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PubMed2001

Bronchiectasis: still a problem.

The prevalence of bronchiectasis (BR) has decreased significantly in industrialized countries, but is still commonplace in developing countries. We evaluated the causes and clinical features of BR in 23 children (13 boys (57%) and 10 girls (43%), with a mean age of 8.45 +/- 4.02 years). Infection was the major cause of BR in our region. In 8 patients, BR developed after tuberculosis or pneumonia, was associated with immune deficiency syndromes in 4 children, and with asthma in 4. Cystic fibrosis was diagnosed in 4 cases and ciliary dyskinesia in 3. In 10 patients, only one lobe was involved. Bronchiectatic lesions were most commonly found in the left lower lobe and were observed in 7 patients. Multilobar involvement was found in 13 patients. The initial treatment was primarily medical, but in 2 patients whose medical therapy failed, pulmonary resection was carried out. Three patients died from severe pulmonary infection and respiratory failure.

Adolescent↗

PubMed1980

Bronchiectasis in childhood. Comparison of chest roentgenograms, bronchography and lung scintigraphy.

In 76 children, 102 unilateral bronchograms were compared with the results of chest radiography and Tc-99m perfusion scintigraphy to assess the presence of bronchiectasis. Additional Kr-81m ventilation lung scintigraphy was obtained in 16 patients. For the chest radiograph the sensitivity was 73% and the specificity was 76%. For lung scintigraphy the sensitivity was 92% and the specificity 60%. When both chest radiography and lung scintigraphy were considered, sensitivity reached 96% and specificity fell to 53%. Forty per cent of the bronchrograms which were performed might have been avoided on the basis of the normal chest radiograph and lung scintigraphy. The combination of lung scintigraphy and chest radiography is an excellent screening method when a decision on the advisability of bronchography is being taken in children.

Adolescent↗

PubMed1993

Bronchiectasis in children: assessment by CT.

The prevalence of bronchiectasis (BR), in general, is decreasing, yet the disease can produce significant morbidity in children. In the pediatric age group the classical investigation by bronchography implies general anaesthesia, thus carrying an additional risk of complications. CT has proved highly accurate in the diagnosis of BR in adults. It is also considered a reliable modality for the diagnosis of BR in children. This conclusion was reached by analysing the radiographic and the CT findings in 40 children with the clinical suspicion of BR in 25 of whom the CT examination was positive. Nine patients of this last group had bronchography as well. There was complete correlation in the diagnosis and location between the CT and the bronchographic findings. Thus, it seems that the occurrence of this disease is still high in the pediatric population in the appropriate clinical and radiological setting. The imaging evaluation of BR should include chest radiographs, computerized tomography and, if surgery is planned, bronchography as well.

Adolescent↗

PubMed2001

CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.

In order to investigate the incidence of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations and unclassified variants in chronic pulmonary disease in children and adults, we studied 20 patients with asthma, 19 with disseminated bronchiectasis (DB) of unknown aetiology, and 12 patients with chronic obstructive pulmonary disease (COPD), and compared the results to 52 subjects from the general Greek population. Analysis of the whole coding region of the CFTR gene and its flanking intronic regions revealed that the proportion of CFTR mutations was 45% in asthma (P<0.05), 26.3% in DB (P>0.05), 16.7% in COPD (P>0.05), compared to 15.4% in the general population. Seventeen different molecular defects involved in disease predisposition were identified in 16 patients. Three potentially disease-causing mutations, T388 M, M1R and V11I, are novel, found so far only in three asthma patients. The hyperactive M470 allele was found more frequently in COPD patients (frequency 70.8%, P<0.01) than in the controls. The study of the TGmTnM470 V polyvariant CFTR allele revealed the presence of CFTR function-modulating haplotypes TG13/T5/M470, TG11/T5/M470, TG12/T5/V470 and TG12/T7, combined with M470 or V470, in six asthma patients, four DB patients (P<0.01), and two COPD patients (P<0.05). These results confirm the involvement of the CFTR gene in asthma, DB and possibly in COPD.

Adolescent↗

PubMed1999

Defective antibody production in patients with rheumatoid arthritis and bronchiectasis.

