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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.

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Nowadays is death an extremely invazie de helmint pe termen lung occurrence. 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.

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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 the clinical presentation and the natural history of patients with different forms of PAP in patients of all age groups, including neonates, children and adults.

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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 WLL 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 invazie de helmint pe termen lung the PAP epidemiology, and, most importantly, 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. Curr Opin Pulm Med 15 5 : Pulmonary alveolar proteinosis: progress in the first 44 years. Characteristics of a invazie de helmint pe termen lung cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Pulmonary alveolar proteinosis: New insights from a single-center cohort of 70 patients.

Respir Med 12 : Assessment and management of pulmonary alveolar proteinosis in a reference center. Orphanet J Rare Dis 8: Pulmonary alveolar proteinosis in Invazie de helmint pe termen lung a systematic review of cases.

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Respirology 14 5 : Experience with treatment of pulmonary alveolar proteinosis from a tertiary care centre in north India. Carey B, Trapnell BC.

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The molecular basis of pulmonary alveolar proteinosis. Clinical immunology 2 : Clinical features of secondary pulmonary alveolar proteinosis: pre-mortem cases in Japan. Eur Respir J 37 2 : Alveolar lipoproteinosis syndromes. In: Cordier JF, ed.

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European Respiratory Society, ; pp. Costabel U, Guzman J. Pulmonary alveolar proteinosis: a new autoimmune disease. J Exp Med 6 : Gm-CSF regulates pulmonary surfactant homeostasis and alveolar macrophage-mediated innate host defense. Annu Rev Physiol Hereditary pulmonary alveolar proteinosis: pathogenesis, presentation, diagnosis, and therapy.

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 and 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.

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J Appl Physiol 2 : Blood 92 4 : Secondary pulmonary invazie de helmint pe termen lung proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan. BMC Pulm Med Experimental 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.

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Respir Res 6: Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease. Am J Med Genet A 1 : Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene.

Medicament pentru giardiaza preț In general, severitatea simptomelor depinde de varsta, de perioada de timp de la debutul a bolii si gravitatea conditiei. Simptomele pot fi atat de usoare si pot aparea atat de lent, incat pot trece neobservate ani de zile. Odata cu inaintarea in varsta, simptomele sunt mai notabile. Copiii cu risc sunt cei care nu acordă suficientă atenție igienei.

Pediatr Res 57 1 : Acta Paediatr 96 2 : Bioorg Med Chem 14 17 : A large kindred of pulmonary fibrosis associated with a novel ABCA3 gene variant. Respir Res Heterogeneous pulmonary phenotypes associated with mutations in the thyroid transcription factor gene NKX Chest 3 : Chest 4 : Bronchoalveolar lavage in other interstitial lung diseases.

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Semin Respir Crit Care Med 28 5 : In: Non-neoplastic disorders of the lower respiratory tract. Atlas of Non-tumor Pathology.

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Pulmonary surfactant protein D in sera and bronchoalveolar lavage fluids. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis.

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Thorax 55 1 : Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review. Mayo Clin Proc Digital clubbing associated with primary alveolar proteinosis: possible implication of growth factors. Presse medicale Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan.

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Respirology 11 Suppl: 55SS. Pulmonary alveolar proteinosis: clinical features and outcomes.

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Chest 5 panacur gatti giardia Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations. Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis.

Radiographics 23 6 : Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings. Radiology 1 : High-resolution CT in the evaluation of clinically suspected Pneumocystis carinii pneumonia in AIDS patients with normal, equivocal, or nonspecific radiographic findings.

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Radiology 3 : Chest 91 3 : Pneumocystis carinii pneumonia: spectrum of parenchymal CT findings. 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 lavage fluid in pulmonary alveolar proteinosis.

Eur Respir J 8 5 : CYFRA as a disease severity marker for autoimmune pulmonary alveolar proteinosis.

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Respirology Respir Investig 50 1 : Serum KL-6 level as a monitoring marker in a patient with pulmonary alveolar proteinosis.