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Bronchiectasis
Selected AbstractsDistal esophagitis in patients with mustard-gas induced chronic coughDISEASES OF THE ESOPHAGUS, Issue 4 2006M. Ghanei SUMMARY., Although confounded by some factors such as medications or surgical complications, the relationship between esophageal pathology and pulmonary disorders has been the subject of many studies. The present study sought to investigate the said relationship in patients inflicted by respiratory disorders induced by mustard gas (MG). A case group of patients complaining of respiratory complications and chronic coughs following MG exposure, and a control group of patients with chronic coughs but without a history of MG exposure were studied. All the case and control subjects had symptoms of gastro-esophageal reflux disease. Chest high resolution tomography (HRCT) was performed to evaluate the existence of pulmonary disorders. Endoscopy and histological studies were carried out to determine the severity of esophagitis in both groups presenting with gastroesophageal reflux. Ninety male patients, who had met our criteria, along with 40 male control cases underwent the diagnostic procedures. The frequency of endoscopic esophagitis findings in the chemically exposed group was significantly higher than that in the control group (70.0%vs. 42.5%). A pathological evaluation revealed that the frequency of esophagitis in the cases was more than that in the controls (32.3%vs. 14.2%). Chest HRCT evaluation demonstrated that half the case group had more than 25% air trapping in expiratory films, mostly compatible with bronchiolitis obliterans (BO). In addition, they were suffering from asthma, chronic bronchitis and bronchiectasis. Bronchiolitis obliterans, along with other lung disorders, can be considered as contributors in the pathogenesis of esophagitis in MG exposed patients. [source] Effect of long-term nebulized colistin on lung function and quality of life in patients with chronic bronchial sepsisINTERNAL MEDICINE JOURNAL, Issue 7 2007D. P. Steinfort Abstract Recurrent Gram-negative bacterial infection is a significant cause of death in patients with bronchiectasis and severe chronic obstructive pulmonary disease (COPD). Nebulized colistin in cystic fibrosis has shown maintenance of pulmonary function and improved symptom scores. We prospectively followed 18 patients with chronic bronchial sepsis treated with nebulized colistin 30 mg daily. Mean decline in forced expiratory volume in 1 s was significantly slower following commencement of inhaled colistin (44 mL/year vs 104 mL/year, P = 0.035). Mean decline in forced vital capacity was also significantly slower following commencement of colistin (48 mL/year vs 110 mL/year, P = 0.033). Patient-reported quality of life improved following commencement of colistin (3.6 vs 6.2, P = 0.001). No patient had isolates resistant to colistin. No side-effects were reported by patients in the cohort. Use of inhaled colistin in the treatment of bronchiectasis and severe (COPD) in patients with recurrent Gram-negative infections is safe. Inhaled colistin may improve quality of life and slow decline in forced expiratory volume in 1 s and forced vital capacity. [source] Transporter associated with antigen processing deficiency: an additional condition associated with bronchiectasisINTERNAL MEDICINE JOURNAL, Issue 3 2007J Zimmer No abstract is available for this article. [source] Computer-aided diagnosis system for the detection of bronchiectasis in chest computed tomography imagesINTERNATIONAL JOURNAL OF IMAGING SYSTEMS AND TECHNOLOGY, Issue 4 2009D. Shiloah Elizabeth Abstract A computer-aided diagnosis (CAD) system has been developed for the detection of bronchiectasis from computed tomography (CT) images of chest. A set of CT images of the chest with known diagnosis were collected and these images were first denoised using Wiener filter. The lung tissue was then segmented using optimal thresholding. The Pathology Bearing Regions (PBRs) were then extracted by applying pixel-based segmentation. For each PBR, a gray level co-occurrence matrix (GLCM) was constructed. From the GLCM texture features were extracted and feature vectors were constructed. A probabilistic neural network (PNN) was constructed and trained using this set of feature vectors. The images together with the PBRs and the corresponding feature vector and diagnosis were stored in an image database. Rules for diagnosis and for determining the severity of the disease were generated by analyzing the images known to be affected by bronchiectasis. The rules were then validated by a human expert. The validated rules were stored in the Knowledge Base. When a physician gives a CT image to the CAD system, it first transforms the image into a set of feature vectors, one for each PBR in the image. It then performs the diagnosis using two techniques: PNN and mahalanobis distance measure. The final diagnosis and the severity of the disease are determined by correlating the diagnosis determined by both the techniques in consultation with the knowledge base. The system also retrieves similar cases from the database. Thus, this system would aid the physicians in diagnosing bronchiectasis. © 2009 Wiley Periodicals, Inc. Int J Imaging Syst Technol, 19, 290,298, 2009 [source] Paediatric bronchiectasis in the twenty-first century: Experience of a tertiary children's hospital in New ZealandJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 2 2003EA Edwards Objective: Despite its decline in developed