bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clear cell sarcoma of the kidney: Patients' characteristics and improved outcome in developing countries
Authors: Zekri Wael , Alfaar Ahmad Samir , Yehia Dina , Elshafie Maged M. , Zaghloul Mohamed Saad , El‐Kinaai Naglaa , Taha Hala , Refaat Amal , Younes Alaa A. 
Published: August 2014

BackgroundClear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor accounting for 5% of pediatric renal tumors with an incidence of 20 patients per year in the USA. It is bone metastasizing with poor prognosis. Our aim was to show characteristics of patients in relation to improved outcome in one of the developing countries.ProcedureWe included all patients diagnosed as CCSK in the period between July 2007 and March 2012 at Children's Cancer Hospital, Egypt. Patients' demographics, clinical presentation, pathology, and management were reviewed. Follow up was continued until April 2013.ResultsTwenty‐five patients were identified in the defined time interval, accounting for 7% all renal tumors diagnosed at the hospital. Mean age was 36 months. Abdominal swelling and hematuria were the most common presentations. Stages I, II, III, IV, and V represented 9 (36%), 3 (12%), 8 (32%), 3 (12%), and 2 (8%), respectively. Twenty‐four patients had radical nephrectomy either upfront or after neo‐adjuvant chemotherapy. Surgery was followed by adjuvant chemotherapy. Abdominal radiotherapy was given for local stages II and III. Twenty‐two patients reached complete remission, while one patient had stationary disease and two patients died due to progression and relapse. Overall survival was 88.5% and event‐free survival was 87.8% at 45 months.ConclusionAlthough previous studies indicate poor prognosis of CCSK, our experience shows that those patients can be treated using extensive chemotherapy combined with proper local control. Pediatr Blood Cancer 2014;61:2185–2190. © 2014 Wiley Periodicals, Inc.
Family life events in the first year of acute lymphoblastic leukemia therapy: A children's oncology group report
Authors: Lau Samantha , Lu Xiaomin , Balsamo Lyn , Devidas Meenakshi , Winick Naomi , Hunger Stephen P. , Carroll William , Stork Linda , Maloney Kelly , Kadan‐Lottick Nina 
Published: August 2014

BackgroundDespite higher cure rates, childhood acute lymphoblastic leukemia (ALL) may continue to result in considerable family strain. We sought to (i) measure incidence of divorce, reduced career opportunities, changes to work hours, home relocation, and changes to family planning at one year after ALL diagnosis; and (ii) Identify family and patient factors associated with these events.ProcedureWe conducted a prospective cohort study of 159 children with average risk‐ALL enrolled and treated on COG protocol AALL0331 at 31 selected sites. Eligibility criteria included age ≥2 years and English or Spanish comprehension. Parents completed surveys at three time points during the first 12 months of therapy.ResultsParents were at significantly increased risk of loss of employment (46% vs. 9.1%, P ≤ 0.001) than peers nationally. 13% divorced/separated, 27% relocated homes, 22% decided not to have more children, 51% declined occupational opportunities, and 68% decreased work hours. In adjusted analyses, relocation correlated with less maternal education (OR: 4.27 [95% CI: 1.43–12.82]). Declining parental opportunities associated with family income <$50,000 (OR: 4.25 [95% CI: 1.50–12.02]) and child <5 years old (OR: 4.21 [95% CI: 1.73–10.25]). Deciding not to have more children correlated with smaller family size 2–3 versus 4–5 (OR: 3.62 [95% CI: 1.10–11.96]).ConclusionFamilies experience a high incidence of major life changes in the first year of ALL treatment. Understanding these burdens helps health care providers to provide appropriate anticipatory guidance and support. No unifying factor was associated with the different family events. Ongoing follow‐up is planned to measure long‐term outcomes. Pediatr Blood Cancer 2014;61:2277–2284. © 2014 Wiley Periodicals, Inc.
Design of a drug‐drug interaction study of vincristine with azole antifungals in pediatric cancer patients using clinical trial simulation
Authors: van Hasselt J. G. Coen , van Eijkelenburg Natasha K. A. , Beijnen Jos H. , Schellens Jan H.M. , Huitema Alwin D. R. 
Published: August 2014

