TL;DR: Hemoptysis is a common symptom with a good prognosis in most cases, however, patients exhibiting massive bleeding or those with lung malignancy and patients with bleeding diathesis had a poorer prognosis.
TL;DR: A detailed literature review of the genes which have been investigated as potential risk factors for chronic obstructive pulmonary disease is presented.
Abstract: Cigarette smoking is the major risk factor for chronic obstructive pulmonary disease (COPD). However, only a minority of cigarette smokers develop symptomatic disease. Studies of families and twins suggest that genetic factors also contribute to the development of COPD. We present a detailed literature review of the genes which have been investigated as potential risk factors for this disease. The only established genetic risk factor for COPD is homozygosity for the Z allele of the alpha1-antitrypsin gene. Heterozygotes for the Z allele may also be at increased risk. Other mutations affecting the structure of alpha1-antitrypsin or the regulation of gene expression have been identified as risk factors. Genes, including those for alpha1-antichymotrypsin, alpha2-macroglobulin, vitamin D-binding protein and blood group antigens, have also been associated with the development of COPD. Variants of the cystic fibrosis transmembrane regulator gene have been identified as risk factors for disseminated bronchiectasis. The genetic basis to chronic obstructive pulmonary disease has begun to be elucidated and it is likely that several genes will be implicated in the pathogenesis of this disease. The knowledge gained from such studies may also prove relevant to other inflammatory diseases.
TL;DR: 3He MRI is a promising new modality for the assessment of pulmonary ventilation and its abnormalities and additional studies are needed to determine its potential clinical role.
Abstract: The purpose of this study was to describe the 3He MRI findings of normal pulmonary ventilation in healthy volunteers and to evaluate abnormalities in patients with different lung diseases. Hyperpolarized 3He gas (300 ml, 3 x 10(5) Pa, polarized to 35-45% by optical pumping, provided in special glass cells) was inhaled by 8 healthy volunteers and 10 patients with different lung diseases. Imaging was performed with a three-dimensional fast low-angle shot (FLASH) sequence (TR = 11.8 msec; TE = 5 msec; transmitter amplitude, 5-8 V; corresponding flip angle, < 5 degrees) in a single breath-hold (22-42 seconds). Clinical and radiological examinations were available for correlation. The studies were performed successfully in eight of eight volunteers and in 8 of 10 patients. The lung parenchyma of volunteers with normal ventilatory function exhibited rather homogeneous intermediate to high signal, whereas patients with chronic obstructive lung disease or bronchiectasis presented with severe signal inhomogeneities with patchy or wedge-shaped defects. The mass effect of bronchogenic carcinoma, chronic empyema, lymphadenopathy, or pleural effusion caused large signal defects, representing the lesion and adjacent hypoventilation, the extent of which had not been presumed from chest x-ray or CT. 3He MRI is a promising new modality for the assessment of pulmonary ventilation and its abnormalities. Additional studies are needed to determine its potential clinical role.
TL;DR: Pulmonary nocardiosis is difficult to diagnose, diagnosis is frequently delayed and a high level of suspicion is, thus, required in patients with underlying diseases or chronic corticosteroid therapy; there is frequent dissemination and high mortality; and antimicrobial combinations with proven synergy, such as imipenem and amikacin, are recommended for initial therapy.
Abstract: Pulmonary nocardiosis (PN) is an infrequent and severe infection due to Nocardia spp., microorganisms that may behave both as opportunists and as primary pathogens. The aim of this study and review was to evaluate the clinical features, evolution and prognostic factors of PN. The study group comprised 10 consecutive patients with pulmonary nocardiosis acquired in a community setting, diagnosed and followed in a tertiary teaching hospital. Chronic obstructive pulmonary disease (COPD), neoplastic disease and human immunodeficiency virus (HIV) infection were the most frequent predisposing factors. Four patients were receiving corticosteroid treatment. Clinical course was chronic and diagnosis was delayed 3 weeks or more in seven of the patients. Lobar or multilobar condensation was the most frequent radiographic pattern. Antimicrobial susceptibility testing showed: 100% sensitivity for amikacin; 83% for imipenem; 71% for cefotaxime; and 71% for trimethoprim-sulphamethoxazole. The disease remained localized in the lung in five cases, with a trend toward chronicity in one with bronchiectasis. In the other five, the disease disseminated, affecting subcutaneous tissue, the central nervous system and the kidney. Three patients died, one with disseminated disease and two who were receiving corticosteroid therapy. The following conclusions were reached: 1) pulmonary nocardiosis is difficult to diagnose, diagnosis is frequently delayed and a high level of suspicion is, thus, required in patients with underlying diseases or chronic corticosteroid therapy; 2) there is frequent dissemination and high mortality; and 3) antimicrobial combinations with proven synergy, such as imipenem and amikacin, are recommended for initial therapy.