countries, bronchiectasis appeared to be a common diagnosis in Auckland, New Zealand children. The aims of this study were: to document the number of children in Auckland with bronchiectasis, their severity, clinical characteristics and possible aetiologies; to assess whether there was a relationship between ethnicity and poverty; and to estimate a crude bronchiectasis prevalence rate for New Zealand. Methods: A retrospective review of the case histories of all children attending a tertiary children's hospital in Auckland with bronchiectasis diagnosed by high-resolution chest computed tomography (CT) scan, during the period 1998,2000 was undertaken. Data collected included patient demographics, number of hospitalizations pre- and post-diagnosis, lung function tests, radiology and investigations. The New Zealand deprivation 1996 index was applied to the data to obtain a measure of socio-economic status. Results: Bronchiectasis was found to be common, with an estimated prevalence of approximately one in 6000 in the Auckland paediatric population. It was disproportionately more common in the Pacific Island and Maori children. In Pacific Island children, bronchiectasis not caused by cystic fibrosis was nearly twice as common in the general population than cystic fibrosis. Socio-economic deprivation and low immunization rates may be significant contributing factors. The bronchiectasis seen was extensive. Ninety-three percent had bilateral disease and 64% had involvement of four or more lobes on chest CT scan. A wide range of comorbidities and underlying aetiologies were evident. Conclusions: Paediatric bronchiectasis in Auckland, New Zealand, is common but underresourced. Only the most severe cases are being recognized, providing a significant challenge for paediatric health professionals. [source] United airways again: high prevalence of rhinosinusitis and nasal polyps in bronchiectasisALLERGY, Issue 5 2009J. M. Guilemany Background:, Although various relationships between the lower and upper airways have been found, the association of bronchiectasis with chronic rhinosinusitis and nasal polyps has not been thoroughly evaluated. This study was undertaken to examine the association of idiopathic and postinfective bronchiectasis with chronic rhinosinusitis and nasal polyposis. Methods:, In a prospective study, 56 patients with idiopathic and 32 with postinfective bronchiectasis were evaluated for chronic rhinosinusitis and nasal polyposis by using EP3OS criteria and assessing: symptoms score, nasal endoscopy, sinonasal and chest CT scan, nasal and lung function and nasal and exhaled NO. Results:, Most bronchiectasis patients (77%) satisfied the EP3OS criteria for chronic rhinosinusitis, with anterior (98.5%) and posterior (91%) rhinorrhea and nasal congestion (90%) being the major symptoms. Patients presented maxillary, ethmoidal and ostiomeatal complex occupancy with a total CT score of 8.4 ± 0.4 (0,24). Using endoscopy, nasal polyps with a moderate score of 1.6 ± 0.1 (0,3) were found in 25% of patients. Nasal NO was significantly lower in patients with nasal polyposis (347 ± 62 ppb) than in those without them (683 ± 76 ppb; P < 0.001), and inversely correlated (R = ,0.36; P < 0.01) with the ostiomeatal complex occupancy. In the chest CT scan, patients with chronic rhinosinusitis showed a higher bronchiectasis severity score (7.2 ± 0.5; P < 0.001) than patients without (3.7 ± 0.7). The prevalence of chronic rhinosinusitis, nasal polyps and other outcomes were similar in idiopathic and postinfective bronchiectasis. Conclusions:, The frequent association of chronic rhinosinusitis and nasal polyposis with idiopathic and postinfective BQ supports the united airways concept, and it suggests that the two type of bronchiectasis share common etiopathogenic mechanisms. [source] Common variable immunodeficiency: 20-yr experience at a single centrePEDIATRIC ALLERGY AND IMMUNOLOGY, Issue 2 2009Ma Pilar Llobet Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency. It can present at any age in patients with a history of recurrent bacterial infections, with or without a family history of other primary immunodeficiencies (PID), and shows a wide range of clinical manifestations and immunological data. Diagnosis is based on low IgG, IgM and/or IgA levels. Delayed diagnosis and therapy can lead to bronchiectasis and malabsorption. The aim of this study was to describe a paediatric population diagnosed of CVID and its evolution in the population. Memory B-cell (MB) classification carried out in these patients was correlated with clinical manifestations and outcome. Clinical and immunological data of 22 CVID children under 18 yr treated at our centre between 1985 and 2005 are presented. Immunological studies included those for diagnosis and MB quantification. Differences in form of presentation, familial incidence and MB classification were reviewed. A statistical descriptive analysis was made. Infections were the commonest manifestation, affecting mainly respiratory (19/22) and gastrointestinal (10/22) tracts. Bronchiectasis was present in seven cases, and detected prior to CVID diagnosis in five. Replacement therapy led to a significant reduction in the number of infections. Severe complications appeared mostly in patients without MB. Patients of the same family share the same MB group. Family members had also been diagnosed of CVID in seven cases. Early diagnosis and therapy are essential to improve outcome in these patients. MB studies are useful in children to orient prognosis and further genetic