Farelerde interferon ve steroid uygulamasının karaciğer, dalak ve kemik iliğindeki regülatuvar t-hücrelerine etkisi

Objectives: Regulatory T-cells (T-regs) maintain immune tolerance by affecting other cells of the immune system. They play an important role in autoimmune diseases and the prevention of graft rejection. Steroids suppress the immune system, especially inhibiting cytokine secretion of T-lymphocytes, initiation of the cell- mediated immune response, and stimulation of T-regs. Interferons (IFN) also have immunomodulatory, antiviral, and anti-proliferative effects. They activate macrophages and cytotoxic T-cells and stimulate the differentiation of T-regs. The aim of this study was to evaluate the effects of IFN and steroids on T-regs in the liver, spleen, and bone marrow in a mouse model, and to determine if they exert their immunosuppresive/immunomodulatory effects through T-regs. Materials and Methods: A total of 24 mice were randomly separated into 3 groups and administered an intraperitoneal injection for five days. The control group received 0.1 mL saline every day, the IFN group received IFN-alpha-2b 20,000 IU on the first, third, and fifth days, and only 0.1 mL saline on the other days, and the steroids group received 5 mg/kg dexamethasone in 0.1 mL saline every day. Two days after the end of therapy, each mouse was anesthetized, the portal vein was explored via laparotomy, and 5 mL bovine serum albumin (BSA) was administered through the portal vein. The inferior vena cava was cut to allow BSA perfusion of the liver, and then the mice were sacrificed. The liver, spleen, and bone marrow were removed for analysis. T-regs were identified and counted using flow cytometry. Results: The flow cytometry count results showed no significant difference between the IFN, steroid, and control groups. Conclusion: IFN and steroid use do not seem to affect the quantity of T-regs.Amaç: Regülatuvar T-hücreleri (T-reg) immün sistemde görevli birçok hücre çeşidine etki ederek immünolojik toleransı sağlayan hücrelerdir. Otoimmün hastalıklar, greft rejeksiyonunun önlenmesi ve enfeksiyon hastalıklarında önemli role sahiptirler. Steroidler, immün sistemi baskılarlar; özellikle T-lenfositlerin sitokin salgılamasını ve hücresel immünolojik yanıtın başlamasını önlerler ve T-reg’leri de stimüle ederler. Diğer yandan interferonlar (İFN) immünomodülatör, antiviral ve anti-proliferatif etkiye sahiptirler. Makrofajları ve sitotoksik T-hücrelerini aktive ederler ve T-reg’lerin diferansiyasyonunu uyarırlar. Biz bu çalışmamızda İFN ve steroidin karaciğer, dalak ve kemik iliğindeki T-reg’lere etkisini, bilinen immünosüpresif/immün düzenleyici etkilerini T-reg’ler üzerinden yapıp yapmadıklarını değerlendirmeyi amaçladık. Gereç ve Yöntemler: Bunun için 24 fareye 5 gün boyunca intraperitoneal enjeksiyon yapıldı. Kontrol grubuna 0,1 cc serum fizyolojik her gün uygulandı. İFN grubuna İFN-alfa-2b 20.000 IU 0,1 cc olacak şekilde serum fizyolojik ile sulandırılarak gün aşırı 3 kez diğer günler 0,1 mL serum fizyolojik uygulandı. Steroid grubuna deksametazon 5 mg/kg 0,1 mL olacak şekilde serum fizyolojik ile sulandırılarak her gün uygulandı. Enjeksiyonlar bittikten sonra 2 gün beklendi. Farelere genel anestezi uygulandı, laparotomi yapılıp portal ven açığa çıkarıldı, portal venden 5 mL bovine serum albümin (BSA) verildi, inferior vena kava kesilerek karaciğerin BSA ile perfüzyonu sağlandı, fareler feda edilmiş oldu. Karaciğer, dalak ve kemik ilikleri elde edildikten sonra T-reg’lerin ayrımı yapıldı ve akım sitometrisi ile sayıldı. Bulgular: Akım sitometrisi ile sayımda İFN, steroid ve kontrol grubunda T-reg sayılarında istatiksel olarak anlamlı bir farlılık bulunamadı. Sonuç: Sağlıklı farelerde İFN ve steroid kullanımının karaciğer, dalak ve kemik iliğindeki T-reg’lerin miktarına etki etmediği düşünüldü

A rare cause of globus pallidus and dentate nucleus hyperintensity in childhood: MBOAT mutation

Mutations in mammalian membrane-bound O-acyltransferase domain-containing (MBOAT) 7 gene are a rare cause for intellectual disability, developmental delay, autistic findings, epilepsy, truncal hypotonia with appendicular hypertonia, and below-average head sizes. Pathogenic variants in MBOAT7 gene show these nonspecific clinical features that are seen in many other neurometabolic diseases. Therefore, specific neuroimaging findings can be valuable key factors for differential diagnosis. Magnetic resonance imaging (MRI) findings of T2 hyperintensity in bilateral globus pallidi and dentate nuclei are seen in a few neurometabolic diseases with similar clinical features of developmental delay and hypotonia, as in our cases. While evaluating the patients with similar phenotypes and specific MRI findings, MBOAT7 deficiency should be kept in mind. Here, we identified two brothers who had a novel homozygous variant in MBOAT7 gene and aimed to raise awareness about this newly described disease

The impact of COVID-19 pandemic and quarantine process a center in Turkey on anxiety levels of pediatric patients with epilepsy

Introduction: In this study, we aimed to compare the levels of state and trait anxiety in pediatric patients with epilepsy and healthy controls during the COVID-19 outbreak in Turkey. Materials and Methods: In this study, the state (STAI-I) and trait (STAI-II) anxiety scales were applied to patients between the ages of 10-17 years that had been followed due to epilepsy and were in quarantine. Anxiety status and potential risk factors were compared with healthy age and gender-matched control group. Results: This study included 40 epilepsy patients and 40 controls. Among the patients in the epilepsy group 92.5% had moderate and 7.5% had severe anxiety (mean value of STAI-I and STAI-II were 40.3±4.8 and 40.9±10.5, respectively). There was no significant difference between epilepsy and controls groups in terms of mean STAI-I and STAI-II scale scores (p=0.756, 0.914). When the state anxiety scores were categorized as low, moderate, and high anxiety levels, moderate-to-high state anxiety was detected in the epilepsy group, and moderate-to-low state anxiety in the control group. The state anxiety level was found to be high in patients with high seizure frequency (p=0.045). No significant relationship was found between state and trait anxiety scale scores and factors such as epilepsy duration, quarantine duration, seizure type, drug resistance, and type of antiepileptics. Conclusion: In situations that commonly affect community health, clinicians should focus also on the mental health of epileptic patients. Therefore, we believe that mental health support should be provided to pediatric patients with epilepsy