Unexpectedly High Prevalence of Low Alpha-Galactosidase A Enzyme Activity in Patients with Focal Segmental Glomerulosclerosis

Hasbal, Nuri Baris/0000-0002-2229-5140WOS: 000576771000001PubMed: 32997080OBJECTIVES: Fabry disease (FD) is a rare disease associated with sphingolipid accumulation. Sphingolipids are components of plasma membranes that are important in podocyte function and accumulate in various glomerular diseases such as focal segmental glomerulosclerosis (FSGS). Both FD and FSGS can cause podocyte damage and are classified as podocytopathies. in this respect, FD and FSGS share the same pathophysiologic pathways. Previous screening studies have shown that a significant proportion of end-stage renal disease (ESRD) patients receiving hemodialysis (HD) have unsuspected FD, and the prevalence of low alpha-galactosidase A (aGLA) enzyme activity in these patients is higher than that in the normal population. We aimed to compare aGLA enzyme activity in patients with biopsy-proven FSGS and ESRD receiving HD. METHODS: the records of 232 patients [62 FSGS (F/M: 33/29); 170 HD (M/F: 93/79)] were evaluated retrospectively. the screening was performed based on the aGLA enzyme activity on a dried blood spot, with the confirmation of plasma LysoGb3 levels, and the known GLA mutations were tested in patients with low enzyme activities. the two groups were compared using these parameters. RESULTS: the mean level of aGLA enzyme activity was found to be lower in FSGS patients than in the HD group (2.88 +/- 1.2 mmol/L/h versus 3.79 +/- 1.9 mmol/L/h, po0.001). There was no significant relationship between the two groups with regard to the plasma LysoGb3 levels (2.2 +/- 1.22 ng/ml versus 1.7 +/- 0.66 ng/ml, p: 0.4). in the analysis of GLA mutations, a D313Y mutation [C(937G4T) in exon p] was found in one patient from the FSGS group. CONCLUSIONS: We found that aGAL activity in patients with FSGS is lower than that in patients undergoing HD. the low enzyme activity in patients with FSGS may be explained by considering the similar pathogenesis of FSGS and FD, which may also lead to sphingolipid deposition and podocyte injury

The Role of Ankaferd Blood Stopper and Oxytocin as Potential Therapeutic Agents in Endometriosis: A Rat Model

WOS: 000549619600020PubMed: 32681259To evaluate the potential effect of Ankaferd Blood Stopper (ABS) and oxytocin (OT) in an experimental endometriosis model, 18 female Sprague Dawley rats were used in this study. the animals were divided randomly into three groups after surgical induction of endometriosis: group 1: control group (isotonic NaCl, 1 mL/kg/day, intramuscular,n=6); group 2: OT group (OT, 80 U/kg/day, intramuscular,n=6); group 3: ABS group (ABS, 1.5 mL/kg/day, intraperitoneal,n=6). Each group was treated for four weeks (two times per week). Volumes of endometriotic explants were measured in biopsy samples for histopathological analysis. Vascular endothelial growth factor (VEGF), monocyte chemotactic protein-1 (MCP-1), and tumour necrosis factor (TNF-alpha) levels were measured in plasma and peritoneal fluid. Endometriotic explant volumes were significantly decreased after OT administration (P<0.0001). the epithelial score was significantly decreased in both treatment groups compared to the control group (P<0.05). TUNEL immunohistochemistry showed more apoptotic changes in the endometriosis foci (gland epithelium and surrounding tissue) in the OT group than in the control group (P<0.05). the levels of VEGF, MCP-1, and TNF-alpha were significantly reduced in the OT group (P<0.05), whereas no significant changes in protein levels were found in the ABS-applied group. the results indicate that OT has greater potential as a therapeutic agent in experimentally induced peritoneal endometriosis, where ABS, which is a VEGF modulator, appears to act through different mechanisms to show its palliative effects on a rat model of peritoneal endometriosis

Impact of an Established National Donor Registry on Transplant Outcome: Turkok Experience in Turkey

Transplantation and Cellular Therapy (TCT) Meetings of ASTCT and CIBMTR -- FEB 19-23, 2020 -- Orlando, FLWOS: 000516887900445[No abstract available]Amer Soc Transplantat & Cellular Therapy, CIBMT

Cerebral Amyloid Angiopathy Related Inflammation Presenting as Steroid Responsive Brain Mass