studies. [source] Evaluation and management of pulmonary disease in ataxia-telangiectasiaPEDIATRIC PULMONOLOGY, Issue 9 2010Sharon A. McGrath-Morrow MD Abstract Ataxia-telangiectasia (A-T) is a rare autosomal recessive disorder caused by mutations in the ATM gene, resulting in faulty repair of breakages in double-stranded DNA. The clinical phenotype is complex and is characterized by neurologic abnormalities, immunodeficiencies, susceptibility to malignancies, recurrent sinopulmonary infections, and cutaneous abnormalities. Lung disease is common in patients with A-T and often progresses with age and neurological decline. Diseases of the respiratory system cause significant morbidity and are a frequent cause of death in the A-T population. Lung disease in this population is thought to exhibit features of one or more of the following phenotypes: recurrent sinopulmonary infections with bronchiectasis, interstitial lung disease, and lung disease associated with neurological abnormalities. Here, we review available evidence and present expert opinion on the diagnosis, evaluation, and management of lung disease in A-T, as discussed in a recent multidisciplinary workshop. Although more data are emerging on this unique population, many recommendations are made based on similarities to other more well-studied diseases. Gaps in current knowledge and areas for future research in the field of pulmonary disease in A-T are also outlined. Pediatr. Pulmonol. 2010; 45:847,859. © 2010 Wiley-Liss, Inc. [source] Resolution of extensive severe bronchiectasis in an infantPEDIATRIC PULMONOLOGY, Issue 7 2010Suzanne Crowley DM Abstract Bronchiectasis is, by definition, an irreversible condition. Following recent reports of reversible bronchiectasis in children, it has been suggested that the definition be broadened to include pre-bronchiectasis and transitional reversible states. We describe the case of a young infant who had extensive, severe bronchiectasis of unknown etiology that resolved following prolonged treatment with antibiotics and a tapering course of oral steroids. We suggest that the prolonged treatment may have played a role, perhaps by eradicating infection and thus enabling regeneration of bronchial anatomy. Pediatr Pulmonol. 2010; 45:717,720. © 2010 Wiley-Liss, Inc. [source] The use of high resolution computerized tomography (HRCT) of the chest in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis lung disease,PEDIATRIC PULMONOLOGY, Issue 5 2010Samya Z. Nasr MD Abstract Objectives To compare the usefulness of HRCT of the chest versus spirometric measures (PFTs) in evaluating the effect of tobramycin solution for inhalation (TSI) in cystic fibrosis (CF). Methods Thirty-two CF patients with mostly mild lung disease age ,6 years, were enrolled in a double-blind, placebo-controlled pilot study. Patients were chronically colonized with Pseudomonas aeruginosa for at least 6 months prior to and at enrollment. If patients were on TSI, they were taken off for at least 3 months prior to enrollment. Duration was 6 months; 31 subjects completed the study. HRCT and PFTs were evaluated at baseline, after 28 days of treatment and at the end of the study. Study medication was administered as 5,ml nebulized treatment twice a day for 28 days followed by 28 days off (one cycle). Study consisted of three cycles. Two radiologists scored all films using a validated system. A total HRCT score consists of the sum of subscores: linear opacities, hyperinflation, nodular opacities, peribronchial thickening, mucous plugging, and bronchiectasis; each subscore could range from 0 to 80, with potential total scores varying from 0 to 480. The percent of the maximum possible HRCT score was then calculated and used for all comparisons. Results Using two tailed paired t -test, the percent maximum HRCT score decreased by 1.4,±,2.6% (mean,±,SD) (P,=,0.049) and 0.3,±,2.8% (P,=,0.63) for the TSI group and decreased by 0.1,±,1.5% (P,=,0.74) and increased by 0.6,±,1.8% (P,=,0.23) for the placebo group between visits 1 and 2, and visits 1 and 3, respectively. The data were then analyzed using a mixed model utilizing changes in scores over the durations of the study for each group. The change of HRCT score for the TSI group was ,0.24/day (P,=,0.02) and ,0.03/day (P,=,0.22), and for the control group the change was ,0.01 (P,=,0.93) and 0.02 (P,=,0.29) between visits 1 and 2, and visits 1 and 3 respectively. FEF25,75% and FEV1% changes were not statistically significant using both analyses. Conclusion HRCT seems to be more sensitive in detecting treatment effect than PFT in CF patients with mild lung disease, especially following the first treatment period (visit 2). Total HRCT score showed some improvement at the end of the study, though not statistically significant. This is probably due to obtaining the HRCT an average of 30 days after completion of the TSI treatment, and selection of study population with mostly mild lung disease. This could indicate that the most significant improvement in the total HRCT score in this patient population occurs after the first treatment period with TSI. Pediatr Pulmonol. 2010; 45:440,449. © 2010 Wiley-Liss, Inc. [source] Early adolescent primary ciliary dyskinesia associated with broncholithiasisPEDIATRIC PULMONOLOGY, Issue 7 2008Koray Hekimoglu MD Abstract Primary ciliary dyskinesia (PCD), is a rare congenital disease group, and contained Kartageners syndrome. This syndrome is characterized with situs viscerum inversus, sinusitis and bronchiectasis. In some elderly patients, broncholithiasis may be associated with PCD. The coexistence of early adolescence PCD and broncolithiasis has not been reported yet. We report here a case of a 14-year-old early adolescent male who had typically presented as PCD-based Kartageners syndrome and developed broncholithiasis which diagnosed with computed tomography. Pediatr Pulmonol. 