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PubMed1989

Long term venous access using a totally implantable drug delivery system in patients with cystic fibrosis and bronchiectasis.

A completely implantable, subcutaneous, venous access system (Port-a-Cath) has been used for antibiotic therapy in 26 patients with cystic fibrosis or bronchiectasis over a period of 45 months. During this period there were ten complications in eight patients and in four patients all or part of the system had to be replaced. Nevertheless, the actuarial median functional survival of the implanted system exceeded 30 months. The Port-a-Cath system provides longer periods of venous access and has a lower complication rate than conventional, percutaneous central venous lines.

Adolescent↗
PubMed2002

Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system.

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are associated with different related disorders such as congenital bilateral absence of the vas deferens, chronic idiopathic pancreatitis, or disseminated bronchiectasis (DB). Many different disease-causing mutations are associated with DB, particularly the 5T allele (IVS 8 polyT tract), a variant of the splice acceptor site at the end of intron 8 that affects the efficiency with which the site is used. It therefore affects the accuracy of exon 9 splicing and hence expression of the functional CFTR protein. In this study we quantified transcripts from nasal biopsies of patients with DB compared to normal controls. We developed a real-time quantitative reverse transcription polymerase chain reaction assay (using the TaqMan system) to evaluate the relative amounts of accurately spliced transcript, and transcript lacking exon 9. Patients with the 5T allele had increased amounts of aberrant transcript: in genotypes 5T/7T, 7T/7T and 9T/7T, mean fractions of 38.4%, 3.5% and 0.6%, respectively, of transcripts had been spliced incorrectly. There was also some evidence that nasal biopsies can provide similar information on transcripts to bronchial biopsies. This functional test is of interest for monitoring the amount of CFTR transcript in different clinical situations or to monitor the effect of drugs on CFTR transcription.

Bronchiectasis↗
PubMed2002

Soluble membrane-type 1 matrix metalloproteinase (MT1-MMP) and gelatinase A (MMP-2) in induced sputum and bronchoalveolar lavage fluid of human bronchial asthma and bronchiectasis.

The aim of this study was to investigate the involvement of the MT1-MMP/MMP-2 cascade in induced sputum (IS) and bronchoalveolar lavage fluid (BALF) from bronchial asthma (BA) and bronchiectasis (BE) patients and healthy controls. The molecular forms and cellular origins of MT1-MMP and MMP-2 were determined by Western immunoblotting, immunohistochemistry and in situ hybridization. Elevated levels of soluble activated and autocatalyzed MT1-MMP species as well as activated forms of MMP-2 in IS and BALF samples from BA and BE patients were evidenced. The activation degrees of soluble MT1-MMP and MMP-2 were significantly correlated in BA and BE IS and BALF. Only low levels of both these MMPs were observed in healthy control IS and BALF. The co-expression of MMP-2 with MT1-MMP was evidenced by double immunostaining in bronchial epithelial cells, submucosal glandular cells, smooth muscle cells and monocyte/macrophages. The MT1-MMP/MMP-2 cascade is present and active in human inflammatory lung disease fluid and tissue samples. This cascade seemingly reflects the active destructive phases of these chronic lung diseases.

Adolescent↗
PubMed2025

High expression of Rex-orf-I and HBZ mRNAs and bronchiectasis in lung of HTLV-1A/C infected macaques.