WOS: 000551172600024PubMed: 30649812Cerebral amyloid angiopathy (CAA) is a common but often asymptomatic disease, characterized by the deposition of amyloid proteins within brain parenchyma and leptomeningeal-cortical vessels. It can occur as a sporadic disorder or accompany Alzheimer disease (AD). the usual presentation of CAA is spontaneous lobar hemorrhage, Unlike CAA, cerebral amyloid angiopathy-related inflammation (CAA-ri), a subtype of CAA, can show various presentations and responds to steroid (or immuno suppressive) treatment. We report a patient with mixed dementia who showed subacute progression in cognitive impairment and had a mass lesion on brain magnetic resonance imaging (MRI). the lesion was identified as CAA related inflammation and the cognitive status of the patient improved significantly after steroid treatment. in this case report, we aimed to emphasize that CAA-ri is one of the possible diagnoses that should be considered in demented patients with rapid cognitive deterioration and showing brain lesions resembling neoplasms on the MRI. Therefore, steroids or other immunosuppresive treatments, which may lead to a dramatic clinical improvement, could be administered without delay

Prevalance and Clinical Characteristics of Pain in Patients with Obstructive Sleep Apnea Syndrome

WOS: 000579120800007Objective: It has been shown that pain, depression, and fatigue related to Obstructive Sleep Apnea syndrome (OSAS) results in a significant socioeconomic burden. We aimed to determine the prevalence, demographic, and clinical properties of pain symptoms in OSAS patients. Materials and Methods: We evaluated 84 OSAS patients diagnosed after full-night polysomnography with the Brief Pain Inventory, Beck Depression Rating scale (BDS), Pittsburg Sleep Quality index (PSQI), Epworth Sleepiness scale (ESS), Fatigue Impact scale (FIS), and Fatigue Severity scale (FSS). Results: A total of 84 patients (62 males, 22 females) with a mean age of 54.37 +/- 11.03 years were included. Headache in 25 (43.1%), myofascial pain in 22 (37.9%), and other types of pain in 11 patients were defined. ESS, FSS, FIS total, physical, cognitive, and psychosocial scores were found to be significantly higher in patients with pain. the presence of pain did not differ according to the severity of OSAS, and no significant difference was found in any parameters between mild, moderate, and severe OSAS except for minimal O-2 saturation. A positive correlation between last week pain severity and BDS scores, a negative correlation between PSQI scores and the decrease in pain rate as a result of the treatment, and a weak positive correlation between FIS-cognitive sub-score and pain intensity scores were found. Conclusion: in our study, excessive daytime sleepiness and the impact and severity of fatigue were found to be higher in patients with pain than in those without pain. the most obvious difference was seen in terms of cognitive, and psychosocial fatigue. Contrary to expectations, it was observed that OSAS severity did not affect pain according to our data

Long Term Outcomes of Freestyle Stentless Aortic Bioprosthesis: A Single Center Experience

EZELSOY, MEHMET/0000-0002-8423-5071WOS: 000518864700007PubMed: 32118540Background: the study is presenting our long-term clinical results after freestyle stentless aortic root bioprosthesis replacement in patients with severe aortic insufficiency with ascending aortic aneurysm. Methods: Seventy-seven patients with ascending aortic aneurysms and aortic valve insufficiency underwent a total root replacement procedure using a stentless "Freestyle" valve (Medtronic Inc., Minneapolis, Minnesota). There were 50 (64.9%) men and 27 (35.1%) women. Mean age was 68.7 +/- 11.1 years. the surgical procedure used a complete root replacement. Concomitant procedures included coronary artery bypass grafting in 15 (19.5%) patients. Results: the mean cardiopulmonary bypass time was 130.3 +/- 26.4 minutes and total aortic cross clamp time was 99.5 +/- 23.6 minutes. Hospital mortality was 2.6%. the median follow-up time was 11.2 years. the 5- and 10-years freedom from aortic valve reoperation were 97.4 +/- 1.2% and 93.4 +/- 4.9%, respectively. During 10 years follow up, there were 14 late deaths; 4 deaths were cardiac, and 10 deaths were noncardiac. Valve-related deaths were attributable to thromboembolism in 1 patient, endocarditis in 2 patients, and congestive heart failure in 1 patient. Conclusion: the freestyle stentless aortic root bioprosthesis offered good clinical outcomes, in terms of survival and structural valve deterioration. the Freestyle valve is a viable option for use in patients undergoing bioprosthetic aortic valve replacement and expected desire for long-term durability

