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Biomedical subjects

M Demedts

Publications and source records attributed to M Demedts.

At least 19 recordsLinked to original sources

Necrotising sarcoid granulomatosis: clinical, functional, endoscopical and radiographical evaluations.

Necrotising sarcoid granulomatosis (NSG) is a rare disease diagnosed on the basis of pathological features. The present study reports the characteristics of 14 cases of NSG. The mean age at the appearance of first symptoms was 37 yrs and the mean delay between first symptoms and diagnosis was 1 yr. Extrarespiratory symptoms were more common (12 out of 14) than respiratory symptoms (eight out of 14). Seven patients had inflammatory syndrome. Bronchoalveolar lavage was performed in eight patients and found to be normal in three cases. Respiratory function was normal in 13 patients, but carbon monoxide diffusing capacity was slightly decreased in eight of the 11 patients tested. A computed tomography scan showed a solitary nodule in four out of 14 cases, bilateral nodules in three and infiltrates in seven. One patient died from neurological complications despite treatment with corticosteroids and immunosuppressive drugs. Two cases of relapse were observed in patients initially treated with corticosteroids, and there were two cases of relapse after surgery. No relapse occurred in the five untreated patients. During the follow-up, lung cancer was detected at 26 months and 8 yrs, respectively, after NSG diagnosis in two patients. In conclusion, no one treatment is associated with a better outcome than the others, although lung biopsy might be necessary in case of isolated nodule or cavitation. Greater vigilance is required during the follow-up.

Adolescent↗

Exhaled nitric oxide after lung transplantation: impact of the native lung.

It has already been demonstrated that exhaled nitric oxide (eNO) is increased in lung transplant patients with chronic rejection, although it is not known whether the diseased native lung after single lung transplantation (SLTx) contributes to the increased eNO values. This study aimed to compare the eNO values in stable lung transplant patients (SLTx versus sequential (S)SLTx and heart (H)LTx) and in patients with established chronic rejection. Altogether, 42 LTx patients (25 females, 13 SLTx, 18 SSLTx, 11 HLTx), with a mean follow-up of 1149 days and a mean age of 44.6 yrs at transplantation, were included. Twenty-six patients had no signs of chronic rejection (five SLTx and 21 SSLTx/HLTx). There was no difference in their eNO values (10.2 in SLTx versus 12.2 (parts per billion) ppb in SSLTx/HLTx). Sixteen patients (eight SLTx and eight SSLTx/HLTx) had a chronic rejection (eight bronchiolitis obliterans syndrome (BOS) potential stage, four BOS stage 1, three BOS stage 2 and one BOS stage 3). Their eNOs were 18.1 (SLTx) and 17.0 (SSLTx/HLTx) ppb, respectively, which were significantly different to the stable LTx patients and showed a trend towards significance for SSLTx/HLTx. There was no significant difference in eNO between the patients with chronic rejection who underwent SLTx and those who underwent SSLTx/HLTx. The diseased native lung after single lung transplantation probably does not contribute much to the exhaled nitric oxide values, either in stable lung transplant patients or in lung transplant patients with chronic rejection.

Adolescent↗

Treatment of chronic respiratory failure in kyphoscoliosis: oxygen or ventilation?

