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Biomedical subjects

B Wallaert

Publications and source records attributed to B Wallaert.

At least 109 records · Page 6Linked to original sources

[Uveitis, arthralgia and pseudo-jaundice in a HIV seronegative patient due to rifabutin].

Rifabutin is an effective drug in the treatment of Mycobacterium avium complex (MAC). Rare adverse effects have been described in non immunocompromised patients. We report the case of a 35 year-old woman, negative for the human immunodeficiency virus (HIV), who had an isolated pulmonary infection caused by MAC. Under Rifabutin (600 mg/day), Clarithromycin and Ethambutol, an uveitis, associated with a pseudojaundice and polyarthralgia, appeared, while pulmonary infection improved. Improvement of adverse effects was obtained with non-steroid anti-inflammatory drugs, local steroid treatment for uveitis and lower doses of Rifabutin (300 mg/day). Those adverse effects have not been previously described with this dose of Rifabutin in a non HIV patient. The mechanism remained unknown. The decrease of Rifabutin doses associated with symptomatic treatments allow the preservation of an effective treatment for MAC infection.

Adult↗

Tuberculin-induced delayed-type hypersensitivity reaction in a model of hu-PBMC-SCID mice grafted with autologous skin.

We have developed an animal model to study human delayed-type hypersensitivity reactions. Previous studies in humans have shown after tuberculin injection the presence of a mononuclear cell infiltration, with almost no eosinophils, associated with a preferential Th-1-type cytokine profile. Human skin graft obtained from tuberculin-reactive donors was grafted onto the back of severe combined immunodeficient mice. After healing, mice were reconstituted intraperitoneally with peripheral mononuclear cells. Tuberculin and diluent were injected intradermally, and skin biopsies were performed 72 hours later. Skin grafts were divided into two parts, one for immunohistochemistry and one for in situ hybridization studies. Immunohistochemistry was performed on cryostat sections using the alkaline phosphatase anti-alkaline phosphatase technique. In the tuberculin-injected sites as compared with the diluent-injected sites, there were significant increases in the number of CD45+ pan leukocytes and CD4+, CD8+, CD45RO+ T cells but not in CD68+ monocytes/macrophages and EG2 or MBP+ eosinophils. The activation markers CD25 and HLA-DR were up-regulated in the tuberculin-injected sites. In situ hybridization was performed using 35S-labeled riboprobes for interleukin (IL)-2, interferon (IFN)-gamma, IL-4, and IL-5. After tuberculin injection, a preferential Th-1-type cytokine profile was observed with significant increases in the numbers of IL-2 and IFN-gamma mRNA-expressing cells. These results are similar to those reported after tuberculin-induced delayed-type hypersensitivity in humans, suggesting that this model might be useful to study cutaneous inflammatory reaction.

Animals↗

Pulmonary involvement in polymyositis and in dermatomyositis.

OBJECTIVE: To assess prevalence, characteristics, and predictive factors of pulmonary involvement in polymyositis (PM) and dermatomyositis (DM). METHODS: The medical records of 55 consecutive patients with PM and DM between 1983 and 1996 were reviewed. The criteria for diagnosis of PM and DM were based upon Bohan and Peter criteria. RESULTS: Twenty-two patients (40%) developed lung impairment with a mean of 17 months after onset of PM and DM. The 3 main types of pulmonary disorders were interstitial lung disease (41%), ventilatory insufficiency with bronchopneumonia (22.8%), and alveolitis (with pulmonary function tests revealing restrictive pattern, low diffusing capacity, and normal computerized tomography) (36.2%). Both morbidity and mortality rates were as high as 40 and 27%, respectively, in PM and DM patients with lung involvement. Moreover, for the group with pulmonary diseases, serum GOT and ferritin levels were higher (p = 0.01 and p = 0.02) and the presence of anti-Jo-1 antibody and characteristic microangiopathy were more frequent (p < 0.05 and p = 0.0002). CONCLUSION: This series reveals both high prevalence (40%) and severity of pulmonary involvement in PM and DM. Our findings also suggest that high serum GOT and ferritin levels, presence of anti-Jo-1 antibody and characteristic microangiopathy may have predictive value and should encourage the search for pulmonary dysfunction and interstitial lung disease in patients with PM and DM.

