Metastatic adult-type non-rhabdomyosarcoma soft tissue sarcomas in children and adolescents: A cohort study from the European paediatric Soft tissue sarcoma Study Group

Background

Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined.

Methods

This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group. Treatment programs were originally designed for patients with metastatic rhabdomyosarcoma, i.e., nine courses of multidrug chemotherapy (with or without bevacizumab in the BERNIE study), followed by 12 cycles of maintenance therapy, whereas radiotherapy and/or surgery (on primary tumor and/or metastases) were delayed until after seven courses of chemotherapy had been administered.

Results

The study included 61 patients <21 years old treated from July 2008 to December 2016. The lung was the site of metastases in 75% of the cases. All patients received multi-agent chemotherapy, 44% had local therapy to primary tumor, and 18% had treatment of metastases. Median time to progression/relapse was 6 months. A high rate of tumor progression was observed during the initial part of the chemotherapy program. With a median follow-up of 41.5 months (range, 2–111 months), 3-year event-free survival and overall survival were 15.4% (95% confidence interval [CI], 7.6–25.7) and 34.9% (95% CI, 22.7–47.5), respectively. There were no statistically significant differences in outcome depending on the type of treatment administered.

Conclusions

The study confirmed the overall poor outcome for patients with metastatic NRSTS, whose treatment remains a challenge.

Plain Language Summary

• Pediatric non-rhabdomyosarcoma soft tissue sarcomas form a heterogeneous group of rare tumors.
• Although recent international studies have defined the standard of care for patients with localized disease, limited data are available on the clinical behavior of patients with distant metastases.
• This study on 61 metastatic cases treated on two prospective European protocols confirms that the chances of survival of such patients are often dismal and a standard treatment is still lacking.

     

Novel insights into the SLC7A11-mediated ferroptosis signaling pathways in preeclampsia patients: identifying pannexin 1 and toll-like receptor 4 as innovative prospective diagnostic biomarkers

Journal of Assisted Reproduction and Genetics - Ferroptosis is associated with oxidative stress (OS) and is caused by iron-dependent lipid-peroxidative damage, but its role in PE is unclear. The...     

Working memory training and brain structure and function in extremely preterm or extremely low birth weight children

This study in children born extremely preterm (EP; <28 weeks’ gestational age) or extremely low birth weight (ELBW; <1,000 g) investigated whether adaptive working memory training using Cogmed® is associated with structural and/or functional brain changes compared with a placebo program. Ninety‐one EP/ELBW children were recruited at a mean (standard deviation) age of 7.8 (0.4) years. Children were randomly allocated to Cogmed or placebo (45‐min sessions, 5 days a week over 5–7 weeks). A subset had usable magnetic resonance imaging (MRI) data pretraining and 2 weeks posttraining (structural, n = 48; diffusion, n = 43; task‐based functional, n = 18). Statistical analyses examined whether cortical morphometry, white matter microstructure and blood oxygenation level‐dependent (BOLD) signal during an n‐back working memory task changed from pretraining to posttraining in the Cogmed and placebo groups separately. Interaction analyses between time point and group were then performed. There was a significant increase in neurite density in several white matter regions from pretraining to posttraining in both the Cogmed and placebo groups. BOLD signal in the posterior cingulate and precuneus cortices during the n‐back task increased from pretraining to posttraining in the Cogmed but not placebo group. Evidence for group‐by‐time interactions for the MRI measures was weak, suggesting that brain changes generally did not differ between Cogmed and placebo groups. Overall, while some structural and functional MRI changes between the pretraining and posttraining period in EP/ELBW children were observed, there was little evidence of training‐induced neuroplasticity, with changes generally identified in both groups. Trial registration Australian New Zealand Clinical Trials Registry, anzctr.org.au ; ACTRN12612000124831.     

Study of the gut Microbiome Profile in Children with Autism Spectrum Disorder: a Single Tertiary Hospital Experience

The role of gut microbiome was recently raised in the pathogenesis of neurodevelopmental disorders including autism spectrum disorder (ASD). The aim of this study was to elucidate changes in gut microbiome in Egyptian autistic children and its possible correlation with the severity of autism and gastrointestinal (GI) symptoms. The gut bacterial microbiome of 41 ASD children, 45 siblings, and 45 healthy controls were analyzed using quantitative SYBR Green real-time PCR technique targeting 16S rRNA of selected bacteria. The gut microbiome of ASD children and their siblings contained a higher relative abundance of Bacteroides as well as Ruminococcus than controls. Prevotella/Bacteroides (P/B) ratio and Firmicutes/Bacteroidetes (F/B) were significantly lower in both ASD cases and their siblings. The only difference between the autistic cases and their siblings was the significantly higher level of Bifidobacterium in siblings, which appears to offer them a protective role. There was no correlation between the altered gut microbiome and the severity of autism or GI symptoms. The current study showed an evidence of changes in the gut microbiome of autistic children compared to the unrelated control. However, the microbiome profile of siblings was more like that of autistic children than that of unrelated controls indicating that gut microbiota is affected by dietary habits, living conditions together with host genetic factors.     