TL;DR: The fact that the prognosis for bronchiectatic patients is poorer than that for asthmatics points to a continued need for focused care and follow-up, particularly in the presence of additional illnesses.
Abstract: The prognosis and risk factors for bronchiectasis are at present poorly known. The aim of this study was to examine the long-term prognosis and cause of death in this disease. The National Hospital Discharge Register was used to search for patients aged 35-74 yrs, with newly-diagnosed bronchiectasis in the period 1982-1986. Each of the 842 patients identified was matched with an asthmatic patient and a patient with chronic obstructive pulmonary disease (COPD), who were of the same age and sex and who had been treated in hospital at the same time. The use of hospital services by these subjects was examined up to the end of 1992, and mortality to the end of 1993. The prognosis for the bronchiectatic patients treated in hospital was better than that for the COPD patients but poorer than that for the asthmatics; the risk of death being 1.25 (95% confidence interval (95% CI) 1.15-1.36) for the COPD patients and 0.79 (95% CI 0.71-0.87) for the asthmatics, relative to the bronchiectatic patients. Bronchiectasis was the main cause of death in 13% of bronchiectatic patients, the risk of death being increased by a factor of 1.21 in the presence of asthma as the main secondary diagnosis, by 1.31 with COPD, by 1.35 with tuberculosis and its sequelae, and by 1.32 with some other secondary diagnosis, as compared with cases for which no secondary diagnosis was indicated. The fact that the prognosis for bronchiectatic patients is poorer than that for asthmatics points to a continued need for focused care and follow-up, particularly in the presence of additional illnesses.
TL;DR: High-resolution CT of adult patients with cystic fibrosis during acute exacerbations with asymptomatic patients with CF was compared to evaluate reversibility of HRCT abnormalities after exacerbations, and to correlate HRCT with clinical parameters.
Abstract: The aims of this study were threefold: to compare high-resolution CT (HRCT) of adult patients with cystic fibrosis (CF) during acute exacerbations with asymptomatic patients with CF, to evaluate reversibility of HRCT abnormalities after exacerbations, and to correlate HRCT with clinical parameters.Nineteen symptomatic and eight asymptomatic patients were prospectively evaluated by HRCT and pulmonary function tests (PFTs). Symptomatic patients were reassessed 2 weeks after the exacerbation. Studies were independently reviewed by two radiologists using a modified Bhalla scoring system, noting the presence, extent, and severity of bronchiectasis, peribronchial thickening, mucus plugging, and atelectasis or consolidation. Modifications to the Bhalla system included evaluation of the presence and profusion of centrilobular nodules and air-fluid levels within bronchiectatic cavities. The highest possible score was 24 points. Higher scores indicated greater severity. Mosaic perfusion was noted but not included i...
TL;DR: Low mucus salinity contributes to its retention in bronchiectasis due to cystic fibrosis and other causes, probably by affecting its rheology, and therapies that increase the osmolality of lung mucus might benefit patients with mucus retention.