HTLV-1 type-A rarely causes lung disease in humans, whereas HTLV-1 type-C is more frequently associated with respiratory failure and premature death. We investigated the genetic basis of HTLV-1C morbidity by constructing a chimeric HTLV-1A/CoI-L encompassing the highly divergent type C orf-I. We demonstrate that systemic infectivity of HTLV-1A and HTLV-1A/CoI-L is equivalent in macaques, but viral expression in lungs is significantly higher in HTLV-1A/CoI-L infection. In addition, bronchoalveolar-lavage immune cell dynamics differs greatly with neutrophils and monocytes producing TNF-α in HTLV-1A/CoI-L, but producing IL-10 in HTLV-1A infection. Animals infected with HTLV-1A/CoI-L develops bronchiectasis at 10 months from infection, but at the same timepoint those infected with HTLV-1A do not. HTLV-1A/CoI-L expressed a 16 kDa fusion protein (p16C) via a doubly spliced, Rex-orf-IC, mRNA able to shield T-cells from efferocytosis, a monocyte function that mitigates inflammation via clearance of apoptotic cells. The Rex-orf-IC mRNA is expressed as more frequent in the lung of HTLV-1A/CoI-L than HTLV-1A infected animals. Since defective efferocytosis is associated with lung obstructive pathologies, the data raise the hypothesis that p16C may contribute to the lung morbidity observed in HTLV-1C infection.

Animals↗
PubMed1988

Neutrophil chemotaxis in bronchiectasis: a study of peripheral cells and lung secretions.

1. Some bronchiectatic patients persistently expectorate purulent secretions containing many polymorphonuclear leucocytes (PMN) and bacteria, suggesting ineffective microbial clearing from the lung. We have therefore studied several aspects of PMN recruitment to the lung by chemotaxis. 2. Circulating PMN from patients with bronchiectasis show a variable chemotactic response to the synthetic peptide N-formyl-L-methionyl-L-leucyl-L-phenylalanine. This is similar to the response of PMN from healthy controls and suggests that failure to clear microbial organisms is not due to defective chemotaxis as measured here. 3. Purulent sputum solphase demonstrates chemotactic activity against control PMN. This activity is greater (P less than 0.001) than that found with mucoid sputum solphase, which is consistent with the neutrophil content of the secretions in vivo. 4. Successful antibiotic therapy reduces the chemotactic activity of purulent sputum solphase from a median value of 183 (range 28-201) cells/high power field to 33.5 (range 0.7-64) cells/high power field (P less than 0.0025). This indicates that the chemotactic activity is largely associated with bacterial load.

Adult↗
PubMed1975

[Surgical therapy of bronchiectasy and its results (author's transl)].

69 patients suffering from bronchiectasies underwent surgery at the II. Surgical Clinic of the University of Vienna from 1958 to 1972. Analysis of results revealed three groups: 1. 67% of the patients cured completely, 2. 23% significantly improved, 3. 10% no improvement. The evaluation of spirometric measurements of not improved patients showed extensive preoperative obstructive ventilation disturbance.

Bronchiectasis↗
PubMed2002

Antioxidant effect of beta-carotene in cystic fibrosis and bronchiectasis: clinical and laboratory parameters of a pilot study.

UNLABELLED: The carotenoids are potent antioxidants with the ability to quench singlet oxygen and other toxic oxygen species. The aim of this pilot study was to investigate the protective effect of beta-carotene on oxidant system in patients with cystic fibrosis (CF) and in patients with bronchiectasis (BE) caused by a reason other than CF. Eighteen children with CF and 15 children with BE followed in the Pediatric Chest Disease Unit of Hacettepe University, and 15 healthy children participated in the study. Compared with the controls, significantly lower plasma levels of beta-carotene were found in the CF group and significantly lower plasma levels of vitamin E in the CF and BE groups. The standardization of carotenoid levels for total cholesterol did not significantly attenuate these differences. In addition, there were significantly higher levels of malondialdehyde (a marker of lipid peroxidation) and tumour necrosis factor-alpha (TNF-alpha) in children with CF and in children with BE than in normal subjects. After 6 mo of beta-carotene supplementation, the plasma levels of beta-carotene and vitamin E increased and the plasma levels of TNF-alpha and malondialdehyde decreased in both groups. CONCLUSION: Potent antioxidants, beta-carotene and vitamin E are deficient in patients with CF and in patients with BE, and they are more susceptible to oxidative damage. These patients may benefit from beta-carotene supplementation.

