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

Alpha-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 infl...

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Published in:	Frontiers in immunology Vol. 8; p. 1491
Main Authors:	Carvalho, Magda Oliveira Seixas, Souza, André Luís Carvalho Santos, Carvalho, Mauricio Batista, Pacheco, Ana Paula Almeida Souza, Rocha, Larissa Carneiro, do Nascimento, Valma Maria Lopes, Figueiredo, Camylla Vilas Boas, Guarda, Caroline Conceição, Santiago, Rayra Pereira, Adekile, Adekunle, Goncalves, Marilda de Souza
Format:	Journal Article
Language:	English
Published:	Switzerland Frontiers Media S.A 06-11-2017
Subjects:	alpha-1 antitrypsin biomarkers Immunology inflammation SERPINA1 sickle cell disease alpha-1 antitrypsin SERPINA1 inflammation biomarkers sickle cell disease
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Summary:	Alpha-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 of gene 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, = 0.02), gallstones (OR = 1.75, CI: 1.03-2.97, = 0.02), and had more blood transfusions (OR = 2.35, CI: 1.51-3.65, = 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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 Edited by: Jixin Zhong, Case Western Reserve University, United States Specialty section: This article was submitted to Inflammation, a section of the journal Frontiers in Immunology Reviewed by: Yonglin Chen, Yale University, United States; Juerg Hamacher, Lindenhof Hospital, Switzerland
ISSN:	1664-3224 1664-3224
DOI:	10.3389/fimmu.2017.01491