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Sample records for oncologia pediatrica aieop

  1. Heterogeneity of Disease Classified as Stage III in Wilms Tumor: A Report From the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP)

    SciTech Connect

    Spreafico, Filippo; Gandola, Lorenza; Terenziani, Monica; Collini, Paola; Bianchi, Maurizio; Provenzi, Massimo; Indolfi, Paolo; Pession, Andrea; Nantron, Marilina; Di Cataldo, Andrea; Marchiano, Alfonso; Piva, Luigi

    2012-01-01

    Purpose: We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems. Methods and Materials: Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT). Results: Ninety-nine WT patients met the criteria for classification as Stage III according to a revised version of the National Wilms Tumor Study-3 staging system (51 patients in CNR-92, 48 patients in TW-2003). Regional lymph nodes (LN) were not biopsied in 16 patients. After a median follow-up of 66 months, the 4-year disease-free survival (DFS) and overall survival (OS) rates were 85% {+-} 4% and 92% {+-} 3%, respectively, for the whole group. For 38 children with positive LN, the 4-year DFS rate was 73% {+-} 7%, as opposed to 98% {+-} 2% for the 45 children with Stage III WT according to the other criteria but with negative biopsied LN (p = 0.001). The subgroup with the worst prognosis consisted of children more than 2 years old with positive LN (DFS 67% {+-} 8%). A delay between surgery and RT > 30 days had an adverse impact on the abdominal tumor relapse rate. Conclusions: This study provides further evidence that Stage III tumors with LN metastases might be distinguished from WTs meeting the other criteria for classification as Stage III. The worse outcome of the former may warrant a prospective study on the effects of intensified therapy. A subclassification of Stage III tumors is discussed.

  2. Adolescents with Cancer in Italy: Improving Access to National Cooperative Pediatric Oncology Group (AIEOP) Centers.

    PubMed

    Ferrari, Andrea; Rondelli, Roberto; Pession, Andrea; Mascarin, Maurizio; Buzzoni, Carlotta; Mosso, Maria Luisa; Maule, Milena; Barisone, Elena; Bertolotti, Marina; Clerici, Carlo Alfredo; Jankovic, Momcilo; Fagioli, Franca; Biondi, Andrea

    2016-06-01

    This analysis compared the numbers of patients treated at Italian pediatric oncology group (Associazione Italiana Ematologia Oncologia Pediatrica [AIEOP]) centers with the numbers of cases predicted according to the population-based registry. It considered 32,431 patients registered in the AIEOP database (1989-2012). The ratio of observed (O) to expected (E) cases was 0.79 for children (0-14 years old) and 0.15 for adolescents (15-19 years old). The proportion of adolescents increased significantly over the years, however, from 0.05 in the earliest period to 0.10, 0.18, and then 0.28 in the latest period of observation, suggesting a greater efficacy of local/national programs dedicated to adolescents.

  3. Infant Ependymoma in a 10-Year AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica) Experience With Omitted or Deferred Radiotherapy

    SciTech Connect

    Massimino, Maura; Gandola, Lorenza; Barra, Salvina; Giangaspero, Felice; Casali, Cecilia; Potepan, Paolo; Di Rocco, Concezio; Nozza, Paolo; Collini, Paola; Viscardi, Elisabetta; Bertin, Daniele; Biassoni, Veronica; Cama, Armando; Milanaccio, Claudia; Modena, Piergiorgio; Balter, Rita; Tamburrini, Giampiero; Peretta, Paola; Mascarin, Maurizio; Scarzello, Giovanni

