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        "resumen" => "<span id="as0005" class="elsevierStyleSection elsevierViewall"><p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">A alfa-1 antitripsina &#40;AAT&#41; &#233; sintetizada pelo f&#237;ga-do&#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="sp0010" 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<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#46; O limiar de protec&#231;&#227;o &#233; 80<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#46; O ta-bagismo aumenta francamente o risco de enfisema nestes doentes&#46;</p><p id="sp0015" 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="sp0020" 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 se-manal com prolastina&#174;&#46;</p><p id="sp0025" 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="sp0030" class="elsevierStyleSimplePara elsevierViewall">A reposi&#231;&#227;o com prolastina&#174; &#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 mortalida-de&#44; ainda n&#227;o foi poss&#237;vel fazer essa infer&#234;ncia na nossa pequena amostra&#46;</p><p id="sp0035" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2009&#59; XV &#40;3&#41;&#58; 473-481</span></p></span>"
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        "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0040" 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="sp0045" 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<span class="elsevierStyleHsp" style=""></span>mg&#47; dl&#46; The &#8220;protective threshold&#8221; is 80<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#46; Smoking increases the risk of emphysema&#46;</p><p id="sp0050" 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="sp0055" 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&#174;&#46; We performed a clinical&#44; respiratory functional and radiological evaluation between 2003 and 2007&#46;</p><p id="sp0060" 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="sp0065" 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; <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><p id="sp0070" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2009&#59; XV &#40;3&#41;&#58; 473-481</span></p></span>"
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Vol. 15. Issue 3.
Pages 473-482 (May - June 2009)
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Vol. 15. Issue 3.
Pages 473-482 (May - June 2009)
Artigo de Revisão/Review Article
Open Access
Défice de alfa-1 antitripsina. A experiência do Hospital de Pulido Valente com a terapêutica de reposição
Alpha-1 antitrypsin deficiency. The experience of Pulido Valente Hospital with augmentation therapy
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Carla Alves Costa1, Cristina Santos2
1 Interna do Internato Complementar de Pneumologia, Hospital de Dia de Insuficientes Respiratórios; Unidade de Reabilitação Respiratória, Responsável: Fátima Rodrigues Departamento de Pneumologia do Hospital de Pulido Valente, CHLN, EPE
2 Assistente Hospitalar Graduada de Pneumologia, Hospital de Dia de Insuficientes Respiratórios; Unidade de Reabilitação Respiratória, Responsável: Fátima Rodrigues Departamento de Pneumologia do Hospital de Pulido Valente, CHLN, EPE
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Resumo

A alfa-1 antitripsina (AAT) é sintetizada pelo fíga-do, com uma semivida plasmática de 4-5 dias. Apresenta acção inibidora das proteases, com particular afinidade para a elastase dos neutrófilos. A sua deficiência está associada a uma menor protecção pulmonar da acção das enzimas dos neutrófilos activados.

A deficiência de AAT é uma doença genética resultante da herança de dois alelos deficientes. Dos alelos deficientes, o mais frequente é o Pi*Z, sendo a forma homozigótica Pi*ZZ responsável por níveis séricos mais baixos, habitualmente inferiores a 50mg/dl. O limiar de protecção é 80mg/dl. O ta-bagismo aumenta francamente o risco de enfisema nestes doentes.

O objectivo da terapêutica de reposição é a manuten-ção de níveis séricos de AAT acima do limiar protector, retardando a progressão da doença.

Os autores apresentam a experiência do Hospital de Dia de Insuficientes Respiratórios do Hospital de Pulido Valente, de cinco doentes com enfisema por deficiência de AAT, fazendo reposição endovenosa se-manal com prolastina®.

Foi efectuada uma avaliação clínica, funcional respiratória e radiológica dos doentes entre 2003 e 2007. Verificou-se estabilidade clínica e radiológica e menor declínio anual de FEV1 após início do tratamento.

A reposição com prolastina® é um tratamento de custos elevados, havendo falta de estudos aleatorizados e controlados que demonstrem a sua eficácia clínica. A evidência do benefício é baseada em estudos observacionais. A nossa experiência é positiva, com benefícios clínicos, funcionais e radiológicos. Apesar de estar descrita na literatura uma redução da mortalida-de, ainda não foi possível fazer essa inferência na nossa pequena amostra.

Rev Port Pneumol 2009; XV (3): 473-481

Palavras-chave:
lfa-1 antitripsina
terapêutica de reposição
enfisema
Abstract

Alpha-1 antitrypsin (AAT) is synthesised in the liver and has half-life of 4-5 days. AAT has antiprotease activity, with particular affinity for neutrophil elastase. Its deficiency leads to a lack of effective lung protection against activated neutrophil enzymes.

Deficiency of AAT is a genetic disorder that occurs as a result of the inheritance of two protease inhibitor deficient alleles. Of the deficient alleles, Pi*Z is the most common, and the homozygous form Pi*ZZ results in the lowest serum levels, usually below 50mg/ dl. The “protective threshold” is 80mg/dl. Smoking increases the risk of emphysema.

The current goal of augmentation therapy is to raise the plasma levels, above protective threshold and slow disease progression.

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, receiving weekly intravenous treatment with Prolastin®. We performed a clinical, respiratory functional and radiological evaluation between 2003 and 2007.

The results point to a slower progression of the disease, with clinical and radiological stability and a reduced rate of FEV1 decline.

Augmentation therapy is an expensive treatment and its use is lacking supportive evidence of efficacy by randomized controlled clinical trials. Evidence that it confers benefits is based on observational studies. Our experience is positive, showing clinical, radiological and functional benefits. The literature available points to a decrease in mortality, but we could not affirm so in our small population.

Rev Port Pneumol 2009; XV (3): 473-481

Key-words:
Alpha-1 antitrypsin
augmentation therapy
emphysema
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Copyright © 2009. Sociedade Portuguesa de Pneumologia/SPP
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