bluuebunny/crossref_metadata_2025_split huggingface dataset:
Marrow Failure
Authors: D’Andrea Alan D. , Dahl Niklas , Guinan Eva C. , Shimamura Akiko 
Published: August 2003

AbstractThis chapter describes the clinical presentation and molecular basis of two inherited bone marrow failure syndromes, Fanconi anemia (FA), and Diamond-Blackfan anemia (DBA). It also provides an update on diagnostic and therapeutic approaches to bone marrow failure of all types (inherited and acquired) in pediatric patients.In Section I, Dr. Alan D’Andrea reviews the wide range of clinical manifestations of Fanconi anemia. Significant advances have been made in understanding the molecular pathogenesis of FA. On the basis of these advances, new diagnostic assays and treatment options are now available.In Section II, Dr. Niklas Dahl examines the clinical features and molecular pathogenesis of Diamond-Blackfan anemia. The possible links between the RPS19 gene (DBA gene) and the erythropoiesis defect are considered.In Section III, Drs. Eva Guinan and Akiko Shimamura provide an algorithm for the diagnostic evaluation and treatment of children with inherited or acquired aplastic anemia. Through the presentation of a case study of a pediatric patient with bone marrow failure, he provides an overview of the newest tests and treatment options.
Acute Myeloid Leukemia
Authors: Giles Francis J. , Keating Armand , Goldstone Anthony H. , Avivi Irit , Willman Cheryl L. , Kantarjian Hagop M. 
Published: August 2003

AbstractIn this chapter, Drs. Keating and Willman review recent advances in our understanding of the pathophysiology of acute myeloid leukemia (AML) and allied conditions, including the advanced myelodysplastic syndromes (MDS), while Drs. Goldstone, Avivi, Giles, and Kantarjian focus on therapeutic data with an emphasis on current patient care and future research studies.In Section I, Dr. Armand Keating reviews the role of the hematopoietic microenvironment in the initiation and progression of leukemia. He also discusses recent data on the stromal, or nonhematopoietic, marrow mesenchymal cell population and its possible role in AML.In Section II, Drs. Anthony Goldstone and Irit Avivi review the current role of stem cell transplantation as therapy for AML and MDS. They focus on data generated on recent Medical Research Council studies and promising investigation approaches.In Section III, Dr. Cheryl Willman reviews the current role of molecular genetics and gene expression analysis as tools to assist in AML disease classification systems, modeling of gene expression profiles associated with response or resistance to various interventions, and identifying novel therapeutic targets.In Section IV, Drs. Hagop Kantarjian and Francis Giles review some promising agents and strategies under investigation in the therapy of AML and MDS with an emphasis on novel delivery systems for cytotoxic therapy and on targeted biologic agents.
Incidence of probable occupational asthma and changes in airway calibre and responsiveness in apprentice welders
Authors: El-Zein M. , Malo J-L. , Infante-Rivard C. , Gautrin D. 
Published: August 2003

The majority of cross-sectional studies have shown a higher prevalence of ventilatory impairment in welders while only few longitudinal studies were able to detect chronic effects on spirometry or bronchial responsiveness.The aim of the study was to determine the incidence of probable occupational asthma (OA), bronchial obstruction and hyperresponsiveness among 286 students entering an apprenticeship programme in the welding profession.This epidemiological prospective cohort study consisted of a baseline assessment survey and two follow-up assessments. A respiratory symptom questionnaire was administered at each visit. Spirometry and methacholine bronchial challenge test results, conducted once prior to onset of exposure and later after an average of 15 months of apprenticeship, were available for 194 subjects.The incidence of probable OA was ∼3% (6 of 194). The incidence of bronchial hyperresponsiveness, defined as a ≥3.2‐fold decrease in the provocative concentration causing a 20% fall in the forced expiratory volume in one second from baseline to the end of the study was 11.9%. A statistically significant difference was found between the baseline and end of study for the lung function values. In particular, the forced expiratory volume per cent predicted had significantly dropped by 8.4% on average.The significance of these early pulmonary function changes in relation to possible chronic effects of exposure to welding fumes and gases remains to be explored.
Diagnostic implications of telomerase activity in pleural effusions
Authors: Dikmen G. , Dikmen E. , Kara M. , Şahin E. , Doğan P. , Özdemir N. 
Published: August 2003

