bluuebunny/crossref_metadata_2025_split huggingface dataset:
Bevacizumab for symptomatic radiation‐induced tumor enlargement in pediatric low grade gliomas
Authors: Foster Kimberly A. , Ares William J. , Pollack Ian F. , Jakacki Regina I. 
Published: November 2014

BackgroundRadiation therapy (RT)‐induced effects in children treated for low grade glioma (LGG) can result in worsening of neurologic symptoms and clinical and radiographic deterioration. Treatment for radiation‐induced tumor enlargement is based on symptom control and usually involves steroids.ProcedureWe conducted a retrospective review of children with LGG treated with RT who developed symptomatic radiation‐induced tumor enlargement and were managed with bevacizumab. Charts were abstracted for onset and duration of RT changes, toxicity and doses of dexamethasone and bevacizumab. Tumor volumes prior to RT, at maximal size following RT, after bevacizumab administration, and at follow‐up were evaluated.ResultsFive children were treated with bevacizumab for symptomatic radiation‐induced tumor enlargement following RT for LGG at a median of 4.2 months (range, 1–11 months) after completion of RT. The median increase in volume of tumor was 195.4% (range, 115.5–309%) compared to the pre‐RT volume. Bevacizumab 5–10 mg/kg was administered IV q 2–4 weeks as primary treatment (n = 1) or to assist in weaning patients off steroids (n = 4). All children on high dose steroids (n = 4) were weaned off or to physiologic doses of hydrocortisone. Two children developed avascular necrosis after prolonged steroid use and while on bevacizumab. Radiographically, all children showed significant improvement and are now a median of 31 months (range, 18–50 months) from the completion of radiation without requiring additional tumor‐related therapy.ConclusionsBevacizumab can play an important role in children with symptomatic radiation changes following LGG treatment, allowing patients to avoid or minimize the toxicity of long‐term steroid use. Pediatr Blood Cancer 2015;62:240–245. © 2014 Wiley Periodicals, Inc.
Comparison of pain outcomes between two anti‐GD2 antibodies in patients with neuroblastoma
Authors: Anghelescu Doralina L. , Goldberg Jacob L. , Faughnan Lane G. , Wu Jianrong , Mao Shenghua , Furman Wayne L. , Santana Victor M. , Navid Fariba 
Published: November 2014

BackgroundAddition of anti‐GD2 antibody ch14.18 to the treatment of neuroblastoma has improved outcomes. The most common side effect of ch14.18 is neuropathic pain, which may in part be complement‐mediated. Hu14.18K322A is a humanized anti‐GD2 antibody designed to diminish complement activation and induce less pain. We compare the pain outcomes in patients treated with ch14.18 and those treated with hu14.18K322A, and explore dose‐dependent relationships between pain scores, opioid requirements, and complement levels in patients treated with hu14.18K322A.ProcedureOpioid (morphine equivalent mg/kg) and anxiolytic requirements during course 1 (4 days) in patients treated with hu14.18K322A and ch14.18 were reviewed. Correlations between antibody dose and pain scores, opioid requirements, and complement levels were examined for patients receiving hu14.18K322A.ResultsPatients treated with hu14.18K322A (n = 19) had lower opioid requirements than those who received ch14.18 (n = 9). The differences in median opioid requirements (mg/kg) were statistically significant for the overall course (1.57 vs. 2.41, P = 0.019) as well as for Days 3 (0.34 vs. 0.65, P = 0.005), and 4 (0.32 vs. 0.64, P = 0.010). No difference in anxiolytic use was observed between the two groups. In the group treated with hu14.18K322A, we found a positive correlation between antibody dose administered and pain scores, but no correlation between antibody dose and opioid requirements or changes in complement levels.ConclusionsIn this retrospective analysis, hu14.18K322A induced less pain than ch14.18 based on opioid requirements. Pediatr Blood Cancer 2015;62:224–228. © 2014 Wiley Periodicals, Inc.
Fanconi anemia and solid malignancies in childhood: A national retrospective study
Authors: Malric Aurore , Defachelles Anne‐Sophie , Leblanc Thierry , Lescoeur Brigitte , Lacour Brigitte , Peuchmaur Michel , Maurage Claude‐Alain , Pierron Gaëlle , Guillemot Delphine , d'Enghien Catherine Dubois , Soulier Jean , Stoppa‐Lyonnet Dominique , Bourdeaut Franck 
Published: November 2014

