Evaluation of Alpha-1 Antitrypsin Levels and SERPINA1 Gene Polymorphisms in Sickle Cell Disease

dc.creatorCarvalho, Magda Oliveira Seixas
dc.creatorSouza, André Luís Carvalho Santos
dc.creatorCarvalho, Mauricio Batista
dc.creatorPacheco, Ana Paula Almeida Souza
dc.creatorRocha, Larissa Carneiro
dc.creatorNascimento, Valma Maria Lopes do
dc.creatorFigueiredo, Camylla Vilas Boas
dc.creatorGuarda, Caroline Conceição
dc.creatorSantiago, Rayra Pereira
dc.creatorAdekile, Adekunle D.
dc.creatorGoncalves, Marilda de Souza
dc.date2018-02-27T17:22:46Z
dc.date2018-02-27T17:22:46Z
dc.date2017
dc.date.accessioned2023-09-26T22:11:51Z
dc.date.available2023-09-26T22:11:51Z
dc.identifierCARVALHO, Magda Oliveira Seixas et al. Evaluation of Alpha-1 Antitrypsin Levels andSERPINA1Gene Polymorphisms in Sickle Cell Disease. Frontiers in Immunology, v. 8, Article 1491, p. 1-11, Nov. 2017.
dc.identifier1664-3224
dc.identifierhttps://www.arca.fiocruz.br/handle/icict/24985
dc.identifier10.3389/fimmu.2017.01491
dc.identifier.urihttps://repositorioslatinoamericanos.uchile.cl/handle/2250/8876101
dc.descriptionBrazilian National Council of Research (CNPq) (311888/2013-5) (MG); the Foundation of Research and Extension of Bahia (FAPESB) (3626/2013, 1431040053063, and 9073/2007) (MG); and PPSUS/ FAPESB (020/2013 EFP_00007295) (MG) and MCD/CNPq/MS-SCTIE-DECIT (409800/2006-6) (MG).
dc.descriptionAlpha-1 antitrypsin (AAT) is an inhibitor of neutrophil elastase and a member of the serine proteinase inhibitor (serpin) superfamily, and little is known about its activity in sickle cell disease (SCD). We hypothesize that AAT may undergo changes in SCD because of the high oxidative stress and inflammation associated with the disease. We have found high AAT levels in SCD patients compared to controls, while mutant genotypes ofSERPINA1gene had decreased AAT levels, in both groups. AAT showed negative correlation with red blood cells, hemoglobin (Hb), hematocrit, high-density lipoprotein cholesterol, urea, creatinine, and albumin and was positively correlated with mean corpuscular Hb concentration, white blood cells, neutrophils, Hb S, bilirubin, lactate dehydrogenase, ferritin, and C-reactive protein. Patients with higher levels of AAT had more infection episodes (OR = 1.71, CI: 1.05-2.65,p = 0.02), gallstones (OR = 1.75, CI: 1.03-2.97,p = 0.02), and had more blood transfusions (OR = 2.35, CI: 1.51-3.65,p = 0.0001). Our data on AAT association with laboratory indices of hemolysis and inflammation suggest that it may be positively associated with SCD severity; the negative correlations with renal parameters suggest a cytoprotective mechanism in SCD patients. In summary, AAT may need to be included in studies related to SCD and in the discussion of further therapeutic strategies.
dc.formatapplication/pdf
dc.languageeng
dc.publisherFrontiers Media
dc.rightsopen access
dc.subjectDoença Falciforme
dc.subjectAlfa-1 antitripsina
dc.subjectSERPINA1
dc.subjectBiomarcadores
dc.subjectInflamação
dc.subjectSickle Cell Disease
dc.subjectAlpha-1 antitrypsin
dc.subjectSERPINA1
dc.subjectBiomarkers
dc.subjectInflammation
dc.titleEvaluation of Alpha-1 Antitrypsin Levels and SERPINA1 Gene Polymorphisms in Sickle Cell Disease
dc.typeArticle


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