Bronchiectasis (BR) occurs in about 3% of patients with rheumatoid arthritis (RA). Defective antibody production is a rare but well-recognised cause of both BR and inflammatory arthritis. We examined the hypothesis that subtle specific antibody defects might play a role in the pathogenesis of BR associated with RA. Identification of defects in antibody production is important because substantial benefits may be gained from immunoglobulin replacement. Specific antibody production was assessed in 20 patients with RA and BR, 20 with BR alone, 20 with RA alone and 20 healthy controls (all groups matched for age and sex). All had normal total IgG. IgA and IgM and IgG subclass levels. Specific antibody production was assessed by assay of antibodies to representative polysaccharide and protein antigens. Subjects with subprotective titres were challenged with the appropriate vaccine. Defective antibody production was defined as a subprotective level despite immunisation. Three out of 20 patients with RA and BR had a defective IgG2 response to the polysaccharide antigen, but normal responses to the protein antigen. All of the subjects in the BR alone or healthy control group had normal antibody production. Two out of 20 patients with RA alone had defective production of antibodies against both protein and polysaccharide antigens; both were receiving gold therapy, a recognised cause of functional antibody defects. It was concluded that some patients with RA and BR have functional antibody defects and may benefit from antibody replacement. An unexpectedly high proportion of patients with RA alone also have functional antibody defects, possibly secondary to gold therapy.

Adult↗

PubMed1987

The detection of 5-lipoxygenase and cyclo-oxygenase products in sputum of patients with chronic bronchitis and bronchiectasis.

Leukotrienes (LTs) and prostanoids (Ps) were detected in sputum of patients with chronic bronchitis and/or bronchiectasis (CB/B) using selective superfusion bioassay and radioimmunoassay (RIA) techniques. Analysis of sputum extracts showed a 4-fold increase in the level of LTB4 compared to the cysteinyl-containing LTs (LTC4/LTD4). The measurement of cyclo-oxygenase products (COPs) indicated relatively greater amounts of the vasodilator prostaglandin E2 (PGE2) and prostacyclin (PGI2) compared to the vasoconstrictor prostaglandin F2 alpha (PGF2 alpha) and thromboxane A2 (TxA2) agents (70:30% of total COPs respectively). The presence of eicosanoids (LTs and Ps) in sputum of patients with CB/B suggest that these biologically active substances may act as mediators of bronchoconstriction and inflammation in these diseases.

Adult↗

PubMed1989

Multiple brain abscesses secondary to bronchiectasis. A case of 34 discrete abscesses in one brain.

Multiple brain abscesses are a rare complication which may occur in a number of conditions including intra-thoracic sepsis. Computerized tomography has had an advantageous impact on the management of this condition by facilitating earlier diagnosis. However, the treatment modalities are inadequate and mortality remains high. A case of multiple brain abscesses in a patient with bronchiectasis is reported which exemplifies this often fatal condition. The management of multiple brain abscesses is discussed.

Adult↗

PubMed1993

Effect of antibiotics on sputum inflammatory contents in acute exacerbations of bronchiectasis.

We studied the changes in sputum neutrophil chemotactic activity (NCA) and elastolytic activity (EA) in acute exacerbations of bronchiectasis before and after treatment with oral antibiotics. Twelve patients who chronically produced sputum were assessed in the stable state, and when they subsequently developed symptoms of acute exacerbations, prior to initiation of antibiotics, during 2 weeks of antibiotics, and at 2 and 6 weeks after stopping antibiotics. NCA was measured using modified Boyden's technique with multiwell chemotaxis chamber, and EA with N-succinyl-trialanine-p-nitroanilide as elastase substrate. All 12 patients had NCA (49.3 +/- 8.69% FMLP response) and EA (50.5 +/- 17.1 mU per 100 microliters) in their sputum in the stable state. At acute exacerbation, there was significant increase in NCA (P < 0.001) and EA (P < 0.05). All responded clinically after 1 week of antibiotics, and this was associated with a decrease in NCA and EA back to the levels in stable state. A further week of antibiotics did not result in further decline of NCA or EA. Three patients had another acute exacerbation clinically between 2-6 weeks after stopping antibiotics and their NCA and EA rose again. In the other nine patients, both NCA and EA at 2 and 6 weeks post-treatment were similar to pre-exacerbation levels. Our findings suggest that short course antibiotics effectively control the upsurge in inflammatory activity in acute exacerbations, but has little effect on chronic airway inflammation.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗

PubMed2007

Bronchiectasis: correlation of high-resolution CT findings with health-related quality of life.

AIM: To evaluate the relationship between the severity of bronchiectatic diseases, as evident on high-resolution computed tomography (HRCT) and the patient's quality of life measured using the St George's Respiratory Questionnaire (SGRQ). METHODS AND MATERIALS: Forty-six patients (25 women, 21 men, mean age: 63 years) with bronchiectatic disease as evident on recent HRCT examinations were recruited. Each patient completed the SGRQ and underwent respiratory function tests. HRCT findings were blindly and independently scored by two radiologists, using the modified Bhalla scoring system. The relationships between HRCT scores, SGRQ scores and pulmonary function tests were evaluated. RESULTS: The patients' total CT score did not correlate with the SGRQ scores. However, patients with more advanced disease on HRCT, significantly differed in their SGRQ scores from patients with milder bronchiectatic disease. A significant correlation was found between the CT scores for the middle and distal lung zones and the activity, impacts and total SGRQ scores. No correlation was found between CT scores and respiratory function test indices. However, a significant correlation was found between the SGRQ scores and most of the respiratory function test indices. CONCLUSION: A correlation between the severity of bronchiectatic disease as expressed in HRCT and the health-related quality of life exists in patients with a more severe bronchiectatic disease but not in patients with mild disease. Such correlation depends on the location of the bronchiectasis in the pulmonary tree.