BackgroundFanconi anemia (FA) predisposes to hematologic disorders and myeloid neoplasia in childhood and to solid cancers, mainly oral carcinomas, in early adulthood. Few cases of solid cancers have been reported in childhood.ProceduresWe conducted a national retrospective study of solid tumors occurring in patients registered with or determined to have FA during childhood in France. Phenotypic features, tumor type, cancer treatment, and outcome were analyzed. Whenever available, fresh‐frozen tumors were analyzed by microarray‐based comparative genomics hybridization.ResultsWe identified eight patients with FA with solid tumor from 1986 to 2012. For two patients, the diagnosis of FA was unknown at the time of cancer diagnosis. Moreover, we identified one fetus with a brain tumor. All patients showed failure to thrive and had dysmorphic features and abnormal skin pigmentation. Seven patients had BRCA2/FANCD1 mutations; five of these featured more than one malignancy and the median age at the time of cancer diagnosis was 11 months (range 0.4–3 years). Solid tumor types included five nephroblastomas, two rhabdomyosarcomas, two neuroblastomas, and three brain tumors. Two children died from the toxic effects of chemotherapy, two patients from the cancer, and one patient from secondary leukemia. Only one BRCA2 patient was alive more than 3 years after diagnosis, after tailored chemotherapy.ConclusionSolid tumors are rare in FA during childhood, except in patients with BRCA2/FANCD1 mutations. The proper genetic diagnosis is mandatory to tailor the treatment. Pediatr Blood Cancer 2015;62:463–470. © 2014 Wiley Periodicals, Inc.
Implementation of transcranial Doppler ultrasonography screening and primary stroke prevention in urban and rural sickle cell disease populations
Authors: Hussain Samiya , Nichols Fenwick , Bowman Latanya , Xu Hongyan , Neunert Cindy 
Published: November 2014

BackgroundTranscranial Doppler (TCD) ultrasonography identifies children with sickle cell disease (SCD) at increased risk of stroke. Initiation of chronic transfusions as primary stroke prevention in children with abnormal TCD significantly reduces stroke risk. Here, we report the results describing the implementation of TCD screening and primary stroke prevention in both urban and rural clinical practices.ProcedureRetrospective chart review identified children ages 2–16 years with Hgb SS or Sß0‐thalassemia and no history of stroke followed in either the local urban or rural SCD clinics at Georgia Regents University. We defined standard of care (SOC) as having one TCD performed annually between January 2010 and December 2012 starting at age 2 years.ResultsA total of 195 patients were included in the evaluation of SOC screening, overall 41% achieved SOC. There was no difference in SOC between the two clinics (35% urban and 47.4% rural). The majority of patients with abnormal TCDs are on chronic transfusions (83%), and none have experienced a stroke. Monitoring of effects of transfusion was difficult with 38% and 31% of rural patients lacking documentation of Hgb S% and ferritin levels, respectively, in the past year.ConclusionsWe report here data describing primary stroke prophylaxis in rural patients. SOC rates are similar between the two clinical settings. While implementation of primary stroke prevention in rural patients was difficult, rural TCD screening is feasible and can achieve SOC equal to that in an urban setting. This suggests that barriers exist in provided primary stroke prevention to all patients. Pediatr Blood Cancer 2015;62:219–223. © 2014 Wiley Periodicals, Inc.
Influence of bony resection margins and surgicopathological factors on outcomes in limb‐sparing surgery for extremity osteosarcoma
Authors: Loh Amos H.P. , Wu Huiyun , Bahrami Armita , Navid Fariba , McCarville M. Beth , Wang Chong , Wu Jianrong , Bishop Michael W. , Daw Najat C. , Neel Michael D. , Rao Bhaskar N. 
Published: November 2014

