A 27 year old white woman with a history of chronic eczema and episodes of serious infection of the chest, skin, and bone presented with acute respiratory failure. She was found to have a spontaneous ...right pneumothorax and a pneumatocele in the left upper lobe. Despite a left upper lobectomy she was left with chronic respiratory failure, bullous lung disease, and bilateral bronchiectasis. The hyperimmunoglobulinaemia E and recurrent infections syndrome was diagnosed only in adult life.
Pulmonary alveolar proteinosis Briens, E; Delaval, Ph; Mairesse, M P ...
Revue des maladies respiratoires
19, Issue:
2 Pt1
Journal Article
Peer reviewed
Pulmonary alveolar proteinosis (PAP) is a rare, diffuse infiltrative lung disease of unknown etiology characterized by accumulation of abnormal lung surfactant in the alveoli and distal airways. ...Although it was first described in 1958, few large scale studies have been published. The diagnosis is conventionally based on lung biopsy and the value of less invasive procedures such as bronchoalveolar lavage (BAL) remains controversial. A multi-center retrospective study was conducted in an attempt to update epidemiological, clinical and long term outcome and to assess the usefulness of chest CT scan and BAL in the management of a large homogeneous series of 41 patients. Inclusion criteria required a clinical picture compatible with PAP and either histological confirmation, or a BAL with a milky macroscopic appearance coupled with positive staining to PAS. Mean age was 48.8 years with a 4: 1 male predominance. The prevalence of smokers (80%) and occupational exposure to various inhaled dusts (39%) was high. The clinical, roentgenographic and functional features were in keeping with previous series. Worsening dyspnea was the most common symptom. Other common manifestations were dry cough, recurrent lung infections, weight loss, finger clubbing, crackles and cyanosis. LDH serum levels were increased in 51% of cases. Lung function tests usually showed a mild restrictive ventilatory defect with hypoxemia and reduced DLCO. Chest X ray revealed bilateral ground glass opacities with consolidation or air bronchogram involving peri-hilar or lower lobes. Chest CT scan, performed in 38 patients, revealed a similar pattern in most of them with diffuse intra-alveolar ground glass opacities (33/38) and interlobular thickening (35/38), resulting in a crazy paving appearance in the typical geographic pattern (32/38). This latter aspect was highly suggestive of the diagnosis. Segmental BAL led to a definitive diagnosis in 62% and lung biopsy was only required in 38% of cases. These data suggest that careful BAL analysis can be sufficient to provide a diagnosis without the need for a lung biopsy. During follow-up, spontaneous remission or improvement was reported in 26%. Segmental (n=3) or whole lung lavage (n=22) was required in 62.5% with a favorable course (complete remission 37%, improvement 34%, stable course 21%) in 92% of the cases. In this series the overall prognosis for PAP remained good, although unpredictable.
The beta-blocker (BB) prescription remains insufficient despite guidelines, especially, for chronic heart failure. Patients suffering chronic obstructive pulmonary disease (COPD) are particularly ...less treated by BB. The level of evidence for BB prescription is however especially high and as we will focus on, the level of evidence for the safety of BB in the COPD context is convincing enough. We, thus, propose to review the existing literature in regard to this prescription of BB in the chronic heart failure, in the coronary artery disease and for high blood pressure in COPD patients. We then propose our approach to improve the level of prescription of BB in COPD patient really justifying this prescription in cardiology.
Monoclonal antibody (McAb) Po66 has been obtained by immunisation of mice against a human lung squamous cell carcinoma. The in vitro reactivity of the antibody with cancer cells and its ability to ...localise in human lung cancer xenografts growing in nude mice have been reported earlier. Presented here is the first clinical evaluation of the antibody for scintigraphic detection of tumours. Thirty-three patients with histologically confirmed primary non-small cell lung carcinoma were investigated. Twenty-seven of them were explored at the preoperative stage and six at 6 months after surgery. Biodistribution results were obtained from seven operated patients by combining injections of 131I-radiolabelled Po66 and of 125I-labelled unrelated immunoglobulin. The localisation index was three times higher for this specific antibody. Immunoscintigraphy detected 78% of primary tumours and 100% of recurrences. In this short series of patients, immunoscintigraphy proved helpful in the assessment of tumour spread in four patients by visualising localisations in the mediastinum or the contralateral lung which the CT scan had failed to demonstrate. Immunoscintigraphy was also more efficient than plain chest X-ray for the detection of local tumour recurrences.
To study the frequency of a factor of immunodepression in patients with tuberculosis, the differences in presentation, and the diagnosis and therapeutic management according to the immune status.
