account for baseline differences in patient and disease characteristics. There is no current professional consensus on the use of case-mix adjustment methods for use in comparing mortality rates in CF. Characteristics used for this case-mix adjustment should include those that are different across institutions and are associated with patient survival. They should not include characteristics of disease severity that may be a consequence of effectiveness of treatment. The goal of these analyses was to identify a set of these characteristics of patients or disease that would be useful for case-mix adjustment of CF mortality rates. Data from the Cystic Fibrosis Foundation Patient Registry and from the United States Census of the Population (1990) were used in these analyses. Kaplan-Meier techniques, the log-rank test, and Cox proportional hazards regression were used to estimate survivorship, calculate hazard ratios (HR), 95% confidence intervals (CI(95%)), and to conduct tests of statistical significance. The data set included all 30,469 CF patients seen at CF Care Centers from 1982-1998. There were 5,906 deaths during 508,721 person-years of follow-up. In multivariate analyses, female gender (HR 1.30, CI(95%) (1.16, 1,47), P < 0.001), nonwhite race (HR 1.48, CI(95%) (1.07, 2.04), P = 0.018), Hispanic ethnicity (HR 1.85, CI(95%) (1.42, 2.43), P < 0.001), and symptomatic presentation (respiratory, gastrointestinal, respiratory and gastrointestinal, meconium ileus, and other symptomatic presentations; HRs 1.38-1.83; P values, 0.028 to < 0.001) were associated with higher risk of death. The homozygous Delta F508 genotype (HR 1.36, CI(95%) (1.19, 1.55), P < 0.001) and neither mutation being Delta F508 (HR 1.40, CI(95%) (1.15, 1.71), P = 0.001) were also associated with higher risk of death. Patients diagnosed after 36 months of age had almost 50% reduction in risk of death compared to those diagnosed before 6 months of age (HR 0.52 CI(95%) (0.44, 0.61), P < 0.001). When patients living in zip codes with a median household income > $50,000/year (corrected for the 1999 consumer price index) were compared with those living in areas with a median household income < $20,000/year, it was apparent that those in the wealthier areas had a 40% reduced risk of death (HR 0.60, CI(95%) (0.44, 0.82), P = 0.001). All of these characteristics were independently significant predictors of death, and all of these characteristics differed significantly across the CF Care Centers. This case-mix adjustment model uses patient and disease characteristics available at the time of diagnosis of CF, and is not believed to be influenced by subsequent treatment to predict the risk of death. If these case-mix adjustment methods are adopted broadly, they will make it possible to study treatment effects and differences in mortality outcomes, while adjusting for baseline differences in patient and disease characteristics.

Age Factors↗

PubMed1981

Serum bactericidal effect on Pseudomonas aeruginosa isolates from cystic fibrosis patients.

The bactericidal activity against Pseudomonas aeruginosa strains isolated from cystic fibrosis patients was determined in a 10% concentration of normal serum or autologous cystic fibrosis serum. Of the 167 strains tested, 77 (46%) were sensitive (greater than 95% killed) in normal serum. Mucoid strains were more frequently sensitive than nonmucoid strains. Twenty-three sensitive strains tested in ethyleneglycoltetraacetic acid-chelated serum were resistant (less than 10% killed), suggesting only classical pathway activation. Absorption of cystic fibrosis serum with the autologous P. aeruginosa strain resulted in decreased killing by that serum. All sera, including the chelated and absorbed sera, had comparable total hemolytic complement levels. Patients in poor clinical condition (5 out of 12), in contrast to patients in good or moderate condition(1 out of 30), were more likely to have P. aeruginosa strains that were serum resistant in autologous serum but sensitive in normal serum. Sera from these five patients in poor clinical condition were capable of killing heterologous P. aeruginosa strains. These results suggest the presence of a protective or "blocking" activity in serum from some patients in poor clinical conditions. This association of a blocking activity with clinical condition may signal a transition point in the progression of cystic fibrosis lung disease and thus may be another contributory factor in the failure of the cystic fibrosis host to control infection.

Antibodies, Bacterial↗

PubMed1978

Antibodies to proteases and exotoxin A of Pseudomonas aeruginosa in patients with cystic fibrosis: Demonstration by radioimmunoassay.