BackgroundLimb‐sparing surgery for osteosarcoma requires taking wide bony resection margins while maximizing preservation of native bone and joint. However, the optimal bony margin and factors associated with recurrence and survival outcomes in these patients are not well established.ProcedureWe conducted a retrospective review of outcomes in children and adolescents with newly diagnosed osteosarcoma from 1986 to 2012, where bony resection margins for limb‐sparing surgeries were decreased serially from 5 to 1.5 cm. The association between bony margins and other surgicopathological factors with survival and recurrence outcomes was determined.ResultsIn 181 limb‐sparing surgeries in 173 patients, planned and actual bony resection margins were not significantly associated with local recurrence‐free survival (LRFS), event‐free survival (EFS), and overall survival (OS)—at median 5.8 years follow‐up, decreasing planned bony resection margins from 5 to 1.5 cm did not significantly decrease survival outcomes. Multivariable analysis showed that the presence of distant metastases at diagnosis was associated with decreased LRFS, EFS, and OS (P = 0.002, 0.005, and <0.0001, respectively). Post‐chemotherapy tumor necrosis ≤90% was associated with decreased EFS and OS (P = 0.001 and 0.022, respectively). Earlier years of treatment and pathologic fractures were associated with decreased OS only (P = 0.018 and 0.008, respectively); previous cancer history and male gender were associated with decreased EFS only (P = 0.043 and 0.023, respectively).ConclusionWe did not observe significant increase in adverse survival outcomes with reduction of longitudinal bony resection margins to 1.5 cm. Established prognostic factors, particularly histologic response to chemotherapy and metastases at diagnosis, remain relevant in limb‐sparing patients. Pediatr Blood Cancer 2015;62:246–251. © 2014 Wiley Periodicals, Inc.
Incidence and clinical presentation of primary hemophagocytic lymphohistiocytosis in Sweden
Authors: Meeths Marie , Horne AnnaCarin , Sabel Magnus , Bryceson Yenan T. , Henter Jan‐Inge 
Published: November 2014

BackgroundPrimary hemophagocytic lymphohistiocytosis (HLH) represents a group of inherited hyperinflammatory immunodeficiencies, including familial HLH (FHL), Griscelli syndrome type 2 (GS2), and X‐linked lymphoproliferative syndrome (XLP). We previously reported an annual incidence of suspected primary HLH in Sweden 1971–1986 of 0.12 per 100,000 children. Here, we determined if the incidence had increased with concomitant awareness.ProcedureChildren <15 years old presenting with HLH 1987–2006 in Sweden were identified through the national mortality registry as well as by nation‐wide inquiries to all pediatric centers. HLH was diagnosed according to the HLH‐2004 diagnostic guidelines (in case of missing data of at least three of the eight diagnostic criteria, fulfillment of four was sufficient for inclusion). We defined primary HLH as patients presenting with HLH requiring transplantation or dying of disease.ResultsRemarkably, the minimal annual incidence rate of primary HLH remained 0.12 per 100,000 children, equating to 1.8 per 100,000 live births. Notably, an increased overall survival was observed in 1997–2006, relative to the period 1987–1996. During the subsequent 5‐year period, 2007–2011, the incidence of genetically and/or functionally verified primary HLH was 0.15 per 100,000 children per year, suggesting that new assays may aid the identification of patients with primary HLH.ConclusionThe annual incidence of primary HLH in Sweden is 0.12–0.15 per 100,000 children per year. Pediatr Blood Cancer 2015;62:346–352. © 2014 Wiley Periodicals, Inc.
A novel trial of topotecan, ifosfamide, and carboplatin (TIC) in children with recurrent solid tumors
Authors: Radhakrishnan Kavita , Lee Alice , Harrison Lauren A. , Morris Erin , Shen Violet , Gates Laura , Wells Robert J. , Wolff Johannes E. , Garvin James H. , Cairo Mitchell S. 
Published: November 2014