Abstract: Mucus retention in the lungs is an important feature of several respiratory diseases (Regnis, J.A., M. Robinson, D.L. Bailey, P. Cook, P. Hooper, H.K. Chan, I. Gonda, G. Bautovich, and P.T.P. Bye. 1994. Am. J. Respir. Crit. Care Med. 150:66-71 and Currie, D.C., D. Pavia, J.E. Agnew, M.T. Lopez-Vidriero, P.D. Diamond, P.J. Cole, and S.W. Clarke. 1987. Thorax. 42:126-130). On the mucus-depleted bovine trachea, the ciliary transport rate of sputum from patients with cystic fibrosis and bronchiectasis of other causes was slow, but the rate was doubled by increasing the sodium chloride content by 90 mM. Increasing the sputum osmolality by inspissation or by the addition of nonelectrolytes had a similar effect. The viscoelasticity of sputum, but not the bovine ciliary beat frequency, was markedly saline dependent over the pathophysiological range. This suggests that low mucus salinity, not (as is generally assumed) its under-hydration, contributes to its retention in bronchiectasis due to cystic fibrosis and other causes, probably by affecting its rheology. It also indicates how the genetic defect in cystic fibrosis might lead to impaired mucus clearance. Therapies that increase the osmolality of lung mucus might benefit patients with mucus retention.
TL;DR: Bilateral lobar transplantation from a single donor lung is possible in small adults or children when there is a large size discrepancy with the donor, and may help resolve the problem of donor availability in the pediatric population.
TL;DR: It is shown that the bronchial wall was more significantly thickened in BA but that it did not correlate well with the clinical features, lung functions and PC20M, which suggested the possibility that HRCT can be used for the differentiation of BA from COPD or healthy controls.
Abstract: The high-resolution computed tomography (HRCT) studies for bronchial asthma (BA) have revealed abnormal radiologic findings such as bronchial wall thickening, bronchiectasis, emphysema and mosaic pattern of lung attenuation. But the clinical significance of these findings are not yet clarified. In this study, we quantified the bronchial wall thickness and evaluated HRCT features in 57 BA subjects (338 bronchi) who had precipitating factors of irreversible airway remodeling, 19 COPD subjects (70 bronchi) and 10 healthy subjects (23 bronchi). Then we correlated HRCT findings with the clinical features, pulmonary functions and methacholine PC20 (PC20M) and studied their clinical significance. The bronchial wall for BA was about 1.48 mm thicker than that for COPD and about 2.34 mm thicker than for healthy controls (p < 0.0001, respectively). But the individual mean ratio of bronchial wall thickness to luminal diameter (BWT/LD) in asthmatics did not correlate with the clinical features, lung functions and PC20M. Abnormal HRCT findings, such as bronchiectasis (17.5%), emphysema (5.3%) and mosaic pattern of lung attenuation (17.5%) were found in BA. These findings were more common in BA with moderate to severe airflow limitation (FEV1 < 80%, p < 0.05) and patients with these changes had a more prolonged history of asthma (p < 0.05). PC20M was higher in BA with these abnormal changes (p < 0.001) but these patients' FEV1 (p < 0.05), FEF25-75 (p < 0.05) and specific airway conductance (p < 0.05) were lower than those having BA without such findings. In this study we showed that the bronchial wall was more significantly thickened in BA but that it did not correlate well with the clinical features, lung functions and PC20M. Additionally, patients having BA with abnormal airway and air space HRCT findings had a prolonged history of asthmatic symptoms, loss of lung functions and decreased bronchial hyperresponsiveness. These results suggested the possibility that HRCT can be used for the differentiation of BA from COPD or healthy controls. Furthermore, patients having BA with abnormal HRCT changes demonstrate poor lung function and less hyperreactive bronchi than those without. We concluded that HRCT may be useful for the prognosis and treatment of bronchial asthma cases who have the precipitating factors of irreversible airway remodelling.
TL;DR: It is concluded that there is a significant occurrence of bronchiectasis in children with AIDS and pulmonary disease, especially in children developing LIP, recurrent pneumonia and unresolved pneumonia, and CD4+ T-cell counts < 100 cells per cubic millimeter.
TL;DR: Computed tomography of the thorax in patients with ABPA provides a sensitive method for the assessment of bronchial, parenchymal and pleural abnormalities, and should constitute a part of the diagnostic work of the disease.
TL;DR: Patients with bronchiectasis infected by P aeruginosa have more extensive and severe bronchial wall thickening and dilatation and more extensive decreased attenuation was seen in the Pa +ve group, robust on multivariate analysis.