Adolescent↗

[Acute silicosis. Clinical, radiologic, functional, and cytologic characteristics of the broncho-alveolar fluids. Observations of 6 cases].

We describe six cases of acute silicosis which were characterised by an intense exposure to pure silica; there was a rapid onset with diffuse abnormalities on the radiological image and severe respiratory abnormalities. The duration of occupational exposure to silica was four to eighteen years. Three patients presented with a cough associated with disabling dyspnoea (stage III and IV). The physical examination showed finger clubbing in three patients and rales and crepitations on auscultation in two patients. There was general debility in four of the cases with weight loss. The chest x-ray showed a rapid progress with confluence of the diffuse nodular opacities. Respiratory function tests showed a restrictive ventilatory defect (mean +/- standard error of mean: TLC 67 +/- 5%, Vital Capacity +/- 7%, FEV1 63.5 +/- 6%) and alteration in the transfer factor for carbon monoxide (DLCO: 49.5 +/- 5%). The initial broncho-alveolar lavage showed a lymphocytosis (23.6 +/- 4.2%) associated with an alveolar neutrophilia (7 +/- 2.5%). A lung biopsy was carried out in three patients and a transbronchial biopsy in one patient confirming the diagnosis of silicosis in three cases and of silicoproteinosis in one case. After a period of eighteen months to 14 years, four patients died either due to cardiopulmonary complications of their disease (two cases) or to intercurrent disorders (two cases). The last two patients are currently stable.

Adult↗

[Management of hemoptysis in invasive pulmonary aspergillosis].

Invasive pulmonary aspergillosis is an opportunistic infection occurring in a background of severe immune depression. The majority of cases occur in patients who have malignant hematologic disease, particularly during chemotherapy induction or consolidations phases for acute non-lymphocytic leukemia. The principal risk factors are profound (PN < 500 per mm3) and prolonged (very high risk beyond 20 days) neutropenia, perturbed phagocyte function and cellular immune deficiency (AIDS, immunosuppressive treatment in organ and bone marrow recipients). Clinically, invasive pulmonary aspergillosis presents as acute non-specific pneumonia with cough, chest pain and fever. The severe infection rapidly becomes life-threatening. The development of massive hemoptysis is a major risk. We report four cases of invasive pulmonary aspergillosis in patients who had hemoptysis. All four patients developed non-specific pneumonia resistant to broad-spectrum antibiotics during post-chemotherapy aplasia. Computed tomography of the thorax and bronchoscopy with bronchoalveolar lavage was performed due to the occurrence of hemoptysis. In the first two cases, the patients were recovering from aplasia. The thoracic CT scan showed evidence of a cavitating mass with peripheral vessels. Bronchoscopy findings suggested mucosal lesions. The patients were managed surgically. Pathology confirmed the diagnosis of invasive pulmonary aspergillosis with the presence of ischemic necrosis of the pulmonary parenchyma harboring numerous aspergillus filaments. Outcome was favorable and chemotherapy was re-initiated in one case. These two patient died from their hematological disease a few months later. The other two patients remained in aplasia. A CT of the thorax showed multifocal infiltration with vascular contact. Bronchoscopy was again suggestive. One patient developed massive hemoptysis with respiratory distress. Embolization was performed but the patient died two days after onset of hemoptysis. In the last case, embolization was successful and outcome was favorable enabling a bone marrow allograft; the patient died a few months later from the hematological disease. The potential gravity of hemoptysis in the course of invasive pulmonary aspergillosis should lead to early treatment with emergency CT scan and, if possible, bronchoscopy with bronchoalveolar lavage to establish the therapeutic strategy based on surgical excision or embolization of the pulmonary or bronchial arteries.

Adult↗

[Pollution and allergy: the contributions of animal and in vitro experimentation].