BackgroundThe aim of the current work was to perform a clinical trial simulation (CTS) analysis to optimize a drug‐drug interaction (DDI) study of vincristine in children who also received azole antifungals, taking into account challenges of conducting clinical trials in this population, and, to provide a motivating example of the application of CTS in the design of pediatric oncology clinical trials.ProcedureA pharmacokinetic (PK) model for vincristine in children was used to simulate concentration‐time profiles. A continuous model for body surface area versus age was defined based on pediatric growth curves. Informative sampling time windows were derived using D‐optimal design. The CTS framework was used to different magnitudes of clearance inhibition (10%, 25%, or 40%), sample size (30–500), the impact of missing samples or sampling occasions, and the age distribution, on the power to detect a significant inhibition effect, and in addition, the relative estimation error (REE) of the interaction effect.ResultsA minimum group specific sample size of 38 patients with a total sample size of 150 patients was required to detect a clearance inhibition effect of 40% with 80% power, while in the case of a lower effect of clearance inhibition, a substantially larger sample size was required. However, for the majority of re‐estimated drug effects, the inhibition effect could be estimated precisely (REE < 25%) in even smaller sample sizes and with lower effect sizes.ConclusionThis work demonstrated the utility of CTS for the evaluation of PK clinical trial designs in the pediatric oncology population. Pediatr Blood Cancer 2014;61:2223–2229. © 2014 Wiley Periodicals, Inc.
Nothing but NET: A review of norepinephrine transporter expression and efficacy of 131I‐mIBG therapy
Authors: Streby Keri A. , Shah Nilay , Ranalli Mark A. , Kunkler Anne , Cripe Timothy P. 
Published: August 2014

AbstractNeuroblastoma is unique amongst common pediatric cancers for its expression of the norepinephrine transporter (NET), enabling tumor‐selective imaging and therapy with radioactive analogues of norepinephrine. The majority of neuroblastoma tumors are avid for 123I‐metaiodobenzaguanidine (mIBG) on imaging, yet the therapeutic response to 131I‐mIBG is only 30% in clinical trials, and off‐target effects cause short‐ and long‐term morbidity. We review the contemporary understanding of the tumor‐selective uptake, retention, and efflux of meta‐iodobenzylguanidine (mIBG) and strategies currently in development for improving its efficacy. Combination treatment strategies aimed at enhancing NET are likely necessary to reach the full potential of 131I‐mIBG therapy. Pediatr Blood Cancer 2015;62:5–11 © 2014 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals, Inc.
Subclinical cardiotoxicity in childhood cancer survivors exposed to very low dose anthracycline therapy
Authors: Leger Kasey , Slone Tamra , Lemler Matthew , Leonard David , Cochran Cindy , Bowman W. Paul , Bashore Lisa , Winick Naomi 
Published: August 2014

BackgroundSubclinical cardiotoxicity occurs in childhood cancer survivors following moderate and high anthracycline doses. However, less is known about the subclinical changes in left ventricular (LV) structure that occur after very low anthracycline doses of ≤100 mg/m2. This study was designed to assess LV function and key structural parameters following very low doses of anthracycline.ProcedureConventional 2‐dimensional echocardiograms with Doppler were obtained in 91 survivors of childhood cancer exposed to ≤100 mg/m2,an average of 9.8 years from diagnosis. LV structural measurements were converted into Z‐scores. The Z‐score distributions were compared to those of the normal population. Diastolic and systolic function were quantified.ResultsThe cohort demonstrated a decreased posterior wall thickness (mean Z‐score −1.01) and mildly decreased LV end diastolic (mean Z‐score −0.85) and systolic (mean Z‐score −0.84) dimensions compared to the normal population (P < 0.001). Further, 28% of patients (n = 25) had abnormal LV posterior wall thickness, ≥2 standard deviations below the mean (Z‐score ≤ −2). There were no patients with diastolic dysfunction or symptomatic systolic dysfunction, however four patients demonstrated abnormal SF ≤ 28%.ConclusionsA significant proportion of patients exposed to very low doses of anthracycline demonstrate subclinical abnormalities in LV structure, despite the absence of radiation or other risk factors. While we cannot say whether these structural changes will result in clinically significant cardiac disease, the reported progressive nature of these findings raises concern that there may truly be no safe dose of anthracycline. Pediatr Blood Cancer 2015;62:123–127. © 2014 Wiley Periodicals, Inc.
Population based surveillance in sickle cell disease: Methods, findings and implications from the California registry and surveillance system in hemoglobinopathies project (RuSH)
Authors: Paulukonis Susan T. , Harris William T. , Coates Thomas D. , Neumayr Lynne , Treadwell Marsha , Vichinsky Elliott , Feuchtbaum Lisa B. 
Published: August 2014