The aim of the present study was to investigate the diagnostic efficacy of telomerase activity for discrimination of malignant and benign pleural effusions.Pleural effusions were collected from 109 consecutive patients in whom the diagnosis was confirmed with cytological and/or histological examinations. Cytological samples were classified as malignant (n=63) and benign (n=46). Telomerase activity was determined with the polymerase chain reaction-based telomeric repeat amplification protocol assay.Telomerase activity was detected in 52 (82.5%) and nine (19.6%) samples from the malignant and benign groups, respectively, which was a significant difference. The sensitivity rate of cytological examination when combined with telomerase activity (92.1%) was significantly greater than that of cytological examination alone (53.9%). The sensitivity and specificity of telomerase activity were 82.5 and 80.4%, respectively. Diagnostic accuracy of telomerase activity was 81.6%.Telomerase activity is a highly sensitive diagnostic biomarker for malignancy and may be used as an adjunct to cytological findings in determining malignant pleural effusions.
Trends in asbestos body counts in bronchoalveolar lavage fluid over two decades
Authors: Dumortier P. , Thimpont J. , de Maertelaer V. , De Vuyst P. 
Published: August 2003

As in most western countries, the use of asbestos has decreased in Belgium since the mid 1970's. Successive regulations have lowered the permissible levels of exposure and prohibited the use of various asbestos products.In order to assess the impact of these prevention measures on the pulmonary fibre burden of asbestos-exposed patients, the bronchoalveolar lavage fluid (BALF) asbestos body (AB) analysis database of the authors' laboratory was reviewed for the period 1983–2000.A total of 4,772 cases were considered, of which 95% were males. AB concentration exceeded 1 AB·mL BALF−1in 36.1%. There was essentially no change in the mean concentration of AB over the period evaluated. However, the concentrations in individuals with very high levels decreased over time. This was associated with a shift in exposure categories from primary asbestos workers to those exposed during handling of asbestos-containing materials or to asbestos in place in buildings or industrial sites.This is consistent with epidemiological data indicating that the number of severe cases of asbestosis caused by very high cumulated doses decreases but that benign pleural diseases and mesothelioma remain the most frequent asbestos-related diseases. Past prevention measures are not expected to have a measurable influence on the incidence of mesothelioma in the near future.
Spirometric reference values in a large Middle Eastern population
Authors: Golshan M. , Nematbakhsh M. , Amra B. , Crapo R.O. 
Published: August 2003

Ethnic differences in pulmonary function have been frequently reported. The purposes of this study were to derive equations for the prediction of normative spirometry values for a large population of Persians in Isfahan and compare them to reference values from a White Euro-USA population.Spirometry measurements were obtained from 4,341 randomly selected healthy nonsmoker subjects in Isfahan, Iran, utilising American Thoracic Society guidelines and a vigorous quality assurance program. Measured data from 3,213 subjects were analysed using multiple regression techniques to derive prediction equations for spirometric variables; the remaining 1,128 subjects were used as a control group to test the validity of the derived equations. In addition, predicted values were compared with values derived from recently published equations for the USA.Derived prediction equations showed good performance for most spirometric parameters. Compared with USA Whites, adult Persians have minimally lower forced vital capacities, while the values for children are close to USA Whites.In comparison with reference equations based on European or USA populations, local reference values are more biologically and technically suitable for the interpretation of spirometric data from Iranian populations.
Inflammatory markers in cystic fibrosis patients with transmissiblePseudomonas aeruginosa
Authors: Jones A.M. , Martin L. , Bright-Thomas R.J. , Dodd M.E. , McDowell A , Moffitt K.L. , Elborn J.S. , Webb A.K. 
Published: August 2003