Patients with kyposcoliosis and chronic respiratory insufficiency are treated either with home oxygen therapy or ventilation. Kyphoscoliotic patients demonstrate impaired ventilatory mechanics, consequently ventilation seems to be the treatment of choice. Yet, no randomised controlled trials (CRT) exist to prove it. Most investigators find it difficult to ethically justify a CRT. Therefore, the current authors performed the following retrospective study: survival and pulmonary function were analysed in all consecutive kyphoscoliotic patients who started long-term oxygen therapy (LTO group; n=15, aged 62+/-11 yrs (mean+/-SD)) or LTO plus nocturnal nasal intermittent positive pressure ventilation (nNIPPV group; n=18, aged 61+/-7 yrs) in the Dept of Pulmonology (University Hospital Gasthuisberg, Leuven) between 1990-2002. Prior to treatment partial pressure of oxygen (PO2) was lower, partial pressure of carbon dioxide (PCO2) tended to be higher and vital capacity (VC) tended to be lower in the nNIPPV group than in the LTO group (PO2 5.9+/-1 versus 6.7+/-0.9 kPa (44+/-8 versus 50+/-7 mmHg), PCO2 8+/-1 versus 7.3+/-0.9 kPa (60+/-8 versus 55+/-7 mmHg), VC 32+/-12 versus 40+/-16% predicted, or 645+/-244 versus 970+/-387 mL). In the nNIPPV group the 1-yr survival was higher (100% versus 66%). nNIPPV patients demonstrated an improvement in PO2 (breathing air) +54%, PCO2 (breathing air) -21%, VC +47% and maximal static inspiratory mouth pressure +33%; these improvements were absent in the LTO group. In conclusion, nocturnal nasal intermittent positive pressure ventilation, plus long-term oxygen therapy results in more favourable survival and changes in blood gases and respiratory function than long-term oxygen therapy alone.

Carbon Dioxide↗

Primary pulmonary hypertension after amfepramone (diethylpropion) with BMPR2 mutation.

Primary pulmonary hypertension (PPH) is characterised by sustained elevations of pulmonary arterial pressure without a demonstrable cause, leading to right ventricular failure and death. Hereditary mutations in the bone morphogenetic protein receptor type II (BMPR2) gene result in familial PPH transmitted as an autosomal dominant trait, albeit with low penetrance. The causes in cases without a BMPR2 mutation are unknown, but a syndrome of pulmonary arterial hypertension (PAH) similar to hereditary PPH is associated with systemic connective tissue disease, congenital heart disease, portal hypertension, and human immunodeficiency virus infection, or with the use of appetite-suppressant drugs. The authors identified a BMPR2 gene mutation in a 27-yr-old female who developed PAH after a short course of the appetite-suppressant drug amfepramone (diethylpropion). This allowed molecular genetic counselling and prevention of potentially harmful drug exposure in the patient's son treated for attention deficit disorder with methylphenidate, an amphetamine-related drug. No BMPR2 mutation was found in four additional, unrelated patients with appetite suppressant-related PPH. The findings provide strong evidence that amfepramone can trigger primary pulmonary hypertension in a bone morphogenetic protein receptor type II gene mutation carrier, and indicate that other genes are probably implicated in genetic susceptibility to appetite suppressants.

Adult↗

Evaluation of an individualised asthma programme directed at behavioural change.

An individualised asthma programme directed at behavioural change was evaluated in asthmatic subjects who reported complaints and impairment, despite adequate medical treatment. Mild-to-moderate asthma patients (n=23) were randomly assigned to a programme or waiting list condition. Outcome measures were: McMaster Asthma Quality of Life Questionnaire, Asthma Symptom Checklist, Negative Emotionality Scale, Knowledge, Attitude and Self-Efficacy Asthma Questionnaire, Adherence Scale, and peak flow measurements. Both groups were evaluated at three consecutive moments, each separated by 3 months; the programme was delivered between the first two evaluations. At onset the patient received a workbook containing information, exercises and homework assignments. Psycho-education, behavioural and cognitive techniques were introduced during six 1-h individual sessions. Compared with controls the programme group reported less symptoms (obstruction, fatigue), better quality of life (activity, symptoms, emotions), decreased negative affectivity, and increased adherence, immediately after finishing the programme and at 3 months follow-up. All three cognitive variables (knowledge, attitude towards asthma, self-efficacy) and day and night peak flow ratings improved in the programme group but not in the waiting list group. Participation in an individualised programme resulted in improvement of asthma morbidity, and asthma-related behaviour and cognitions, in subjects reporting symptoms and impairment despite adequate medical therapy.

Adult↗

New classifications and concepts of pathogenesis and management of diffuse interstitial lung diseases.

