Thrombin activatable fibrinolysis inhibitor in Behçet's disease

INTRODUCTION: Thrombin activatable fibrinolysis inhibitor (TAFI) is a procarboxypeptidase downregulating plasmin formation, thereby causing a tendency for thrombosis development. Since, Beh?et's disease (BD) is a systemic vasculitis, which is commonly complicated by arterial and venous thrombosis, we aimed to find out plasma TAFI levels in BD, compared with healthy controls. We also searched whether plasma TAFI levels were significantly different between Beh?et's subgroups with and without thrombosis. MATERIALS AND METHODS: In this study, 105 BD patients (M/F: 64/41; mean age 36+/-1 years), followed up by Ege University Rheumatology Department were enrolled. The exclusion criteria were hemophilia, hyperlipidemia, diabetes mellitus, hepatic diseases renal failure, antiphospholipid positivity, oral contraceptive use and pregnancy. Age-and sex-matched healthy controls (n=53) were also included. Plasma TAFI levels were measured by ELISA. Since TAFI is also an acute-phase reactant, we also measured other inflammatory markers such as C-reactive protein (CRP). RESULTS: Plasma TAFI levels were significantly higher in Beh?et's patients (91.1+/-7.4 ng/ml) compared with healthy controls (14.3+/-4.5 ng/ml) (P<0.001), but there were no significant difference between the subgroups with and without thrombosis. In BD, there was no correlation between plasma TAFI levels and CRP. CONCLUSIONS: Regardless of manifest thrombosis, plasma TAFI levels in BD were significantly higher than in healthy controls. High TAFI levels might possibly contribute to the thrombotic tendency in BD. Future studies investigating TAFI gene polymorphism and functional activity are clearly needed, to clarify the exact role of TAFI in Beh?et's thrombosis.     

The plasma levels of activated thrombin activatable fibrinolysis inhibitor and thrombomodulin in Behçet Disease and their association with thrombosis

Objective

To investigate the plasma levels of activated thrombin activatable fibrinolysis inhibitor (aTAFI) and thrombomodulin (TM) in Behçet disease (BD) and their relationship with thrombosis.

Methods

Plasma aTAFI and TM levels were measured by ELISA in 89 patients with BD (18 having venous thrombosis) and in 86 healthy controls.

Results

Compared with healthy controls, the BD group had significantly lower levels of aTAFI (13.49 ± 8.88 µg/ml vs. 26.76 ± 11.57 µg/ml, p < 0.0001) and significantly higher levels of TM (3.26 ± 1.85 ng/ml vs. 2.6 ± 0.69 ng/ml, p = 0.0003). Neither aTAFI, nor TM levels differed significantly between BD patients with and without thrombosis (p > 0.05). Despite a tendency to positive correlation (r = 0.37, p = 0.0004) between plasma levels of aTAFI and TM in healthy controls, there was a tendency for negative correlation (r = -0.51, p < 0.0001) between these two parameters in BD patients.

Conclusion

The plasma aTAFI and TM levels do not seem to be related with the presence of thrombosis observed in BD. Increased plasma TM levels in BD may simply reflect endothelial cell activation and dysfunction.     

Plasma concentrations of soluble CD40 ligand in smokers with acute myocardial infarction: a pilot study

Coronary artery disease (CAD) is believed to be the single leading cause of death in both men and women in the world. Smoking is the most important risk factor for CAD. Smoking increases platelet aggregation and thrombus formation. CD40 ligand (CD40L) is a transmembrane glycoprotein derived from activated platelets. It participates in thrombus formation during the acute phase of acute myocardial infarction (MI). Elevation of CD40L identifies the patients who are at highest risk for cardiac events and who are likely to benefit from treatment with the glycoprotein IIb/IIIa (GPIIb/IIIa) receptor antagonists. The purpose of this study was to evaluate levels of CD40L in smokers with acute MI. Fifty-seven patients with acute MI were enrolled in this study. Thirty-one smokers were compared with 26 non-smokers. Soluble CD40L level in the plasma was determined by a standard enzyme-linked immunosorbent assay. Circulating levels of CD40L were higher in the smokers’ group. Smokers with acute MI may have increased risk for thrombotic complications during acute MI, and optimal antiaggregant therapy should be administered.     

Modified Ferriman-Gallwey hirsutism score and androgen levels in Turkish women

Objective(s)

To evaluate a modified Ferriman-Gallwey (mFG) scoring system and serum total testosterone (tT) and dehydroepiandrosterone sulfate (DHEAS) levels, in women with polycystic ovary syndrome (PCOS) and in control subjects.

Study design

Women with any gynecological problem who presented to the Kahramanmaras Sutcuimam University Medical Faculty Gynecology and Obstetric Outpatient Clinic in the Mediterranean region of Turkey were assessed prospectively. Oligo-anovulation, clinical and biochemical hyperandrogenism, and PCO appearance on ultrasound that met the 2003 Rotterdam Consensus Criteria were used for PCOS diagnosis. Group 1 consisted of 43 cases with a diagnosis of PCOS and group 2 (control group) consisted of 75 age and BMI matched control subjects without PCOS.

Results

In groups 1 and 2, clinical hyperandrogenism (hirsutism) appearance ratio was 86.0% (37/43) and 18.0% (15/75) (p < 0.001); while biochemical hyperandrogenism (high serum tT or DHEAS level) was 65.1% (28/43) and 36% (27/75) (p < 0.05), respectively. In groups 1 and 2, mean serum tT levels were 115.2 and 73.4 ng/dL (p < 0.001), mean serum DHEAS levels were 256.1 and 177.7 ng/dL (p < 0.001), and mean hirsutism scores were 11.2 and 5.5 (p < 0.001), respectively. Serum mean tT, DHEAS and hirsutism scores for group 1 were significantly higher than those of group 2 (<0.001). No correlation was observed between BMI and the hirsutism scores or the biochemical hyperandrogenism in both groups.

Conclusion(s)

In our study, mean serum androgen levels and hirsutism ratio in the PCOS group were higher than the control group. These values were also substantially higher than those previously published in the literature.     

Patients with Primary Immunodeficiencies in Pediatric Intensive Care Unit: Outcomes and Mortality-Related Risk Factors

Purposes

The aims of this study were to review the frequency, characteristics, and the clinical course of primary immunodeficiency (PID) patients admitted to pediatric intensive care unit (PICU) and attempt to identify factors related with mortality that might predict a poor outcome.

Methods

We performed a retrospective review of children with PID aged 1 month to 18 years and admitted to PICU from January 2002 to January 2012 in our tertiary teaching children’s hospital.

Results

There were a total of 51 patients accounting for 71 admissions to the PICU. The most common diagnosis was severe combined immunodeficiency. Respiratory problems were the leading cause for admission. A total of 20 patients received hematopoietic stem cell transplantation. Immune reconstitution was achieved in 9 (45 %) patients and eight of them did survive. In all 56 % of all admission episodes resulted in survival. Risk factors for mortality included requirement of mechanical ventilation (P?<?.001), number of organ system failure (P?=?.013), need for renal replacement therapy (P?<?.001), use of inotropes (P?<?.001), higher Pediatric Logistic Organ Dysfunction (PELOD) score (P?=?.005), and length of PICU stay (P?<?.001).

Conclusions

This is the first study regarding the outcome and mortality-related risk factors for PID patients requiring PICU admission. We suggest that PICU management is as important as early diagnosis and treatment for these patients. Prediction of those at risk for poorer outcome might be beneficial for accurate intensive care management and survival.