The respiratory pathological symptoms induced by atmospheric pollutants are closely dependent on the action of the aerocontaminants on the cells of the respiratory tract that are exposed to their effects. Two methods of experimental investigation are used to specify the effects of pollutants on the respiratory system: Study of the morphological and functional alterations of animal respiratory systems after exposure to different pollution constituents. Exposure of bronchitic or pulmonary cells to microquantities of pollutants, using different techniques that create direct contact between the pollutant and target cells. In animals gaseous pollutants alter the means of defence (muco-ciliary purification and antibacterial defence), inducing development of a neutrophil inflammatory reaction and, for ozone and NO2 favouring sensitisation by allergen-dependent IgE; diesel particles are responsible for restrictive ventilation problems, an inflammatory reaction, a sensitisation develops to pneumoallergens and in some cases of the development of pulmonary tumours. In vitro studies specify the cellular mechanisms and the molecules that are responsible for the observed phenomena: increase in the synthesis and expression of messenger DNA the codes for the pro-inflammatory cytokines, adherance molecules and chimiokines.

Air Pollutants↗

[Tuberculosis, mycobacterium infection and hairy cell leukemia].

OBJECTIVES: Diagnosis of tuberculosis and/or mycobacteria infection is particularly difficult in immunocompromised patients. PATIENTS AND METHODS: We examined the clinical presentation, means of diagnosis, treatment and outcome of tuberculosis in a retrospective study of 6 patients among 75 with hairy cell leukemia diagnosed from 1982 to 1995. RESULTS: Hearlding symptoms of tuberculosis diagnosis were: fever (6/6), weight loss (4/6), pleural effusion (1/6), superficial adenopathy (1/6), persistence of cytopenia or splenomegaly during the treatment of hairy cell leukemia. Pulmonary symptoms were present in only two cases. Diagnosis was obtained by positive culture of mycobacteria in 2 cases (Mycobacterium tuberculosis in pleural effusion, Mycobacterium kansaii in adenopathy). Microbiological diagnosis was never obtained from sputum (6/6). Diagnosis was obtained by histopathology in all cases: from bone marrow (2 cases), lymph nodes (2 cases), liver (1 case), spleen (1 case), umbilical fat (1 case). Tuberculosis was disseminated in all cases. By clinical, biological, microbiological histopathological means and response to treatment, tuberculosis was considered as: hematopoietic in all cases, hepatic (in 4/6), pleural (1/6), pulmonary (1/6). A favorable outcome of tuberculosis was observed in all cases. No death was observed. CONCLUSIONS: Tuberculosis was found in 8% of hair cell leukemia patients. In hairy cell leukemia, tuberculosis is characterized by few pulmonary symptoms and scarse microbiological documentation. In contrast, histopathology is very interesting to confirm diagnosis. Tuberculosis is in most cases disseminated and in particular hematopoietic diffusions is always present. In spite of existensive localization, the prognosis remains excellent and all patients can be cured. In our opinion, this good prognosis may be linked to the improvement of hairy cell leukemia treatment observed since the advent of interferon pentostatin and 2cdA.

Adult↗

Sequential evaluation of pulmonary function and bronchial hyperresponsiveness in patients with nasal polyposis: a prospective study.

Nasal polyposis (NP) is commonly associated with nonspecific bronchial hyperresponsiveness (BHR) and/or asthma. The aim of this prospective study was to investigate the changes of pulmonary function and BHR in patients with nasal polyposis. Forty-four consecutive patients with NP were included in the study and were followed for 12 mo. Nonspecific BHR was assessed by a carbachol challenge test to determine the provocating dose (PD20) necessary to decrease FEV1 by 20% from baseline values; 17 of 22 patients who demonstrated BHR also exhibited asthma. Spirometric measurements and carbachol challenge were performed before initiating any treatment and 12 mo later. All patients were treated first with beclomethasone (600 microg/d). Intranasal ethmoidectomy was performed in 23 patients who did not improve when treated with topical steroids alone (nonresponders); in contrast, 21 patients were successfully treated with beclomethasone alone (responders). PD20 significantly decreased in the group of nonresponders (p = 0.018), whereas it remained unchanged in responders (p = 0.95). FEV1 (% pred) and FEF25-75 (% pred) significantly decreased in nonresponders (p < 0.001), whether BHR existed or not, whereas no significant change was observed in responders. Our results demonstrate that nonresponders who required nasal surgery exhibited an enhancement of BHR and a slight but significant decrease of FEV1 and FEF25-75 values. However, no change in pulmonary symptoms and/or asthma severity occurred. Clinical and functional follow-up of these patients should assess the long-term evolution of these parameters and their clinical relevance.

