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PubMed1983

Biliary tree in cystic fibrosis. Biliary tract abnormalities in cystic fibrosis demonstrated by endoscopic retrograde cholangiography.

Two female adolescents with cystic fibrosis were investigated by endoscopic retrograde cholangiography for recurrent abdominal pain. The cholangiogram of 1 patient demonstrated multiple irregular filling defects throughout the biliary tree representing thickened bile and mucus as well as stones. The cholangiogram of the other case illustrated cystic dilatation of the intrahepatic bile ducts with intrahepatic cholelithiasis as well as extensive irregularities of the smaller proximal ductules secondary to recurrent cholangitis or focal biliary cirrhosis, or both. Endoscopic papillotomy resulted in drainage of tenacious bile and mucus and stones in both cases. Sustained clinical improvement did not follow papillotomy in either case, whereas radiologic improvement was demonstrated in 1 case.

Abdomen↗
PubMed2000

Nebulised anti-pseudomonal antibiotics for cystic fibrosis.

BACKGROUND: Lung damage associated with persistent infection by Pseudomonas aeruginosa is the major cause of morbidity and mortality in people with cystic fibrosis. Nebulised antibiotics are commonly used for treatment of this infection. OBJECTIVES: To examine the evidence that nebulised anti-pseudomonal antibiotic treatment in patients with cystic fibrosis reduces frequency of exacerbations of infection, improves lung function, quality of life and survival. To assess adverse effects of nebulised anti-pseudomonal antibiotic treatment. SEARCH STRATEGY: Trials were identified from the Cochrane Cystic Fibrosis and Genetic Disorders Group clinical trials register. Companies which marketed nebulised anti-pseudomonal antibiotics were contacted for information on unpublished trials. Date of the most recent search of the Group's specialised register: November 1999. SELECTION CRITERIA: Trials were selected if, nebulised anti-pseudomonal antibiotics treatment was used in patients with cystic fibrosis, treatment was used for four weeks or more, allocation to treatment was randomised or pseudo-randomised, and there was a placebo or a no placebo control group. DATA COLLECTION AND ANALYSIS: References to nebulised antibiotics were retrieved from the register. Two reviewers independently selected trials from this sample according to the criteria. Each judged the quality of selected trials. One reviewer extracted data from these trials. MAIN RESULTS: Ten trials with 758 participants were included in the review. Sixty eight % of participants were in one trial. Duration of trials ranged from one to 32 months. There was large variation in study design and outcome measures which limited meta-analysis. Lung function measured as forced expired volume in one second (FEV1) was generally better in the treated groups than in control groups but a pooled estimate of effect was not possible. In the largest trial of 520 participants the FEV1 was 11.9% predicted (95% confidence interval 8.1to 15.6) higher in the treated group than in the placebo control group after 5 months. In three trials with 581 participants the number of participants having at least one hospital admission during a trial was reduced in the groups treated with nebulised antibiotics, odds ratio 0.69 (95% confidence Interval 0.50 to 0.96). In two trials with 591 participants the number of participants having and at least one course of antibiotic therapy was reduced in the treated group, odds ratio 0.62 (95% confidence interval 0.45 to 0.87). There was no evidence of renal or auditory toxicity. Resistance to antibiotic increased more in the antibiotic treated group than in placebo group. The most evidence for a single drug is for tobramycin, which was used in total daily doses from 40mg to 1800mg. REVIEWER'S CONCLUSIONS: Nebulised anti-pseudomonal antibiotic treatment improves lung function and reduces frequency of exacerbations of infection in people with cystic fibrosis. There is a need for more evidence for effect on quality of life and survival, for longer duration trials to determine if this benefit is maintained and to determine the significance of development of antibiotic resistant organisms.

Administration, Inhalation↗
PubMed1992

Cystic fibrosis. Part I. Frequency of the delta F508 mutation in South African families with cystic fibrosis.

Cystic fibrosis (CF) is a common autosomal recessive disorder among people of European origin. With the localisation of the gene locus to chromosome 7q31 and the identification of closely linked polymorphic markers in 1985, it became possible to offer prenatal testing to couples at risk of having CF children, provided a live affected individual from that family was available for investigation. The CF gene, named CFTR, was cloned and sequenced in 1989 and the most common CF-causing mutation, delta F508, identified. A search for this mutation has been carried out in 81 South African white CF families of European origin. Using the polymerase chain reaction (PCR) technique the frequency of delta F508 was found to be 0.81. This mutation was not found in the 1 negroid and the 1 Indian CF family investigated.