BackgroundIfosfamide, carboplatin, and etoposide (ICE) in children with refractory or recurrent solid tumors and lymphomas has resulted in good overall response rates (ORR). Etoposide, a topoisomerase‐II inhibitor, however, has been associated with a significant increase in secondary leukemia. The rationale for substituting topotecan, a topoisomerase‐I inhibitor, for etoposide in this regimen, a topoisomerase‐II inhibitor, includes its limited toxicity profile and decreased leukemogenicity. Furthermore, topotecan in combination with both alkylators and platinating agents are additive and/or synergistic against a variety of solid tumors.ProcedurePatients with relapsed/refractory solid tumors received ifosfamide (9 g/m2) and carboplatin (area under the curve: 3 mg/ml/min). Topotecan was also administered at 0.5 mg/m2/day × 3 days (N = 12) and in a small cohort (N = 3) at 0.75 mg/m2/day.ResultsFifteen patients were entered onto study. Two patients developed seizures/encephalitis secondary to ifosfamide. One patient had dose‐limiting thrombocytopenia secondary to TIC that resolved with supportive care. Patients received a median of three cycles (1–3) of TIC. Of the 14 evaluable patients for response, 4/14 had a complete response (CR), 2/14 had a partial response (PR), and 1/14 patients had stable disease (SD). The ORR (CR + PR) was 43%.ConclusionTIC chemotherapy is feasible and tolerable in children and adolescents with refractory/recurrent solid tumors and lymphomas and results in a 43% excellent ORR in this poor‐risk group of patients. A larger cohort of patients, especially in Wilms tumor and central nervous system (CNS) tumors, should be studied in the future to attempt to confirm these preliminary findings. Pediatr Blood Cancer 2015;62:274–278. © 2014 Wiley Periodicals, Inc.
Risk factors for abandonment of Wilms tumor therapy in Kenya
Authors: Libes Jaime , Oruko Oliver , Abdallah Fatmah , Githanga Jessie , Ndung'u James , Musimbi Joyce , Njuguna Festus , Patel Kirtika , White John , Axt Jason R. , O'Neill James A. , Shrubsole Martha , Li Ming , Lovvorn Harold N. 
Published: November 2014

BackgroundSurvival from Wilms tumor (WT) in sub‐Saharan Africa remains dismal as a result of on‐therapy mortality and treatment abandonment. Review of patients diagnosed from 2008 to 2011 in our Kenyan Wilms Tumor Registry showed a loss to follow up (LTFU) rate approaching 50%. The purpose of this study was to trace those LTFU, estimate the survival rate, and identify risk factors for treatment abandonment.ProcedureWe administered a comprehensive survey to parents of patients with WT at the two largest referral hospitals in Kenya to identify barriers to care. We also telephoned families who had abandoned care to determine vital status and identify risk factors for treatment abandonment.ResultsOf 136 registered patients, 77 were confirmed dead (56.7%), 38 remained alive (27.9%), and the vital status of 21 patients remains unknown (15.4%). After contacting 33 of the patients who either abandoned curative treatment (n = 34) or did not attend off‐therapy visits (n = 20), the best estimate of 2‐year overall survival of patients with WT in Kenya approaches 36%. Sixty‐three percent of parents misunderstood treatment plans and 55% encountered financial barriers. When asked how to increase comfort with the child's treatment, 27% of parents volunteered improving inefficient services and 26% volunteered reducing drug‐unavailability.ConclusionsTreatment abandonment remains a significant problem contributing to increased mortality from WT in developing countries. This multi‐center survey identified the barriers to treatment completion from the parental perspective to be lack of education about WT and treatment, financial constraints, need for quality improvement, and drug‐unavailability. Pediatr Blood Cancer 2015;62:252–256. © 2014 Wiley Periodicals, Inc.
Homopolymerization and copolymerization of N‐substituted maleimides with chiral anionic initiators
Authors: Huang Yi‐Ling , Cao Jing , Hu Wei , Liang Sai 
Published: November 2014