髋臼横韧带定位髋臼假体前倾校正骨盆旋转

目的通过观察后外侧入路全髋关节置换术中骨盆的旋转变化，探讨术中骨盆旋转对髋臼假体前倾角植入的影响，评估使用髋臼横韧带作髋臼假体前倾定位的准确性以及对骨盆旋转角度变化的校正作用。 方法2015年1月至2016年1月河池市第三人民医院关节外科收治的行初次THA的40例44髋的髋关节疾病患者纳入本研究。纳入标准为：初次THA术的患者，术前、术后CT扫描质量符合标准、能确定髋臼解剖前倾角、髋臼假体前倾角的患者。排除标准：髋臼发育不良、强直性脊柱炎、既往有髋关节严重创伤手术史以及翻修术等，术前、术后双髋关节CT扫描，CT横断面上骨盆明显倾斜、两侧髋关节的中心显示明显不在同一层面、难以确定水平线测量前倾角的患者，予以排除。手术均采用侧卧位后外侧入路，切皮前将1枚施氏针以垂直于地面方向打入髂骨嵴，术中髋臼假体的前倾角，以髋臼横韧带为参照标志，通过直接参照或间接参照髋臼横韧带进行髋臼挫磨及安装臼杯，使髋臼假体开口平行韧带进行安放，在此过程中用摄像机记录施氏针相对于地面的角度变化，确定手术过程中骨盆旋转度数，同时测量并记录挫磨髋臼及安放假体时，相对于身体长轴髋臼手术前倾角的数值。术后通过CT测量髋臼假体前倾角，对术中手术前倾角和术后髋臼假体的前倾角、术前髋臼解剖前倾角数据进行t检验分析。 结果术中骨盆旋转发生在本研究中的平均度数为(18±4)°。44个髋关节中，所有的病例都能对髋臼横韧带进行辨认，术中手术前倾角平均为(33±5)°，有93%(41髋)的病例大于Lewinnek提出的"安全区"的前倾角上限25°，余下的7%(3个髋)也全部大于24°。术后CT测量髋臼假体的解剖前倾角为(21±10)°，与术前髋臼解剖前倾角度(19±7)°比较，差异无统计学意义(t＝1.264，P >0.05)。 结论在后外侧入路THA术中，体位改变骨盆前旋转会影响髋臼假体植入的准确性，使用髋臼横韧带作为解剖标志指导髋臼假体前倾角度的植入，可以排除患者体位改变骨盆旋转对前倾角的影响，提高髋臼假体放置的准确性。     

Optimal Combination of Soy,Buffalo, and Cow's Milk in Bioyogurt for Optimal Chemical,Nutritional, and Health Benefits

Objective: Soy milk is enriched with nutritive elements such as proteins, unsaturated fatty acids, lecithins, isoflavones, mineral substances, free amino acids, and polypeptides. The aim of this study was to increase the health and nutritional values of bioyogurt by using ABT-5 culture and mixing soy milk with buffalo and cow's milk.

Methods: Five treatments of yogurt were made from soy, buffalo, and cow's milk and from 75% buffalo + 25% soy milk mixture and 75% cow + 25% soy milk mixture using ABT-5 culture.

Results: Utilization of soy milk in yogurt production decreased acidity, redox potential (E_h), total solids (TS), fat, total nitrogen, ash, total volatile fatty acids (TVFAs), saturated fatty acids (SFAs), monounsaturated fatty acids (MUFAs), and total amino acids contents. In contrast, the highest levels of unsaturated fatty acids (USFAs), polyunsaturated fatty acids (PUFAs), linoleic acid, and α-linolenic acid were detected. Higher numbers of L. acidophilus and bifidobacteria were found in soy milk yogurt than in buffalo or cow's milk. Blending of buffalo or cow's milk with soy milk increased the sensory evaluation scores of yogurt.

Conclusions: Mixtures of 25% soy milk + 75% buffalo milk, 25% soy milk + 75% cow's milk and ABT-5 culture could be successfully used in production of bioyogurt characterized by high health and nutritional properties. These optimal combinations highly enhanced probiotic bacteria. The recommended level of 10⁷ cfu.g^?1 of bifidobacteria as a probiotic was exceeded for soy milk yogurt treatments.     

