HLA‐matched family hematopoetic stem cell transplantation in children with beta thalassemia major: The experience of the Turkish Pediatric Bone Marrow Transplantation Group

Yesilipek MA, Ertem M, Cetin M, Öniz H, Kansoy S, Tanyeli A, Anak S, Kurekci E, Hazar V. HLA‐matched family hematopoetic stem cell transplantation in children with beta thalassemia major: The experience of the Turkish Pediatric Bone Marrow Transplantation Group. Abstract: From January 1991 to June 2009, 245 children with beta thalassemia major who underwent their first allogeneic HSCT in Turkey and who were followed for a minimum of one yr post‐transplantation were enrolled this study. The median age of the patients was 6.6 yr old (range, 1–22 yr). The distribution of Pesaro risk class I, II, and III categories was 41, 130, and 63 children, respectively. The median serum ferritin level was 2203 ng/mL. Eighty‐eight patients received bone marrow (BM) stem cells; 137, peripheral blood (PB) stem cells; and 20, cord blood (CB) stem cells. The donors were HLA‐matched siblings or parents. Median engraftment times were shorter in PBSCT patients compared with the BMT group (p < 0.001). Grade II‐IV acute GvHD was observed in 33 children (13.5%), while cGvHD was observed in 28 patients (12.5%), eight of whom had the extensive form. Thalassemic reconstitution was observed in 43 (17%) of the transplant patients. Post‐transplant aplasia occurred in three patients, and the TRM rate was 7.75%. Seventeen patients were lost after 100 days. The thalassemia‐free survival and OS rates were 68% (95% CI, 61.8–74.2) and 85.0% (95% CI, 80.2–89.8), respectively. We believe that this study is important because it is the first multicenter national data for children with beta thalassemia major receiving HSCT.     

An iatrogenic femoral nerve injury after open reduction and displacement iliac osteotomy for hip dysplasia: a case report

In this report, we present a 4-year-old female patient who came to our clinic complaining of symptoms that were then attributed to a right femoral nerve injury, 15 months after open reduction, and innominate osteotomy operations performed at another orthopedic center. The operations were performed using the Smith–Peterson incision technique and led to a neurotmetic femoral nerve injury. In our clinic, we repaired damaged femoral nerve by sural nerve graft using interfascicular technique. After 6 years, she was walking without additional device or hand to stabilize the knee.     

Schizophrenia and the efficacy of qEEG-guided neurofeedback treatment: a clinical case series

Schizophrenia is sometimes considered one of the most devastating of mental illnesses because its onset is early in a patient's life and its symptoms can be destructive to the patient, the family, and friends. Schizophrenia affects 1 in 100 people at some point during their lives, and while there is no cure, it is treatable with antipsychotic medications. According to the Clinical Antipsychotic Trials for Interventions Effectiveness (CATIE), about 74% of the patients who have discontinued the first medication prescribed within a year will have a relapse afterward. This shows an enormous need for developing better treatment methods and better ways to manage the disease, since current therapies do not have sufficient impact on negative symptoms, cognitive dysfunction, and compliance to treatment. In this clinical case series, we investigate the efficacy of quantitative electroencephalography (qEEG)-guided neurofeedback (NF) treatment in this population, and whether this method has an effect on concurrent medical treatment and on the patients. Fifty-one participants (25 males and 26 females) ranging from 17 to 54 years of age (mean: 28.82 years and SD: 7.94 years) were included. Signed consent was received from all patients. Most of the participants were previously diagnosed with chronic schizophrenia, and their symptoms did not improve with medication. All 51 patients were evaluated using qEEG, which was recorded at baseline and following treatment. Before recording the qEEG, participants were washed out for up to 7 half-lives of the medication. After Food and Drug Administration (FDA)-approved Nx-Link Neurometric analysis, qEEGs suggested a diagnosis of chronic schizophrenia for all participants. This was consistent with the clinical judgment of the authors. The participants' symptoms were assessed by means of the Positive and Negative Syndrome Scale (PANSS). Besides the PANSS, 33 out of 51 participants were also evaluated by the Minnesota Multiphasic Personality Inventory (MMPI) and the Test of Variables of Attention (TOVA), both at baseline and following treatment. Each participant was prescribed an NF treatment protocol based on the results of their qEEG neurometric analysis. Each session was 60 minutes in duration, with 1 to 2 sessions per day. When 2 sessions were administered during a single day, a 30-minute rest was given between the sessions. Changes in the PANSS, MMPI, and TOVA were analyzed to evaluate the effectiveness of NF treatment. The mean number of sessions completed by the participants was 58.5 sessions within 24 to 91 days. Three dropped out of treatment between 30 and 40 sessions of NF, and one did not show any response. Of the remaining 48 participants 47 showed clinical improvement after NF treatment, based on changes in their PANSS scores. The participants who were able to take the MMPI and the TOVA showed significant improvements in these measures as well. Forty were followed up for more than 22 months, 2 for 1 year, 1 for 9 months, and 3 for between 1 and 3 months after completion of NF. Overall NF was shown to be effective. This study provides the first evidence for positive effects of NF in schizophrenia.     

巢式逆转录-聚合酶链反应扩增人骨形成蛋白2全长cDNA及其真核表达载体的构建

目的:利用巢式逆转录-聚合酶链反应扩增的方法,从肌肉组织中扩增人骨形成蛋白2全长cDNA并构建真核表达载体系统。方法:实验于2003-10/2005-10在苏州大学基因工程教研室和北京大学第三医院骨科实验室完成。提取成人肌肉组织内的总RNA,设计内外两对引物以巢式逆转录-聚合酶链反应扩增方法分两次扩增出人骨形成蛋白2全长1188bp基因,经T-A克隆装入pUCM-T质粒载体内,测序验证后,将克隆质粒以Hind Ⅲ和Xba Ⅰ双酶切后与pcDNA3.0载体相连接,构建真核表达载体系统。结果:利用巢式逆转录-聚合酶链反应扩增方法能从成人肌肉组织内扩增出1188bp的人骨形成蛋白2全长cDNA基因,其测序结果显示与Genebank报道序列完全相符。将扩增序列双酶切后与pcDNA3.0载体相连接,经电泳验证,能构建人骨形成蛋白2全长基因的真核表达系统。结论:巢式逆转录-聚合酶链反应扩增方法能从成人肌肉组织内扩增出人骨形成蛋白2全长cDNA基因,并克隆构建真核表达载体系统,为下一步基因组织工程人工骨实验奠定基础。     

黄褐毛忍冬皂甙对对乙酰氨基酚致小鼠肝脏毒性的保护作用

黄褐毛忍冬皂甙对对乙酰氨基酚致小鼠肝脏毒性的保护作用时京珍，刘耕陶（贵阳贵州省中医研究所５５０００２；中国医学科学院药物研究所１０００５０）我们已报道黄褐毛忍冬总皂甙对实验性肝损伤有保护作用［１］。经进一步实验发现其中的两种成分，即Ｈ和Ｓ均为保肝作用...