    2011-07-01

    Purpose: The protocols of the 1990s omitted or delayed irradiation, using upfront chemotherapy to spare the youngest children with ependymoma the sequelae of radiotherapy (RT). We treated 41 children under the age of 3 years with intracranial ependymoma between 1994 and 2003. Patients and Methods: After surgery, chemotherapy was given as follows: regimen I with four blocks of vincristine, high-dose methotrexate 5 g/m{sup 2}, and cyclophosphamide 1.5 g/m{sup 2} alternating with cisplatin 90 mg/m{sup 2} plus VP16 450 mg/m{sup 2} for 14 months; subsequently, regimen II was used: VEC (VCR, VP16 300 mg/m{sup 2}, and cyclophosphamide 3 g/m{sup 2}) for 6 months. Radiotherapy was planned for residual tumor after the completion of chemotherapy or for progression. Results: We treated 23 boys and 18 girls who were a median 22 months old; 14 were given regimen I, 27 were given regimen II; 22 underwent complete resection, 19 had residual tumor. Ependymoma was Grade 2 in 25 patients and Grade 3 in 16; tumors were infratentorial in 37 patients and supratentorial in 4. One child had intracranial metastases; 29 had progressed locally after a median 9 months. Event-free survival was 26% at 3 and 5 years and 23% at 8 years. One child died of sepsis, and another developed a glioblastoma 72 months after RT. Progression-free survival was 27% at 3, 5, and 8 years, and overall survival was 48%, 37%, and 28% at 3, 5, and 8 years, respectively. Of the 13 survivors, 6 never received RT; their intellectual outcome did not differ significantly in those children than in those without RT. Conclusions: Our results confirm poor rates of event-free survival and overall survival for up-front chemotherapy in infant ependymoma. No better neurocognitive outcome was demonstrated in the few survivors who never received RT.

  4. Detection of prognostic factors in children and adolescents with Burkitt and Diffuse Large B-Cell Lymphoma treated with the AIEOP LNH-97 protocol.

    PubMed

    Pillon, Marta; Mussolin, Lara; Carraro, Elisa; Conter, Valentino; Aricò, Maurizio; Vinti, Luciana; Garaventa, Alberto; Piglione, Matilde; Buffardi, Salvatore; Sala, Alessandra; Santoro, Nicola; Lo Nigro, Luca; Mura, Rossella; Tondo, Annalisa; Casale, Fiorina; Farruggia, Piero; Pierani, Paolo; Cesaro, Simone; d'Amore, Emanuele S G; Basso, Giuseppe

    2016-11-01

    Burkitt lymphoma (BL) and Diffuse Large B-Cell Lymphoma (DLBCL) account for most cases of non-Hodgkin lymphoma (NHL) in childhood. We report the clinical characteristics, outcome and prognostic factors in children with BL or DLBCL treated according to the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) LNH-97 protocol. Patients aged up to 18 years that were newly diagnosed with BL/DLBCL were included in the study. Therapy consisted of pre-phase followed by 2-6 high-dose chemotherapy courses tailored according to lactate dehydrogenase (LDH) value and disease stage. A total of 442 patients (379 BL, 63 DLBCL) were enrolled between 1997 and 2014, of whom 18 failed to achieve remission, 6 experienced treatment-related death, 2 developed second malignancy and 20 relapsed. At a median follow-up time of 5 years, overall survival was 93% (±1%) and event-free survival was 90% (±1%). LDH value above the median value had an independently negative prognostic value (P < 0·0001). However, in the subgroup of 128 patients in which minimal disseminated disease (MDD) was analysed, MDD-positivity became the only unfavourable prognostic factor for progression-free survival. Tailored chemotherapy could be extremely effective with limited toxicity. Identification of MDD as a hallmark of a higher risk of treatment failure may provide a target population for treatment intensification by anti-CD20.

  5. Results of the AIEOP AML 2002/01 multicenter prospective trial for the treatment of children with acute myeloid leukemia.

    PubMed

    Pession, Andrea; Masetti, Riccardo; Rizzari, Carmelo; Putti, Maria Caterina; Casale, Fiorina; Fagioli, Franca; Luciani, Matteo; Lo Nigro, Luca; Menna, Giuseppe; Micalizzi, Concetta; Santoro, Nicola; Testi, Anna Maria; Zecca, Marco; Biondi, Andrea; Pigazzi, Martina; Rutella, Sergio; Rondelli, Roberto; Basso, Giuseppe; Locatelli, Franco