Adolescent↗

Absence of DNA from mycobacteria of the M. tuberculosis complex in sarcoidosis.

To evaluate the role of mycobacterial infection in the pathogenesis of sarcoidosis, several groups have attempted to identify mycobacterial DNA in clinical samples from these patients by polymerase chain reaction (PCR), but widely divergent results have been obtained. It has been suggested that differences in the sensitivity of the procedures used may explain these discrepant results. To test this possibility, the presence of mycobacterial DNA was sought in biopsies from patients with sarcoidosis using sequence capture-PCR, a procedure that is 100-fold more sensitive in detecting mycobacterial DNA in paucibacillary samples than standard PCR protocols. Using this approach, DNA corresponding to two different sequences specific for organisms of the Mycobacterium tuberculosis complex (the 1S6110 insertion element and the DR region) could not be detected in any of the 15 biopsies from patients with sarcoidosis, whereas a high proportion of positive results was obtained for tissue biopsies and other clinical samples from patients with active tuberculosis, including samples that were smear-negative/culture-positive and smear-negative/culture-negative. These results support prior studies suggesting that M. tuberculosis does not play a pathogenic role in sarcoidosis in most patients.

Biopsy↗

Long-term follow-up of pulmonary function in chronic eosinophilic pneumonia. Groupe d'Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord.

The prognosis of chronic eosinophilic pneumonia (CEP) is usually good under corticosteroid therapy (CST). The main complications are relapses when treatment is tapered or discontinued. The aim of this retrospective, multicentre study was to evaluate the long-term consequences of CEP on pulmonary function tests. Nineteen patients (mean +/- SEM age 51 +/- 16 yrs) with CEP were studied. Lung function tests were performed at the time of diagnosis and at follow-up, and included flow-volume curve. The results of the first pulmonary function test were normal in six patients, restrictive in nine, and obstructive in four. Relapses (recurrence of initial signs) occurred in nine patients. The last evaluation (mean +/- SEM follow-up of 49 +/- 44 months, range 12-142 months) showed a complete recovery in 8 of the 19 patients. One patient developed bilateral apical fibrosis. The remaining 10 patients, exhibited obstructive pulmonary function without relapse of CEP at this time. Bronchoalveolar lavage (BAL) eosinophilia at the time of the initial evaluation tended to be higher (p = 0.05) in these 10 patients than in those with normal pulmonary function findings at follow-up. This study demonstrates: firstly, that the development of an obstructive ventilatory defect is a common finding during the course of chronic eosinophilic pneumonia (CEP); secondly, that bronchial obstruction might appear despite the absence of clinical and radiological signs of relapse; and, thirdly, that a markedly increased bronchoalveolar lavage eosinophilia at the initial evaluation is associated with a higher risk of development of bronchial obstruction. These results suggest that pulmonary function tests should be included in the management of chronic eosinophilic pneumonia.