Amniocentesis↗
PubMed1990

Acute and long-term amiloride inhalation in cystic fibrosis lung disease. A rational approach to cystic fibrosis therapy.

Cystic fibrosis (CF) is the most common inherited fatal disorder among Caucasians. Bronchial mucus in CF contains more potassium and less sodium, which may be due to increased sodium absorption, resulting in a reduced airway water content. We studied 23 patients with CF after inhalation of normal saline or amiloride (10(-3) M), a sodium transport blocker. Mucociliary clearance (MC) and cough clearance (CC) were determined with a gamma camera that traced the movement of 99mTc-labeled, hardened erythrocytes over a 1-h period after the patients inhaled these particles as an aerosol. Before and after each investigation pulmonary function tests (PFT) and blood pressure (BP) were measured. Sputum thread formation was measured by means of a filancemeter. Six of the patients also completed a 3-wk trial of amiloride inhalation therapy. MC increased significantly (p less than 0.001) after acute amiloride inhalation (bronchial deposition, 0.07 mg amiloride) compared with that in the saline control. CC also increased, but not as much as MC. After 3 wk of amiloride inhalation (2 times a day) clearance values (both MC and CC) were markedly enhanced (p less than 0.01); after a similar period of saline inhalation, clearance values were not different from baseline. Sputum filance values also decreased significantly after amiloride inhalation. There were no adverse effects of the amiloride inhalation compared with saline. We conclude that amiloride inhalation administered as a single dose or as long-term therapy is able to increase MC and CC in CF airways and that the effect of 10(-3) M amiloride inhalation on MC lasts at least 40 min. (ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Inhalation↗
PubMed1977

Pseudomonas aeruginosa infection in cystic fibrosis. Bactericidal effect of serum from normal individuals and patients with cystic fibrosis on P. aeruginosa strains from patients with cystic fibrosis or other diseases.

P. aeruginosa strains originating from the respiratory tract of patients with cystic fibrosis (CF) and patients with other diseases (non-CF) were analysed with regard to their sensitivity to the bactericidal activity of human serum. P. aeruginosa strains isolated from CF patients were more sensitive than strains from non-CF patients to the bactericidal activity of normal human serum. The bactericidal activity was heatlabile. As regards the sensitivity to normal human serum, mucoid and non-mucoid variants were not found to differ. Strains originating from chronically infected CF patients with many precipitins against these bacteria did not differ with respect to serum sensitivity from strains originating from intermittently colonized CF patients without P. aeruginosa precipitins. Compared with normal sera, CF sera showed similar or higher bactericidal activity against a panel of P. aeruginosa strains. In this respect, any difference between CF sera with precipitins and CF sera without precipitins against P. aeurginosa was not found. Sera from three CF patients chronically infected with P. aeruginosa, and with many precipitins against these bacteria, showed a selective inability in bactericidal activity against the patients' own P. aeruginosa isolate possibly reflecting the presence of "bactericidal blocking" antibodies.

Adolescent↗
PubMed1995

Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells.

Cystic fibrosis airway epithelia exhibit a spectrum of ion transport properties that differ from normal, including not only defective cAMP-mediated Cl- secretion, but also increased Na+ absorption and increased Ca(2+)-mediated Cl- secretion. In the present study, we examined whether adenovirus-mediated (Ad5) transduction of CFTR can correct all of these CF ion transport abnormalities. Polarized primary cultures of human CF and normal nasal epithelial cells were infected with Ad5-CBCFTR at an moi (10(4)) which transduced virtually all cells or Ad5-CMV lacZ as a control. Consistent with previous reports, Ad5-CBCFTR, but not Ad5-CMV lacZ, corrected defective CF cAMP-mediated Cl- secretion. Basal Na+ transport rates (basal Ieq) in CF airway epithelial sheets (-78.5 +/- 9.8 microA/cm2) were reduced to levels measured in normal epithelial sheets (-30.0 +/- 2.0 microA/cm2) by Ad5-CBCFTR (-36.9 +/- 4.8 microA/cm2), but not Ad5-CMV lacZ (-65.8 +/- 6.1 microA/cm2). Surprisingly, a significant reduction in delta Ieq in response to ionomycin, a measure of Ca(2+)-mediated Cl- secretion, was observed in CFTR-expressing (corrected) CF epithelial sheets (-6.9 +/- 11.8 microA/cm2) when compared to uninfected CF epithelial sheets (-76.2 +/- 15.1 microA/cm2). Dose response effects of Ad5-CBCFTR on basal Na+ transport rates and Ca(2+)-mediated Cl- secretion suggest that the mechanism of regulation of these two ion transport functions by CFTR may be different. In conclusion, efficient transduction of CFTR corrects hyperabsorption of Na+ in primary CF airway epithelial cells and restores Ca(2+)-mediated Cl- secretion to levels observed in normal airway epithelial cells. Moreover, assessment of these ion transport abnormalities may represent important endpoints for testing the efficacy of gene therapy for cystic fibrosis.