ChronicPseudomonas aeruginosainfection in cystic fibrosis (CF) leads to a damaging host inflammatory response. There are an increasing number of reports ofP. aeruginosacross-infection at CF centres. The clinical significance of acquisition of a transmissible strain for patients who already harbourP. aeruginosais unclear. In this study, levels of inflammatory markers in clinically stable adult CF patients who harbour transmissible and sporadic strains ofP. aeruginosahave been compared.Patients with CF and chronicP. aeruginosainfection were grouped into those who harbour a transmissibleP. aeruginosaand those who harbour their own sporadic strains. Total white cell and differential counts, sputum neutrophil elastase (NE), interleukin (IL)‐8, tumour necrosis factor (TNF)‐α, plasma IL‐6 and NE/α1‐antitrypsin complexes, serum C‐reactive protein, and urine TNF receptor 1 were all measured in clinically stable patients 4–6 weeks following completion of intravenous antibiotic therapy.The two groups (both n=20) were well matched for per cent predicted forced expiratory volume in one second, per cent predicted forced vital capacity and body mass index. There were no significant differences in levels of white cell counts or inflammatory markers between the two groups.At times of clinical stability, cystic fibrosis patients infected with transmissiblePseudomonas aeruginosado not have a heightened inflammatory response above that of those harbouring sporadic strains.
Is bronchoscopic photodynamic therapy a therapeutic option in lung cancer?
Authors: Moghissi K. , Dixon K. 
Published: August 2003

This study addresses whether photodynamic therapy (PDT) is a valid therapeutic option in lung cancer treatment.A total of 24 articles were reviewed in two categories: advanced (G1) and early (G2) disease. Details considered included the following: 1) number of patients in each series; 2) staging; 3) methodology; 4) mortality; 5) morbidity; 6) survival; 7) relief of symptoms; and 8) concomitant treatments. G1 (636 patients) had severe endobronchial obstructive cancer and PDT was primarily for symptom relief. G2 (517 patients) had early stage cancer and were treated with PDT for curative intent.There was no procedure-related mortality in either group. G1 had a 5–28% incidence of skin sensitivity. Haemoptysis occurred in two series; one fatal, an incidence of 2.2%. Almost all patients had symptomatic relief. Patients with lower disease stage and better performance status had improved survival rates. G2 had a 8–28% incidence of sunburn. Three patients in one series (38 patients) had haemoptysis. Survival after 5 yrs in complete remission/response patients was 70%.This review suggests that bronchoscopic photodynamic therapy has indications in selected lung cancer patients with early or advanced stage disease. However, in the absence of a formal comparative study, no claim can be made of its superiority over other endobronchial therapies.
Melioidosis: an important cause of pneumonia in residents of and travellers returned from endemic regions
Authors: Currie B.J. 
Published: August 2003

Melioidosis is endemic in South East Asia, Asia and northern Australia. Infection usually follows percutaneous inoculation or inhalation of the causative bacterium,Burkholderia pseudomallei, which is present in soil and surface water in the endemic region. While 20–36% of melioidosis cases have no evident predisposing risk factor, the vast majority of fatal cases have an identified risk factor, the most important of which are diabetes, alcoholism and chronic renal disease.Half of all cases present with pneumonia, but there is great clinical diversity, from localised skin ulcers or abscesses without systemic illness to fulminant septic shock with multiple abscesses in the lungs, liver, spleen and kidneys. At least 10% of cases present with a chronic respiratory illness (sick >2 months) mimicking tuberculosis and often with upper lobe infiltrates and/or cavities on chest radiography. As with tuberculosis, latency with reactivation decades after infection can also occur, although this is rare.Confirmation of diagnosis is by culture ofB. pseudomalleifrom blood, sputum, throat swab or other samples. Microbiology laboratories need to be informed of the possibility of melioidosis, as those not familiar with it can misidentify the organism. Antibiotic therapy is initial intensive therapy withi.v.ceftazidime or meropenem or imipenem +/− cotrimoxazole for ≥10 days, followed by eradication therapy with cotrimoxazole +/− doxycycline +/− chloramphenicol (first 4 weeks only) for ≥3 months.Melioidosis has been increasingly recognised in returning travellers in Europe and recently melioidosis and colonisation withB. pseudomalleihave been documented in cystic fibrosis patients visiting or resident in endemic areas.
Primary pulmonary hypertension, Castleman's disease and human herpesvirus‐8
Authors: Bull T.M. , Cool C.D. , Serls A.E. , Rai P.R. , Parr J. , Neid J.M. , Geraci M.W. , Campbell T.B. , Voelkel N.F. , Badesch D.B. 
Published: August 2003