Adult↗

Cytokine mRNA gene expression in active and nonactive pulmonary sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease associated with the expansion and activation of CD4+ T-lymphocytes and macrophages. To investigate the immunopathology of active and nonactive pulmonary sarcoidosis, we have examined the expression of cytokine gene transcripts in bronchoalveolar lavage cells from 15 patients with active pulmonary sarcoidosis, eight patients with non-active pulmonary sarcoidosis, and nine normal controls. Using in situ hybridization, the percentage of cells expressing messenger ribonucleic acid (mRNA) for interleukin (IL)-1 beta, IL-2, IL-3, IL-4, IL-5, IL-6, IL-10, IL-12 and interferon-gamma (IFN-gamma) were compared in the groups studied. In individuals with active sarcoidosis, there were significantly greater proportions of cells expressing mRNA for IL-2, IL-10, IL-12 and IFN-gamma than in subjects with nonactive disease and normal controls (p < 0.01). There was no significant difference in the percentage of positive cells expressing IL-10 and IL-12 mRNA in the nonactive group compared to the normal controls (p > 0.05). No significant differences in the percentages of IL-3, IL-4 and IL-5 mRNA positive cells were observed between active and nonactive sarcoidosis patients and normal controls (p > 0.05). These results demonstrate that there is a preferential expression of T-helper type 1 cytokines in pulmonary sarcoidosis, and that cytokines related to macrophage activation are the most prominent. In addition, these data implicate an elevated expression of interleukin-2, -10 and -12 and interferon-gamma in active compared to nonactive sarcoidosis.

Adult↗

Rothia dentocariosa: two new cases of pneumonia revealing lung cancer.

Pneumonia due to Rothia dentocariosa is extremely rare: only 1 case of pneumonia due to this pathogen has been reported in an immunocompromised patient with acute myelocytic leukemia. In this report, 2 new cases of Rothia dentocariosa pneumonia are described in 2 patients with lung cancer. This opportunistic pulmonary agent, belonging to the oro-pharyngeal flora, is also known to cause endocarditis in patients with underlying cardiac pathology.

Actinomycetales Infections↗

MCP-1 secretion in lung from nonsmoking patients with coal worker's pneumoconiosis.

Exposure to coal dust leads to the development of coal worker's pneumoconiosis (CWP), a disease associated with an accumulation of macrophages in the lower respiratory tract. Mechanisms controlling monocyte recruitment are still poorly understood. Since monocyte chemoattractant protein-1 (MCP-1) is recognized as a potent chemotactic factor for blood monocytes, we analysed the presence of MCP-1 in the pulmonary compartment of patients with CWP. Bronchoalveolar lavage fluid (BALF) from 16 nonsmoking control subjects and 27 nonsmoking CWP patients (16 with simple pneumoconiosis (SP) and 11 with progressive massive fibrosis (PMF)) was analysed. Alveolar macrophages (AMs) were purified by adherence and BALF was concentrated tenfold by lyophilization. MCP-1 was measured in BALF and in 3 h AM supernatants using a sandwich enzyme-linked immunosorbent assay (ELISA). The localization of MCP-1 in lung tissue was determined by immunohistochemistry on tissue sections from three patients with CWP and two control subjects. MCP-1 levels were significantly higher in concentrated BALF from patients with SP or PMF (median 370 and 555 pg x mL-1, respectively) than in those from control subjects (median 11 pg x mL-1) (p<0.001). Released MCP-1 in AM supernatants was enhanced in patients with CWP (median 83 pg x mL-1) but compared to controls (median 41 pg x mL-1) this level did not reach significance. Although significantly increased, AM counts in BALF from patients with CWP did not correlate with MCP-1 levels. MCP-1 levels in BALF correlated with MCP-1 levels in AM supernatants (p=0.47; p<0.02). In control lung specimens, MCP-1 was expressed by a few AMs, type II pneumocytes and perivascular smooth muscle cells. CWP sections were characterized by an increased number of AMs and mainly by the presence of fibroblasts (in the myogenic area of fibrotic lesions) and hyperplastic type II pneumocytes, which were strongly immunostained for MCP-1. Our data demonstrate that: 1) patients with coal worker's pneumoconiosis have a marked pulmonary overproduction of monocyte chemoattractant protein-1; and 2) in addition to alveolar macrophages, fibroblasts (probably myofibroblasts) and hyperplastic type II pneumocytes may also be responsible for this increased level of monocyte chemoattractant protein-1 in coal worker's pneumoconiosis.