AbstractNovel optically
active anionic initiators bearing a chiral oxazole substituent on the fluorene ring, (S)‐1‐(9H‐fluoren‐2‐yl)‐4‐isopropyl‐4,5‐dihydrooxazole lithium (Li‐(S)‐1‐FIDH) and (S)‐2‐(9H‐fluoren‐2‐yl)‐4‐isopropyl‐4,5‐dihydrooxazole lithium (Li‐(S)‐2‐FIDH), were prepared. Anionic homopolymerizations of achiral N‐substituted maleimide (RMI) with the chiral initiators were investigated. The optically active polymers obtained were attributed to asymmetric induction of the chiral initiators. The very crowded chiral initiator Li‐(S)‐1‐FIDH was found to play a better asymmetric induction role in the polymers than Li‐(S)‐2‐FIDH. Anionic copolymerization of (R)‐(+)‐N‐1‐phenylethyl maleimide and optically inactive RMI with Li‐(S)‐1‐FIDH were also studied. © 2014 Society of Chemical Industry
A benzophenone‐naphthalimide derivative as versatile photoinitiator of polymerization under near UV and visible lights
Authors: Zhang Jing , Zivic Nicolas , Dumur Frédéric , Xiao Pu , Graff Bernadette , Gigmes Didier , Fouassier Jean Pierre , Lalevée Jacques 
Published: November 2014

ABSTRACTA benzophenone‐naphthalimide derivative (BPND) bearing tertiary amine groups has been developed as a high‐performance photoinitiator in combination with 2,4,6‐tris(trichloromethyl)‐1,3,5‐triazine or an iodonium salt for both the free radical polymerization (FRP) of acrylates and the cationic polymerization (CP) of epoxides upon exposure to near UV and visible LEDs (385–470 nm). BPND can even produce radicals without any added hydrogen donor. The photochemical mechanisms are studied by molecular orbital calculations, steady state photolysis, electron spin resonance spin trapping, fluorescence, cyclic voltammetry and laser flash photolysis techniques. These novel BPND based photoinitiating systems exhibit an efficiency higher than that of the well‐known camphorquinone‐based systems (FRP and CP) or comparable to that of bis(2,4,6‐trimethylbenzoyl)‐phenylphosphineoxide (FRP at λ ≤ 455 nm). © 2014 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2015, 53, 445–451
Comparison of monomethoxy‐, dimethoxy‐, and trimethoxysilane anchor groups for surface‐initiated RAFT polymerization from silica surfaces
Authors: Huebner Dennis , Koch Vanessa , Ebeling Bastian , Mechau Jannik , Steinhoff Judith Elisabeth , Vana Philipp 
Published: November 2014

ABSTRACTThe immobilization of reversible addition–fragmentation chain transfer (RAFT) agents on silica for surface‐initiated RAFT polymerizations (SI‐RAFT) via the Z‐group approach was studied systematically in dependence of the functionality of the RAFT‐agent anchor group. Monoalkoxy‐, dialkoxy‐, and trialkoxy silyl ether groups were incorporated into trithiocarbonate‐type RAFT agents and bound to planar silica surfaces as well as to silica nanoparticles. The immobilization efficiency and the structure of the bound RAFT‐agent film varied strongly in dependence of the used solvent (toluene vs. 1,2‐dimethoxyethane) and the anchor group functionality, as evidenced by atomic force microscopy, transmission electron microscopy, dynamic light scattering, and UV/Vis spectroscopy. Surface‐initiated RAFT polymerizations using functionalized silica nanoparticles revealed that grafted oligomers, which often occur in SI‐RAFT, are not formed within the crosslinked structures that originate from the immobilization, and that RAFT‐agent films that show less aggregation during the immobilization are more efficient during SI‐RAFT in terms of polymer grafting density. © 2014 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2015, 53, 103–113
Molecular damage inFabry disease: Characterization and prediction of alpha‐galactosidaseApathological mutations
Authors: Riera Casandra , Lois Sergio , Domínguez Carmen , Fernandez‐Cadenas Israel , Montaner Joan , Rodríguez‐Sureda Victor , de la Cruz Xavier 
Published: November 2014