Adenoviridae↗
PubMed1997

Height and weight in cystic fibrosis: a cross sectional study. UK Cystic Fibrosis Survey Management Committee.

Cross sectional data reporting the height, weight, and body mass index of UK patients with cystic fibrosis are presented. During the first decade of life height and weight in patients with cystic fibrosis are maintained at about 0.5 SD below those of the general population, which reflects an improvement over earlier published observations. Postpubertal stature and weight maintenance in the cystic fibrosis population still show substantial deficits which may be related to treatment.

Adolescent↗
PubMed1992

Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia.

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), a membrane glycoprotein that forms Cl- channels. Previous work has shown that when some CF-associated mutants of CFTR are expressed in heterologous cells, their glycosylation is incomplete. That observation led to the hypothesis that such mutants are not delivered to the plasma membrane where they can mediate Cl- transport. Testing this hypothesis requires localization of CFTR in nonrecombinant cells and a specific determination of whether CFTR is in the apical membrane of normal and CF epithelia. To test the hypothesis, we used primary cultures of airway epithelia grown on permeable supports because they polarize and express the CF defect in apical Cl- permeability. Moreover, their dysfunction contributes to disease. We developed a semiquantitative assay, using nonpermeabilized epithelia, an antibody directed against an extracellular epitope of CFTR, and large (1 microns) fluorescent beads which bound to secondary antibodies. We observed specific binding to airway epithelia from non-CF subjects, indicating that CFTR is located in the apical membrane. In contrast, there was no specific binding to the apical membrane of CF airway epithelia. These data were supported by qualitative studies using confocal microscopy: the most prominent immunostaining was in the apical region of non-CF cells and in cytoplasmic regions of CF cells. The results indicate that CFTR is either missing from the apical membrane of these CF cells or it is present at a much reduced level. The data support the proposed defective delivery of some CF-associated mutants to the plasma membrane and explain the lack of apical Cl- permeability in most CF airway epithelia.

3T3 Cells↗
PubMed1976

Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. II. Electron microscopy and electrophoresis.

Centrifuged pellets of turbid parotid saliva from cystic fibrosis (CF) patients and non-CF subjects, obtained from saliva kept at 2 degrees for 10 min, had the electron microscope appearance of amorphous, round particles, and were thought to be colloidal aggregates of organic material. Drops of turbid saliva, from samples incubated for 2 hr at 2 degrees or 37 degrees, additionally contained discrete, electron-dense crystals having well defined angular morphology: usually cubic, retangular, or approximately hexagonal. The inhibitors, urea, guanidine hydrochloride, and EDTA, resulted in no crystals being observed. Selected area electron diffraction from individual crystals showed predominantly hexagonal, rectangular patterns could be indexed as coming from hydroxyapitite. A transition from the hexagonal to the rectangular pattern and back to the hexagonal pattern could be obtained from individual crystals tilted in the electron microscope. The square diffraction pattern may be from octa-calcium or brushite. Polyacrylamide gel disc electrophoresis of the parotid saliva indicated that the sparingly soluble proteins in the 2 degree and 37 degree pellets comprised proline-rich proteins and a calcium-precipitable, trichloroacetic acid (TCA)-precipitable phosphoprotein, which fluoresced with amido schwarz and Coomassie brilliant blue G250.

Adolescent↗
PubMed1976

Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. I. Physicochemistry.