2008; 43:714,716. © 2008 Wiley-Liss, Inc. [source] Chronic wet cough: Protracted bronchitis, chronic suppurative lung disease and bronchiectasisPEDIATRIC PULMONOLOGY, Issue 6 2008A.B. Chang PhD Abstract The role of persistent and recurrent bacterial infection of the conducting airways (endobronchial infection) in the causation of chronic respiratory symptoms, particularly chronic wet cough, has received very little attention over recent decades other than in the context of cystic fibrosis (CF). This is probably related (at least in part) to the (a) reduction in non-CF bronchiectasis in affluent countries and, (b) intense focus on asthma. In addition failure to characterize endobronchial infections has led to under-recognition and lack of research. The following article describes our current perspective of inter-related endobronchial infections causing chronic wet cough; persistent bacterial bronchitis (PBB), chronic suppurative lung disease (CSLD) and bronchiectasis. In all three conditions, impaired muco-ciliary clearance seems to be the common risk factor that provides organisms the opportunity to colonize the lower airway. Respiratory infections in early childhood would appear to be the most common initiating event but other conditions (e.g., tracheobronchomalacia, neuromuscular disease) increases the risk of bacterial colonization. Clinically these conditions overlap and the eventual diagnosis is evident only with further investigations and long term follow up. However whether these conditions are different conditions or reflect severity as part of a spectrum is yet to be determined. Also misdiagnosis of asthma is common and the diagnostic process is further complicated by the fact that the co-existence of asthma is not uncommon. The principles of managing PBB, CSLD and bronchiectasis are the same. Further work is required to improve recognition, diagnosis and management of these causes of chronic wet cough in children. Pediatr Pulmonol. 2008; 43:519,531. © 2008 Wiley-Liss, Inc. [source] Chronic lung disease in human immunodeficiency virus (HIV) infected children,PEDIATRIC PULMONOLOGY, Issue 1 2008Heather J. Zar Abstract The development of chronic lung disease is common in HIV-infected children. The spectrum of chronic HIV-associated lung disease includes lymphocytic interstitial pneumonia (LIP), chronic infections, immune reconstitution inflammatory syndrome (IRIS), bronchiectasis, malignancies, and interstitial pneumonitis. Chronic lung disease may result from recurrent or persistent pneumonia due to bacterial, mycobacterial, viral, fungal or mixed infections. In high tuberculosis (TB) prevalence areas, M. tuberculosis is an important cause of chronic respiratory illness. With increasing availability of highly active antiretroviral therapy (HAART) for children in developing countries, a rise in the incidence of IRIS due to mycobacterial or other infections is being reported. Diagnosis of chronic lung disease is based on chronic symptoms and persistent chest X-ray changes but definitive diagnosis can be difficult as clinical and radiological findings may be non-specific. Distinguishing LIP from miliary TB remains a difficult challenge in HIV-infected children living in high TB prevalence areas. Treatment includes therapy for specific infections, pulmonary clearance techniques, corticosteroids for children with LIP who are hypoxic or who have airway compression from tuberculous nodes and HAART. Children who are taking TB therapy and HAART need adjustments in their drug regimes to minimize drug interactions and ensure efficacy. Preventative strategies include immunization, chemoprophylaxis, and micronutrient supplementation. Early use of HAART may prevent the development of chronic lung disease. Pediatr Pulmonol. 2008; 43:1,10. © 2007 Wiley-Liss, Inc. [source] Risk factors for the development of bronchiectasis in HIV-infected childrenPEDIATRIC PULMONOLOGY, Issue 10 2007David M. Berman DO Abstract Our objective was to describe the risk factors for the development of bronchiectasis in HIV-1 infected children. This study was a retrospective, case controlled study based upon medical record review of HIV-1 infected children receiving primary care at a single large, urban medical center in Miami, Florida. Cases (HIV-1 infected children who developed bronchiectasis while being cared for between January 1982 and September 2000) were matched 1:3 (birth,±,24 months) with controls (HIV-1 infected children without bronchiectasis). Variables analyzed including number of episodes of pneumonia (including Pneumocystis jiroveci pneumonitis [PCP], lymphoid interstitial pneumonitis (LIP), and CDC category of immunosuppression) were noted in both cases and controls until the age at which the cases developed bronchiectasis. Of the 749 patients whose charts were reviewed, 43 met the case definition for bronchiectasis and 19 met the eligibility criteria for this study. Fifty-seven controls were randomly selected from the patients without bronchiectasis. Cases were more likely to have experienced recurrent pneumonia than the controls; 17 (89.5%) versus 5 children (8.8%) respectively (P -value ,0.001) as well as a greater mean number of episodes of pneumonia 8.2 (range, 4,13) versus 1.45 (range, 0,9) respectively (CI,=,(5.58,7.82); P -value ,0.001). Cases were more likely to have progressed to CDC immunological category 3 than the controls; 19 (100%) versus 32 (56%) children respectively (P -value <0.001). LIP occurred more frequently in the cases than in the controls; 14/19 (73.6%) versus 19/57 (33.3%), respectively (P -value,=,0.005). HIV-1 infected children with a history of recurrent pneumonia, profound immuno-suppression (CDC immunologic category 3), and LIP appear to have a higher risk of developing bronchiectasis. Pediatr Pulmonol. 