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Insulin receptor (INSR) mutations lead to heterogeneous disorders that may be as severe as Donohue syndrome or as mild as “type A insulin resistance syndrome”. Patients with severe disorders usually harbor homozygous or compound heterozygous mutations. In contrast, type A insulin resistance syndrome has been associated with heterozygous mutations; homozygous mutations are rarely responsible for this condition. We report a novel, homozygous mutation, p.Leu260Arg in exon 3, of the INSR gene in a female adolescent patient with type A insulin resistance syndrome together with clinical details of her medical follow-up. Different mutations in the INSR gene cause different phenotype and vary depending on the inheritance pattern. This report adds to the literature, increases understanding of the disease mechanism and aids in genetic counseling.

Othello, aşk ve kıskançlığa dair bir oyun olduğu kadar kelimelerin gücünü de ortaya koyan bir oyundur. Oyunda hem Othello hem de Iago güçlü bir dile sahiptir. Ne var ki Othello, kullandığı şiirsel dille ün kazanmış olmasına rağmen, Iago Othello’ya göre dilin belirsiz doğasını ve sonsuz katmanlarını daha iyi kavramıştır. Dolayısıyla iki karakterin dile yaklaşımları oldukça farklıdır. Othello dile geleneksel bir bakış açışıyla yaklaşır. Saussure’un gösterge kavramından yola çıkarsak, Othello her gösterenin doğrudan doğruya bir gösterilene bağlı olduğunu düşünür ve ikisini sıkı sıkıya eşleştirme çabası içindedir. Oysa Iago, post-yapısalcı Jacques Derrida gibi dilin çok daha oynak olduğunu bilir. Kelimelerin birbirleriyle olan etkileşiminin ve bu durumun yarattığı muğlaklığın içgüdüsel olarak farkında olan Iago, oyun boyunca planlarını dilin bu belirsiz doğası üzerinden yapar. Othello’nun dile dair kör noktasını keşfeden Iago, oyun boyunca bu farkındalıkla hareket eder ve Othello’nun zihnini bulanıklaştırmayı başarır. Bu bilgiler ışığında, bu çalışmada her iki karakterin oyun boyunca dili nasıl kullandığı incelenecektir. Iago’nun dili kullanma biçimi, Fransız filozof Derrida’nın dile dair ortaya sürdüğü düşünceler doğrultusunda ele alınacaktır. Othello’nun tuzağa düşüp savunmasız kalması ve nihayetinde trajedisine kucak açması kendisinin dilin doğasına dair sahip olduğu yanılgılar çerçevesinde ele alınacaktır.

Background Total body irradiation (TBI) is the cornerstone of conditioning regimens in pediatric hematopoietic stem cell transplantation for acute lymphoblastic leukemia. As the late effects and survival comparison between TBI and chemotherapy were well analyzed before, in this study, we aim to focus on the first 100 days and early complications of transplantation. Methods This retrospective study involves 72 pediatric patients (0 to 18 y) underwent first hematopoietic stem cell transplantation for acute lymphoblastic leukemia between October 2015 and May 2019. Patients are divided into 2 groups regarding conditioning regimens. Conditionings includes either TBI 1200 cGy/6 fractions/3 days and etoposide phosphate or busulfan, fludarabine, and thiotepa. Busulfan was administered IV and according to body weight. Results The incidences of acute graft versus host disease grade 2 to 4, veno-occlusive disease, capillary leakage syndrome, thrombotic microangiopathy, blood stream infection, hemorrhagic cystitis and posterior reversible encephalopathy syndrome before day 100 were similar for both conditioning regimens; however, patients received TBI-based conditioning had significantly longer neutrophil engraftment time (17.5 vs. 13 d, P=0.001) and tended to have more engraftment syndrome (ES) (45.5% for TBI vs. 24.0% for chemotherapy, P=0.069). Multivariate analysis showed that TBI-based conditioning was associated with a longer neutrophil engraftment time (hazard ratio [HR]=1.20, P=0.006), more cytomegalovirus (CMV) reactivation (HR=3.65, P=0.038) and more ES (HR=3.18, P=0.078). Conclusions Our findings support chemotherapy-based regimens with early neutrophil engraftment, less ES and CMV reactivation compared with TBI. Although there is no impact on survival rates, increased incidence of ES and CMV reactivation should be considered in TBI-based regimens.

BACKGROUND The posterior cervical keyhole (KH) laminoforaminotomy has been described to involve the lateral portion of cervical laminae of the upper vertebra alone (small KH) or of both upper and lower vertebrae (large KH). OBJECTIVE To microscopically compare the two keyhole techniques in terms of their ability to expose the corresponding cervical roots. METHODS Ten cadaveric specimens were operated bilaterally from C3-4 to C6-7 level to expose a total of 80 nerve roots. The large KH was applied to the left side, the small KH to the right side. The maximal length of exposed nerve roots was measured under microscope. The virtual optimal KH surface area was determined using digital software. Each root was inspected for exposure of its root and axilla. RESULTS The maximal exposed nerve root length on the large KH side was significantly larger than on the small KH side at C3-4, C5-6, and C6-7 levels (P = .031, P = .002, P = .003). No significance was reported for C4-5 (P = .06). We could expose right axillae in (3/40) and left axillae in (33/40; P

Background: Atherosclerotic cardiovascular disease influences young patients as well as the elderly. A large proportion of patients with coronary artery disease (CAD) have metabolic syndrome (MS), although the frequency and association of its different components are not clear. To the best of our knowledge, there is no any study about the prevalence of MS among young patients with ST-elevation myocardial infarction (STEMI). We sought to determine the prevalence of MS in patients below 46 years of age who presented with STEMI and underwent primary percutaneous coronary intervention. Methods: In the present prospective study, 141 consecutive patients with STEMI