Two types of turbidity were found in parotid saliva from both cystic fibrosis (CF) patients and non-CF subjects. On cooling saliva, a rapidly forming, reversible, cold-dependent turbidity appeared in increasing amounts with decreasing temperature and increasing protein concentration. At 37 degrees, a slowly forming, stable turbidity appeared in increased amounts in parotid saliva samples containing increased amounts of calcium. The 2 degree centrifuged pellet consisted predominantly of protein, whereas the 37 degree pellet contained calcium, inorganic phosphate, and protein. The cold-dependent turbidity at 2 degrees was not inhibited by EDTA, but 37 degrees turbidity was dramatically inhibited. Urea and guanidine hydrochloride reduced 2 degree turbidity, and, to a lesser extent, inhibited 37 degree turbidity. The tendency towards higher levels of protein, amylase, and calcium in CF compared with child control parotid saliva (4, 6) causes a greater incidence and degree of turbidity formation in saliva of CF patients. In this paper only the nature of the turbidity has been investigated, not its relative occurrence in each group of subjects.

Adolescent↗
PubMed1985

Survival of cystic fibrosis patients in South Australia. Evidence that cystic fibrosis centre care leads to better survival.

Life tables were calculated for 205 South Australians with cystic fibrosis. An improvement in survival was noted between 1948 and 1982. Ninety-three per cent of patients who were diagnosed as having cystic fibrosis after 1973 were alive at 14 years of age, compared with 40% of those who were diagnosed between 1948 and 1973. A Cystic Fibrosis Clinic was established in 1973 and much of this improvement is attributed to the care provided by this centre. Deaths from meconium ileus fell from 58% of infants with this complication between 1948 and 1973 to only 8% between 1973 and 1983, in spite of the increasing incidence of patients who were chronically colonized with Pseudomonas aeruginosa (currently 68% of patients). These figures are similar to those from Victoria and from other cystic fibrosis centres in North America. The improvement in survival means that adults now comprise a quarter of the patients with cystic fibrosis in South Australia, and that adult institutions need to be aware of these patients and their needs.

Actuarial Analysis↗
PubMed2000

[Adult care centers for patients with cystic fibrosis: prerequisites and practical organization. Cystic Fibrosis Care Centers of Rennes and Lille].

In the past 40 years there has been an increase in the median age of survival of patients with cystic fibrosis (CF), attributed to advances in the diagnosis and treatment of patients with this disease. This progress was also due to the level of care provided by pediatricians and now by adult physicians in CF care centers. They centralize and integrate knowledge and skills of pulmonary medicine, gastroenterology, nutrition, nursing, respiratory therapy, social work, and genetics to provide specialized care to patients with CF from childhood to adulthood. They are also expected to develop teaching and research, particularly the development of new life-extending therapies. The continuity of care of this multisystemic disease is best carried out through a close collaboration between the patient's primary care physician or pulmonologist and the specialized staff of the CF care center.

Adult↗
PubMed1998

Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition.

OBJECTIVES: The objectives of this study were to determine growth status and to identify malnutrition with various anthropometric indicators in children with cystic fibrosis (CF) based on cross-sectional analysis of the 1993 National CF Patient Registry data. METHODS: Heights and weights of 13,116 children with CF were evaluated with percentile, percent of reference median, Z-score, and percent ideal weight-for-height based on National Center for Health Statistics/Centers for Disease Control growth references. Malnutrition was defined by four criteria: (1) height-for-age <5th percentile ("stunting") or weight-for-age <5th percentile ("wasting") (2) height-for-age <90% of reference median or weight-for-age <80% of reference median, (3) height-for-age <5th percentile or percent ideal weight-for-height <85%, and (4) height-for-age <90% of reference median or weight-for-height <85% of reference median. RESULTS: Mean and median height- and weight-for-age were found to be at the 30th and 20th percentiles in children with CF. Malnutrition (height- or weight-for-age <5th percentile) was particularly pronounced in infants (47%) and adolescents (34%) and patients with newly diagnosed CF (44%). A significant sex difference (p < 0.01) in the occurrence of stunting (height-for-age <5th percentile) was observed during adolescence: boys 11 to 14 years of age showed lower occurrence of stunting (19%) compared with girls (29%), whereas the opposite trend was observed at 15 to 18 years (34% in male patients vs 28% in female patients). CONCLUSION: Twenty percent of all children in the 1993 National CF Patient Registry were <5th percentile for height- or weight-for-age. A significant discrepancy was found when different criteria were used to distinguish "stunting" versus "wasting" in malnourished children with CF.

Adolescent↗
PubMed2003

Pseudomonas cross-infection from cystic fibrosis patients to non-cystic fibrosis patients: implications for inpatient care of respiratory patients.