Primary pulmonary hypertension (PPH) and Castleman's disease (CD) are rare conditions infrequently encountered in clinical practice.In this paper, two patients diagnosed with both of these diseases are reported. The authors speculate that rather than being a chance occurrence, these conditions are linked by a common angio-proliferative mechanism. Therefore, an association between infection with the human herpesvirus‐8 and the diseases of PPH and CD was sought.Evidence of human herpesvirus‐8 infection was found in the lung tissue and, specifically, in the plexiform lesions from one of the patients.
Bleomycin induces cellular senescence in alveolar epithelial cells
Authors: Aoshiba K. , Tsuji T. , Nagai A. 
Published: August 2003

Cellular senescence is a state of irreversible growth arrest. In this paper the authors examined whether bleomycin, an agent that causes pulmonary fibrosis, induces the senescence of alveolar epithelial cells.Type II-like alveolar epithelial (A549) cells or rat primary type II cells were exposed to bleomycin and then evaluated for markers of cellular senescence. Bleomycin was also administered intratracheally in C57BL/6 mice.The authors found that exposure to bleomycin induced cellular senescence in A549 cells and rat primary type II cells. The senescence was characterised by a dose- and time-dependent increase in senescence-associated β‐galactosidase activity, senescence-associated changes in cell morphology, an increase in cell size and lysosomal mass, the overexpression of p21 protein, and irreversible growth arrest. The intratracheal injection of bleomycin in mice induced an increase in senescence-associated β‐galactosidase activity in type II epithelial cells, reaching a maximum at day 7.These results suggest that bleomycin induces a phenotype that is indistinguishable from that of senescence in alveolar epithelial cells. The induction of epithelial senescence by bleomycin may contribute to the pathway of impaired re-epithelialisation leading to pulmonary fibrosis.
Expression of the serotonin 1b receptor in experimental pulmonary hypertension
Authors: Rondelet B. , Van Beneden R. , Kerbaul F. , Motte S. , Fesler P. , McEntee K. , Brimioulle S. , Ketelslegers J-M. , Naeije R. 
Published: August 2003

The pathogenesis of pulmonary arterial hypertension (PAH) remains uncertain. Both the serotonin and endothelin (ET) systems are believed to be involved. Recent studies pointed to the importance of the serotonin 2B receptor as a limiting step.The current authors investigated the lung tissue expression of serotonin receptors and of the serotonin transporter (5‐HTT) by real-time-quantitative polymerase chain reaction in chronic overcirculation-induced PAH in growing piglets, with and without treatment with the dual ET receptor blocker bosentan. Pulmonary haemodynamic changes were described by pulmonary arterial impedance spectra.Three months after the surgical anastomosis of the left subclavian artery to the pulmonary arterial trunk, there was a shift of the impedance spectra to higher ratios of pressure and flow moduli, with increases in both 0 Hz impedance and characteristic impedance, and these changes were completely prevented by bosentan therapy. There was an increase in the expression of the serotonin 1B receptor. There was no change in the expression of the 5‐HTT, and of the serotonin 2B, 1D, and 4 receptors. The overexpression of the serotonin 1B receptor was partially prevented by bosentan therapy.The present authors conclude that this early pulmonary arterial hypertension model is characterised by an endothelin receptor-dependent increased expression of the serotonin 1B receptor.
Cellular profiles of induced sputum in children with stable cystic fibrosis: comparison with BAL
Authors: Reinhardt N. , Chen C.I.U. , Loppow D. , Schink T. , Kleinau I. , Jörres R.A. , Wahn U. , Magnussen H. , Paul K.P. 
Published: August 2003