Aged↗

[Erasmus syndrome: clinical, tomographic, respiratory function and bronchoalveolar lavage characteristics].

The Erasmus syndrome describes the association of generalised progressive scleroderma following exposure to silica with or without silicosis. This is a case report on four patients presenting with the Erasmus syndrome who were admitted to hospital. The analysis of the four cases enables an assessment of the cause of the dyspnoea during the course of the Erasmus syndrome. The dyspnoea presents more as scleroderma (pulmonary fibrosis in two cases, pulmonary artery hypertension in one case and localised thoracic skin disease in one case) than of pneumoconiosis. Pulmonary fibrosis should be considered where there is an association of progressive effort dyspnoea, fine crackles on auscultation and a radiological appearance either of honeycombing and/or a ground glass appearance predominantly in the posterior regions which does not exist in isolated cases of silicosis. The functional repercussion of the fibrosis is evident by a restrictive ventilatory defect which is not specific but more severe than in a case of silicosis alone. Bronchoalveolar lavage showed, in two cases of pulmonary fibrosis, an unusual polymorphonuclear neutrophilia during the course of the silicosis. The presence of ausculatory anomalies, the atypical aspects of pneumoconiosis on computed tomography and an unusual form of LBA should suggest the existence of pulmonary fibrosis associated with pneumoconiosis.

Adult↗

[Pulmonary artery hypertension caused by carcinomatous lymphangitis ].

A 58 year old man without significant past medical history developed a cough and effort dyspnoea of a few weeks. A computed tomographic scan showed bilateral interstitial disease with linear thickening of the septa and also a ground glass effect in the lung parenchyma. Echocardiography and right heart catheterisation confirmed the existence of pulmonary arterial hypertension (mean pulmonary artery pressure 45 mmHg) with a normal pulmonary artery wedge pressure (12 mmHg). There was a rapidly progressive worsening with an unfavourable outcome. A necropsy examination showed the development of bilateral pulmonary lymphangitis carcinomatosis secondary to an adenocarcinoma of the head of the pancreas. There was no metastatic emboli of the vessels but there was lymphatic infiltration and the physiopathological hypothesis suggested a compression of the pulmonary arterial vessels secondary to lymphatic obstruction.

Adenocarcinoma↗

Interleukin-8 secretion in patients with allergic rhinitis after an allergen challenge: interleukin-8 is not the main chemotactic factor present in nasal lavages.

BACKGROUND: Interleukin-8 (IL-8) is a chemotactic cytokine for neutrophils and primed eosinophils. In allergic rhinitis, allergen exposure triggers leucocyte recruitment. OBJECTIVE: We evaluated in this study IL-8 secretion and the neutrophil chemotactic activity in nasal lavages collected after a nasal allergen challenge. Moreover, the participation of IL-8 in the neutrophil chemotactic activity was quantified. METHODS: Four healthy subjects and 19 patients with allergic rhinitis were exposed to a nasal allergen challenge. As a control, saline challenge was performed in four patients with allergic rhinitis. Concentration of IL-8 was measured by ELISA in nasal lavages collected before and after challenge. Neutrophil chemotactic assay was developed using a 48-well chemotaxis microassembly. RESULTS: After allergen challenge, the healthy subjects, the four patients receiving saline and one patient exposed to allergen did not respond; seven patients presented a single early reaction and 11 patients a dual response. For healthy subjects and the four patients exposed to saline, the level of IL-8 did not increase after challenge in comparison with that at baseline. After allergen challenge, two peaks of IL-8 release were observed for patients with allergic rhinitis during the early (30 min to 1 h 30 min) and the late periods (3 h 30 min to 9 h 30 min), however the difference was not significant for the early period. During the late period, a significant increase in IL-8 concentrations was detected for the patients developing a dual response, whereas the difference was not significant for those presenting only an early reaction. The neutrophil chemotactic activity of nasal lavages from patients with allergic rhinitis collected during the early and the late reactions (17 +/- 2.1 and 23.3 +/- 2.8 neutrophils per high power field (hpf), respectively) was significantly higher than the activity of lavage fluid collected at baseline (9.2 +/- 1.8 neutrophils per hpf). Nevertheless, the addition of a neutralizing anti-IL-8 antibody inhibited weakly the chemotactic activity of lavage fluid from rhinitic patients collected during the early or the late periods (18 and 11% of inhibition) (P = NS). CONCLUSION: These data show that allergen challenge increased significantly the secretion of IL-8 for the patients with allergic rhinitis. However, neutralization of IL-8 in nasal lavages by a specific antibody revealed that the role of this chemokine in granulocyte infiltrate was limited, suggesting that IL-8 acts in connection with other chemotactic factors in this recruitment.