A 14-year-old boy with bronchiectasis secondary to chronic aspiration developed multiresistant Pseudomonas aeruginosa lower respiratory disease following several inpatient periods where accommodation and physiotherapy services were shared with cystic fibrosis (CF) patients known to be infected with the genetically identical strain of P. aeruginosa. Cross-infection with P. aeruginosa between CF patients and non-CF patients has not previously been described, and this finding raises significant issues relevant to the treatment of patients with non-CF suppurative lung disease.

Adolescent↗
PubMed1997

Incidence, population, and survival of cystic fibrosis in the UK, 1968-95. UK Cystic Fibrosis Survey Management Committee.

The UK Cystic Fibrosis Survey holds data on all people resident in the UK who were diagnosed as having cystic fibrosis and born either since 1968 or before 1968 and alive in 1977. Thus, incidence may be reported from 1968 and prevalence from 1977. The previous estimates are updated to the end of 1995 from data held in the database on 23 August 1996. The incidence is now calculated as one in 2415 live births. The 1992 mid-year population was 6500 people with 65% aged under 16 years. Births outnumber deaths by 160 per year, which suggests a population of 7750 by the year 2000, with all the increase being in the adult age range. The survival of successive cohorts continues to be better than earlier cohorts, the linear descent of the curves is still evident. The infant mortality rate for cystic fibrosis is now under 20 per thousand per year and early childhood mortality is under five per thousand per year. The crude mortality rate for 1995 was 21 per thousand per year, but the standardised mortality ratio was about 3300.

Adolescent↗
PubMed2004

Reversal of cystic fibrosis phenotype in a cultured Delta508 cystic fibrosis transmembrane conductance regulator cell line by oligonucleotide insertion.

Cystic fibrosis (CF) is a lethal genetic disorder that is due to mutations in the gene encoding the cAMP-activated anion CF transmembrane conductance regulator (CFTR) channel. A three-nucleotide base deletion (TTT), encoding phenylalanine in position 508 of the translatable CFTR sequence (accompanied by a C to T replacement immediately 5' to the deletion), accounts for approximately 75% of cases of the disease. In the present study, an oligonucleotide complex (CF4-CF6, 2'-0-methyl RNA-unmodified RNA oligonucleotide duplex, respectively) was used to restore CFTR function by insertion of missing bases in Delta508 CFTR mRNA from a cultured (Delta508) cell line. cAMP-activated whole-cell currents and Cl- transport were detected in CF4-CF6-treated, but not control Delta508, cells by patch-clamp and 6-methoxy-N-(3-sulfopropyl)quinolinium fluorescence (SPQ) quenching analyses, respectively. Further, the nucleotide addition in the deleted region of Delta508 CFTR was determined after amplification by RT-PCR. Insertion of UGU and replacement of U by C immediately 5' to the deletion site in Delta508 mRNA appear to have taken place, with phenotypic but not genotypic reversion in tissue culture of treated cells. The mechanism of insertion of nucleotides has yet to be determined.

Animals↗
PubMedDate not supplied

Immunohistochemical investigation with carbohydrate monoclonal antibodies in cystic fibrosis.

BACKGROUND: Cystic fibrosis is one of the most common inherited diseases in Europe. In patients with cystic fibrosis, the serum levels of the tumour marker antigens CA19-9 and CA50, which are normally found in the epithelium of many organs, are increased. No such finding has been reported for CA242, another ubiquitous tumour marker antigen. We set out to investigate immunohistochemically the correlation of CA19-9 and CA50 with cystic fibrosis and to compare the effect of antibodies against these three tumour markers with regard to improved immunohistochemical results. MATERIALS AND METHODS: Paraffin-embedded tissue from different organs of 18 patients with cystic fibrosis was studied. Immunohistochemical reactions were performed with antibodies against CA19-9, CA50 and CA242 followed by visualisation with the ABC method. RESULTS: Immunohistochemical reactions in different tissues were uniform. Anti-CA19-9 reacted less intensive than anti-CA50 and anti-CA242. The antibodies generally bound to cell membranes and compounds of the cytoplasm. CONCLUSIONS: This study demonstrates immunohistochemically the epitopes of monoclonal carbohydrate antigens (CA-19-9, CA-50 and CA 242) in cystic fibrosis for the first time. We also supposed that cystic fibrosis patients show increased serum levels of CA242. However, none of the three antibodies investigated seems suitable for the immunohistochemical diagnosis of cystic fibrosis.

Antibodies, Monoclonal↗