2007; 42:871,875. © 2007 Wiley-Liss, Inc. [source] Markers of airway inflammation in primary ciliary dyskinesia studied using exhaled breath condensatePEDIATRIC PULMONOLOGY, Issue 6 2006MRCPCH, Nadwa Zihlif MD Abstract Macroscopically, the airways in primary ciliary dyskinesia (PCD) are inflamed and infected, and the eventual result is bronchiectasis. The measurement of noninvasive markers of inflammation in PCD may allow determination of mechanisms of tissue damage, and even allow monitoring of therapy. The aim of this study was to measure in exhaled breath condensate (EBC) of children with PCD the concentrations of the neutrophil chemoattractants leukotriene (LT) B4 and interleukin (IL)-8 and the marker of oxidative stress 8-isoprostane (8-IP), and to try determining whether these markers can be used to assess mechanisms of airway inflammation in these patients. Concentrations of LTB4, IL-8, and 8-IP in the EBC of 23 PCD and 11 age-matched healthy children were measured using an enzyme immunoassay (EIA). The children also performed spirometry and underwent sputum induction, the latter for differential cell count. The concentrations of 8-IP in EBC of children with stable PCD were significantly increased compared to normal controls (median, 7.8 pg/ml vs. 3.1 pg/ml; P,=,0.004). There was no difference in the median concentrations of EBC LTB4 between PCD subjects and healthy controls (28 pg/ml vs. 28 pg/ml; P,=,0.5). IL-8 levels were below the detection limit of the assay, and were not analyzed further. There was no correlation between concentrations of either 8-IP or LTB4 in EBC and forced expired volume in 1 sec in PCD children. Sputum induction was successful in 83% of the subjects; the median induced sputum neutrophil count was 69% (interquartile range, 59.3,73.6). No significant correlation was found between sputum neutrophils and either EBC 8-IP or LTB4 concentrations in PCD children. This study showed that oxidative stress, as reflected by increased exhaled 8-IP concentration, is increased in PCD children. The mechanism of airway neutrophilia is unclear, but is unlikely to be related to increased production of LTB4, at least in stable PCD patients. Pediatr Pulmonol. © 2006 Wiley-Liss, Inc. [source] Retrospective review of children presenting with non cystic fibrosis bronchiectasis: HRCT features and clinical relationships,PEDIATRIC PULMONOLOGY, Issue 2 2003E.A. Edwards FRACP Abstract Non cystic fibrosis (CF) bronchiectasis in children presents with a spectrum of disease severity. Our aims were to document the extent and severity of disease in children with non-CF bronchiectasis, to review the inter- and intraobserver agreement for the high-resolution computed tomography (HRCT) features examined, and to assess correlations between HRCT features and clinical measures of severity. We performed a retrospective review of 56 children from the Starship Children's Hospital. HRCT scans were scored by a modified Bhalla system, and the chest X-rays using the Brasfield score. Scores were correlated with demographics, number of hospitalizations, disease duration, pulmonary function, clinical examination, and chronic sputum infection. The bronchiectasis seen was widespread and severe, particularly in Maori and Pacific Island children. The kappa coefficient for intraobserver agreement was better than that for interobserver agreement. Comparisons between HRCT scan and lung function parameters showed that the strongest relationships were between forced expiratory volume in 1 sec (FEV1) and forced expiratory flow between 25,75% of forced vital capacity (FEF25,75) with the extent of bronchiectasis, bronchial wall thickening, and air trapping. Children with digital clubbing and chest deformity showed significantly higher scores for extent of bronchiectasis, bronchial wall dilatation and thickness, and overall computed tomography (CT) score. No relationship was demonstrated between chronic sputum infection and CT score. The HRCT score demonstrated a stronger correlation between the extent and severity of bronchiectasis, and spirometry values, than the chest X-ray score. In conclusion, pediatric non-CF bronchiectasis in Auckland is extensive and severe. The good intraobserver ratings mean that consistency of scoring is possible on repeated scans. This study cannot comment on the relationships of CT and less severe disease. Pediatr Pulmonol. 