Abstract: BACKGROUND: Pseudomonas aeruginosa commonly infects the airways of patients with bronchiectasis. A study was undertaken to examine the relationship between infection of the airways with this pathogen, the morphological pattern of bronchiectasis on thin section computed tomographic (CT) scanning, symptom duration, smoking habits of the patients, and the presence of airflow obstruction. METHODS: Thin section CT scans of 22 adult patients with bronchiectasis and concurrent sputum infected by P aeruginosa (Pa +ve) and those of 45 randomly selected patients not infected by P aeruginosa (Pa -ve) were analysed independently by two thoracic radiologists. Patients with cystic fibrosis were excluded. Each scan was scored at a lobar level for extent of bronchiectasis, severity of bronchial wall thickening and dilatation, predominant pattern of bronchiectasis, presence of mucus plugging, and degree of decreased attenuation of the lung parenchyma. RESULTS: The Pa +ve group had more extensive bronchiectasis and a greater degree of bronchial wall thickening and dilatation on the CT scan than the Pa -ve group; more extensive decreased attenuation was seen in the Pa +ve group. These findings were robust on multivariate analysis; decreased attenuation was also independently related to the duration of sputum production. CONCLUSION: Patients with bronchiectasis infected by P aeruginosa have more extensive and severe bronchiectasis on thin section CT scanning than those without P aeruginosa infection. The bronchi and small airways are both involved, reflecting the end result of complex interactions between host airways and the numerous virulence factors produced by P aeruginosa.
TL;DR: The postoperative complication rate of pneumonectomy for destroyed lung is acceptably low, however, it is increased by preoperative empyema, tuberculosis, and right-sided resection.
TL;DR: The preferred drug for the treatment of Pasteurella infections is penicillin, and doxycycline is highly effective against P multocida.
Abstract: Pasteurella multocida, a gram-negative coccobacillus which colonizes the nasopharynx and gastrointestinal tract of many animals, is a well known cause of soft tissue infection after animal bites. Human infection can also occur after non-bite animal exposure, usually via inhalation of contaminated secretions. The respiratory tract is the second most common site of Pasteurella infection after soft tissue infection. Most patients with Pasteurella pulmonary infection are elderly with underlying lung disease, either COPD, bronchiectasis, or malignancy. The spectrum of disease includes pneumonia, tracheobronchitis, lung abscess, and empyema. Clinical features of Pasteurella respiratory tract infections are indistinguishable from other pathogens. A history of cat or dog exposure should alert the clinician to consider Pasteurella as a potential pulmonary pathogen in an elderly patient with chronic lung disease. The preferred drug for the treatment of Pasteurella infections is penicillin. Alternately, doxycycline is highly effective against P multocida.
TL;DR: In most cases, thin-section CT allows reliable distinction of patients with cylindrical bronchiectasis from healthy subjects.
Abstract: The aim of this study was to determine the frequency of diagnostic findings of bronchiectasis on thin-section CT and the usefulness of those findings in distinguishing patients with cylindrical bronchiectasis from healthy subjects.The study was retrospective and included 26 healthy adults, 10 consecutive patients with surgically proven cylindrical bronchiectasis, and 49 patients who had been prospectively diagnosed at three institutions as having cylindrical bronchiectasis. All patients had 1.0- to 1.5-mm-collimation CT scans obtained at 10-mm intervals through the chest. The CT scans were independently reviewed in random order by two chest radiologists.Findings in patients with bronchiectasis that were not seen by either observer in any of the healthy subjects included visualization of a bronchus within 1 cm of the costal pleura and visualization of a bronchus abutting the mediastinal pleura. The two observers saw these findings on 96 (81%) and 63 (53%) of 118 CT scans in the 59 patients with bronchiecta...
TL;DR: In this article, the radiologic, histologic, and clinical findings of the spectrum of eosinophilic lung disease from the previous reports and their experiences are reviewed and compared.
