A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study

Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment.We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1­75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE.     

Compliance With Dyslipidemia Guidelines in Daily Practice: How Effective Is Cardiovascular Risk Prevention?

Purpose

Cardiovascular disease (CVD) due to atherosclerosis is the leading cause of early mortality and morbidity. The current European guidelines on CVD prevention in clinical practice recommend the use of the Systematic Coronary Risk Estimation (SCORE) system. The current American Heart Association guidelines recommend the use of the new pooled cohort risk assessment equations to estimate the 10-year atherosclerotic CVD risk. The purpose of this article was to investigate the compliance of dyslipidemia guidelines in daily practice in patients with dyslipidemia or who have risk factors for CVD.

The cognitive aspect of formal thought disorder and its relationship with global social functioning and the quality of life in schizophrenia

Purpose

It was expected that using a comprehensive scale like the Thought and Language Disorder Scale (TALD) for measurement of FTD would enable assessing its heterogeneity and its associations with cognitive impairment and functionality. This study has aimed to analyze the relationship between formal thought disorder (FTD) and cognitive functions, functionality, and quality of life in schizophrenia.

Methods

This cross-sectional exploratory study included 46 clinical participants meeting the DSM-5 diagnostic criteria for schizophrenia and 35 healthy individuals as the control groups. Data were acquired by means of the Turkish language version of the TALD, the Positive and Negative Syndrome Scale, the Clinical Global Impression Scale, the Functioning Assessment Short Test, the Social Functioning Scale, the World Health Organization Quality of Life Instrument-Short Form, and a neuropsychological test battery on executive functions, working memory, verbal fluency, abstract thinking, and response inhibition. Correlation analyses were conducted to detect significant relationships.

Results

The clinical group scored failures in all cognitive tests. The objective positive FTD was associated with deficits in executive functions and social functioning. The objective negative FTD was associated with poor performance in all cognitive domains, physical quality of life, and social and global functioning. The subjective negative FTD was negatively correlated with psychological quality of life.

Conclusion

This study demonstrated that objective FTD factors reflect different underlying cognitive deficits and correlate with different functioning domains. Significant correlation was determined between subjective negative FTD and psychological quality of life. Given the close relationship of FTD with functioning and quality of life, the FTD-related cognitive deficits should be the key treatment goal in schizophrenia.

     

Correlation of molecular biomarker concentrations between synovial fluid and saliva of the patients with temporomandibular disorders

Objectives

The synovial membrane and fluid are involved in the pathogenesis of temporomandibular joint (TMJ) disorders. This study aims to assess the relationship between matrix metalloproteinase-2 (MMP-2), chemerin and prostaglandin (PGE₂) levels in the synovial fluid (SF) and saliva of patients with TMJ disorder regarding their role in inflammation and the value of being a candidate for predictive biomarkers in the disease. Also, it is aimed to find out whether chemerin’s main function triggers the formation inflammatory cytokine markers in the associated area.

Materials and methods

Thirty-two samples of SF and saliva were obtained from patients with disc displacement without reduction with limited opening (DDWORwLO). Mann-Whitney-U test was used for the comparisons of the biomarker levels in SF and saliva. The correlation between chemerin and BMI (Body Mass Index) is analyzed by non-parametric Spearman’s rho correlation coefficient.

Results

For all of the three biomarkers, statistically significant differences were found between SF and saliva. An unexpectedly high level expression of chemerin was observed in SF. A statistically significant, positive correlation was observed between PGE₂ -MMP-2, and chemerin-PGE₂ in saliva, chemerin and MMP-2 in SF, respectively (p = 0.031, r = 0.382 / p = 0.039, r = 0.366 / p = 0.032, r = 0.379). A positive correlation was determined between saliva and SF levels of PGE₂ (p = 0.016, r = 0.421).

Conclusions

Chemerin, MMP-2, and PGE₂ can play a role as an inflammatory factor for the development of TMJ disorder.

Clinical relevance

The search for molecular markers in TMJ and the inhibition of the associated molecular signaling mechanism is important to reduce joint inflammation and cartilage degradation.

     

Single-stage endovascular treatment in patients with severe extracranial large vessel stenosis and concomitant ipsilateral unruptured intracranial aneurysm

PURPOSE

We aimed to evaluate the safety and effectiveness of single-stage endovascular treatment in patients with severe extracranial large vessel stenosis and concomitant ipsilateral unruptured intracranial aneurysm.

METHODS

Hospital database was screened for patients who underwent single-stage endovascular treatment between February 2008 and June 2013 and seven patients were identified. The procedures included unilateral carotid artery stenting (CAS) (n=4), bilateral CAS (n=2), and proximal left subclavian artery stenting (n=1) along with ipsilateral intracranial aneurysm treatment (n=7). The mean internal carotid artery stenosis was 81.6% (range, 70%–95%), and the subclavian artery stenosis was 90%. All aneurysms were unruptured. The mean aneurysm diameter was 7.7 mm (range, 5–13 mm). The aneurysms were ipsilateral to the internal carotid artery stenosis (internal carotid artery aneurysm) in five patients, and in the anterior communicating artery in one patient. The patient with subclavian artery stenosis had a fenestration aneurysm in the proximal basilar artery. Stenting of the extracranial large vessel stenosis was performed before aneurysm treatment in all patients. In two patients who underwent bilateral CAS, the contralateral carotid artery stenosis, which had no aneurysm distally, was treated initially.

RESULTS

There were no procedure-related complications or technical failure. The mean clinical follow-up period was 18 months (range, 9–34 months). One patient who underwent unilateral CAS experienced contralateral transient ischemic attack during the clinical follow-up. There was no restenosis on six-month follow-up angiograms, and all aneurysms were adequately occluded.

CONCLUSION

A single-stage procedure appears to be feasible for treatment of patients with severe extracranial large vessel stenosis and concomitant ipsilateral intracranial aneurysm.The concomitance of severe extracranial large vessel stenosis and unruptured ipsilateral distal intracranial aneurysm is often detected incidentally and their management is not clear (1). Although there are many studies in the literature that report different treatment approaches, there is no definite consensus on the management of the concomitant lesions (2–14). Various treatment options have been suggested, such as initial treatment of the aneurysm before revascularization of the stenosis, treating both lesions in the same surgical session and correcting the stenosis without treating the aneurysm (1, 5, 6, 9–11, 14–16). Few studies have reported single-stage endovascular treatment of both lesions as an effective method (17–19). On the other hand, the treatment of each lesion by this technique may lead to procedure-related undesired events such as cerebral ischemia/stroke or aneurysm rupture.In this study, we aimed to present the radiologic and clinical results of seven consecutive patients who underwent single-stage endovascular treatment of severe extracranial large vessel stenosis and concomitant unruptured ipsilateral intracranial aneurysm and discuss the safety and feasibility of this approach. In addition, distinct from the limited number of similar studies in the literature, we present our experience with bilateral carotid artery stenting (CAS) and proximal subclavian artery stenting during single-stage endovascular treatment.