    2013-07-11

    We evaluated the outcome of 482 children with acute myeloid leukemia (AML) enrolled in the Associazione Italiana di Ematologia e Oncologia Pediatrica AML 2002/01 trial. Treatment was stratified according to risk group; hematopoietic stem cell transplantation (HSCT) was used in high-risk (HR) children. Patients with core binding factor leukemia achieving complete remission (CR) after the first induction course were considered standard risk (SR; 99 patients), whereas the others (n = 383) were assigned to the HR group. Allogeneic (ALLO) or autologous (AUTO) HSCT was employed, respectively, in 141 and 102 HR patients after consolidation therapy. CR, early death, and induction failure rates were 87%, 3%, and 10%, respectively. Relapse occurred in 24% of patients achieving CR. The 8-year overall survival (OS), event-free survival (EFS), and disease-free survival (DFS) were 68%, 55%, and 63%, respectively. OS, EFS, and DFS for SR and HR patients were 83%, 63%, and 66% and 64%, 53%, and 62%. DFS was 63% and 73% for HR patients given AUTO-HSCT and ALLO-HSCT, respectively. In multivariate analysis, risk group, white blood cell >100 × 10(9)/L at diagnosis, and monosomal karyotype predicted poorer EFS. Risk-oriented treatment and broad use of HSCT result in a long-term EFS comparing favorably with previously published studies on childhood AML.

  6. Outcome of children with acute myeloid leukaemia (AML) experiencing primary induction failure in the AIEOP AML 2002/01 clinical trial.

    PubMed

    Quarello, Paola; Fagioli, Franca; Basso, Giuseppe; Putti, Maria C; Berger, Massimo; Luciani, Matteo; Rizzari, Carmelo; Menna, Giuseppe; Masetti, Riccardo; Locatelli, Franco

    2015-11-01

    Paediatric patients with acute myeloid leukaemia (AML) who fail induction due to primary resistance to chemotherapy account for a significant proportion of cases and have a particularly dismal prognosis. We report the clinical and biological data, and final outcome of 48 paediatric patients with primary-resistant AML enrolled in the Associazione Italiana di Ematologia e Oncologia Pediatrica AML 2002/01 clinical trial. These patients had a significantly higher white blood cell count at diagnosis compared to other AML patients. Cytogenetic and molecular features did not differ between patients with primary induction failure and patients allocated to the high-risk group. For the whole patient population, the probability of overall survival, event-free survival (EFS) and disease-free survival (DFS) was 21·8% ± 6·2, 20·4% ± 5·9, and 49·5% ± 11·3, respectively. Twenty-eight (58%) patients received haematopoietic stem cell transplantation (HSCT); 3 were autologous and 25 were allogeneic. Patients who underwent HSCT had improved EFS (31·2% vs. 5%, P < 0·0001). Only one of the 20 patients who did not receive HSCT is alive and disease free. The 19 patients in complete remission at time of HSCT showed significantly better DFS than the 9 with active disease (46% vs. 0%, P = 0·02). This study represents one of the largest series with long-term follow up of paediatric AML patients with primary refractory disease. Children who underwent transplantation had an encouraging long-term outcome. Disease recurrence remains the major cause of treatment failure; a better understanding of the disease biology is desirable to develop more effective treatment strategies.

  7. Prognostic significance of flow-cytometry evaluation of minimal residual disease in children with acute myeloid leukaemia treated according to the AIEOP-AML 2002/01 study protocol.

    PubMed

    Buldini, Barbara; Rizzati, Frida; Masetti, Riccardo; Fagioli, Franca; Menna, Giuseppe; Micalizzi, Concetta; Putti, Maria Caterina; Rizzari, Carmelo; Santoro, Nicola; Zecca, Marco; Disarò, Silvia; Rondelli, Roberto; Merli, Pietro; Pigazzi, Martina; Pession, Andrea; Locatelli, Franco; Basso, Giuseppe

    2017-04-01

    In children with acute myeloid leukaemia (AML), assessment of initial treatment response is an essential prognostic factor; methods more sensitive than morphology are still under evaluation. We report on the measurement of minimal residual disease (MRD), by multicolour flow-cytometry in one centralized laboratory, in 142 children with newly diagnosed AML enrolled in the Associazione Italiana di EmatoOncologia Pediatrica-AML 2002/01 trial. At the end of the first induction course, MRD was <0·1% in 69, 0·1-1% in 16 and >1% in 51 patients. The 8-year disease-free survival (DFS) of 125 children in morphological complete remission and with MRD <0·1%, 0·1-1% and ≥1% was 73·1 ± 5·6%, 37·8 ± 12·1% and 34·1 ± 8·8%, respectively (P < 0·01). MRD was also available after the second induction course in 92/142 patients. MRD was ≥0·1% at the end of the first induction course in 36 patients; 13 reached an MRD <0·1% after the second one and their DFS was 45·4 ± 16·7% vs. 22·8 ± 8·9% in patients with persisting MRD ≥0·1% (P = 0·037). Multivariate analysis demonstrated that MRD ≥0·1% after first induction course was, together with a monosomal karyotype, an independent adverse prognostic factor for DFS. Our results show that MRD detected by flow-cytometry after induction therapy predicts outcome in patients with childhood AML and can help stratifying post-remission treatment.