Abstract: Purpose Eosinophilic lung disease includes various disease entities. Each disease manifests different radiologic findings. The purpose of this review is to present the radiologic findings of the spectrum of eosinophilic lung disease. Method We reviewed the radiologic, histologic, and clinical findings of the spectrum of eosinophilic lung disease from the previous reports and our experiences. Results Simple pulmonary eosinophilia is characterized by transient and migrating opacities on chest radiography. Acute eosinophilic pneumonia is characterized by acute clinical symptoms and signs and rapid changes of radiographic diffuse reticular lesions. Chronic eosinophilic pneumonia, with more prolonged symptom duration, history of asthma, occurrence of relapse, and radiologic features of subpleural consolidation, can be differentiated from acute eosinophilic pneumonia. Allergic bronchopulmonary aspergillosis presents with bilateral central bronchiectasis with or without mucoid impaction. Although these diseases show specific radiographic findings, some show overlapping radiographic features. High-resolution CT enables characterization of parenchymal lesions further by showing internal and marginal features and the exact extent of the lesions. Extrapulmonary organs are involved in Churg-Strauss syndrome and idiopathic hypereosinophilic syndrome. Asthma is associated with Churg-Strauss syndrome, allergic bronchopulmonary aspergillosis, chronic eosinophilic pneumonia, and bronchocentric granulomatosis. Conclusion Integration of clinical, laboratory, and radiologic findings enables initial and differential diagnoses of various eosinophilic lung diseases.
TL;DR: In this paper, the authors evaluated the effect of specific risk factors on postoperative complications of pneumonectomy for destroyed lung, including preoperative empyema, tuberculosis, and right-sided resection.
Abstract: Background. The presence of specific risk factors can increase the postoperative complication rate of pneumonectomy for destroyed lung. Methods. Our experience in 118 consecutive patients who underwent pneumonectomy for destroyed lung over a 10-year period was retrospectively analyzed to evaluate the effect of specific risk factors on postoperative complications. The significance of tuberculosis, right pneumonectomy, preoperative empyema, and duration of illness longer than 36 months was examined by univariate analyses. Results. The most common underlying diseases were nonspecific bronchiectasis (n = 52) and tuberculosis (n = 43). Sixty-day or in-hospital morbidity and mortality rates were 11.9% and 5.9%, respectively. The combined morbidity and mortality rate was significantly higher in patients with preoperative empyema (p < 0.003), tuberculosis (p < 0.03), and right pneumonectomy (p < 0.03). The prevalence of bronchopleural fistula was higher in patients with preoperative empyema (p < 0.02) and patients with tuberculosis (p < 0.03). Conclusions. The postoperative complication rate of pneumonectomy for destroyed lung is acceptably low. However, it is increased by preoperative empyema, tuberculosis, and right-sided resection.
TL;DR: Correlation with clinical factors suggests a higher risk group needing more aggressive management, and HRCT is a useful adjunct to demonstrate the extent and severity of lung disease at diagnosis and during therapy.
Abstract: Objective. To assess the value of high-resolution computed tomography (HRCT) in determining the extent and significance of lung disease in children with antibody deficiency states. Materials and methods. Seventy HRCT scans performed on 37 children with various antibody deficiency disorders over a 5-year period were retrospectively scored using a previously described demerit scoring system (0–25 with 0 = worst, 25 = best). Points are subtracted from 25 with increasing severity of disease. The potential correlations between CT scores and clinical factors, including age at diagnosis, age at CT, type of immunoglobulin deficiency, length of respiratory symptoms before diagnosis, number of pneumonias before diagnosis, type, length and success of therapy, patient compliance and pulmonary function tests (PFTs), were assessed. Results. Of the 37 children, a demonstrated 22 abnormal scans (CT score ≤ 22). All nine demonstrated bronchiectasis with a lower lobe and right middle lobe predominance. Statistically significant correlations were seen between severity of lung disease (CT score) and length of respiratory symptoms before diagnosis (p = 0.01), success of therapy (P = 0.001) and PFTs (P = 0.0008). Of seven children who were followed with repeated scans, 4 of the 7 demonstrated CT scores which improved on high-dose intravenous immunoglobulin replacement therapy. Conclusion. HRCT is a useful adjunct to demonstrate the extent and severity of lung disease at diagnosis and during therapy. Correlation with clinical factors suggests a higher risk group needing more aggressive management.