Adolescent↗
PubMed1993

Comparison of the efficacy and safety of ceftibuten and cefaclor in the treatment of pneumonia and bronchiectasis.

In a multicentre, international study of 187 adult patients with bacterial pneumonia or bronchiectasis, the safety and efficacy of a regimen of 200 mg ceftibuten administered twice-daily was compared with cefaclor given in a dosage of 500 mg three times a day. Of the 94 evaluable patients, 66 received ceftibuten and 28 received cefaclor. The overall bacteriological response was similar in the two treatment groups with elimination of the original pathogen in 91% and 89% of the patients receiving ceftibuten and cefaclor, respectively. The overall clinical response mirrored the bacteriological results with a successful clinical outcome in 92% of ceftibuten-treated patients compared with 93% in patients receiving cefaclor. Adverse experiences were, in general, few and mild, being reported in 8% and 17% of patients receiving ceftibuten and cefaclor, respectively.

Adolescent↗
PubMed2026

Lpb. plantarum inhalation powders for the reduction of lung inflammation and S. aureus growth control in non-CF bronchiectasis.

INTRODUCTION: The reduced diversity of the lung microbiome in respiratory conditions, including bronchiectasis, promotes bacterial infection and inflammation, contributing to worsening clinical outcomes. Traditional treatments often fail to address both infection and inflammation. Because of their potential dual-action, lactic acid bacteria (LAB) directly administered to the lungs could represent an innovative therapeutic approach for these diseases. RESEARCH DESIGN AND METHODS: Two powders for inhalation containing Lpb. plantarum, lactose, l-leucine with and without raffinose, a prebiotic, were produced by spray drying and in vitro tested. The focus was on investigating their potential in vitro anti-inflammatory and anti-microbial activities against S. aureus. RESULTS: The powders showed a fine particle fraction (<5&#x2009;&#xb5;m) of >40% and allowed the maintenance of anti -inflammatory activity in vitro for both treatment and prevention. Moreover, both powders led to a significant reduction in S. aureus growth. The stability study of the powders in capsules at different storage conditions showed the preservation of the LAB up to 90&#x2009;days in refrigerated conditions (4&#xb0;C/-20&#xb0;C). CONCLUSIONS: This proof-of-concept study shows that inhalable spray-dried live LABs retain biological activity and are suitable for pulmonary delivery, supporting their potential as a microbiota-modulating therapy, which, however, requires further preclinical validation.

Inhalation↗
PubMed2002

Evaluation of allergic bronchopulmonary aspergillosis in patients with and without central bronchiectasis.

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity disorder induced by Aspergillus species colonizing the bronchial tree. Thirty one patients fulfilling criteria of ABPA were evaluated in the present study. Eleven patients were diagnosed as ABPA-S (serological positive) and 20 patients as ABPA-CB (with central bronchiectasis). The two groups of patients were compared on the basis of clinical, serological, and radiographic observations. Serum anti Aspergillus fumigatus IgG was positive in 72% of cases of ABPA-S and 85% of ABPA-CB patients at the time of presentation. Specific IgE against A. fumigatus and total IgE were significantly lower in ABPA-S (specific IgE= 7.42 IU and total IgE= 1127 ng/mL) as compared to ABPA-CB (specific IgE = 44 IU and total IgE = 2874 ng/mL). The spirometric changes in ABPA-S (normal 80%, mild obstruction 10%, and severe obstruction 10%) were milder than in ABPA-CB (normal 40%, mild obstruction 10%, moderate obstruction 20%, and severe obstruction 30%). These patients were monitored closely for seasonal exacerbation with new pulmonary infiltrates which gave lower recordings in ABPA-S patients. No patient in the ABPA-S group progressed to end-stage lung disease. This may be due to early recognition and treatment. The present data suggest that ABPA-S represents the early stage of an apparently less aggressive form of ABPA than ABPA-CB.

Adolescent↗
PubMed2000

Use of meropenem 3 g once daily for outpatient treatment of infective exacerbations of bronchiectasis.