Neutrophil-dominated endobronchial inflammation is a major characteristic of cystic fibrosis (CF) and there is increasing demand for easy-to-perform noninvasive monitoring for prediction and intervention.Fourteen stable paediatric CF patients (8–17 yrs; mean forced expiratory volume in one second 86.7% of the predicted value) were investigated once by fractional bronchoalveolar lavage (BAL) and by sputum induction on three occasions, 2–6 weeks apart. Sputum was induced by consecutive 10‐min inhalations of 3, 4 and 5% saline. CFsputum cellular profiles were compared with BAL fluid cell counts and samples from age-matched healthy children, and between different time points to assess reproducibility.Adequate sputum was recovered on >95% of occasions. In all sputum fractions, CF patients showed higher neutrophil counts than healthy children. Neutrophil percentages were highest in the first BAL fraction (median 92%), followed by sputum, in which the percentages decreased in consecutive fractions (72, 66 and 64%), whereas counts were lowest in the pooled BAL fraction (53%). Increasing percentages of macrophages mirrored the decreases in neutrophil percentage. Results of sputum induction at different time points in the CF patients showed good reproducibility and nonoverlap with counts from healthy children.In conclusion, the results of sputum induction in children with mild stable cystic fibrosis adequately describe airway inflammation by providing cellular profiles with lower relative neutrophil counts than in the first (“bronchial”) bronchoalveolar lavage fraction and higher relative neutrophil counts than in subsequent pooled (“more peripheral”) bronchoalveolar lavage fractions.
Controversies in epidemiology of occupational asthma
Authors: Gautrin D. , Newman-Taylor A.J. , Nordman H. , Malo J-L. 
Published: August 2003

Epidemiology is the study of the distribution, determinants and outcome of disease. In this article, the recently acquired knowledge of the epidemiology of occupational asthma is described, as well as current areas of controversy.Incidence figures obtained from field studies in high-risk workplaces, medicolegal statistics and sentinel programmes indicate that ∼10% of adult-onset asthma is attributable to the workplace. The strategy to identify cases through questionnaires and tools that address functional, immunological and physiopathological issues needs to be improved.Although few in number and limited to a handful of workplaces, cohort studies found that the risk of developing occupational asthma is determined less by individual susceptibility (e.g.atopy, tobacco smoking, human leukocyte antigen phenotype) and more by the level of exposure to its causes; in general, the higher the exposure, the greater the risk, and, by implication, lowering the level of exposure reduces the incidence of disease.Occupational asthma can be used as a satisfactory model for the development of adult-onset asthma. There is a great need to develop intervention strategies through adequate surveillance programmes in high-risk workplaces.
Predominant emphysema phenotype in chronic obstructive pulmonary disease patients
Authors: Boschetto P. , Miniati M. , Miotto D. , Braccioni F. , De Rosa E. , Bononi I. , Papi A. , Saetta M. , Fabbri L.M. , Mapp C.E. 
Published: August 2003

Patients with fixed airflow limitation are grouped under the heading of chronic obstructive pulmonary disease (COPD). The authors investigated whether COPD patients have distinct functional, radiological and sputum cells characteristics depending on the presence or absence of emphysema.Twenty-four COPD outpatients, 12 with and 12 without emphysema on high-resolution computed tomography scan of the chest, were examined. Patients underwent chest radiography, pulmonary function tests and sputum induction and analysis.Subjects with documented emphysema had lower forced expiratory volume in one second (FEV1), FEV1/forced vital capacity ratio, and lower carbon monoxide diffusion constant (KCO), compared with subjects without emphysema. Chest radiograph score of emphysema was higher, chest radiograph score of chronic bronchitis was lower, and the number of sputum lymphocytes was increased in patients with emphysema, who also showed a negative correlation betweenKCOand pack-yrs.Chronic obstructive pulmonary disease patients with emphysema, documented by high-resolution computed tomography scan, have a different disease phenotype compared with patients without emphysema. Identification of chronic obstructive pulmonary disease-related phenotypes may improve understanding of the natural history and treatment of the disease.