TL;DR: Three patients with acute chest syndrome and a protracted clinical course due to plastic bronchitis are described and the cases of three children with sickle cell disease and ACS unresponsive to traditional medical management, who required bronchoscopy for the removal of fibromucinous casts are reported.
Abstract: Plastic bronchitis is reported as a rare entity characterized by the formation of branching mucoid bronchial casts.1,2 These endobronchial casts of rubber-like consistency are organized aggregates of mucofibrinous substrate mixed with inflammatory cells. They may become firmly wedged and occlude the tracheobronchial tree at many levels.3 Obstruction involving both main stem bronchi is rare.4 Bowen et al2 reported that before 1985 there had been approximately 40 known cases of plastic bronchitis in children. Subsequently, in 1989, Perez-Soler5 reported an additional 72 cases of plastic bronchitis collected from his own personal experience.
Galen described a similar condition in the second century AD.1,3,6 Although the condition is usually a sign of an underlying bronchopulmonary disorder such as asthma1-46-8 or allergy,1-3 the cause of cast formation remains an enigma in other cases. Respiratory infections with Corynebacterium diphtheria , Haemophilus influenzae , Klebsiella pneumoniae , Mycobacterium tuberculosis , and viral illnesses have been associated with the formation of bronchial casts.8 Other processes associated with bronchial cast formation include bronchiectasis,3,8cystic fibrosis,3,4,6,8 and as a complication after the Fontan procedure.9
To our knowledge this entity has not been described in pediatric patients with sickle cell disease. In this report, we describe three patients with acute chest syndrome (ACS) and a protracted clinical course due to plastic bronchitis.
The cases of three children with sickle cell disease and ACS unresponsive to traditional medical management, who required bronchoscopy for the removal of fibromucinous casts are reported. The three patients varied in age and symptomatology at presentation (Table 1). Significant past medical history included previous episodes of ACS in all three patients and asthma in two of the patients. Each patient …
TL;DR: Utilizing a diagnostic protocol based on the anatomy of the cough reflex, Irwin and co-workers were able to diagnose and treat successfully the majority of patients who presented with previously unexplained chronic cough.
TL;DR: Although antibiotic therapy is beneficial for the management of bacterial respiratory tract infections in CF patients, gene therapy may provide the ultimate cure as new treatment strategies will emerge as the pathogenesis of cystic fibrosis is better elucidated.
Abstract: Objective. To describe the current understanding of the pathogenesis of infections in cystic fibrosis (CF). The key element in the pathogenesis of infections in CF is the abnormal mucin resulting from a defective chloride channel. Abnormal mucin and the lack of hydration of respiratory secretions entraps bacteria, permitting colonization and subsequent infection. Normal physiologic insults, such as microaspiration, and pollution evoke mucin secretion with bacteria-mucin aggregates causing infection of small airways. Microcolonies of pulmotropic bacteria (i.e. Haemophilus and Staphylococcus) in the trapped mucin cause mucosal injury which predisposes the patient to Pseudomonas aeruginosa infection. Eventually there is obstruction of medium-sized and small airways by inflammatory exudate and mucus. Ultimately these chronic endobronchial bacterial infections cause significant loss of pulmonary function with morbidity and a decrease in the life span of patients with CF. Although antibiotic therapy is beneficial for the management of bacterial respiratory tract infections in CF patients, gene therapy may provide the ultimate cure. Conclusion. New treatment strategies will emerge as the pathogenesis of cystic fibrosis is better elucidated.
TL;DR: The serum HGF concentration in patients with digital clubbing was significantly higher when compared to that of lung cancer patients without digitalClubbing, suggesting that HGF may play a role in the formation of digitalclubbing.