The diffuse interstitial lung diseases (ILD) are a very complex group of disorders, of which more than 200 specific disease entities are known. All ILD are characterized by a homogenous or heterogenous affection of the lung parenchyma. In recent years there was a renewed interest in the ILD because new concepts have been developed on aetiologic influences, on pathogenetic mechanisms (of inflammation versus active fibrosis) and on genetic susceptibility and consequently on the diagnostic-therapeutic management. Yet, there remain many questions still, e.g. concerning the exact incidence and prevalence of the majority of these diseases and concerning the genetic aspects. More specifically much research has been carried out on the important group of "idiopathic interstitial pneumonias" (IIP) and in particular on "idiopathic pulmonary fibrosis" (IPF). Recently several international consensus reports have been published with guidelines on the histologic and clinical definitions and on the classification and the diagnostic-therapeutic management.

Genetic Predisposition to Disease↗

Impact of age and radiographic presentation on the presumptive diagnosis of pulmonary tuberculosis.

We compared delay in presumptive diagnosing pulmonary tuberculosis (PTB) in elderly and younger patents, yield of diagnosis and whether the yield is influenced by the radiographic presentation, even when PTB was suspected. Time from first complaints to first consideration of PTB was determined as suspicion interval (SI) and from first consideration to diagnosis as recognition interval (RI). Presumptive diagnosis was defined as positive staining for acid-fast bacilli or presence of granulomatous lesions in pulmonary specimens. Inthe elderly and in the younger patients, the mean SI was 111.1 and 878 days respectively (P = NS), and the mean RI was 5.9 and 8.3 days, respectively (P = NS). The mean RI was longer in uncharacteristic than in characteristic radiographic findings in both elderly (8.2 and 4.6 days; P = 0.007) and younger patients (10.6 and 3.9 days; P=0.0001). A diagnosis was obtained in 89/113 elderly (79%) and in 109/138 younger (79%) patients (P=NS) and also in 59/80 (73%) patients with uncharacteristic findings and in 139/170 (82%) patients with characteristic findings (P = NS). In the latter, sputum contributed for 66% ofdiagnosis, whereas it was only 31% in patients with uncharacteristic findings (P < 0.005). In elderly patients with uncharacteristic radiographic findings, diagnosis was obtained from sputum in 41% and from other specimens in 35% (P = NS); in the younger group diagnosis was obtained from sputum in 23% and from other specimens in 48% (P < 0.05). In conclusion, there was no difference in SI and RI in elderly patients in comparison with younger patients. Uncharacteristic radiographic findings increased RI in both age groups. Age or radiographic presentation did not influence diagnosing PTB. In patients with characteristic radiographic findings, diagnosis was especially made from examination of sputum, whereas in those with uncharacteristic findings, diagnosis was more often obtained from the complementary investigation of other specimens.

Adolescent↗

Pertechnegas lung clearance in different forms of interstitial lung disease.

Interstitial lung diseases (ILD) are characterized by an acute or chronic inflammation of the alveolar capillary membrane, which affects the permeability of this membrane. A possible way to measure the permeability of the membrane is by radionuclide aerosol imaging. Pertechnegas, a gas composed of technetium-labelled carbon particles, has recently been proposed as a new ventilation agent to measure this lung clearance. The clearance by pertechnegas in the four most common forms of ILD (eight patients with connective tissue disease, 10 with hypersensitivity pneumonitis, nine with idiopathic interstitial pneumonia and 10 with sarcoidosis) was measured and compared with 10 nonactive smoking controls. Because forced vital capacity (FVC), total lung capacity (TLC) and carbon monoxide diffusing capacity of the lung (DL,CO) are used in the assessment of functional severity of the ILD, the pertechnegas clearance was correlated with these lung-function indices. It was found that the time to half clearance of pertechnegas of the lung is significantly decreased in idiopathic interstitial pneumonia (p<0.0001), hypersensitivity pneumonitis (p=0.0005) and connective tissue disease (p=0.002) but not in sarcoidosis when compared with 10 nonsmoking controls. A significant correlation is also found between time to half clearance and FVC (r=0.76; p<0.0001), TLC (r=0.63; p<0.0001) and DL,CO (r=0.75; p<0.0001) for all groups together. For all subjects as a group, the time to half clearance is shorter in the upper lung zones than in the lower zones (p<0.0001) and the ratio between both zones is not significantly different between the different types of disease. These results indicate that pertechnegas clearance is increased in idiopathic interstitial pneumonia, hypersensitivity pneumonitis and connective tissue disease, but not in sarcoidosis and is related to the functional severity of the disease.