Adult↗

["Primary" diffuse interstitial fibrosis in coal miners: a new entity? Study Group on Interstitial Pathology of the Society of Thoracic Pathology of the North].

It is well known that silica exposure leads in an experimental model to the development of an acute fibrotic process. In human beings two main observations have already been done: (1) silica exposure is frequently associated with the development of connective tissue disease (CTD), especially progressive systemic sclerosis; (2) 10 to 20% patients with CTD developed pulmonary fibrosis. In this context we report 26 cases of coal miners who presented with clinical, radiological, biological and functional characteristics mimicking idiopathic pulmonary fibrosis (IPF), with or without associated coal worker's pneumoconiosis (CWP). All were men; mean age was 68 +/- 9.2 years. Twenty-three were smokers. Duration of exposure was 28.8 +/- 9.1 years. All the patients had dyspnea (stage III, IV in the NHYA classification) and diffuse crackles. Eleven out of 26 had finger clubbing. Computed tomography showed honeycombing (23 cases), and/or ground glass opacities (6 cases) with bronchiectasis (3 cases) predominant in the lower lobes; 19 had radiological signs of CWP, micronodules (n = 16) and nodules (n = 3) predominant in the upper lobes. BAL exhibited an increased % of neutrophils (11.9 +/- 16.1%). Lung function demonstrated a restrictive pattern (TLC = 73 +/- 15.6% and VC = 80 +/- 18% of predicted values) associated with a decreased DLCO (51.8 +/- 23.6% of predicted values) and hypoxemia (at rest = 66.5 +/- 11.2 mmHg, upon effort = 56 +/- 12 mmHg). Lung biopsies were performed in four cases and demonstrated interstitial fibrosis of intraalveolar septum with an accumulation of immune and inflammatory cells similar to the one described in IPF. The association between IPF and silica exposure with or without associated CWP points out the problem of legal recognition of idiopathic-like pulmonary fibrosis as a complication of the occupational exposure of coal workers.

Adult↗

[Chylothorax and sarcoidosis].

The association of sarcoidosis and chylothorax is rare. A patient aged 64 presented with mediastinal sarcoidosis. Two years after the diagnosis he developed a right chylothorax. The computed tomographic examination showed a retraction of the right hemithorax associated with large mediastinal nodes, without signs of interstitial lung disease. Pulmonary function tests revealed a restrictive ventilatory defect with a Forced Vital Capacity (FVC) of 53% and a Forced Expired Volume in one second (FEV1) of 54% from the predicted values; the FEV1/FVC ratio was 78%. Steroid therapy was started at 1 mg per kilogram per day for three months then progressively decreased to 15 mg per day. Inspite of the steroid therapy there was as well biological evidence of continued disease activity (bronchoalveolar lavage lymphocytosis and increased serum angiotensin converting enzyme), as persistence of pleural effusion and mediastinal adenopathy; the contraction of the right hemithorax increased. Restrictive ventilatory defect worsened with a FVC of 44% and an FEV1 of 47% of the predicted values. The presence of a significant contraction of the hemithorax and of a severe restrictive ventilatory defect suggested the existence both of pleural fibrosis and of a compression of the main lymphatic pathways responsible for the chylothorax.

Adrenal Cortex Hormones↗