Abstract: The cause of digital clubbing is unknown. Hepatocyte growth factor (HGF) is a pleotrophic factor which has various biological effects. We measured serum HGF in 12 patients with digital clubbing; the underlying diseases of these patients were: lung cancer, 2; cystic fibrosis, 2; idiopathic pulmonary fibrosis, 3; lung cancer with idiopathic pulmonary fibrosis, 1; chronic hepatitis, 1; interstitial pneumonia with collagen disease, 2; and bronchiectasis, 1; nine hundred and fifty-seven normal volunteers and 17 lung cancer patients without clubbing served as the control. As a result, the serum HGF concentration in patients with digital clubbing (0.47 +/- 0.29 ng/ml) was significantly higher when compared to that of lung cancer patients without digital clubbing (0.15 +/- 0.04, p < 0.01). Therefore, we suggest that HGF may play a role in the formation of digital clubbing.
TL;DR: Morphologic changes depicted by HRCT correlate with decreased lung pefusion on SPECT, however, HRCT changes accurately predict regional lung function only in the most normal and severely diseased lung zones, which constituted 106 of 120 zones.
Abstract: Objective. To evaluate the relationship between morphologic findings seen on high-resolution computed tomography (HRCT) of the lung and regional lung perfusion depicted on single photon-emission computed tomography (SPECT) pulmonary perfusion imaging in patients with cystic fibrosis. Materials and methods. Ten HRCT and 10 technetium-99 m macroaggregated albumin SPECT pulmonary perfusion imaging studies were performed on eight young adult patients who were considered to be clinically well and have mild to moderate cystic fibrosis. HRCT scans of the chest were evaluated using a CT scoring system which included grading of bronchiectasis, peribronchial thickening, hyperlucency, bullae, collapse/consolidation, and mucus plugging. Each lung was divided into six anatomic zones which were independently scored. A lung perfusion score (between 0 and 100), reflecting the percentage of compromised lung, was estimated for each zone. Axial lung perfusion SPECT images were matched anatomically to HRCT images. Lung function was considered compromised when the counts per pixel were less than 25 % of the count level seen in an area of the same patient's lung which was judged to be normal. Results. There was a statistically significant relationship (P = 0.0001) between HRCT total scores and SPECT lung perfusion scores as well as between hyperlucency scores by HRCT and the SPECT lung perfusion scores. However, the HRCT score was a poor predictor of the lung perfusion score in zones with intermediate HRCT scores, which constituted 106 of 120 zones. Conclusion. Morphologic changes depicted by HRCT correlate with decreased lung pefusion on SPECT. However, HRCT changes accurately predict regional lung function only in the most normal and severely diseased lung zones.
TL;DR: Bronchiectasis may be an under-recognised manifestation of chronic graft versus host disease of the lung in patients after allogeneic bone marrow transplantation for haematological malignancy.
Abstract: Two patients are described with clinical and radiographic bronchiectasis which occurred after allogeneic bone marrow transplantation for haematological malignancy. Both had evidence of chronic graft versus host disease in other organs. Increased immunosuppression with corticosteroids resulted in clinical response, although both patients persisted with chronic mucopurulent sputum production and one had progressive airflow obstruction. Bronchiectasis may be an under-recognised manifestation of chronic graft versus host disease of the lung.
TL;DR: The association of elevated levels of BAL neutrophils and decreased diffusing capacity with bronchial dilatation suggests that the neutrophil may be associated with airway damage and lung destruction in patients who are infected with HIV.
Abstract: Symptoms of airway disease occur in patients infected with HIV, and bronchiectasis has been reported in patients with AIDS. We evaluated thin-section thoracic CT scans in HIV-positive individuals for bronchial dilatation and correlated imaging findings with pulmonary function abnormalities and findings at bronchoalveolar lavage (BAL).Sixty-one subjects, 50 of whom were HIV-positive and 11 of whom were HIV-negative, underwent thin-section CT, BAL, and pulmonary function tests. Two radiologists evaluated the CT scans on two separate occasions for bronchial dilatation in each lobe. BAL and pulmonary function test data in the subjects with bronchial dilatation were compared with such data in subjects with normal bronchi seen on CT scans.Eighteen of the 50 HIV-positive subjects and none of the HIV-negative subjects had bronchial dilatation revealed by CT. Subjects with bronchial dilatation revealed by CT had significantly higher BAL neutrophil counts (p = .014) and significantly lower diffusing capacity (p = ....