2003; 36:87,93. © 2003 Wiley-Liss, Inc. [source] Bronchiectasis in children: Orphan disease or persistent problem?,PEDIATRIC PULMONOLOGY, Issue 6 2002Charles W. Callahan DO Abstract More than a decade ago, bronchiectasis unrelated to cystic fibrosis was termed an "orphan disease", because it had become an uncommon clinical entity among children in the developed world. Bronchiectasis is more common among children in lower socioeconomic classes and in developing countries, presumably due to more frequent and recurrent respiratory infections, environmental airway irritants, poor immunization rates, and malnutrition. Reports from the Southern Pacific and from Alaska Native children reveal persistently high rates of childhood bronchiectasis. Better epidemiologic data throughout the world are needed to reassess the importance of this condition. The pathophysiology includes airway inflammation, mucus production, and regional airway obstruction, yet the reasons why some children develop bronchiectasis while other do not is unclear. The coexistence of asthma with bronchiectasis is associated with more severe disease, yet the impact of asthma therapy in children with both disorders has not been studied. Similarly, the pattern of antibiotic use for children with bronchiectasis varies by region with little data to justify one particular approach. It may be that public health measures aimed at improving living conditions for children and prevention of respiratory infections with antiviral vaccines will have more impact on childhood bronchiectasis than medical treatments in the future. Pediatr Pulmonol. 2002; 33:492,496. © 2002 Wiley-Liss, Inc. [source] Improved outcome of pediatric kidney transplantations in the Netherlands , Effect of the introduction of mycophenolate mofetil?PEDIATRIC TRANSPLANTATION, Issue 1 2005Karlien Cransberg Abstract:, Collaboration of the Dutch centers for kidney transplantation in children started in 1997 with a shared immunosuppressive protocol, aimed at improving graft survival by diminishing the incidence of acute rejections. This study compares the results of transplantations in these patients to those in a historical reference group. Ninety-six consecutive patients receiving a first kidney transplant were treated with an immunosuppressive regimen consisting of mycophenolate mofetil, cyclosporine and corticosteroids. The results were compared with those of historic controls (first transplants between 1985 and 1995, n = 207), treated with different combinations of corticosteroids, cyclosporine A and/or azathioprine. Cytomegalovirus (CMV) prophylaxis was prescribed to high-risk patients in the study group, and only a small proportion of the reference group. The graft survival at 1 yr improved significantly: 92% in the study group, vs. 73% in the reference group (p < 0.001). In the study group 63% of patients remained rejection-free during the first year; in the reference group 28% (p < 0.001). After statistical adjustment of differences in baseline data, as cold ischemia time, the proportion of LRD, preemptive transplantation, and young donors, the difference between study and reference group in graft survival (RR 0.33, p = 0.003) and incidence of acute rejection (RR 0.37, p < 0.001), as the only factor, remained statistically significant, indicating the effect of the immunosuppressive therapy. In the first year one case of malignancy occurred in each group. CMV disease occurred less frequently in the study group (11%) than in the reference group (26%, p = 0.02). As a new complication in 4 patients bronchiectasis was diagnosed. A new consensus protocol, including the introduction of mycophenolate mofetil, considerably improved the outcome of pediatric kidney transplantation in the Netherlands, measured as reduction of the incidence of acute rejection and improved graft survival. [source] The causes of haemoptysis in Malaysian patients aged over 60 and the diagnostic yield of different investigationsRESPIROLOGY, Issue 1 2003Catherine Mee-Ming WONG Objective: In southeast Asia, pulmonary tuberculosis (TB) is the most frequently presumed diagnosis for haemoptysis. This study was designed to assess the causes of haemoptysis, the diagnostic yield of causes in different diagnostic modalities and the distribution of older patients. Methods: All patients presenting to the University of Malaya Medical Centre, Kuala Lumpur, Malaysia with haemoptysis were recruited prospectively and evaluated. Results: One hundred and sixty patients were evaluated for haemoptysis; 71 (44.4%) were aged 60 years or more. Significantly more patients smoked in the older age group (P = 0.002). The main causes of haemoptysis in the older patients were bronchogenic carcinoma (49.3%), pneumonia (11.3%), bronchiectasis (8.6%), cryptogenic (5.6%) and active TB (4.2%). Significantly more older patients had carcinoma (P < 0.001), while the younger patients more often had TB (P < 0.001). Chest pain was significantly more common in the older patients (P = 0.025), particularly in patients with carcinoma. Bronchoscopy alone or combined with CT of the thorax was significantly more diagnostic in the older patient (P = 0.006). Conclusion: Bronchogenic carcinoma is the commonest cause of haemoptysis in patients aged 60 years and above. Presumptive anti-TB therapy should not be encouraged despite the regional high prevalence of TB. [source] Allergic bronchopulmonary aspergillosis: New concepts of pathogenesis and treatmentRESPIROLOGY, Issue 1 2001Peter A. B. Wark Allergic bronchopulmonary aspergillosis (ABPA) is a condition that results from a hypersensitivity reaction to the fungus Aspergillus fumigatus. The purpose of the present review is to examine the pathogenesis of this condition and the evidence for treatments available. Allergic bronchopulmonary aspergillosis is characterized by an intense airway inflammation with eosinophils and the formation of mucus plugs. Clinically, there are periods of exacerbation and remission that may lead to proximal bronchiectasis and fibrotic lung disease. New evidence confirms the role of intense airway inflammation with eosinophils, but also suggests a role for interleukin (IL)-8/neutrophil-mediated inflammation in this process, and the potential deficiency of anti-inflammatory cytokines such as reduced IL-10. Treatment for ABPA has so far focused on corticosteroids to suppress eosinophilic airway inflammation. An expanding knowledge of the pathology of ABPA also suggests other therapies may be of potential benefit, particularly the use of azole antifungal agents. Allergic bronchopulmonary aspergillosis is itself an important complication of asthma and cystic fibrosis. A greater understanding of the condition is required to improve management and well-designed clinical trials need to be carried out to critically assess new and current treatments. In addition, the information gained from the studies of its pathogenesis has the potential to benefit our understanding of the disease processes in asthma and bronchiectasis. [source] Ciliary assessment in bronchiectasisRESPIROLOGY, Issue 2 2000Kenneth Wt Tsang Objective: Bronchiectasis is a common condition among the Oriental population and affected patients suffer from chronic sputum production punctuated by recurrent infective exacerbations. Cilia are minute structures present on the surface of respiratory and other epithelial cells that beat continuously to maintain a sterile mucosal surface in the respiratory tract. Patients with primary ciliary dyskinesia could potentially develop recurrent sinotrachrobronchitis, bronchiectasis, serous otitis media, hydrocephalus, and male infertility. The assessment of cilia has, however, received little attention until recently and generally involves elaborate methods that require complex and expensive technology. This brief article discusses application of the saccharine test, light microscopy assessment of ciliary beat, and transmission electron microscopy assessment of the ultrastructure of cilia. The rationale and indications for ciliary assessment are also listed along with illustrations showing ciliary structure, equipment required for sampling and assessment of cilia, and transmission electron micrographs of ciliary ultrastructural abnormalities. [source] A prospective evaluation of hemoptysis cases in a tertiary referral hospitalTHE CLINICAL RESPIRATORY JOURNAL, Issue 3 2010uz Uzun Abstract Background and Aims:, Hemoptysis is symptomatic of a potentially serious and life-threatening thoracic disease. The purpose of this study was to evaluate the relative frequency of the different causes of hemoptysis, the change of the frequency of diseases, the value of the evaluation process and the outcome in a tertiary referral hospital. Methods:, A prospective study was carried out on consecutive patients presented with hemoptysis. Results:, A total of 178 patients (136 male, 42 female) were included to the study. Lung cancer (51), pulmonary embolism (23) and bronchiectasis (23) constituted most of the diagnosis. The most frequent cause of hemoptysis in males was by far lung carcinoma (50). Twelve cases of bronchiectasis and 11 cases of pulmonary embolism were observed in females. While lung cancer and pulmonary embolism were associated with mild to moderate amounts of bleeding (84% and 100%, respectively), patients with active tuberculosis and pulmonary vasculitis had severe to massive hemoptysis (50% and 44%, respectively). Transthoracic and other organ biopsies, spiral computed tomography (CT) angiography (X pres/GX model TSX-002a, Toshiba, Tochigi Ken, Japan) and aortography yielded high diagnostic results in our group (100%, 67%, 59% and 100%, respectively). The most frequent final diagnosis in patients with normal chest radiograph was pulmonary embolism (seven cases). Conclusions:, Lung cancer, pulmonary embolism and bronchiectasis were the main causes of hemoptysis in this prospective cohort; however, this is the first report showing pulmonary embolism as a leading cause of hemoptysis. CT angiography with high-resolution CT should be the primary diagnostic modality if the initial investigation is inconclusive in hemoptysis cases. Please cite this paper as: Uzun O, Atasoy Y, Findik S, Atici AG and Erkan L. A prospective evaluation of hemoptysis cases in a tertiary referral hospital. The Clinical Respiratory Journal 2010; 4: 131,138. [source] Disease-Specific Survival Benefit of Lung Transplantation in Adults: A National Cohort StudyAMERICAN JOURNAL OF TRANSPLANTATION, Issue 7 2009A. Titman The lung transplantation candidate population is heterogeneous and survival benefit has not been established for all patient groups. UK data from a cohort of 1997 adult (aged , 16), first lung transplant candidates (listed July 1995 to July 2006, follow-up to December 2007) were analyzed by diagnosis, to assess mortality relative to continued listing. Donor lungs were primarily allocated according to local criteria. Diagnosis groups studied were cystic fibrosis (430), bronchiectasis (123), pulmonary hypertension (74), diffuse parenchymal lung disease (564), chronic obstructive pulmonary disease (COPD, 647) and other (159). The proportion of patients in each group who died while listed varied significantly (respectively 37%, 48%, 41%, 49%, 19%, 38%). All groups had an increased risk of death at transplant, which fell below waiting list risk of death within 4.3 months. Thereafter, the hazard ratio for death relative to listing ranged from 0.34 for cystic fibrosis to 0.64 for COPD (p < 0.05 all groups except pulmonary hypertension). Mortality reduction was greater after bilateral lung transplantation in pulmonary fibrosis patients (p = 0.049), but not in COPD patients. Transplantation appeared