Sera from 33 patients with cystic fibrosis and two pediatric patients being treated for chronic pulmonary infections not related to cystic fibrosis and six sera or serum pools from uninfected individuals were tested with a microtiter radioimmunoassay for reactivity against exotoxin A and two proteases from Pseudomonas aeruginosa. Exotoxin A was purified from a low-protease strain of P. aeruginosa and shown to have adenosine diphosphate-ribose transferase activity and mouse lethality. Proteases were purified from an isolate of P. aeruginosa from a patient with cystic fibrosis and had proteolytic activity against elastin and collagen in an assay employing dimethylated protein substrates. The antibody responses of the patients detected using 125I-labeled antibody to human immunoglobulin were correlated with clinical evaluations expressed as a composite score based on pulmonary findings, case histories, growth and nutrition, and chest X rays. Values in the radioimmunoassay for patients' sera were compared with those of a control serum pool and expressed as the ratio of counts per minute (cpm) in patient serum to the cpm in the control pool. Inverse correlations were found between these ratios for each of the pseudomonas exoproducts and clinical scores; highest ratios occurred in patients showing the lowest clinical scores. These results confirm that proteases and exotoxin A of P. aeruginosa are produced in cystic fibrosis pulmonary infections due to P. aeruginosa and suggest that they may serve as significant virulence factors in these chronic infectious states.

Adolescent↗

PubMed2005

Long-term azithromycin in cystic fibrosis: another possible mechanism of action?

Azithromycin is used for the treatment of cystic fibrosis lung disease, although its mechanisms of action are not completely understood. Besides its antiinflammatory and antimicrobial activities, one possibility could be the overexpression induction of the multidrug resistance-associated protein (MRP), which could affect chloride transport, thus overcoming the ion transport defect of cystic fibrosis. Seven patients were evaluated before and after 4 weeks of azithromycin treatment (500 mg once daily). Ion transport was studied in vivo by measuring nasal potential difference (NPD). MRP mRNA expression was studied in nasal cells by an internal standard-based semiquantitative RT-PCR assay. NPD was consistent with cystic fibrosis before treatment. After azithromycin treatment, sodium transport was still impaired, whereas a significant increase in chloride conductance was observed (p = 0.03). A significant direct correlation was found between MRP mRNA expression levels and NPD chloride response after azithromycin treatment (p = 0.04, r = 0.78). In conclusion, azithromycin may induce MRP overexpression and restore chloride conductance in the airways of cystic fibrosis patients. These findings suggest a new potential role of azithromycin in the treatment of cystic fibrosis pulmonary disease, i.e. the possibility to upregulate proteins whose function may, at least in part, compensate for the basic defect of cystic fibrosis.

Adolescent↗

PubMed1997

Disruption of monolayer integrity enables activation of a cystic fibrosis "bypass" channel in human airway epithelia.

Cystic fibrosis (CF) is a genetic disease characterized by marked reduction in Cl- conductance across many epithelia. Two kinds of Cl- channels have been associated with CF. One channel, termed the cystic fibrosis transmembrane conductance regulator (CFTR), is directly coded by the CF gene. The other channel is an outwardly rectifying depolarization induced Cl- channel (ORDIC) that is distinguished from other outwardly rectifying chloride channels (ORCCs) because its activity is induced most reliably by patch excision and depolarization. An issue in current CF research is whether ORDIC channels are indirectly activated by CFTR to contribute a significant portion of apical membrane Cl- conductance in airway cells. We now show that ORDIC channels are readily activated in patches excised and depolarized from isolated cells, but are rarer or refractory to activation in patches from the apical membranes of confluent human airway epithelia. These findings have important implications for proposed therapies that would bypass the CFTR conductance by activating ORDIC channels.

Calcium↗

PubMed1960

Cystic fibrosis.

To determine the actual number of deaths from cystic fibrosis reported in 1957 in California, all death certificates mentioning any one of the various terms used to describe the condition were reviewed. As a cause of death, cystic fibrosis is infrequently mentioned on death certificates. In 1957, only 42 of a total of 124,082 death certificates mentioned cystic fibrosis. Half of the deaths occurred in persons less than 12 months of age. There was no significant difference by sex between deaths due to cystic fibrosis and deaths from all causes. All deaths occurred in the white population.

California↗

PubMed2004

Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration.

AIMS: To assess the incidence of intestinal inflammation in children with cystic fibrosis and to investigate whether probiotics decrease it. STUDY DESIGN: In this two-phase, controlled, prospective study, faecal calprotectin was measured by enzyme-linked immunosorbent assay in 30 children with cystic fibrosis, 30 healthy controls and 15 children with active inflammatory bowel disease. Ten children with cystic fibrosis received Lactobacillus GG, and faecal calprotectin was re-measured 4 weeks later. Rectal nitric oxide production was measured with the rectal dialysis bag technique in 20 children with cystic fibrosis, 20 healthy controls and 15 children with inflammatory bowel disease. Five children with cystic fibrosis received Lactobacillus GG and nitric oxide was re-measured 4 weeks later. RESULTS: Mean faecal calprotectin was significantly higher in the two groups of patients than in controls. Abnormal values were detected in 27 of 30 cystic fibrosis and in 15 of 15 inflammatory bowel disease children. Also mean nitric oxide production was increased in both group of patients, and abnormal values were detected in 19 of 20 cystic fibrosis and in 15 of 15 inflammatory bowel disease children. Calprotectin and nitric oxide concentrations were reduced after probiotics administration. CONCLUSIONS: Intestinal inflammation is a major feature of cystic fibrosis and is reduced by probiotics. The latter finding suggests that intestinal microflora play a major role in intestinal inflammation in cystic fibrosis children.