Adolescent↗

Blood gas analysis: POCT versus central laboratory on samples sent by a pneumatic tube system.

BACKGROUND: We aimed to compare the results of blood gas analyses, performed at point-of-care and in the central laboratory (CL), on samples sent via a pneumatic tube system (PTS). METHOD: Specimens from two locations (lung function laboratory (LFL) and pneumology wards) were first analysed locally and then sent to the CL via PTS. RESULTS: While none of the blood gas samples from the LFL had air bubbles, 21 samples from the wards (n=31) had air bubbles in them. The mean time difference between the first (POCT) and the second (CL) determinations from LFL was 13.3+/-5.4 min (n=27) and from the wards 20.2+/-11 min. For pO2 the differences between LFL and CL results, for patients undergoing a 100% O2 test, were unacceptably large. For pO2 range 41-407 mm Hg, the difference was -2.4+/-3.2 (n=25). For the samples from the wards, the difference in pO2 between ward (range 37-183 mm Hg) and CL was -13+/-18 mm Hg. CONCLUSION: Irrespective of air bubbles, the transport by PTS has very little or no effect on pH and pCO2. If air bubbles cannot be excluded with certainty, PTS is not an appropriate transport medium for measurement of pO2 on blood gas samples.

Blood Gas Analysis↗

Pulmonary sequestration: a comparison between pediatric and adult patients.

OBJECTIVE: Modern large single institutional reports on pulmonary sequestration (PS) are extremely rare. We were interested in comparing patients with PS referred by our pediatric versus adult pulmonologists. METHODS: Hospital notes of all patients operated on between 1978 and 1997 for a congenital broncho-pulmonary malformation were reviewed. In 28 patients, the parenchymal lesion was vascularized by a systemic artery and was separated from the bronchial tree, thus matching the strict definition of PS. Patient characteristics and outcome were analyzed comparing the pediatric group (< or =16 years: n=13; mean age, 3+/-5 years) versus the adult group (>16 years: n=15; mean age, 33+/-13 years). RESULTS: No significant differences between both groups were observed in sex, side, type of sequestration, pulmonary venous drainage, associated anomalies, hospital and late outcome, and patient's overall score. Patients (n=21) with the intralobar type of sequestration presented significantly more often with an infection when compared with patients (n=7) with the extralobar type (91 versus 14%; P=0.0033). When compared with the pediatric group, patients in the adult group had significantly more respiratory infections (87 versus 38%; P=0.016), and also required a lobectomy more often (67 versus 31%; P=0.056). CONCLUSIONS: The extralobar type of sequestration often remains asymptomatic, and is usually an incidental finding during infancy. The intralobar type mostly presents with recurrent infections in adulthood resulting in more lobectomies. We believe these findings support our current policy to remove any pulmonary malformation whenever diagnosed in order to: (1), prevent infection and other potentially serious late complications which may compromise the surgical outcome; and (2), enhance the chance of a parenchymal-sparing resection.

Adolescent↗

Long-term safety and efficacy of a chlorofluorocarbon-free beclomethasone dipropionate extrafine aerosol.