TL;DR: Asthma is common in hospitalized bronchiectasis patients and appears to be consequent upon this disease, according to data from the Finnish Hospital Discharge Register recorded in 1972–92.
Abstract: In view of the conflicting notions of the relationship between bronchiectasis and asthma, we have analysed the use of hospital services by bronchiectasis and asthma patients and evaluated the links between these diseases, employing data from the Finnish Hospital Discharge Register of over 21 million hospitalization periods recorded in 1972-92. We conclude that asthma is common in hospitalized bronchiectasis patients and appears to be consequent upon this disease.
TL;DR: HR-CT is the most sensitive radiographic method with which to image emphysema and has been shown to be more sensitive than pulmonary function tests or TL,CO [15‐18].
Abstract: Computed tomography (CT) scan has been used in the diagnosis of airways diseases [1] because of the high degree of anatomical detail provided. Most abnormalities, such as bronchiectasis and emphysema, seen on a CT scan have been anatomically validated, but their clinical significance is not always fully understood. Highresolution CT (HR-CT) has been used to study asthmatics [2‐8]. It has been consistently observed that more abnormalities related to fixed airways remodelling, such as bronchial dilatation and bronchiectasis are observed in asthmatic subjects than in normal subjects, although the clinical consequences of these findings are not clear. HRCT scans have also been used to measure the internal size of the airways at baseline, during challenge or after bronchodilatation [9‐12]. However, there is no data on lung density in patients with acute and chronic asthma. This was the aim of the paper published in the present issue of the European Respiratory Journal [13]. The relationship between asthma and emphysema was suggested in 1952 by ROYLE [14] who found emphysema in patients with severe asthma. However, the study was flawed since chest radiographs failed to recognize mild emphysema [15] and most of the patients studied were current or exsmokers. Importance of CT scan in the diagnosis of emphysema Pulmonary emphysema is a pathological diagnosis, but correlations between results of lung function tests and the extent of emphysema are poor. Lung function tests are also nonspecific for emphysema, and alteration of transfer factor of the lung for carbon monoxide (TL,CO) may be due to cardiogenic oedema or interstitial disease. The diagnosis of pulmonary emphysema can be made with relative confidence on the basis of clinical and radiological criteria. Despite the fact that very mild emphysema goes undetected and the severity of the disease may be underestimated, HR-CT is the most sensitive radiographic method with which to image emphysema and has been shown to be more sensitive than pulmonary function tests or TL,CO [15‐18]. Many studies have demonstrated significant correlations between CT scan data and pathological observations of lungs obtained at autopsy or after surgery for both centrilobular and panacinar emphysema [19‐23]. However, occasionally CT scans give false-positive findings and the criteria used
TL;DR: This case confirms the occurrence of neuro endocrine proliferations, in the setting of bronchiectasis, ranging from neuroendocrine cell hyperplasia to carcinoids, and suggests tumourlets as an appropriate model for neuroendocrin lung tumour genesis.
Abstract: A 66 year old woman underwent a left lower lobectomy for bronchiectasis. Histology revealed the presence of multiple endocrine lesions, such as neuroendocrine cell hyperplasia, tumourlets and microcarcinoids, which were widespread in bronchi, bronchioles and alveolar tissue. This case confirms the occurrence of neuroendocrine proliferations, in the setting of bronchiectasis, ranging from neuroendocrine cell hyperplasia to carcinoids, and suggests tumourlets as an appropriate model for neuroendocrine lung tumour genesis.
TL;DR: From scanty to copious, nebulised terbutaline also increased the radioaerosol clearance rate in patients with bronchiectasis.
Abstract: from scanty to copious. Patients with bronchiectasis have a number of other symptoms ‐ pectorated fluid/mucus by a small amount, similar to the weight of the nebulised solution some are breathless with impaired lung function, usually predominantly an obstructive (only a 25% increase in the expectorated weight compared with physiotherapy alone). Interestventilatory defect, and asthma is frequent with an incidence of 28% (10 of 36) and 39% (nine ingly, nebulised terbutaline also increased the radioaerosol clearance rate in patients with