Cerebral Venous Sinus Thrombosis in Women: Subgroup Analysis of the VENOST Study

Sahin, Sevki/0000-0003-2016-9965; Uzuner, Nevzat/0000-0002-4961-4332; UZUNER, NEVZAT/0000-0002-4961-4332; Yayla, Vildan/0000-0002-4188-0898WOS: 000570429300001PubMed: 32953038Background. Early diagnosis of cerebral venous sinus thrombosis (CVST) associated with reproductive health-related risk factors (RHRF) including pregnancy, puerperium, and oral contraceptive (OC) use can prevent severe neurological sequelae; thus, the symptoms must be documented in detail for each group.Methods. Out of 1144 patients with CVST, a total of 777 women were enrolled from a multicenter for the study of cerebral venous sinus thrombosis (VENOST). Demographic, biochemical, clinical, and radiological aspects were compared for 324 cases with RHRF and 453 cases without RHRF.Results. the mean age of the RHRF (-) group (43.2 +/- 13 years) was significantly higher than of the RHRF (+) group (34 +/- 9years). A previous history of deep venous thrombosis (3%), isolated cavernous sinus involvement (1%), cranial neuropathy (13%), comorbid malignancy (7%), and its disability scores after 12 months (9%) were significantly higher in the RHRF (-) group. the RHRF (+) group consisted of 44% cases of puerperium, 33% cases of OC users and 23% of pregnant women. the mean age was found to be higher in OC users (38 +/- 9years). A previous history of deep venous thrombosis was slightly higher in the pregnancy subgroup (4%). Epileptic seizures were more common in the puerperium group (44%).Conclusion. the results of our study indicate that the risk of CSVT increases parallel to age, OC use, and puerperium period. in addition, when considering the frequency of findings and symptoms, epileptic seizures in the puerperium subgroup of the RHRF (+) group and malignancies in the RHRF (-) group may accompany the CSVT. in daily practice, predicting these risks for the CSVT and early recognition of the symptoms will provide significant benefits to patients.NIA NIH HHSUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute on Aging (NIA) [U54 AG044170] Funding Source: Medlin

Assessment of Patients with Intracerebral Hemorrhage or Hemorrhagic Transformation in the VENOST Study

PubMed: 33130674Introduction: Cerebral venous and sinus thrombosis (CVST) may lead to cerebral edema and increased intracranial pressure; besides, ischemic or hemorrhagic lesions may develop. Intracerebral hemorrhages occur in approximately one-third of CVST patients. We assessed and compared the findings of the cerebral hemorrhage (CH) group and the CVST group. Materials and Methods: in the VENOST study, medical records of 1,193 patients with CVST, aged over 18 years, were obtained from 35 national stroke centers. Demographic characteristics, clinical symptoms, signs at the admission, radiological findings, etiologic factors, acute and maintenance treatment, and outcome results were reported. the number of involved sinuses or veins, localizations of thrombus, and lesions on CT and MRI scans were recorded. Results: CH was detected in the brain imaging of 241 (21.1%) patients, as hemorrhagic infarction in 198 patients and intracerebral hemorrhage in 43 patients. Gynecologic causes comprised the largest percentage (41.7%) of etiology and risk factors in the CVST group. in the CH group, headache associated with other neurological symptoms was more frequent. These neurological symptoms were epileptic seizures (46.9%), nausea and/or vomiting (36.5%), altered consciousness (36.5%), and focal neurological deficits (33.6%). mRS was ?3 in 23.1% of the patients in the CH group. Discussion and Conclusion: CVST, an important cause of stroke in the young, should be monitored closely if the patients have additional symptoms of headache, multiple sinus involvement, and CH. Older age and parenchymal lesion, either hemorrhagic infarction or intracerebral hemorrhage, imply poor outcome. © 2020 S. Karger AG, Basel. All rights reserved

Neuromyelitisoptica spectrum disorders: the evaluation of 66 patients followed by Istanbul Bilim University, Department of Neurology

Conference of the Pan-Asian-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (PACTRIMS) -- NOV 13-15, 2019 -- Singapore, SINGAPOREWOS: 000556862300090[No abstract available]Pan Asian Comm Treatment & Res Multiple Sclerosi

The Influence of Class III Obesity on Subarachnoid Depth of Turkish Parturient: A Prospective Observational Study

Seyhan, Tulay Ozkan/0000-0002-7070-8827WOS: 000535666500001Introduction: This observational study aimed to investigate the effects of morbid obesity on the subarachnoid depth and spinal anaesthesia technique. Methods: Sixty American Society of Anesthesiologists Classification II women with term pregnancy who were candidates for elective caesarean section under spinal anaesthesia were enrolled in this prospective, observational study. Only patients with a Body Mass index (BMI) of = 40 kg/m(2) (obesity group) were included in the study. Spinal anaesthesia was performed in the sitting position via a midline approach at either L3-4 or L4-5 level by using a 25G 90-mm Quincke spinal needle with an introducer. Demographic data of the parturient, visual characteristics of the lumbosacral region, palpation of landmarks, depth of the spine, technical characteristics of the block, time of block performance and satisfaction of patients were recorded. Results: the spinal depth of the control and obesity groups were 51.7 +/- 4.4 and 69 +/- 10.4 mm, respectively (p<0.001). Although needle change was not necessary for any of the patients in the control group, a 120-mm long needle change was required in six patients in the obesity group (p<0.024). We found that the incidence of patients with landmarks that were difficult to palpate was higher in the obesity group, and significantly increased attempt number, skin puncture and needle pass were also required in this group. Conclusion: Anaesthesiologists should be prepared for a longer attempt in patients with obesity but should not be discouraged as the increase in the number of attempts or prolonged initiation time of spinal anaesthesia was not associated with patient dissatisfaction or discomfort