ABSTRACTLoss‐of‐function mutations of the enzyme alpha‐galactosidase A (GLA) causes Fabry disease (FD), that is a rare and potentially fatal disease. Identification of these pathological mutations by sequencing is important because it allows an early treatment of the disease. However, before taking any treatment decision, if the mutation identified is unknown, we first need to establish if it is pathological or not. General bioinformatic tools (PolyPhen‐2, SIFT, Condel, etc.) can be used for this purpose, but their performance is still limited. Here we present a new tool, specifically derived for the assessment of GLA mutations. We first compared mutations of this enzyme known to cause FD with neutral sequence variants, using several structure and sequence properties. Then, we used these properties to develop a family of prediction methods adapted to different quality requirements. Trained and tested on a set of known Fabry mutations, our methods have a performance (Matthews correlation: 0.56–0.72) comparable or better than that of the more complex method, Polyphen‐2 (Matthews correlation: 0.61), and better than those of SIFT (Matthews correl.: 0.54) and Condel (Matthews correl.: 0.51). This result is validated in an independent set of 65 pathological mutations, for which our method displayed the best success rate (91.0%, 87.7%, and 73.8%, for our method, PolyPhen‐2 and SIFT, respectively). These data confirmed that our specific approach can effectively contribute to the identification of pathological mutations in GLA, and therefore enhance the use of sequence information in the identification of undiagnosed Fabry patients. Proteins 2015; 83:91–104. © 2014 Wiley Periodicals, Inc.
Approche motivationnelle auprès des auteurs de violences sexuelles : revue de la littérature et aspects cliniques
Authors: Bertsch I. , Cano J. 
Published: November 2014

En France, le suivi sanitaire des sujets condamnés pour des infractions sexuelles en milieu ouvert ambulatoire s’effectue généralement dans le cadre des soins pénalement ordonnés.Les prises en charge de ces auteurs se heurtent régulièrement à des difficultés diverses comme la question sensible de la formulation de la demande de suivi par les patients a partie de la laquelle émergent les dimensions de volonté de changement et de motivation au traitement. Considérant le modèle motivationnel de Miller et Rollnick [1], il est fréquent de constater que les auteurs de violences sexuelles se situent généralement aux stades de pré-contemplation et de contemplation de leurs difficultés et que les facteurs motivationnels au changement sont surtout extrinsèques.La motivation étant un phénomène complexe divers facteurs semblent impliqués dans l’amorce du changement. La littérature reconnaît la présence de facteurs sociodémographiques, liés à l’individu et liés à l’environnement.Parmi les solutions proposées à l’accès au changement la thérapie sous contrainte semble être privilégiée. On attend de celle-ci qu’elle soit le moteur d’une évolution du fonctionnement du patient qui se traduirait par exemple par une modification du discours les faits, la considération de la victime par l’auteur… [2].L’adaptation des techniques d’entretien motivationnel apporterait des bénéfices dans le suivi de ces patients, en termes d’adaptation aux rythmes individuels, d’engagement et de responsabilisation [3]. Cela proposerait un outil thérapeutique pertinent, et resituerait l’approche motivationnelle dans le mouvement d’approches dites « positives » développées depuis quelques années.Ce poster se propose d’aborder la question de la motivation au changement des auteurs de violences sexuelles à travers des rappels théoriques de la théorie du changement, des facteurs impliqués dans le changement des AVS et une application clinique de cette méthode du changement.