Adult↗

PubMed2004

Diagnosis of cystic fibrosis in adults with diffuse bronchiectasis.

We assessed the contribution of the sweat test, genotyping and nasal potential difference (NPD) in the diagnosis of cystic fibrosis (CF) in adults with diffuse bronchiectasis (DB). Among 601 adults referred for DB from 1992 to 2001, 46 were diagnosed with CF. The sweat test was positive in 37 patients and normal or intermediate in nine patients. Two CF mutations were identified in 18 patients (39%) by screening for 31 mutations and in 36 patients (78%) after complete genetic analysis. NPD was suggestive of CF in 71% of the patients. The combination of the sweat test and genetic analysis led to the diagnosis of CF in 45 patients. In the nine patients with normal or intermediate sweat test, the diagnosis was confirmed by screening for 31 mutations in five, by complete genetic screening in three, and by NPD in the remaining patient. Searching for CF should start with sweat test. If the sweat test is normal or intermediate, screening for 31 mutations may help to diagnose CF. A complete genetic analysis is indicated when only one mutation is detected and/or when other clinical features, such as obstructive azoospermia or pancreatic insufficiency, are suggestive of CF. NPD measurement is indicated in controversial cases.

Adolescent↗

PubMed2006

Bronchiectasis in rheumatoid arthritis: report of four cases and a review of the literature--implications for management with biologic response modifiers.

OBJECTIVE: To describe patients with rheumatoid arthritis (RA) who subsequently developed bronchiectasis (BR) and to review the literature on biologic response modifiers (BRM) in relation to infectious complications in the management of these patients. METHODS: We describe 4 patients with RA who were diagnosed with BR out of a cohort of 170 patients. We then performed a comprehensive review of the English language literature on the major clinical trials for RA that involved the BRMs etanercept, infliximab, anakinra, and adalimumab. We focused on inclusion/exclusion criteria involving pulmonary disease and infectious complications in these trials. RESULTS: Of the 4 patients we describe, all developed BR after the diagnosis of RA was established, had positive cyclic citrullinated peptide antibodies, had extra-articular manifestations, and had clinical courses complicated by pneumonia. Management strategies were influenced by these factors in all of the patients described. Of the 16 clinical trials on BRMs reviewed, few studies mentioned BR as an exclusion criteria or reported pneumonia as a specific infectious complication. CONCLUSIONS: BR may be considered as an extra-articular pulmonary manifestation of RA. The infectious complications associated with BR in these patients underscore the management challenge, especially in choosing whether or not to treat with BRMs. Further studies are needed to analyze the infectious complications in RA trials with BRMs, specifically, to assess the risk of patients with BR. Risk stratification in these patients may require screening them for the presence of underlying BR.

Aged↗

PubMed1976

Hyperimmunoglobulinemia E in a child with allergic bronchopulmonary aspergillosis and bronchiectasis.

A 12-year-old boy was hospitalized for resection of a bronchiectatic lesion. Investigation of an elevated cencentration of serum IgE led to a diagnosis of allergic bronchopulmonary aspergillosis. ABPA has rarely been described in the pediatric age group. This hypersensitivity lung disease is characterized by intermittent wheezing, fever, recurrent pulmonary infiltrates, eosinophilia, hyperimmunoglobulinemia E, and Type I (allergic) skin reactivity to aspergillus extract. Hyphae of aspergillus may also be found in expectorated brown mucus plugs. Type III (Arthus) skin test response and presence of precipitating antibody to this fungus may be demonstrated. Central bronchiectasis or pulmonary fibrosis may result from uncontrolled progression of this disease.

Aspergillosis↗

PubMed1993

[Bronchiectasis following colectomy for hemorrhagic rectocolitis].

Pulmonary disease is an uncommon extraintestinal manifestation of inflammatory bowel disease. We report the case of a patient in whom colectomy for ulcerative colitis was followed by development of bronchiectasis. A discussion of the relation between ulcerative colitis and bronchial disease is presented.

Aged↗

PubMed1999

Imaging the airways. Hemoptysis, bronchiectasis, and small airways disease.