Case-Control Studies↗

PubMed2000

Quorum-sensing signals indicate that cystic fibrosis lungs are infected with bacterial biofilms.

The bacterium Pseudomonas aeruginosa permanently colonizes cystic fibrosis lungs despite aggressive antibiotic treatment. This suggests that P. aeruginosa might exist as biofilms--structured communities of bacteria encased in a self-produced polymeric matrix--in the cystic fibrosis lung. Consistent with this hypothesis, microscopy of cystic fibrosis sputum shows that P. aeruginosa are in biofilm-like structures. P. aeruginosa uses extracellular quorum-sensing signals (extracellular chemical signals that cue cell-density-dependent gene expression) to coordinate biofilm formation. Here we found that cystic fibrosis sputum produces the two principal P. aeruginosa quorum-sensing signals; however, the relative abundance of these signals was opposite to that of the standard P. aeruginosa strain PAO1 in laboratory broth culture. When P. aeruginosa sputum isolates were grown in broth, some showed quorum-sensing signal ratios like those of the laboratory strain. When we grew these isolates and PAO1 in a laboratory biofilm model, the signal ratios were like those in cystic fibrosis sputum. Our data support the hypothesis that P. aeruginosa are in a biofilm in cystic fibrosis sputum. Moreover, quorum-sensing signal profiling of specific P. aeruginosa strains may serve as a biomarker in screens to identify agents that interfere with biofilm development.

4-Butyrolactone↗

PubMed2005

Susceptibility to typhoid fever is associated with a polymorphism in the cystic fibrosis transmembrane conductance regulator (CFTR).

The cystic fibrosis transmembrane conductance regulator (CFTR) is the affected protein in cystic fibrosis (CF). The high rate of CF carriers has led to speculation that there must be, similar to the sickle cell haemoglobin advantage in malaria, a selective advantage for heterozygotes. Such a selective advantage may be conferred through reduced attachment of Salmonella typhi to intestinal mucosa, thus providing resistance to typhoid fever. We tested this hypothesis by genotyping patients and controls in a typhoid endemic area in Indonesia for two highly polymorphic markers in CFTR and the most common CF mutation. We found an association between genotypes in CFTR and susceptibility to typhoid fever (OR=2.6). These analyses suggest that the role CFTR plays in vitro in S. typhi infection is also important for infection in the human population.

Cystic Fibrosis Transmembrane Conductance Regulato↗

PubMed1998

Mutations of the cystic fibrosis gene in patients with chronic pancreatitis.

BACKGROUND: The pancreatic lesions of cystic fibrosis develop in utero and closely resemble those of chronic pancreatitis. Therefore, we hypothesized that mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene may be more common than expected among patients with chronic pancreatitis. METHODS: We studied 134 consecutive patients with chronic pancreatitis (alcohol-related disease in 71, hyperparathyroidism in 2, hypertriglyceridemia in 1, and idiopathic disease in 60). We examined DNA for 22 mutations of the CFTR gene that together account for 95 percent of all mutations in patients with cystic fibrosis in the northwest of England. We also determined the length of the noncoding sequence of thymidines in intron 8, since the shorter the sequence, the lower the proportion of normal CFTR messenger RNA. RESULTS: The 94 male and 40 female patients ranged in age from 16 to 86 years. None had a mutation on both copies of the CFTR gene. Eighteen patients (13.4 percent), including 12 without alcoholism, had a CFTR mutation on one chromosome, as compared with a frequency of 5.3 percent among 600 local unrelated partners of persons with a family history of cystic fibrosis (P<0.001). A total of 10.4 percent of the patients had the 5T allele in intron 8 (14 of 134), which is twice the expected frequency (P=0.008). Four patients were heterozygous for both a CFTR mutation and the 5T allele. Patients with a CFTR mutation were younger than those with no mutations (P=0.03). None had the combination of sinopulmonary disease, high sweat electrolyte concentrations, and low nasal potential-difference values that are diagnostic of cystic fibrosis. CONCLUSIONS: Mutations of the CFTR gene and the 5T genotype are associated with chronic pancreatitis.

Adolescent↗