Meropenem administered as a single iv 3 g dose once every 24 h was used to treat nine ambulatory patients with infective exacerbations of bronchiectasis. Serum meropenem concentrations were measured before dosing and at 30 min after each 30 min infusion. Mean pre-dose concentrations were <0.1 mg/L and mean post-dose concentrations 93.9 +/- 29.5 mg/L (95% confidence interval (CI) 86. 2-101.6, n = 59). A pathogen was cultured from sputum in six patients and eradicated (<100 cfu/g sputum) in all but one by day 6 of therapy. Previous work on animals has shown that a bacteriostatic effect is seen with meropenem when t > MIC is greater than 20-30% of the dose interval. In these nine patients, this could be achieved and was associated with successful outcome for pathogens for which MICs are </= 0.5 mg/L. Therefore, once-a-day meropenem therapy may be a useful option for outpatient treatment for isolates for which MICs are </= 0.5 mg/L.

Aged↗
PubMed1997

Decreased survival in patients with co-existent rheumatoid arthritis and bronchiectasis.

The aim was to compare the 5 yr survival in patients with rheumatoid arthritis (RA) alone, bronchiectasis (Br) alone and RA plus Br (RA-Br). A case-control study was carried out in which 32 patients with RA-Br were matched for age (within 5 yr), sex and (where possible) disease duration with 32 patients with RA alone. An additional comparison group of 31 unselected patients with Br was chosen. All patients were followed for 5 yr. Patients with RA-Br were 7.3 times more likely to die than the general population, 5.0 times more likely than the RA group and 2.4 times more likely than the Br group. An increased risk of death within the RA-Br group was associated with a history of smoking, more severe RA and steroid usage. The co-existence of RA and Br is associated with a poor 5 yr survival.

Adult↗
PubMed1988

Primary structure of neutral oligosaccharides derived from respiratory-mucus glycoproteins of a patient suffering from bronchiectasis, determined by combination of 500-MHz 1H-NMR spectroscopy and quantitative sugar analysis. 1. Structure of 16 oligosaccharides having the Gal beta(1----3)GalNAc-ol core (type 1) or the Gal beta(1----3)[GlcNAc beta(1----6)]GalNac-ol core (type 2).

Carbohydrate chains of respiratory-mucus glycopeptides from a patient (blood group O) suffering from bronchiectasis with a Kartagener's syndrome have been released by alkaline borohydride treatment. Application of high-performance liquid chromatography using subsequently two silica columns, one bonded with aminopropyl groups and the other with octadecyl groups, afforded 39 neutral fractions; 35 oligosaccharide-alditol structures have been characterized by employing 500-MHz 1H-NMR spectroscopy in conjunction with sugar analysis. Here, 16 oligosaccharide structures, possessing a core consisting of Gal beta(1----3)GalNAc-ol branching through a GlcNAc residue linked beta(1----6) to the GalNAc residue (core type 2 or core type 1, respectively), are described. Ten oligosaccharide-alditols with these types of cores (2, 3, 10a, 14, 7, 11a, 15a, 16a, 12 and 16c) have been identified previously in human bronchial mucins of patients suffering from cystic fibrosis [Lamblin, G., Boersma, A., Lhermitte, M., Roussel, P., Mutsaers, J.H.G.M., Van Halbeek, H. and Vliegenthart, J.F.G. (1984) Eur. J. Biochem. 143, 227-236]. Of the remaining six compounds, one is a partial structure of oligosaccharides previously described: (Formula: see text). The structures 17a and 20 contain the Y determinant, i.e., Fuc alpha(1----2)Gal beta(1----4)[Fuc alpha(1----3)]GlcNAc beta(1----). High-resolution 1H-NMR spectroscopy is able to distinguish whether the Y determinant is beta(1----3) or beta(1----6) linked in such oligosaccharide-alditols.