Advances in technology have increased the contribution of radiology in understanding and evaluating diseases of the airways. In patients with hemoptysis, CT is now established as a complementary technique to bronchoscopy, or as an alternative to bronchoscopy in selected cases. The introduction of high-resolution CT has improved the detection and assessment of bronchiectasis and small airways disease, allowed better correlation between pathologic changes and radiologic appearances, and provided new insights into possible links between small airways disease and bronchial disease.

Bronchi↗

PubMed1995

[Bronchiectasis associated with human immunodeficiency virus infection].

The precise incidence of bronchiectasis (BCH) in individuals infected by human immunodeficiency virus (HIV) is unknown. Because such knowledge can imply different diagnostic procedures, prognosis and treatment, we report a relevant case in which clinical and radiological signs led to suspicion of BCH which was confirmed by high resolution computerized axial tomography. As life expectancy improves for HIV-infected patients, the incidence of BCH may rise. We therefore believe it is important to include BCH in the spectrum of likely pulmonary radiological signs in the HIV-infected population, though further studies must be done to assess the epidemiology, etiology, pathogenesis and prognosis of BCH.

AIDS-Related Opportunistic Infections↗

PubMed1999

[Association of severe bronchial disease (bronchial casts, bronchiectasis) and partial abnormal pulmonary venous drainage in 2 children with Turner's syndrome].

UNLABELLED: The prevalence of cardiovascular malformation is high in Turner's syndrome. We report two cases of partial anomalous pulmonary venous drainage associated with a severe bronchial disease. CASE REPORTS: Case 1: a 5-year-old girl with monosomy X presented with acute respiratory failure. The chest X-ray showed an atelectasis of the left lower lobe. The patient improved following bronchoscopic removal of a bronchial cast. During hospitalization, an anomalous pulmonary venous drainage of the right lung was discovered. In spite of complete surgical repair of the cardiac malformation, several episodes of respiratory failure occurred several years after the operation. Case 2: a 9-year-old girl with monosomy X and coarctation of the aorta underwent pre-operative cardiac catheterization. She had a history of bronchiectasis, leading to a lobectomy. Her respiratory condition improved following surgery. A partial anomalous pulmonary venous return was discovered during the cardiac angiography. The coarctation was repaired surgically. The correction of the left-to-right shunt was not necessary. CONCLUSION: The partial anomalous pulmonary venous return is frequently reported in Turner's syndrome. The possible respiratory disease associated with this cardiac malformation is usually described as a consequence of the increase in pulmonary blood flow. Our two cases did not match this hypothesis.

Angiography↗

PubMed1991

Systemic effects of inflammation in bronchiectasis.

A group of bronchiectatic subjects in the clinically stable state were studied for systemic evidence of inflammation. The following parameters were evaluated: body weight, serum albumin, serum globulin, serum alpha 1-antitrypsin (alpha 1 AT) and peripheral white cell count. For serum albumin and globulin, comparison was made between subjects with bronchiectasis and control subjects with no known pulmonary disease matched for sex and age, and for serum alpha 1 AT and peripheral white cell count, matched for smoking habit as well. The bronchiectatic subjects showed systemic effects of inflammation as indicated by lower body weight and serum albumin (P less than 0.01), higher serum globulin (P less than 0.001), serum alpha 1 AT (P less than 0.05) and total leucocyte count (P less than 0.05). Differential white cell count showed that the elevation was distributed in most cell types. Correlation matrix was done for the above systemic parameters and indices of airway inflammation including sputum volume, purulence, and polymorph count and FEV1. There was an inverse correlation between total peripheral WBC count and FEV1 in percentage of predicted (P less than 0.01), and a positive correlation between sputum purulence and sputum polymorph score (P less than 0.05). This suggests that host peripheral leucocyte response may be a factor in the determination of lung function.

Adolescent↗

PubMed1990

IgA subclasses in sputum from patients with bronchiectasis.

The concentrations of IgA1 and IgA2 were measured in the serum and sputum from 27 clinically stable patients with bronchiectasis. Of the 27 patients, nine were regularly producing mucoid secretions, nine mucopurulent and nine purulent secretions. No significant differences were observed in serum IgA2 concentrations between the patient groups, although IgA1 concentrations were higher in the serum from the patients producing mucopurulent sputum than those producing mucoid or purulent sputum. The sputum concentrations of both IgA1 and IgA2 increased significantly with purulence. Furthermore, there was a significant increase in the proportion of IgA2 as the secretions became more purulent. The sputum concentrations of both IgA subclasses indicated significant local synthesis, with increased production (especially of IgA2) in the presence of inflammation due to infection.

Bronchiectasis↗