110 research outputs found

    Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

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    Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

    Cerebellar liponeurocytoma/lipidized medulloblastoma - Case report and review of the literature

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    Cerebellar liponeurocytoma that has been recently identified as a distinct entity by the World Health Organization is characterized by areas of lipomatous differentiation and apparently by a favorable prognosis. In this paper, we described a case of 49-year-old female showing progressive clinical course inspite of a low labeling/mitotic index. We also review the relevant literature. Although, basically all reported cases share a similar histological pattern, i.e. focal accumulations of adiposities in an otherwise typical small cell tumor like central neurocytoma, some clinical properties such as (age, proliferative potential, therapy and survival) are not uniform. The exact biological behavior of this special variant tumor is established. Yet, this needs further confirmation on a large number of cases with longer follow-up periods

    Flow Cytometric Detection of Anti-AB Antibody Titers in Blood Group O Recipients of Blood Group A2 Donor Kidneys

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    Aim. ABO-incompatible kidney transplantation has been accepted for end-stage renal failure patients who have no ready opportunity for a deceased or living donor. Antibody titration for ABO-incompatible renal transplantation is not only difficult but also lacks conformity among laboratories. Herein we analyzed 20 living related renal transplant couples to detect recipient anti-A2 antibody using flow cytometric analysis. Materials and methods. Patients were admitted to our center for renal transplantation between January 1999 and December 2010. All but four of them had undergone a previous renal transplantation from an ABO-compatible donor but experienced graft failure. All donor blood groups were subtyped by our blood bank using a lectin-based dilution assay. To detect recipient anti-A2 antibody titers we used a tube hemagglutination method. A/B antibody titer analysis by flow cytometry incubated serially diluted serum samples with donor erythrocytes. Each analysis was repeated three, times over a 2-week period using an older and the last sera simultaneously. Results. The 13 male and 7 female patients showed our overall mean age of 32 +/- 12 years. All patients had panel-reactive antibody levels below 15%. The level of flow cytometric antibody titers did not vary upon repeated analysis (P = .01). When compared with the tube method there was a discrepancy of the level at which the antibody titer became negative. Discussion. Flow cytometric antibody titration is a practical and rapid technique to determine the amount of anti-A2 antibody in renal recipients

    Relationship of Urothelial Gene Expressions in Urine-Deprived Bladders of Renal Recipients With Posttransplant Urinary Infections

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    Objective. In this study, we analyzed gene expression levels of apoptotic (Fas, FasL, Bcl-2, Bax) and survival (CXCR1, CXCR2, IL-8) signal pathways of the urine-deprived bladder tissues and the relation of urinary tract infections with these pathways. Material and Methods. We included 37 patients admitted for renal transplantation between December 2009 and December 2012. Bladder mucosal samples were obtained at the time of transplantation and 6-8 weeks posttransplantation, at the time of ureteral catheter removal. RNA extraction and cDNA synthesis were done using guanidium-thiocyanate and colon filter methods. Expression analysis was studied with quantitative real-time polymerase chain reaction optimized with ROX dye and internal control beta-actin. Results. According to our findings Fas, FasL, Bcl-2, and Box expression was higher in urine-deprived bladder samples than those in the posttransplant samples (P < .05). Although Fas, FasL, Bcl-2, and Box expression levels increased in pretransplant samples, there was an increase in posttransplant bladder samples; however, this increase was not as marked as those of pretransplant samples. IL-8, CXCR1, and CXCR2 expression was decreased at the pretransplant samples and increased in posttransplant bladder samples. Conclusions. We have found an upregulated apoptotic process and decreased survival signals at the urine-deprived bladder tissue. After transplantation, bladder tissue survival parameters were increased, demonstrating the importance of urinary flow for bladder tissue

