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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A alfa-1 antitripsina &#40;AAT&#41; &#233; sintetizada pelo f&#237;gado&#44; com uma semivida plasm&#225;tica de 4-5 dias&#46; Apresenta ac&#231;&#227;o inibidora das proteases&#44; com particular afinidade para a elastase dos neutr&#243;filos&#46; A sua defici&#234;ncia est&#225; associada a uma menor protec&#231;&#227;o pulmonar da ac&#231;&#227;o das enzimas dos neutr&#243;filos activados&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A defici&#234;ncia de AAT &#233; uma doen&#231;a gen&#233;tica resultante da heran&#231;a de dois alelos deficientes&#46; Dos alelos deficientes&#44; o mais frequente &#233; o Pi&#42;Z&#44; sendo a forma homozig&#243;tica Pi&#42;ZZ respons&#225;vel por n&#237;veis s&#233;ricos mais baixos&#44; habitualmente inferiores a 50 mg&#47;dl&#46; O limiar de protec&#231;&#227;o &#233; 80 mg&#47;dl&#46; O tabagismo aumenta francamente o risco de enfisema nestes doentes&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">O objectivo da terap&#234;utica de reposi&#231;&#227;o &#233; a manuten&#231;&#227;o de n&#237;veis s&#233;ricos de AAT acima do limiar protector&#44; retardando a progress&#227;o da doen&#231;a&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Os autores apresentam a experi&#234;ncia do Hospital de Dia de Insuficientes Respirat&#243;rios do Hospital de Pulido Valente&#44; de cinco doentes com enfisema por defici&#234;ncia de AAT&#44; fazendo reposi&#231;&#227;o endovenosa semanal com prolastina<span class="elsevierStyleSup">&#174;</span>&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Foi efectuada uma avalia&#231;&#227;o cl&#237;nica&#44; funcional respirat&#243;ria e radiol&#243;gica dos doentes entre 2003 e 2007&#46; Verificou-se estabilidade cl&#237;nica e radiol&#243;gica e menor decl&#237;nio anual de FEV<span class="elsevierStyleInf">1</span> ap&#243;s in&#237;cio do tratamento&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">A reposi&#231;&#227;o com prolastina<span class="elsevierStyleSup">&#174;</span> &#233; um tratamento de custos elevados&#44; havendo falta de estudos aleatorizados e controlados que demonstrem a sua efic&#225;cia cl&#237;nica&#46; A evid&#234;ncia do benef&#237;cio &#233; baseada em estudos observacionais&#46; A nossa <span class="elsevierStyleBold">experi&#234;ncia &#233; positiva&#44; com benef&#237;cios cl&#237;nicos&#44; funcionais e radiol&#243;gicos</span>&#46; Apesar de estar descrita na literatura uma redu&#231;&#227;o da mortalidade&#44; ainda n&#227;o foi poss&#237;vel fazer essa infer&#234;ncia na nossa pequena amostra&#46;</p>"
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        "resumen" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Alpha-1 antitrypsin &#40;AAT&#41; is synthesised in the liver and has half-life of 4-5 days&#46; AAT has antiprotease activity&#44; with particular affinity for neutrophil elastase&#46; Its deficiency leads to a lack of effective lung protection against activated neutrophil enzymes&#46;</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Deficiency of AAT is a genetic disorder that occurs as a result of the inheritance of two protease inhibitor deficient alleles&#46; Of the deficient alleles&#44; Pi&#42;Z is the most common&#44; and the homozygous form Pi&#42;ZZ results in the lowest serum levels&#44; usually below 50 mg&#47; dl&#46; The &#8220;protective threshold&#8221; is 80 mg&#47;dl&#46; Smoking increases the risk of emphysema&#46;</p><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">The current goal of augmentation therapy is to raise the plasma levels&#44; above protective threshold and slow disease progression&#46;</p><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">The authors present the experience of the Day Care Hospital of the Pulido Valente Hospital with five male patients presenting emphysema due to AAT deficiency&#44; receiving weekly intravenous treatment with Prolastin<span class="elsevierStyleSup">&#174;</span>&#46; We performed a clinical&#44; respiratory functional and radiological evaluation between 2003 and 2007&#46;</p><p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">The results point to a slower progression of the disease&#44; with clinical and radiological stability and a reduced rate of FEV<span class="elsevierStyleInf">1</span> decline&#46;</p><p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Augmentation therapy is an expensive treatment and its use is lacking supportive evidence of efficacy by randomized controlled clinical trials&#46; Evidence that it confers benefits is based on observational studies&#46;</p><p id="spar0065" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Our experience is positive&#44; showing clinical&#44; radiological and functional benefits</span>&#46; The literature available points to a decrease in mortality&#44; but we could not affirm so in our small population&#46;</p>"
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Défice de alfa-1 antitripsina. A experiência do Hospital de Pulido Valente com a terapêutica de reposição
Carla Alves Costa1, Cristina Santos2, Jaime Pina3
1 Interna do Internato Complementar de Pneumologia
2 Assistente Hospitalar Graduada de Pneumologia
