Abstract
IntroductionPatients with sickle cell anemia (SCA) may present chronic hemolytic anemia, vaso-occlusion and respiratory tract infection (RTI) episodes. Galectin-3 (GAL-3) is a multifunctional protein involved in inflammation, apoptosis, adhesion and resistance to reactive oxygen species. Studies point to a dual role for GAL-3 as both a circulation damage-associated molecular pattern and a cell membrane associated pattern recognition receptor.ObjectiveTo investigate associations between the SNPs of GAL-3 gene (LGALS3) and serum levels with RTI and vaso-occlusive crisis (VOC) in children with SCA.Materials and MethodsSNPs +191 and +292 in LGALS3 were studied using the TaqMan real-time PCR system; GAL-3 serum levels were measured by ELISA. The study included 79 children with SCA ranging from 2 to 12 years old.ResultsGAL-3 serum levels were associated with LGALS3 +191 and +292 genotypes (p <0.0001; p = 0.0169, respectively). LGALS3 +191, AA genotype was associated with low and CC with higher levels of GAL-3. For LGALS3 +292, the CC genotype was associated with lower GAL-3 and AA with higher levels. Patients with Frequency of RTI (FRTI) ≥1 presented higher frequency of +191AA (p = 0.0263) and +292AC/CC genotypes (p = 0.0320). SNP +292 was associated with Frequency of VOC (FVOC) (p = 0.0347), whereas no association was shown with SNP +191 and FVOC. However, CA/AC and AA/CC genotypes with lower GAL-3 levels showed a higher frequency in patients with FRTI ≥1 (p = 0.0170; p = 0.0138, respectively). Also, patients with FVOC ≥1 presented association with CA/AC (p = 0.0228). LGALS3 +191 and +292 combined genotypes related to low (p = 0.0263) and intermediate expression (p = 0.0245) were associated with FRTI ≥1. Lower GAL-3 serum levels were associated with FRTI ≥1 (p = 0.0426) and FVOC ≥1 (p = 0.0012).ConclusionVariation of GAL-3 serum levels related to SNPs at +191 and +292 may constitute a susceptibility factor for RTI and VOC frequency.
Highlights
Patients with sickle cell anemia (SCA) may present chronic hemolytic anemia, vaso-occlusion and respiratory tract infection (RTI) episodes
SNP +292 was associated with Frequency of vaso-occlusive crisis (VOC) (FVOC) (p = 0.0347), whereas no association was shown with SNP +191 and FVOC
Lower GAL-3 serum levels were associated with Frequency of RTI (FRTI) 1 (p = 0.0426) and FVOC 1 (p = 0.0012)
Summary
Seventy-nine children with SCA, aged 2 to 12 years with a median of 5 years (52% males) were studied. The patients enrolled in this study were attended in an ambulatory for hemoglobin disorders and neonatal screening, from 2002 until 2014, diagnosed at the Blood Center of Pernambuco (Hemope Foudation), using electrophoresis of Hemoglobin and High Pressure Liquid Chromatography (HPLC) (BioRad, Hercules, CA, USA). All children were vaccinated against pneumococcus (7 Pneumo-Wyeth Pharmaceuticals Inc. USA) and meningococcus (Meningitec-Wyeth Pharmaceuticals, UK) and used penicillin (4000 U dose/kg/day) prophylactically according to the national protocol. Epidemiological, clinical and laboratory data were obtained from standardized medical records provided by the Hemope Foundation. Clinical data were considered before initiating treatment with hydroxyurea (HU). For assessment of the GAL-3 serum concentration, children who received a transfusion in the last 3 months were excluded
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