  8. AIEOP-BFM consensus guidelines 2016 for flow cytometric immunophenotyping of Pediatric acute lymphoblastic leukemia.

    PubMed

    Dworzak, Michael N; Buldini, Barbara; Gaipa, Giuseppe; Ratei, Richard; Hrusak, Ondrej; Luria, Drorit; Rosenthal, Eti; Bourquin, Jean-Pierre; Sartor, Mary; Schumich, Angela; Karawajew, Leonid; Mejstrikova, Ester; Maglia, Oscar; Mann, Georg; Ludwig, Wolf-Dieter; Biondi, Andrea; Schrappe, Martin; Basso, Giuseppe

    2017-02-10

    Immunophenotyping by flow cytometry (FCM) is a worldwide mainstay in leukemia diagnostics. For concordant multicentric application, however, a gap exists between available classification systems, technologic standardization, and clinical needs. The AIEOP-BFM consortium induced an extensive standardization and validation effort between its nine national reference laboratories collaborating in immunophenotyping of pediatric acute lymphoblastic leukemia (ALL). We elaborated common guidelines which take advantage of the possibilities of multi-color FCM: marker panel requirements, immunological blast gating, in-sample controls, tri-partite antigen expression rating (negative vs. weak or strong positive) with capturing of blast cell heterogeneities and subclone formation, refined ALL subclassification, and a dominant lineage assignment algorithm able to distinguish "simple" from bilineal/"complex" mixed phenotype acute leukemia (MPAL) cases, which is essential for choice of treatment. These guidelines are a first step toward necessary inter-laboratory standardization of pediatric leukemia immunophenotyping for a concordant multicentric application. © 2017 International Clinical Cytometry Society.

  9. Incidence of colonization and bloodstream infection with carbapenem-resistant Enterobacteriaceae in children receiving antineoplastic chemotherapy in Italy.

    PubMed

    Caselli, Desiree; Cesaro, Simone; Fagioli, Franca; Carraro, Francesca; Ziino, Ottavio; Zanazzo, Giulio; Meazza, Cristina; Colombini, Antonella; Castagnola, Elio

    2016-02-01

    Few data are available on the incidence of carbapenemase-producing Enterobacteriaceae (CPE) infection or colonization in children receiving anticancer chemotherapy. We performed a nationwide survey among centers participating in the pediatric hematology-oncology cooperative study group (Associazione Italiana Ematologia Oncologia Pediatrica, AIEOP). During a 2-year observation period, we observed a threefold increase in the colonization rate, and a fourfold increase of bloodstream infection episodes, caused by CPE, with a 90-day mortality of 14%. This first nationwide Italian pediatric survey shows that the circulation of CPE strains in the pediatric hematology-oncology environment is increasing. Given the mortality rate, which is higher than for other bacterial strains, specific monitoring should be applied and the results should have implications for health-care practice in pediatric hematology-oncology.

  10. Pharmacogenetics and induction/consolidation therapy toxicities in acute lymphoblastic leukemia patients treated with AIEOP-BFM ALL 2000 protocol.