AbstractBackgroundThere are no population‐based surveillance systems to determine prevalence, impact or outcomes in sickle cell disease (SCD). Estimates of the SCD population in California range broadly from 4,500 to 7,000, and little is known about their health status, health care utilization or health outcomes. A surveillance strategy was implemented using diverse data sources to develop a multi‐dimensional, state‐based surveillance system for SCD that includes adults and children and describes utilization, treatment and outcomes.ProcedureData from California newborn screening, inpatient and emergency room records, Medi‐Cal/Medicaid claims and two SCD special care centers were collected for 2004–2008. A multi‐step, iterative linkage process was used to link and de‐duplicate these data sources, and case definitions were used to categorize cases.ResultsAfter linking and de‐duplicating, there were 1,975 confirmed cases of SCD, 3,159 probable cases as well as 8,024 possible cases. Among individual data sources, newborn screening and data from clinics contributed the greatest number of unique cases to the total. Select analyses of utilization and treatments for the population are described.ConclusionsUsing linked existing data sources, an estimate of the statewide count of the SCD population is possible. The approach can be used to create an in‐depth health status profile of the affected population by aggregating utilization, treatment, and outcomes data including mortality and morbidity information. This effort sets the stage for development of an on‐going, state‐based surveillance system. Pediatr Blood Cancer 2014;61:2271–2276. © 2014 Wiley Periodicals, Inc.
Ewing‐like sarcomas with BCOR‐CCNB3 fusion transcript: A clinical, radiological and pathological retrospective study from the Société Française des Cancers de L'Enfant
Authors: Cohen‐Gogo Sarah , Cellier Cécile , Coindre Jean‐Michel , Mosseri Véronique , Pierron Gaëlle , Guillemet Cécile , Italiano Antoine , Brugières Laurence , Orbach Daniel , Laurence Valérie , Delattre Olivier , Michon Jean 
Published: August 2014

BackgroundThis retrospective multicenter study assessed the clinical, radiological and pathological presentation, treatment and outcome of 26 patients with Ewing‐like sarcoma harboring BCOR‐CCNB3 gene fusion transcript. Tumor samples had been collected between 1994 and April 2012.ProcedureEligibility criteria included assessment of a BCOR‐CCNB3 transcript‐positive tumor after molecular analysis and availability of minimal clinical and pathological data. Radiological data were also retrieved when possible. Data were analyzed by descriptive statistics and methods for survival analysis.ResultsMedian age at diagnosis was 13.1 years (5.9 to 25.6 years). Most patients (24/26) had localized tumors. All tumors but five were localized to bone. CCNB3 immunochemistry showed strong nuclear staining on all samples. No specific radiological features were found. Most patients received chemotherapy (15 according to protocols designed for Ewing tumors), before and/or after local treatment (surgery and/or radiotherapy, with 46.2% receiving both). Local and metastatic relapses were of poor prognosis. Induction chemotherapy and treatment according to an Ewing protocol might influence survival for patients with localized tumors. Sixteen patients are alive in complete remission with a median follow‐up of 86 months. Five year overall survival and disease‐free survival were respectively 76.5% (95% CI, 58%–95%) and 67.9% (95% CI, 48%–88%).ConclusionsBCOR‐CCNB3 transcript‐positive Ewing‐like sarcoma diagnosis should be discussed for a transcript‐negative small round cell sarcoma in a child, adolescent or young adult patient. Diagnosis needs to be stated through CCNB3 immunochemistry or transcript identification. The exquisite chemosensitivity of these tumors should encourage the use of polychemotherapy for appropriate care, associated with best local tumor control. Pediatr Blood Cancer 2014;61:2191–2198. © 2014 Wiley Periodicals, Inc.
CALR mutation screening in pediatric primary myelofibrosis
Authors: An Wenbin , Wan Yang , Guo Ye , Chen Xiaojuan , Ren Yuanyuan , Zhang Jingliao , Chang Lixian , Wei Wei , Zhang Peihong , Zhu Xiaofan 
Published: August 2014