    Cytotoxic Antibody Detection by Means of Flow-Cytometric Cross-Match

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    Background. Complement-dependent lymphocytotoxicity (CDC-XM) and flowcytometric (FCXM) cross-match are analyzed individually for each donor and recipient pair, because these techniques have fundamental differences for the evaluation of histocompatibility. Lately, cytotoxic flow-cytometric cross-match (cFCXM) has been developed as an alternative to both CDC-XM and FCXM techniques. We evaluated the limits of cFCXM with the use of different positive serum dilutions. Methods. CDC-XM, FCXM, and cFCXM tests were performed with the use of commercially available negative and positive serum samples and lymphocytes from healthy donors. Results. Complement-dependent cell death was successfully detected with the use of cFCXM. Complement-dependent cell death ratios in cFCXM were similar those in CDCXM. With cFCXM, not only complement-dependent cell death but also IgG binding could be detected within a single assay. At higher concentrations of the positive serum, IgG-fluorescein isothiocyanate (FITC) mean fluorescent intensity (MFI) values detected with the use of cFCXM were less than those of conventional FCXM. Correspondingly, for dead cells, MFI values of IgG-FITC were less than those of live cells in higher positive serum concentrations in the cFCXM assay. Moreover, our results demonstrated that in cFCXM analysis, the decreasing ratio of dead cells at increasing positive serum dilutions was not in parallel with the same decrease in IgG-FITC MFI values. Conclusions. The cFCXM technique detects complement-mediated cytotoxic cell death with the additional ability to show IgG binding in the same tube and therefore may reduce the necessary bench time and workload

    Comparison of the Treatment Efficacy of Rituximab and Plasmapheresis/Intravenous Immunoglobulin Combination with Historical Control in Chronic Antibody Mediated Rejection

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    OBJECTIVE: Chronic antibody mediated rejection (CAMR) is a major therapeutic challenge for achieving long-term graft survival; treatment options are limited to several anti-humoral interventions. MATERIAL and METHODS: Efficacy of rituximab combination therapy was retrospectively investigated by comparison with a historical control group for allograft function at six month and overall graft survival/dysfunction. The inclusion criterion was biopsy proven chronic AMR according to the Banff 2007 classification. Nineteen patients found eligible, rituximab group had nine patients (rituximab, plasmapheresis and low dose IVIG); control group had ten recipients. Predictive factors for graft failure also investigated according to Banff scores and renal functions. RESULTS: None of the outcomes were exposed significant efficacy of rituximab, although better treatment response at sixth month (55% vs. 40%, p= 0.51), fewer overall graft failures (33% vs. 60%, p= 0.25) and dysfunctions (66% vs. 80%, p= 0.52). Overall, 47% of patients suffered graft failure. Advanced transplant glomerulopathy was found in 90% of biopsies (all scored = 2). Peritubular capillaritis score (1.67 +/- 0.87 vs. 0.70 +/- 0.94, p= 0.04) and interstitial inflammation score (1.78 +/- 0.44 vs. 1.00 +/- 0.47, p= 0.004) were significantly higher in recipients who suffered graft failure. CONCLUSION: Rituximab could not sufficiently prevent further deterioration of allograft and failed to improve allograft survival in CAMR, especially after settlement of the irreversible transplant glomerulopathy

    Cancer Screening of Renal Transplant Patients Undergoing Long-Term Immunosuppressive Therapy

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    Objective. With this study we aimed to research the effects of immunosuppressive drugs, their cumulative doses, and viral infections on development of malign tumors in patients who have undergone treatment for 5 years. Methods. We examined 100 patients who underwent renal transplantation from 2004 to 2009. Patients had mycophenolate mofetil and steroid in addition to cyclosporine, sirolimus, or tacrolimus as immunosuppressive treatment. For malignancy screening, physical examination, radiologic and endoscopic screening were done, and immunosuppressive drugs and their cumulative doses, age, sex, body mass index (BMI), dialysis history, and viral infection history were investigated. Results. The mean age of patients was 42.03 +/- 11.30 years. There were 1 colon cancer patient, 1 retroperitoneal liposarcoma, 1 renal oncocytoma, 3 Kaposi sarcoma patients treated with cyclosporine; in those treated with Tac there were 1 basal cell carcinoma, 1 Kaposi sarcoma, 2 thyroid carcinoma, 1 breast carcinoma, 1 bladder carcinoma, 1 renal cell carcinoma, and 1 colon carcinoma patients. The mean age of patients having carcinoma was statistically significant compared with those without cancer (P < .01). The prednisolone cumulative dose was significantly higher in carcinoma patients than in patients without carcinoma (P < .01). Results. The use of long-term chronic immunosuppressive therapy may increase the development of cancer. The risk of carcinoma increases with increasing drug dose and time period of the immunosuppressive drug. There was not a negative effect on cancer prevalence in patients with cyclosporine or tacrolimus. But the cumulative dose of steroids significantly increased malignancy occurence