to improve survival for all groups. Differential waiting list and posttransplant mortality by diagnosis suggest further use and development of algorithms to inform lung allocation. [source] Bactericidal activity of neutrophils with reduced oxidative burst from adults with bronchiectasisAPMIS, Issue 2 2009PAUL KING Recent work has shown that the most common abnormality on screening of immune function in cohort of adult subjects with bronchiectasis was a low neutrophil oxidative burst. To assess the functional significance of a low oxidative burst in subjects with idiopathic bronchiectasis. Neutrophils with a low oxidative burst were obtained from six bronchiectasis patients and assessed for their ability to kill Staphylococcus aureus. The results were compared with those obtained using neutrophils from 12 healthy controls subjects and control neutrophils treated with dimethylthiourea (DMTU), an inhibitor of the oxidative burst. The results showed that the bronchiectasis subjects had significantly reduced killing of bacteria compared with controls (p<0.001). The addition of DMTU to neutrophils of control subjects significantly impaired both the oxidative burst and bactericidal activity. The addition of interferon-, enhanced oxidative burst in both groups. Abnormal neutrophil function in some subjects with bronchiectasis may account for their high rate of infection. [source] 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 bronchiectasisAPMIS, Issue 11 2002PÄIVI MAISI 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. [source] Some cases of common variable immunodeficiency may be due to a mutation in the SBDS gene of Shwachman,Diamond syndromeCLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2008S. Khan Summary Known genetic defects currently account for only a small proportion of patients meeting criteria for ,probable' or ,possible' common variable immunodeficiency (CVID). A 59-year-old male with a 12-year history of CVID on intravenous immunoglobulin (IVIG) is presented who developed bronchiectasis, cytopenias and malabsorption that are recognized complications of CVID. Work-up for his malabsorption suggested the possibility of Shwachman,Diamond syndrome, confirmed by mutation testing. With the identification of the molecular defect in Shwachman,Diamond syndrome (SDS), it is becoming clear that not all SDS patients have the prominent features of neutropenia or pancreatic malabsorption. A meta-analysis of published immunological defects in SDS suggests that four of 14 hypogammaglobulinaemic SDS patients meet criteria for ,possible' CVID. Mutations in the SBDS gene may therefore be the fifth identified molecular defect in CVID. [source] Assessing immune function in adult bronchiectasisCLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2006P. T. King Summary Bronchiectasis is characterized by chronic airway infection and damage and remains an important health problem. Recent literature has emphasized the role of host defence and immune deficiency in the pathogenesis of bronchiectasis, but there have been few studies of immune function in adult bronchiectasis. A comprehensive screen of immune function was conducted in 103 adult patients with bronchiectasis, encompassing full blood examinations, immunoglobulins and IgG isotypes, complement levels, lymphocyte subsets and neutrophil function. Full blood examinations were normal in this cohort, as were complement levels. Statistical analysis confirmed that a significant number of subjects had low levels of IgG3 (13 patients), B cell lymphocytes (six patients) and T helper cell lymphocytes (seven patients) when compared with controls (P < 0·05). The most common abnormality was found with testing of the neutrophil oxidative burst. All subjects had a normal neutrophil phagocytic function but 33 of the subjects had an oxidative burst that was below the normal range (P < 0001). Almost half the group (45 subjects) had abnormally low levels of one of these four parameters. The findings of low B cells, Th cells and oxidative burst in bronchiectasis are novel. The results emphasize the importance of immune function assessment for adult bronchiectasis. [source] Lung disease in ataxia-telangiectasiaACTA PAEDIATRICA, Issue 7 2007L Bott Abstract Ataxia-telangiectasia (AT) is a multi-systemic disease caused by mutational inactivation of the ATM gene. We report a retrospective study of lung disease in 15 patients. Patients and methods: A diagnosis of AT was made if the patient met the following criteria: neurological features and at least one the following: oculo-cutaneous telangiectasia, elevated serum ,-feto-protein level. Results: Recurrent sino-pulmonary infections were usually present in 11 of the cases and occurred during the first 2 years of life. Other lung injuries noted were bronchiectasis, obstruction and restriction of the airways, fibrosis, pneumothorax and haemoptysis. Eleven children had immunodeficiencies. Discussion: Recurrent sino-pulmonary manifestations precede neurological complications, but the severity of neuro-degeneration and pulmonary disease were not correlated. Pulmonary status was a prognosis factor. Immunodeficiency was the main, but not the only, aetiology for lung disease in AT. Conclusion: There is little dispute over the role of ATM in lung and respiratory epithelium. To reduce the morbidity associated with AT, there needs to be greater awareness of respiratory complications. Early management and monitoring lung function is necessary to minimize lung damage. [source] | |||||||||||||||||||||||||||||||