Thrombin Activable Fibrinolysis Inhibitor in Beta Thalassemia

Chhikara, Aruna; Sharma, Sunita; Chandra, Jagdish; Nangia, Anita

doi:10.1007/s12098-016-2208-x

Cited by 10 publications

(11 citation statements)

References 27 publications

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“…Both the aPTT and PT were prolonged in patients compared with controls, which is consistent with other reports that attributed those changes to damage to the liver parenchyma caused by iron overload [18,19]. Although fibrinogen remained within the normal range, it was lower in patients than in controls.…”

Section: Discussionsupporting

confidence: 91%

“…Previous studies have reported decreased PC and PS activity in β-TM [15,19,21] and Hb E/β-thal [22], and decreased PC in β-TI [23]. We observed a significant decrease of PC and PS activity in α-TI and α + β-thal and the PC deficiency was consistent with Type II abnormalities.…”

Section: Discussionsupporting

confidence: 79%

“…AT III activity was within the normal reference range, but was reduced in all patient groups compared to controls. Others have reported similar AT III activity levels in β-TM [19] and Hb E/β-thal [22], but did not refer to antigen. The difference might be explained by ongoing subclinical activation of coagulation.…”

Section: Tablementioning

confidence: 89%

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Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin

Huang¹,

Yuan²,

Deng

et al. 2018

Thrombosis Research

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Background: Thalassemia is characterized by a hypercoagulable state in which the protein C (PC) pathway controls thrombosis. We investigated changes in PC, protein S (PS), antithrombin III (AT III) and soluble endothelial protein C receptor (sEPCR) in thalassemia. Methods: A group of 129 patients with β-thalassemia major (β-TM), β-thalassemia intermedia (β-TI), α-thalassemia intermedia (α-TI) and combined α-/β-thalassemia (α + β-thal) were compared with 32 gender-and age-matched controls. PC, PS, AT III, sEPCR, thrombin-antithrombin complex (TAT), and intercellular adhesion molecule1 (ICAM-1) antigens were measured by enzyme-linked immunosorbent assay. PC, AT III, and PS activity were assayed by substrate chromatography and a prothrombin time (PT)-based free protein S assay. Results: PC deficiency was seen in 95.3% of the patients and PS deficiency was seen in 77.5%. Concomitant reductions in PC and AT III antigen and activity were observed in β-TM, β-TI, and α-TI than in controls (p < 0.005). PC activity was lower in β-TM than in α-TI (p = 0.004). PS antigen was elevated in β-TM (p = 0.011) and sEPCR was elevated in α-TI (p = 0.018). Nonsplenectomized patients had lower PC (p = 0.001) and PS (p = 0.006) and higher sEPCR (p = 0.021) than postsplenectomy patients. Transfusion dependent thalassemia (TDT) patients had lower PC levels (p < 0.005) than those with nontransfusion dependent thalassemia (NTDT). ICAM-1 was increased in patient subgroups (p < 0.001), especially those with splenectomies (p = 0.009), and TAT was increased in all patient subgroups compared with controls (p < 0.001) except for α + β-thal. Conclusions: Deficiencies of anticoagulant proteins and elevated sEPCR contributed to chronic hypercoagulability in these thalassemia patients of Chinese origin. Splenectomy alleviated these alterations in this patient cohort with the median duration since splenectomy of two years. Blood transfusion was not ideal for avoiding thrombosis. 1. Introduction Mutations or deletions of globin genes in thalassemia result in an imbalance of globin synthesis, decreased erythropoiesis, and extravascular hemolysis. Hypercoagulability states are common occurrences in thalassemia patients in southeast Asia, the Mediterranean, and the Middle East regions [1-3]. The prevalence of β-thalassemia is approximately 4.91% and that of α-thalassemia is about 14.13% in the Guangxi province of China, and the various causes of coagulation disorders deserve investigation [4]. Accumulation of unmatched globin chains results in premature destruction of erythrocyte precursors in bone marrow, hemolysis of erythrocytes in the peripheral blood, and the appearance of abnormal red blood cells (RBCs). Loss of deformability impairs passage of RBCs through capillaries [5], and the resulting hemostasis may elicit a procoagulant condition [6]. Other reasons may include endothelial injury, platelet activation, increased plasma microparticles, impaired nitric oxide bioavailability, increased blood oxidants, and phosphatidylserine exposure on the outer ...