3 Hospital de Dia de Insuficientes Respiratórios; Unidade de Reabilitação Respiratória, Responsável: Fátima Rodrigues, Departamento de Pneumologia do Hospital de Pulido Valente, CHLN, EPE, Director: Jaime Pina, Departamento de Pneumologia, Hospital de Pulido Valente EPE, Alameda das Linhas Torres, n.° 117, 1769-001 Lisboa
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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A alfa-1 antitripsina &#40;AAT&#41; &#233; sintetizada pelo f&#237;gado&#44; com uma semivida plasm&#225;tica de 4-5 dias&#46; Apresenta ac&#231;&#227;o inibidora das proteases&#44; com particular afinidade para a elastase dos neutr&#243;filos&#46; A sua defici&#234;ncia est&#225; associada a uma menor protec&#231;&#227;o pulmonar da ac&#231;&#227;o das enzimas dos neutr&#243;filos activados&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A defici&#234;ncia de AAT &#233; uma doen&#231;a gen&#233;tica resultante da heran&#231;a de dois alelos deficientes&#46; Dos alelos deficientes&#44; o mais frequente &#233; o Pi&#42;Z&#44; sendo a forma homozig&#243;tica Pi&#42;ZZ respons&#225;vel por n&#237;veis s&#233;ricos mais baixos&#44; habitualmente inferiores a 50 mg&#47;dl&#46; O limiar de protec&#231;&#227;o &#233; 80 mg&#47;dl&#46; O tabagismo aumenta francamente o risco de enfisema nestes doentes&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">O objectivo da terap&#234;utica de reposi&#231;&#227;o &#233; a manuten&#231;&#227;o de n&#237;veis s&#233;ricos de AAT acima do limiar protector&#44; retardando a progress&#227;o da doen&#231;a&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Os autores apresentam a experi&#234;ncia do Hospital de Dia de Insuficientes Respirat&#243;rios do Hospital de Pulido Valente&#44; de cinco doentes com enfisema por defici&#234;ncia de AAT&#44; fazendo reposi&#231;&#227;o endovenosa semanal com prolastina<span class="elsevierStyleSup">&#174;</span>&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Foi efectuada uma avalia&#231;&#227;o cl&#237;nica&#44; funcional respirat&#243;ria e radiol&#243;gica dos doentes entre 2003 e 2007&#46; Verificou-se estabilidade cl&#237;nica e radiol&#243;gica e menor decl&#237;nio anual de FEV<span class="elsevierStyleInf">1</span> ap&#243;s in&#237;cio do tratamento&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">A reposi&#231;&#227;o com prolastina<span class="elsevierStyleSup">&#174;</span> &#233; um tratamento de custos elevados&#44; havendo falta de estudos aleatorizados e controlados que demonstrem a sua efic&#225;cia cl&#237;nica&#46; A evid&#234;ncia do benef&#237;cio &#233; baseada em estudos observacionais&#46; A nossa <span class="elsevierStyleBold">experi&#234;ncia &#233; positiva&#44; com benef&#237;cios cl&#237;nicos&#44; funcionais e radiol&#243;gicos</span>&#46; Apesar de estar descrita na literatura uma redu&#231;&#227;o da mortalidade&#44; ainda n&#227;o foi poss&#237;vel fazer essa infer&#234;ncia na nossa pequena amostra&#46;</p>"
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        "resumen" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Alpha-1 antitrypsin &#40;AAT&#41; is synthesised in the liver and has half-life of 4-5 days&#46; AAT has antiprotease activity&#44; with particular affinity for neutrophil elastase&#46; Its deficiency leads to a lack of effective lung protection against activated neutrophil enzymes&#46;</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Deficiency of AAT is a genetic disorder that occurs as a result of the inheritance of two protease inhibitor deficient alleles&#46; Of the deficient alleles&#44; Pi&#42;Z is the most common&#44; and the homozygous form Pi&#42;ZZ results in the lowest serum levels&#44; usually below 50 mg&#47; dl&#46; The &#8220;protective threshold&#8221; is 80 mg&#47;dl&#46; Smoking increases the risk of emphysema&#46;</p><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">The current goal of augmentation therapy is to raise the plasma levels&#44; above protective threshold and slow disease progression&#46;</p><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">The authors present the experience of the Day Care Hospital of the Pulido Valente Hospital with five male patients presenting emphysema due to AAT deficiency&#44; receiving weekly intravenous treatment with Prolastin<span class="elsevierStyleSup">&#174;</span>&#46; We performed a clinical&#44; respiratory functional and radiological evaluation between 2003 and 2007&#46;</p><p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">The results point to a slower progression of the disease&#44; with clinical and radiological stability and a reduced rate of FEV<span class="elsevierStyleInf">1</span> decline&#46;</p><p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Augmentation therapy is an expensive treatment and its use is lacking supportive evidence of efficacy by randomized controlled clinical trials&#46; Evidence that it confers benefits is based on observational studies&#46;</p><p id="spar0065" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Our experience is positive&#44; showing clinical&#44; radiological and functional benefits</span>&#46; The literature available points to a decrease in mortality&#44; but we could not affirm so in our small population&#46;</p>"
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Pulmonology

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