Minilaparoscopic appendectomy using a new spiral needle

Background

Acute appendicitis is the most frequent abdominal disease and requires urgent surgery. At the present time, laparoscopic appendectomy is a well-accepted emergency procedure at most centers. In this study, we used a new spiral needle to facilitate the procedure, making it easy, minimally invasive, and cost effective.

Methods

The study included 70 patients of both sexes with acute appendicitis that was treated by laparoscopic appendectomy using a new spiral needle. These cases were treated at the General Surgery Department, Zagazig University, Egypt, from May 2012 to August 2013. In the procedure we used only two ports (a 10-mm port directly below the umbilicus for the camera and a 5-mm port at the left iliac fossa at the midclavicular line). The new spiral needle was used to hold the appendix during the procedure and was inserted into the right iliac fossa depending on the site of the appendix.

Results

The mean age of the patients was 27.3 years, mean operative time was 40 min, and mean hospital stay was 1.3 days. Ten patients (14 %) had minimal bleeding at the site of needle passage into the mesoappendix. Wound infection at the site of umbilical port occurred in four patients (5.7 %) postoperatively.

Conclusion

Laparoscopic appendectomy using our new spiral needle is easy, minimally invasive, and cost effective.     

HLA-G Gene Polymorphism in Egyptian Patients with Non-Hodgkin Lymphoma and its Clinical Outcome

Background: Non-Hodgkin lymphoma (NHL) is a major cancer in Egypt and worldwide and has many risk factors including genes involved in the immune response. Aim: we investigated the HLA-G 14bp gene polymorphism as a risk factor for NHL and its clinic pathologic features. The study involved 150 patients with NHL and 100 healthy control. Full histories, clinical examination, C.T scan and laboratory investigations such as CBC, LDH, ?2microglobulin and HCV RNA by qualitative real time PCR were performed for all subjects. HLA-G 14bp ins/del gene polymorphism was determined by PCR. Results: in our study, del/del, ins/del and dominant genotypes increased the risk of NHL by 11.01, 10.55 and 10.88 fold respectively (p<0.001) but the recessive genotype did not increase the risk of NHL (p=0.112). Cases with the del allele had a greater risk of NHL than those with the ins allele (p<0.001). del/del and ins/del genotypes were significantly associated with higher LDH and ?2microglobulin levels (p<0.001), lower Hb and platelet values (p<0.001), extra nodal sites (p=0.001), poor performance status (p=0.04) and relapse (p=0.001). Conclusions: the results suggest that HLA-G 14bp ins/del gene polymorphism is a risk factor for NHL in our Egyptian population and is associated with poor clinical pathological features.

Abbreviations: Non-Hodgkin lymphoma (NHL), Diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL), Epstein-Barr virus (EBV), human T-cell lymphotropic/leukemia virus-1 (HTLV-1)     

HSP70 as a Diagnostic and Prognostic Marker in Egyptian Women With Breast Cancer

BackgroundHeat shock protein 70 (HSP70) is a significant cellular stress response protein that has intrinsic and extrinsic pathways to protect cells against apoptosis. It is one of the most induced proteins in cancer cells. The aim of the present study is to investigate the significant role of the HSP70 expression in Egyptian patients with breast cancer (BC) and its potential to be as a diagnostic and prognostic marker.Materials and MethodsHSP70 was examined in 155 cases in this prospective study; patients were subdivided into 3 groups: 60 patients with malignant metastatic disease, 60 patients with malignant non-metastatic disease, and 35 patients with benign lesions as control. HSP70 expression was detected using enzyme-linked immunosorbent assay (ELISA) and immunohistochemistry (IHC).ResultsMost cases of breast cancer expressed HSP70 in both serum (98.3%) and tumor tissue (90%). A strong positive correlation was found between HSP70 IHC and ELISA (r = 0.811). The mean HSP70 levels, as detected in both patients’ serum by ELISA and tumor tissue by IHC, was significantly higher in patients with BC than in benign cases (P = .001). HSP70 was significantly higher in patients with metastatic BC than in those with non-metastatic BC (P = .001). HSP70 showed positive correlation with tumor size (pT stage) and number of lymph node metastases (P ≤ .001).ConclusionHSP70 is over-expressed in patients with metastatic and non-metastatic BC than in benign cases. A high level of HSP70 either in patient’s serum or in tumor tissue correlated significantly with advanced disease in patients with BC. This present study suggests that HSP70 can serve as a BC biomarker for early screening, diagnosis, and follow-up.