BackgroundPrimary myelofibrosis (PMF) is quite rare in children. Mutations of JAK2V617F or MPLW515K/L were absent in pediatric patients with PMF according to previous studies. Recently, mutations in calreticulin (CALR) were described in adult patients with JAK2/MPL‐unmutated PMF. Our study aimed to analyze the clinical and genetic features of Chinese pediatric patients with PMF.ProceduresWe retrospectively investigated 14 pediatric patients diagnosed as PMF according to WHO 2008 criteria. Direct sequencing was performed for the existence of genetic alterations in JAK2, MPL, TET2, CBL, ASXL1, IDH1, IDH2, SRSF2, EZH2, DNMT3A and CALR.ResultsIn our cohort, all patients had anemia, three patients (21%) had splenomegaly, six patients (43%) had micromegakaryocytes at time of diagnosis. No patient had spontaneous remission and six patients (43%) transformed to acute myelocytic leukemia. In nine patients with evaluable cytogenetic information, three subjects (33%) had abnormal karyotypes. The median survival from time of diagnosis was 28 months. Seven patients (50%) had type 2 mutations of CALR. No patient had mutations in the other candidate genes. There was no statistical differences in age, gender, hemoglobin, WBC, neutrophil and platelet counts, percentage of circulating blast, overall survival and leukemia transformation between patients with and without CALR mutation.ConclusionOur study documented that Chinese pediatric patients with PMF in our cohort had its own clinical characteristics and poor outcome. CALR mutations were detected in 50% of our pediatric patients with PMF. Based on our study, CALR mutations screening could be used as molecular marker for diagnosis of pediatric patients with PMF. Pediatr Blood Cancer 2014;61:2256–2262. © 2014 Wiley Periodicals, Inc.
Utility of bone marrow aspiration and biopsy in initial staging of Ewing sarcoma
Authors: Kopp Lisa M. , Hu Chengcheng , Rozo Beatriz , White‐Collins Andrea , Huh Winston W. , Yarborough Angela , Herzog Cynthia E. , Hingorani Pooja 
Published: August 2014

BackgroundThe current standard of care for initial staging of pediatric Ewing sarcoma (EWS) patients is to obtain a bilateral bone marrow aspiration and biopsy (BMAB). The incidence of bone marrow (BM) disease in patients deemed non‐metastatic by conventional and metabolic imaging and the concordance of BM positivity with other clinical characteristics are not well established.ProcedureThis study is a multi‐institutional retrospective review of newly diagnosed EWS patients less than 40 years of age with initial staging that included imaging and BMAB.ResultsA total of 116 patients were eligible with 85 patients considered non‐metastatic and 31 considered metastatic by imaging. None of the 85 patients with non‐metastatic disease were BMAB positive (0%; 95% CI: 0–4.2%); 13 of the 31 patients with metastases were BMAB positive (41.9%; 95% CI: 24.5–60.9%). Primary tumor size was significantly higher in patients with metastases (P = 0.017). Bone metastasis by imaging had high correlation with BMAB positivity (P = 0.0002). In addition, the number of bony metastatic sites was significantly higher in patients with a positive BMAB as compared to those with a negative BMAB (median 3.5 and 0.0, respectively; P < 0.001).ConclusionsBMAB may not be required for initial staging of pediatric and young adult EWS patients deemed non‐metastatic by imaging. In patients with metastatic disease, there is a high correlation of BM involvement with multiple bone metastases. Pediatr Blood Cancer 2015;62:12–15. © 2014 Wiley Periodicals, Inc.
Capacity building and stroke risk assessment in Nigerian children with sickle cell anaemia
Authors: Soyebi Kofo , Adeyemo Titilope , Ojewunmi Oyesola , James Funmi , Adefalujo Kunle , Akinyanju Olu 
Published: August 2014

BackgroundTranscranial Doppler ultrasonography (TCD) measures blood flow velocities in the large cerebral vessels and thus, detects the risk of stroke. This report describes a capacity building program which enabled the use of TCD for detecting stroke risk and also describes the pattern of non‐imaging TCD examinations seen in Nigerian children with sickle cell anaemia (SCA).ProcedureTen university graduates were trained on the use of TCD in a 5‐day capacity building workshop after which, the three best candidates were employed to provide a 5‐day a week TCD screening service in Lagos. Data from TCD examination collected between March 2011 and September 2013 were analysed and reported.ResultBetween March 2011 and September 2013, 2,331 children with SCA aged 2–16 years had TCD studies. TCD's findings were classified as normal (standard risk) in 70.4%, conditional in 19% and abnormal (high risk) in 9.3%. The majority of children (76.9%) in the high risk category were aged 2–8 years. TCD study was inadequate for risk categorisation in 1.3% of the patients.ConclusionEffective capacity building of middle level manpower is feasible and can provide a credible TCD screening service to communities with a high demand and a shortage of trained professionals. The pattern of TCD abnormalities seen in Africa are comparable to those obtained in several previous worldwide reports. Pediatr Blood Cancer 2014;61:2263–2266. © 2014 Wiley Periodicals, Inc.
Comparison of Soxhlet, Accelerated Solvent and Supercritical Fluid Extraction Techniques for Volatile (GC–MS and GC/FID) and Phenolic Compounds (HPLC–ESI/MS/MS) from Lamiaceae Species
Authors: Rodríguez‐Solana Raquel , Salgado José Manuel , Domínguez José Manuel , Cortés‐Diéguez Sandra 
Published: September 2014

