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.     

Effects of primary selective laser trabeculoplasty on anterior segment parameters

AIM:To investigate the effects of selective laser trabeculoplasty (SLT) on the main numerical parameters of anterior segment with Pentacam rotating Scheimpflug camera in patients with ocular hypertension (OHT) and primary open angle glaucoma (POAG).METHODS: Pentacam measurements of 45 eyes of 25 (15 females and 10 males) patients (12 with OHT, 13 with POAG) before and after SLT were obtained. Measurements were taken before and 1 and 3mo after SLT. Pentacam parameters were compared between OHT and POAG patients, and age groups (60y and older, and younger than 60y).RESULTS: The mean age of the patients was 57.8±13.9 (range 20-77y). Twelve patients (48%) were younger than 60y, while 13 patients (52%) were 60y and older. Measurements of pre-SLT and post-SLT 1mo were significantly different for the parameters of central corneal thickness (CCT) and anterior chamber volume (ACV) (P<0.05). These parameters returned back to pre-SLT values at post-SLT 3mo. Decrease of ACV at post-SLT 1mo was significantly higher in younger than 60y group than 60y and older group. There was no statistically significant difference in Pentacam parameters between OHT and POAG patients at pre- and post-treatment measurements (P>0.05).CONCLUSION:SLT leads to significant increase in CCT and decrease in ACV at the 1^st month of the procedure. Effects of SLT on these anterior segment parameters, especially for CCT that interferes IOP measurement, should be considered to ensure accurate clinical interpretation.     

Caregiver burden and related factors in caregivers of patients with childhood-onset systemic lupus erythematosus

Clinical Rheumatology - Having a child with a chronic illness is a source of stress for the whole family, especially the primary caregiver. The aim of this study was to evaluate the associations...     

Impaired TRAIL-dependent cytotoxicity of CD1c-positive dendritic cells in chronic hepatitis C virus infection

Dendritic cells (DCs) play a central role in antiviral immunity. Conflicting data on DC function have been reported for hepatitis C virus (HCV) infection. In addition to antigen presentation and cytokine secretion, a subset of human DCs displays direct cytotoxic activity. It has been suggested that measles virus and human immunodeficiency virus (HIV) may enhance cytotoxicity of DCs potentially leading to apoptosis of activated T cells and subsequent down-regulation of antiviral immune responses. We demonstrate that CD1c-positive myeloid DCs, but not BDCA-4-positive plasmacytoid DCs, are able to kill different target cells mainly via tumour necrosis factor-related apoptosis-inducing ligand. The ability of CD1c+ DCs to lyze target cells was found to be completely impaired in patients with chronic hepatitis C (10 chronic HCV patients vs 10 healthy controls; P < 0.001) but not in patients with primary biliary cirrhosis. Successful antiviral therapy of chronic hepatitis C rescued the cytotoxicity of DCs. Myeloid DCs of HCV patients and healthy controls had a similar phenotype and endocytotic activity, however, the frequency of mDCs in the peripheral blood was lower (P = 0.004) and the allostimulatory function was weaker (P < 0.001) in chronic hepatitis C. Thus, in contrast to HIV and measles virus studies on monocyte-derived DCs, freshly isolated myeloid DCs of patients with hepatitis C do not show an increased but a completely abolished cytotoxic activity. The impaired DC cytotoxicity could represent a novel mechanism for the increased prevalence of autoimmunity in HCV infection.     

A fistula connecting the right coronary artery to the right atrium: a hitherto undescribed association

A case of double right coronary artery accompanying a fistulous connection is presented. An additional right coronary artery is an extremely rare congenital abnormality. In this case, the second right coronary artery was draining into a cardiac chamber via a fistulous connection. This is the first case in the literature to present these abnormalities coexisting in the same patient.     

A novel PSEN2 p.Ser175Phe variant in a family with Alzheimer’s disease

Neurological Sciences - Alzheimer’s disease (AD) can be either sporadic or familial, and familial forms of AD accounts for only 5% of the cases. So far, autosomal dominantly inherited...     

3-T MRI of the biliary tract variations

Purpose

The gallbladder and the biliary tract are structures in close connection with the adjacent organs and may show a number of variations and anomalies. It is therefore important for surgical purposes to know their anatomy and variations in detail. Various methods are used in the imaging of the variations of the biliary tract and its pathologies, including ultrasonography, computed tomography; direct cholangiographic methods like endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography, intravenous cholangiography and T-tube cholangiography, as well as indirect methods like magnetic resonance cholangiopancreatography (MRCP) or cholescintigraphy. The aim of this study is to investigate the frequency of the anatomic variations of the biliary tract using 3-T MRCP and to compare the findings with the data in the literature.

Materials and methods

For the purposes of this study, patients who underwent MRCP at our hospital (Dicle University Hospital) between November 2009 and February 2012 were investigated retrospectively. A total of 590 patients (between 6 and 88 years of age; mean age: 51 ± 9 years) were included in the study. The MRCP imaging was carried out with an magnetic resonance imaging (MRI) device supplied with 3-T magnetic power and by obtaining T2-weighted images through the single-shot fast spin echo technique using the standard body coil. The axial and coronal source images and the reformatted images were evaluated together in terms of the possible anatomic variations.

Results

Among the 590 patients included in the study, of 233 (39.5 %) showed anatomic variations at different levels in the intra- and extrahepatic biliary tracts. Among these variations, a right posterior hepatic duct insertion to the left hepatic duct at the level of the bifurcation has been observed in 71 patients (12.1 %), trifurcation was observed in 30 patients (5.1 %) and insertion into the main hepatic duct at the proximal aspect of the cystic duct was observed in 18 patients (3.1 %). At the level of the cystic duct, medial insertion of the cystic duct was viewed in 58 patients (9.8 %), distal medial insertion was seen in 40 patients (6.8 %), a short cystic duct was detected in 10 patients (1.7 %), pancreatobiliary junction anomaly was viewed in two patients (0.4 %) and duplicate anatomic variations have been observed in 42 patients (7.2 %).

Conclusion

MRCP studies conducted using 3-T MRI devices may reveal similar or greater numbers of variations when compared to the existing MRCP studies in the literature. 3-T MRI shows a couple of variations. Pointing out these anatomical variations before the surgical intervention may prevent possible iatrogenic traumas. Donors with unsuitable variations for liver transplant may be spotted out at an early phase through the MRCP and certain operations with a high morbidity rate may thus be avoided.     

Neurological manifestations of COVID-19: with emphasis on Iranian patients

Journal of NeuroVirology - The novel coronavirus disease 2019 (COVID-19) caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has instigated a global pandemic as a formidable...