Bronchiectasis↗
PubMed1988

Primary structure of neutral oligosaccharides derived from respiratory-mucus glycoproteins of a patient suffering from bronchiectasis, determined by combination of 500-MHz 1H-NMR spectroscopy and quantitative sugar analysis. 2. Structure of 19 oligosaccharides having the GlcNAc beta(1----3)GalNAc-ol core (type 3) or the GlcNAc beta(1----3)[GlcNAc beta(1----6)]GalNAc-ol core (type 4).

A pool of neutral carbohydrate chains was prepared from respiratory mucins of a patient suffering from bronchiectasis. Fractionation by HPLC led to 35 smaller-size oligosaccharide-alditols; the structure of 16 oligosaccharide-alditols with core type 1 or type 2 has been established (Klein, A., Lamblin, G., Lhermitte, M., Roussel, P., Breg, J., Van Halbeek, H. & Vliegenthart, J.F.G., preceding paper in this journal). In this second part, we identified 19 oligosaccharide-alditols possessing core types 3 and 4. Nine of the structures (1, 5, 9, 6, 10b, 13, 19, 15b and 18.1) have been described previously to be present in cystic fibrosis mucins [Lamblin, G., Boersma, A., Lhermitte, M., Roussel, P., Mutsaers, J.G.H. M., Van Halbeek, H. & Vliegenthart, J.F.G. (1984) Eur. J. Biochem. 143, 227-234]. The remaining ten are new structures isolated from bronchial mucins; they are all extensions of the above-mentioned nine oligosaccharides. These compounds are octassaccharide-alditols containing the Y determinant together with the H determinant of either backbone-type 1 or 2, and partial structures thereof: (Formula: see text) and 21b, which is 23c without Fuc alpha(1----3), 18.2, which is 23c without any Fuc in the 6-branch, and 22b, which is 23c without Fuc in the 3-branch.

Bronchiectasis↗
PubMed1993

Isolation and structural characterization of novel sialylated oligosaccharide-alditols from respiratory-mucus glycoproteins of a patient suffering from bronchiectasis.

The carbohydrate chains of the respiratory-mucus glycoproteins of a patient (blood group O) suffering from bronchiectasis due to Kartagener's syndrome, were released by alkaline borohydride treatment of a pronase digest. The structures of 82 neutral and low-molecular-mass sialylated oligosaccharides have been described previously [van Kuik A., de Waard P., Vliegenthart J. F. G., Klein A., Carnoy C., Lamblin G. Roussel P. (1991) Eur. J. Biochem. 198, 169-182]. In the present work, medium-size sialylated oligosaccharides were obtained after ion-exchange chromatography and were subsequently separated into 36 fractions utilizing gel filtration, HPLC on normal-phase alkylamine-bonded silica and reverse-phase HPLC. From these fractions, the following six sialylated hepta- and octa-saccharide-alditols have been characterized by employing 500-MHz 1H-NMR spectroscopy, in conjunction with fast-atom-bombardment mass spectroscopy and methylation analysis. [formula: see text]

Bronchiectasis↗
PubMed1978

Pregnancy in patients with bronchiectasis.

Pulmonary function was monitored at regular intervals throughout pregnancy in three patients with bronchiectasis. They tolerated pregnancy well, and the underlying lung disease did not appear to have any adverse effect on the outcome of the pregnancy.

Adult↗
PubMed1988

Pulmonary resection for localized bronchiectasis in cystic fibrosis. Report of three cases and review of the literature.

Three children with cystic fibrosis and localized bronchiectasis were treated by surgical removal of the affected lobe, after attempts to clear the obstructed bronchus by medical therapy and bronchoscopy with suction and lavage had been unsuccessful. Three to six years after surgery, the results are excellent. Pulmonary disease is mild in all patients, shown by chest radiographs and pulmonary function tests. A review of 102 cases reported in the literature demonstrates that pulmonary surgery is effective and can be performed safely in selected patients.

Bronchiectasis↗