ABSTRACTIntroductionPlants from the Lamiaceae family have been known traditionally for their beneficial health‐promoting properties, attributed to their anti‐inflammatory, anaesthetic and anti‐microbial effects.ObjectiveThe purposes of this study was to characterise the essential oils from four Lamiaceae plants by applying different extraction techniques.MethodsAccelerated solvent (ASE), Soxhlet and supercritical fluid (SFE) extraction methods were compared for their efficiency in obtaining the essential oils from plants. The volatile compounds were identified by GC–MS and the main chemotype was quantified by GC with flame ionisation detection (FID). Phenolic compounds were identified and quantified by HPLC and electrospray ionisation (ESI) with MS/MS.ResultsThe essential oils Mentha piperita (ct. menthol/menthone), Rosmarinus officinalis L. (ct. eucalyptol/camphor) and Origanum vulgare (ct. carvacrol/thymol), whereas Thymus vulgaris L. was found to be a pure chemotype (ct. thymol). All three extracts also contained six phenolic compounds. The highest extraction yields were achieved by the Soxhlet and ASE techniques, with M. piperita and R. officinalis L. producing the highest concentrations of rosmarinic and carnosic acids. Finally, it was observed that M. piperita and O. vulgare produced the highest total phenolic content, whereas R. officinalis L. and T. vulgaris L. produced the highest anti‐oxidant activity.ConclusionThe ASE and Soxhlet extraction techniques presented the highest yields of volatile and phenolic compounds, showing their suitability to characterise the chemical profile of aromatic plants. Copyright © 2014 John Wiley & Sons, Ltd.
Burning Characteristics of Consolidated Gun Propellants
Authors: Li Yuxiang , Yang Weitao , Ying Sanjiu 
Published: September 2014

AbstractTo improve the combustion behaviors of conventional consolidated propellants, consolidated propellants were prepared using porous propellant grains as fast‐burning filler. The porous propellant grains were prepared by supercritical fluid foaming process and exhibited the high burning rate. The multi‐perforated structure of the consolidated propellants was designed and adopted to obtain high burning progressivity. To investigate the burning characteristics of the consolidated propellants, closed vessel and quenched combustion experiments were carried out. The results show that deconsolidation of the consolidated propellants does not occur, and that the consolidated propellants exhibit high burning rates and high burning progressivities. Besides, the results show that the consolidated propellants burn steadily even at low (−40 °C) and high temperatures (50 °C).
The pluripotency factor Nanog is directly upregulated by the Androgen Receptor in prostate cancer cells
Authors: Kregel Steven , Szmulewitz Russell Z. , Griend Donald J. Vander 
Published: August 2014

BACKGROUNDThe Androgen Receptor (AR) is a nuclear hormone receptor that functions as a critical oncogene in all stages of prostate cancer progression, including progression to castration‐resistance following androgen‐deprivation therapy. Thus, identifying and targeting critical AR‐regulated genes is one potential method to block castration‐resistant cancer proliferation. Of particular importance are transcription factors that regulate stem cell pluripotency; many of these genes are emerging as critical oncogenes in numerous tumor cell types. Of these, Nanog has been previously shown to increase the self‐renewal and stem‐like properties of prostate cancer cells. Thus, we hypothesized that Nanog is a candidate AR target gene that may impart castration‐resistance.METHODSWe modulated AR signaling in LNCaP prostate cancer cells and assayed for Nanog expression. Direct AR binding to the NANOG promoter was tested using AR Chromatin Immunoprecipation (ChIP) and analyses of publically available AR ChIP‐sequencing data‐sets. Nanog over‐expressing cells were analyzed for cell growth and cytotoxicity in response to the AR antagonist enzalutamide and the microtubule stabilizing agent docetaxel.RESULTSAR signaling upregulates Nanog mRNA and protein. AR binds directly to the NANOG promoter, and was not identified within 75 kb of the NANOGP8 pseudogene, suggesting the NANOG gene locus was preferentially activated. Nanog overexpression in LNCaP cells increases overall growth, but does not increase resistance to enzalutamide or docetaxel.CONCLUSIONSNanog is a novel oncogenic AR target gene in prostate cancer cells, and stable expression of Nanog increases proliferation and growth of prostate cancer cells, but not resistance to enzalutamide or docetaxel. Prostate 74:1530–1543, 2014. © 2014 Wiley Periodicals, Inc.