...Retrospective study of the files of patients hospitalised in the University Hospital Centre of Rennes in 1998 for a Mycobacterium tuberculosis infection. Comparison of two populations, immunodepressed versus non immunodepressed.
75 patients aged 20 to 91 were included, 41 patients were considered immunodepressed and 34 non immunodepressed. The causes of immunodepression were: HIV infection (n = 2), diabetes (n = 4), chronic alcoholism (n = 12), chronic respiratory diseases treated with corticosteroids (n = 6), neoplasia (n = 9), and inflammatory diseases (n = 7). Comparison between the 2 populations revealed more a frequent history of tuberculosis in the immunodepressed (p = 0.04), shorter delay before diagnosis (p = 0.04), greater frequency of disseminated forms (p = 0.02) and enhanced mortality (p = 0.01). There was no difference in the 2 groups with regard to the clinical signs having evoked tuberculosis, the diagnostic method, the bacteriological results or the modalities of treatment.
The frequent reactivation of tuberculosis in immunodepressed patients and the severity of the infection in these patients should evoke tuberculosis and the rapid initiation of an efficient treatment in such patients. In the case of alteration in immune defences, prophylactic treatment should help to reduce the number of such reactivations.
Matrix metalloproteinases are particularly potent in degrading basement membrane collagen and other extracellular matrix components. We have investigated the effects of a selective phosphodiesterase ...4 inhibitor, RP 73-401
N-(3,5-dichloropyrid-4-yl)-3-cyclopentyloxy-4-methoxybenzamide, on gelatinase (matrix metalloproteinase-2 and matrix metalloproteinase-9) activity in ovalbumin-sensitized and -challenged mice. Twenty-four hours after the last challenge, matrix metalloproteinase activity was evaluated in the bronchoalveolar lavage fluids by a zymography technique, and a significant increase in matrix metalloproteinase-9, but not matrix metalloproteinase-2, activity was noted. When administered orally (0.3–3 mg/kg) 1 h before each ovalbumin challenge, the selective phosphodiesterase 4 inhibitor, RP 73-401, significantly reduced this increased matrix metalloproteinase-9 activity in bronchoalveolar lavage fluids. Our data suggest that RP 73-401 may modulate tissue remodelling associated with lung inflammatory processes including asthma.
A monoclonal antibody, Po66, recognized an antigen named Po66 carbohydrate binding protein (PO66-CBP), which was homologous to the galectin-8 protein. Two additional isoforms previously not described ...were characterized. The aim of this study was to compare the expression of Po66-CBP and its isoforms in different healthy, tumoral and peritumoral tissues and at last to determine the localization of the protein in tumors and distant tissues.
Reverse transcriptase PCR of Po66-CBP was performed on total RNA extract from eleven healthy and eleven tumoral and peritumoral tissue specimens. Antibody Po66 was used to localize the protein in the tumors and distant tissues by an immunohistochemistry method.
Po66-CBP was expressed by half of the healthy tissues. One of the isoforms, the last often present in healthy tissues, was found in all tumoral and peritumoral tissues studied. Immunohistochemistry evidenced a gradient of protein expression in normal cells depending on the vicinity of tumoral tissue. Po66-CBP expression was modified in cancerous tissue, suggesting the implication of galectins in carcinogenesis.
Monoclonal antibody Po66, produced by immunization against a patient's lung squamous cell carcinoma was found suitable for the scintigraphic detection of human tumours. Surprisingly, the cellular ...antigen recognized by Po66 was abundant in the cytoplasm of tumour cells but could not be detected on the surface membrane. In the present work the biodistribution of radiolabelled Po66 and of an unrelated immunoglobulin were studied comparatively after intravenous injection into nude mice bearing lung squamous cell carcinoma grafts. Radioactivity distribution among mouse organs and tumour was analysed by gamma counting and autohistoradiography. After injection, radiolabelled Po66 decreased rapidly from the blood in tumour-bearing animals whereas, in controls, it remained at a level comparable to that of the unrelated immunoglobulin. The antibody seemed slowly trapped by the tumour and, 12 days after its injection, distribution ratios between tumour and mouse organs reached values of 20-30 as against 1 in animals injected with the non-specific immunoglobulin. Autohistoradiographic investigations in the tumour confirmed the slow diffusion rate of the antibody, which remained in the vascular spaces up to the 24th hour after injection and diffused afterwards throughout the clusters of tumor cells. Furthermore, radioactivity was detected in cells which, unexpectedly, seemed morphologically unaltered. These cells, the viability of which remains to be determined, were predominant in the central area of the tumours. The results presented constitute new evidence of the ability of an in vivo injected monoclonal antibody to reach a cytoplasmic target inside non-necrotic cells and suggest that the cells permeable to the antibody might be in defective nutritional conditions.
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