BACKGROUND: Beclomethasone dipropionate (BDP) extrafine aerosol, a newly developed pressurized metered dose inhaler (pMDI) with a hydrofluoroalkane-134a (HFA) propellant (HFA-BDP; Qvar, 3M Pharmaceuticals, St. Paul, MN), has been shown to be effective in controlling asthma symptoms at approximately half the daily dose of chlorofluorocarbon (CFC)-BDP. OBJECTIVE: This study evaluated the long-term efficacy and safety of switching patients with asthma maintained on a stable dose of CFC-BDP pMDI to therapy with HFA-BDP pMDI at approximately half their previous daily dose of CFC-BDP. METHODS: This was an open-label, randomized, parallel-group multicenter trial. Patients with at least a 6-month history of asthma whose symptoms were controlled on CFC-BDP, 400 to 1600 microg daily, during a 2-week run-in period were randomized in a 1:3 ratio to CFC-BDP at the same daily dose or HFA-BDP at approximately half the daily dose of CFC-BDP for 12 months. RESULTS: A total of 473 patients were randomized: 354 to HFA-BDP, 119 to CFC-BDP. There were no statistically significant differences between groups in mean change from baseline in morning (AM) peak expiratory flow rate or forced expiratory volume in one second throughout the study. There were no consistent differences between treatment groups in individual asthma symptoms or daily beta2-agonist use during the study. There was an increase in the percentage of symptom-free days between baseline and month 12 in the HFA-BDP group (11.5%) and the CFC-BDP group (4.6%). No statistically significant differences in serum osteocalcin levels or adverse events were seen during the study or in AM plasma cortisol levels at month 12. CONCLUSIONS: Asthma control was maintained in patients switched from a stable dose of CFC-BDP (400 to 1600 microg daily) to HFA-BDP at approximately half the CFC-BDP dose (200 to 800 microg daily), and was maintained over the next 12 months. HFA-BDP demonstrated a similar safety profile to CFC-BDP; there were no differences between the agents with regard to systemic effects.

Administration, Inhalation↗

Recurrent flu-like illness with migrating pulmonary infiltrates of unknown aetiology.

Migrating pulmonary infiltrates present a difficult diagnostic and therapeutic challenge. We report on eight patients (mean age 51 years, range 32-78 years, with a prolonged history of migrating pulmonary infiltrates of unknown aetiology despite a very elaborate search for infectious causes, hypersensitivity pneumonitis or inhalation fever due to occupational or domestic exposure to fungi, or to other environmental causes, and for humoral or cellular immunological incompetence. These patients (one male, seven females) presented with recurrent episodes (mean 6, range 2-13) of a flu-like illness, often with cough, wheezing and pleuritic chest pain, but without systemic involvement. Previous medical histories were unremarkable. There was no relation with smoking habits, occupation, drug use or other possible exposures. Biochemical data were non-specific. There was no peripheral nor pulmonary eosinophilia; total IgE was normal, with negative RASTs and precipitins to a variety of antigens. Cultures and serological tests for bacteria, viruses, fungi, etc were non-contributory. Chest X-ray and computed tomography (CT) scan showed bilateral migratory pulmonary infiltrates, with a predilection for the middle and lower lung zones, often with a minor-to-moderate pleural effusion. Lung function tests were usually normal; at the most a slight decrease in diffusing capacity was noted in some patients. There was no or only a slight response to antimicrobials; systemic corticosteroids were not given. Further evolution was benign with patients being asymptomatic between the episodes. Despite elaborate investigations, the cause of these 'pneumonias' remains frustratingly unknown.

Adult↗

Giant cell interstitial pneumonia (hard metal lung disease, cobalt lung).

Hard metal lung disease is a rare form of occupational lung disease that can occur in workers engaged in the manufacture, utilization, or maintenance of tools composed of hard metal [a material composed mainly of tungsten carbide (WC) and cobalt] or diamond-cobalt. Clinically, the condition resembles hypersensitivity pneumonitis, with subacute presentations and possible evolution to pulmonary fibrosis. However, this interstitial lung disease is uniquely characterized by the presence of bizarre ;;cannibalistic'' multinucleated giant cells in the alveoli and the bronchoalveolar lavage. A pathological diagnosis of giant cell interstitial pneumonitis (GIP) is, therefore, specific for hard metal lung disease, even though not all affected subjects exhibit this pathognomonic feature. Cobalt is the critical toxic component causing hard metal lung disease, hence also the term cobalt-lung. Hard metal lung disease is more likely to occur in poorly regulated workplaces, but its occurrence depends mainly on individual susceptibility, rather than on cumulative exposure, so that even young subjects may be affected.

Journal Article↗

Evaluation of impulse oscillation system: comparison with forced oscillation technique and body plethysmography.