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 79%

See 1 more Smart Citation

Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin

Huang¹,

Yuan²,

Deng

et al. 2018

Thrombosis Research

View full text Add to dashboard Cite

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“…Values of naturally occurring anticoagulants in betathalassemia patients are suggested to be low with increased risk of TEE [5][6][7][8]. Nevertheless, such issue is still debatable among researchers [9,10]. While some found low levels of natural anticoagulants [7,8], others found normal values [9,10].…”

Section: Introductionmentioning

confidence: 99%

“…Nevertheless, such issue is still debatable among researchers [9,10]. While some found low levels of natural anticoagulants [7,8], others found normal values [9,10]. In this study, we investigated the level of anti-thrombin-III (AT-III) and protein C among a group of beta-thalassemia children and correlated to different clinical and laboratory features in order to help hematologists to establish of a prophylactic policy for each patient according to his risk.…”

Section: Introductionmentioning

confidence: 99%

Protein C and Anti-Thrombin-III Deficiency in Children With Beta-Thalassemia

et al. 2018

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Background: Thromboembolic events (TEEs) are recently described complications in thalassemia patients. Many mechanisms were postulated for thrombosis. Conflicting results of natural anticoagulants values were reported in previous studies. Our aim was to investigate protein C and anti-thrombin-III (AT-III) levels in thalassemics and to detect risk factors for their decrement. Methods: A cross-sectional study for 60 beta-thalassemia patients (35 major and 25 intermedia) and 35 healthy children were tested for protein C and AT-III levels, liver function tests and Sr. ferritin. Results: A significant reduction in protein C and AT-III levels was noticed in patient group compared to healthy children (82.50% (32-175) vs. 104% (60-204), P = 0.041 and 237.52 ± 53.19 mg/L vs. 322.99 ± 56.57 mg/L, P value ≤ 0.001, respectively). Protein C was lower among older patients (> 10 years) than younger patients (< 10 years), and splenectomized category than non-splenectomized one (P = 0.02 and 0.011, respectively). AT-III was significantly lower among splenectomized patients as compared to those who did not undergo splenectomy (P = 0.04). Significant correlations were found between protein C and AT-III with older age and liver functions. Conclusions: Protein C and AT-III were significantly lower among thalassemics with the main risk factors for their deficiencies being: splenectomy and increasing age. This allows establishment of early prophylactic policy against TEE for the vulnerable group.

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Strain Screening from Traditional Fermented Soybean Foods and Induction of Nattokinase Production in Bacillus subtilis MX-6

Man

Xiang

Zhang

2018

Probiotics & Antimicro. Prot.

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The plasminogen-free fibrin plate assay method was used to isolate Bacillus subtilis MX-6, a strain with high production of nattokinase from Chinese douchi. The presence of aprN, a gene-encoding nattokinase, was verified with PCR method. The predicted amino acid sequence was aligned with homologous sequences, and a phylogenetic tree was constructed. Nattokinase was sublimated with ammonium sulfate, using a DEAE-Sepharose Fast Flow column, a CM-Sepharose Fast Flow column and a Sephadex G-75 gel filtration column. SDS-PAGE analysis indicated that the molecular weight of the purified nattokinase from Bacillus subtilis MX-6 was about 28 kDa. Fermentation of Bacillus subtilis MX-6 nattokinase showed that nattokinase production was maximized after 72 h; the diameter of clear zone reached 21.60 mm on the plasminogen-free fibrin plate. Nattokinase production by Bacillus subtilis MX-6 increased significantly after supplementation with supernatant I, supernatant II and soy peptone but decreased substantially after the addition of amino acids. This result indicated that the nattokinase production by B. subtilis MX-6 might be induced by soybean polypeptides. The addition of MgSO and CaCl increased B. subtilis MX-6 nattokinase production.

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Thrombin Activable Fibrinolysis Inhibitor in Beta Thalassemia

Cited by 10 publications

References 27 publications

Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin

Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin

Protein C and Anti-Thrombin-III Deficiency in Children With Beta-Thalassemia

Strain Screening from Traditional Fermented Soybean Foods and Induction of Nattokinase Production in Bacillus subtilis MX-6

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