    THE EXPRESSION LEVELS OF microRNAs ASSOCIATED WITH T AND B CELL DIFFERENTIATION/STIMULATION IN ANKYLOSING SPONDYLITIS

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    Spondyloarthropathies (SpAs), are a group of chronic inflammatory diseases with a number of genetic, physiopathological, clinical and radiological features. Ankylosing spondylitis (AS) is the most common type of spondylo-arthropathies, and >90.0% of patients with ankylosing spondylitis are human leukocyte antigen-B27 (HLA-B27)-positive. In recent years, non-HLA genetic factors have been reported to have an effect on ankylosing spondylitis. MicroRNAs (miRNAs), are endogenous non coding RNA molecules containing 18-23 nucleotides that play a role in the post-transcriptional regulation of gene expression. In this study, we aimed to determine the expression levels of miRNAs associated with T- and B-cell differentiation/ stimulation in peripheral blood mononuclear cells and their relationship with the etiology of the AS in patients and healthy controls. In a molecular study, peripheral blood mononuclear cell isolation, and total RNA isolation were performed first. In the second step, cDNA synthesis and quantitative real-time PCR (qPCR) expression analysis were completed. Ultimately, in the patient and control group, the expression levels of miR-142-5p and miR-143 were found to be significantly different (p <0.05). According to current knowledge, miR-142-5p and miR-143 expressions were found to be important for those diseases that share similar etiology with AS. We suggest that miR-142-5p and miR-143 may play a role in the pathogenesis, especially miR-142-5p may be a potential biomarker and a target molecule for the treatment

    Association between occurrence of ossicular chain defect and osteoprotegerin gene expression in patients with chronic otitis media

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    Objective Chronic otitis media (COM) is an important debilitating public problem causing hearing loss due to irreversible resorption of the ossicular chain. Activation of osteoprotegerin (OPG) during an acute attack of COM prevents bone resorption. The aim of the study was to investigate the role of OPG gene expression level on ossicular chain resorption in chronic otitis media. Materials and methods Fifty operated COM patients were included in the study. While 20 patients underwent ossiculoplasty, 30 patients underwent type 1 tympanoplasty. For RNA isolation and OPG gene expression analysis, middle ear swabs were taken from nasopharynx in the ostium of the Eustachian tube. RNA was isolated with mRNA easy kit and kept at - 85 degrees C till the cDNA and expression analysis. Expression levels were analyzed with real-time quantitative PCR in comparison with PDGB gene expression level as an internal control. Results Sample Cq measurements of type 1 tympanoplasty group were higher than Cq measurements of the internal control group (p = 0.027; p 0.05). Conclusion Since OPG gene expression level was significantly higher in type 1 tympanoplasty group, OPG gene regulation system may have an effect on ossicular chain destruction in COM

    A New Biomarker on Bone Resorption in Chronic Otitis Media: Osteoprotegerin and NLRP3 Inflammasome Gene Polymorphisms

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    Chronic Otitis Media (COM) is a chronic inflammation of the middle ear and mastoid with persistent membrane perforation and hearing loss. Osteoprotegerin (OPG) and NOD like receptor protein 3 (NLRP3) play an important role in bone metabolism. The aim of the study was to investigate the role of OPG and NLRP3 gene polymorphism on ossicular chain resorption in COM. Fourty COM patients and 20 healhty control group were included in the study. While 20 patients underwent ossiculoplasty, 20 patients underwent type 1 tympanoplasty. DNA was isolated from peripheral blood using the DNA kit. OPG gene c.226A > C (p.Thr76Pro) and NLRP3 gene c.592G > A (p. Val198Met) polymorphisms were genotyped using melting curve analysis technique. NLRP3 gene polymorphism were determined in 6 of 20 patients (30%) in ossiculoplasty group, 4 of 20 patients (20%) in type 1 tympanoplasty group and 3 of 20 patients (15%) in control group. OPG gene polymorphism were determined in 5 of 20 patients (25%) in ossiculoplasty group, 3 of 20 patients (15%) in type 1 tympanoplasty group and 1 of 20 patients (5%) in control group, respectively. There was no statistically significant difference between groups regarding to results. Although the difference was not significant NLRP3 and OPG gene polymorphisms were higher in the ossiculoplasty group. Since NLRP3 and OPG gene polymorphisms were determined to be higher numerically in the ossiculoplasty group, OPG and NLRP3 gene regulation system may have an effect on ossicular chain destruction in COM
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