The impulse oscillation system (IOS) has been developed recently to measure respiratory system resistance (Rrs) and reactance (Xrs) at different frequencies up to > or = 25 Hz. IOS has, however, not been validated against established techniques. This study compared IOS with the classical pseudorandom noise forced oscillation technique (FOT) and body plethysmographic airway resistance (Raw) in 49 subjects with a variety of lung disorders and a wide range of Raw (0.10-1.28 kPa x L(-1) x s). Rrs,IOS was slightly greater than Rrs,FOT, especially at lower frequencies, with a mean +/- SD difference at 5-6 Hz of 0.14 +/- 0.09 kPa x L(-1) x s. Comparisons with the wave-tube technique applied on two analogues indicated an overestimation by IOS. Xrs,IOS and Xrs,FOT were very similar, with a slightly higher resonant frequency with IOS than with FOT (mean difference +/- SD 1.35 +/- 3.40 Hz). Raw was only moderately correlated with Rrn,FOT and Rrs-IOS; although the mean differences were small (0.04 +/- 0.14 kPa x L(-1)s for Rrs6,FOT and -0.10 +/- 0.14 kPa x L(-1) x s for Rrs5,IOS), IOS and FOT markedly underestimated high resistance values. In conclusion, the impulse oscillation system yields respiratory system resistance and reactance values similar, but not identical to those provided by the forced oscillation technique.

Adolescent↗

Comparison of registries of interstitial lung diseases in three European countries.

Few published studies have compared epidemiological data on the prevalence, incidence or relative frequency of the different interstitial lung diseases. In this review, the data of such registries from three countries in Europe (Belgium, Germany and Italy) are compared with those in the USA (Bernalillo County, NM). These registries show some striking similarities, but also discrepancies, which in part may be real, but may also be due to selection bias. Indeed, the registries in the European countries encompass the patients seen by pulmonologists, while the registry in Bernalillo County is based on a general population study in a limited area. In addition, in some studies, prevalences and incidences were registered, while in others, only prevalences or incidences were registered. Finally, most, but not all studies were prospective. Nonetheless, the different studies showed that sarcoidosis, idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, interstitial lung diseases due to collagen vascular diseases and not-defined (or postinflammatory) fibrosis were most frequent. In the majority of disease entities a male predominance was found. Surprisingly large differences in the use of diagnostic techniques, such as high-resolution computed tomography, bronchoalveolar lavage, open lung biopsy and transbronchial lung biopsy, were noted between the different studies.

Adult↗

Interstitial lung diseases: an epidemiological overview.

Epidemiological studies on interstitial lung diseases (ILDs) may be schematically subdivided into the following major types: 1) quantifications of disease, broken down into incidence, prevalence and mortality data; 2) identification of aetiological factors; and 3) clinical epidemiological studies. Epidemiological data may be obtained from different sources or population groups, using different study designs such as systematic national statistics, population-based data and registries, and large case series of specific diseases. Differences in results between epidemiological studies may be due to real differences in incidence, but may also be due to changes in disease definitions and classifications, differences in the epidemiological design of the studies, or even registration bias. Comparative epidemiological data of different ILDs are almost limited to the general population study in Bernalillo County and to national mortality statistics, which should be interpreted with great caution. Also, some, mostly national registries of the different ILDs have been carried out by specific medical profession groups (especially pulmonologists), which clearly underestimate the real incidence of ILDs, but in which the comparison of the relative frequencies is probably accurate. Based on all these comparative studies, sarcoidosis and idiopathic pulmonary fibrosis appear to be the most frequent ILDs, followed by hypersensitivity pneumonitis and ILD in collagen vascular disease, when classical pneumoconioses are not included. There is also a relatively large group of nonspecific fibrosis. Much more data have been published on the epidemiology of specific forms of interstitial lung disease. Most information is available on the epidemiology of sarcoidosis, and those data are probably the most accurate. Data on idiopathic pulmonary fibrosis have the disadvantage of the recent changes in definition and classification of this disease. Hypersensitivity pneumonitis has been studied epidemiologically, especially in some exposure groups such as farmers and pigeon breeders, and in some regions in North America, UK, France and Scandinavia. Estimates of frequencies of interstitial lung disease in collagen vascular disease or of drug-induced interstitial lung disease are less accurate and more variable, depending on diagnostic criteria. Notwithstanding the aforementioned problems, this report tries to provide a balanced overview of the epidemiology of different interstitial lung diseases.

Humans↗