Outcome and prognosis Prognosis of PAP has improved considerably with introduction of whole lung lavage. In the era of therapeutic lavage infectious complications like nocardiosis, cryptococcosis, mucormycosis have became rare.
Nowadays is death an extremely rare occurrence.
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Lung transplantation may be an option for patients developing progressive interstitial pulmonary fibrosis, although recurrence of PAP has been reported in one patient 3 years after double-lung transplantation For secondary PAP associated with hematologic malignancy, the prognosis is linked to the underlying disease and is generally worse than in autoimmune PAP 2, 5, 10, The project was founded by the European Agency for Rare Disease E-RARE project based on cohorts already available in the centers, it is expected the enrollment of one of the largest series of PAP patients ever reported, in order to better understand about the epidemiology of the different forms of PAP in Europe.
The aim is to create a database containing useful information to correlate clinical data with the analysis of biomaterial obtained from PAP patients. The overall perspective of the program is to gain more information about invazie de helmint a bronhiilor clinical presentation and the natural history of patients with invazie de helmint a bronhiilor forms of PAP in patients of all age groups, including neonates, children and adults. In EuPAPNet project, patients have been added to a functional SNP-array, containing genetic variations with proven and presumed functionality, to identify genomic regions and genes in humans which provide candidates for PAP susceptibility; furthermore pediatric PAP forms have been identified and characterized genetically.
Through a proof-of-concept approach it has been found that some biomarkers, like serum KL-6 and YKL40, are valid to predict outcome disease outcome and response to whole lung lavage in PAP Finally, as Invazie de helmint a bronhiilor is not yet a procedure standardised and is still suffering from lack of information, both clinically and technically, an international survey was launched, to answer many open questions related to lavage in PAP In summary the transnational collaboration in EuPAPNet project is a unique opportunity to ensure a translation of basic research into clinical management of PAP patients, and in turn to create the basis for the implementation of the database in a European-wide basis.
This would result in the organization of a European registry for PAP, with the aim of both improving the knowledge of the PAP epidemiology, and, most viermi vărsători, of creating awareness among pulmonologists about this disorder and its management.
Pulmonary alveolar proteinosis. N Engl J Med 23 : Huizar I, Kavuru MS. Alveolar proteinosis syndrome: pathogenesis, diagnosis, and management.
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J Clin Invest 9 : Safety and efficacy of ARA in sarcoidosis patients with symptoms of small fiber neuropathy: a randomized, double-blind pilot study. Mol Med J Exp Med 12 : Conclusion Basic invazie de helmint a bronhiilor translational research in the last decade has allowed an improvement in the diagnosis and classification of alveolar proteinosis disorders.
Additional studies are needed to define clinical and radiological features as well as pathogenetic mechanisms in secondary PAP.
Pulmonary alveolar proteinosis in SCID mice. Alveolar macrophage depletion is associated with increased surfactant pool sizes in adult rats. J Appl Physiol 2 : Blood 92 4 : Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan.
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BMC Pulm Med Invazie de helmint a bronhiilor alveolar lipoproteinosis following the inhalation of silica. J Pathol 4 : Pulmonary alveolar proteinosis in workers at an indium processing facility.
Nat Genet 21 3 : Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report.
Orphanet J Rare Dis 2: Hum Mutat 14 6 : A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds. J Clin Invest 93 4 : Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease. Respir Res 6: Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease.
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- Această peliculă menţine volumul constant al alveolelor şi nu le permite să se strângă.
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Radiology 3 : Chest 91 3 : Pneumocystis carinii pneumonia: spectrum of parenchymal CT findings.
Această peliculă menţine volumul constant al alveolelor şi nu le permite să se strângă. În afară de aceasta, substanţa bioactivă a peliculei neutralizează microorganismele, care pătrund în plămâni odată cu aerul. În caz de pneumonie, tuberculoză şi alte boli pulmonare infecţioase pelicula poate fi vătămată, alveolele pulmonare pot să se strângă şi nu pot participa la metabolismul gazos. La fumători alveolele îşi pierd elasticitatea şi capacitatea de a se curăţi, pelicula devine rigidă din cauza substanţelor toxice din fumul de tutu.
Serum KL-6 is a predictor of outcome in pulmonary alveolar proteinosis. Blood Leukemia 22 4 : Gastroenterology 4 : PUlmonary alveolar proteinosis: shunt fraction and lactic acid dehydrogenase concentration as aids to diagnosis. Am Rev Respir Dis 6 : Thorax 58 3 : Elevation of tumour markers in serum and bronchoalveolar invazie de helmint a bronhiilor fluid in pulmonary alveolar proteinosis.
Eur Respir J 8 5 : CYFRA as a disease severity marker for autoimmune pulmonary alveolar proteinosis. Respirology Respir Investig 50 1 : Serum KL-6 level as a monitoring marker in a patient with pulmonary alveolar proteinosis.
Thorax 53 9 : Clin Chest Med 25 3 invazie de helmint a bronhiilor