    PubMed

    Franca, R; Rebora, P; Bertorello, N; Fagioli, F; Conter, V; Biondi, A; Colombini, A; Micalizzi, C; Zecca, M; Parasole, R; Petruzziello, F; Basso, G; Putti, M C; Locatelli, F; d'Adamo, P; Valsecchi, M G; Decorti, G; Rabusin, M

    2017-01-01

    Drug-related toxicities represent an important clinical concern in chemotherapy, genetic variants could help tailoring treatment to patient. A pharmacogenetic multicentric study was performed on 508 pediatric acute lymphoblastic leukemia patients treated with AIEOP-BFM 2000 protocol: 28 variants were genotyped by VeraCode and Taqman technologies, deletions of GST-M1 and GST-T1 by multiplex PCR. Toxicities were derived from a central database: 251 patients (49.4%) experienced at least one gastrointestinal (GI) or hepatic (HEP) or neurological (NEU) grade III/IV episode during the remission induction phase: GI occurred in 63 patients (12.4%); HEP in 204 (40.2%) and NEU in 44 (8.7%). Logistic regression model adjusted for sex, risk and treatment phase revealed that ITPA rs1127354 homozygous mutated patients showed an increased risk of severe GI and NEU. ABCC1 rs246240 and ADORA2A rs2236624 homozygous mutated genotypes were associated to NEU and HEP, respectively. These three variants could be putative predictive markers for chemotherapy-related toxicities in AIEOP-BFM protocols.

  11. Patterns of domestic migrations and access to childhood cancer care centres in Italy: a report from the hospital based registry of the Italian Association of Pediatric Hematology and Oncology (AIEOP).

    PubMed

    Dama, Elisa; Rondelli, Roberto; De Rosa, Marisa; Aricò, Maurizio; Carli, Modesto; Bellani, Franca Fossati; Magnani, Corrado; Merletti, Franco; Pastore, Guido; Pession, Andrea

    2008-10-01

    Tertiary care centres, grouped in the Italian Association of Paediatric Haematology and Oncology (AIEOP) are unevenly distributed across the country. In an attempt to describe their perceived efficacy, we matched the residence and the location of the treatment centre in 18,441 patients aged AIEOP network between 1989 and 2005. Overall, centres located in the central and southern regions were less appealing than those located in the North, although this trend decreased over the study period. Patients with solid tumours migrated more frequently than those with leukaemia or lymphoma. Information resulting from better knowledge of the non-random migrations for treatment of children with cancer will be useful to refine planning of the national paediatric haematology-oncology network with social and economic implications.

  12. Late MRD response determines relapse risk overall and in subsets of childhood T-cell ALL: results of the AIEOP-BFM-ALL 2000 study.

    PubMed

    Schrappe, Martin; Valsecchi, Maria Grazia; Bartram, Claus R; Schrauder, André; Panzer-Grümayer, Renate; Möricke, Anja; Parasole, Rosanna; Zimmermann, Martin; Dworzak, Michael; Buldini, Barbara; Reiter, Alfred; Basso, Giuseppe; Klingebiel, Thomas; Messina, Chiara; Ratei, Richard; Cazzaniga, Giovanni; Koehler, Rolf; Locatelli, Franco; Schäfer, Beat W; Aricò, Maurizio; Welte, Karl; van Dongen, Jacques J M; Gadner, Helmut; Biondi, Andrea; Conter, Valentino

    2011-08-25

    The prognostic value of MRD in large series of childhood T-ALL has not yet been established. Trial AIEOP-BFM-ALL 2000 introduced standardized quantitative assessment of MRD for stratification, based on immunoglobulin and TCR gene rearrangements as polymerase chain reaction targets: Patients were considered MRD standard risk (MRD-SR) if MRD was negative at day 33 (time point 1 [TP1]) and day 78 (TP2), analyzed by at least 2 sensitive markers; MRD intermediate risk (MRD-IR) if positive either at day 33 or 78 and < 10(-3) at day 78; and MRD high risk (MRD-HR) if ≥ 10(-3) at day 78. A total of 464 patients with T-ALL were stratified by MRD: 16% of them were MRD-SR, 63% MRD-IR, and 21% MRD-HR. Their 7-year event-free-survival (SE) was 91.1% (3.5%), 80.6% (2.3%), and 49.8% (5.1%) (P < .001), respectively. Negativity of MRD at TP1 was the most favorable prognostic factor. An excellent outcome was also obtained in 32% of patients turning MRD negative only at TP2, indicating that early (TP1) MRD levels were irrelevant if MRD at TP2 was negative (48% of all patients). MRD ≥ 10(-3) at TP2 constitutes the most important predictive factor for relapse in childhood T-ALL. The study is registered at http://www.clinicaltrials.gov; "Combination Chemotherapy Based on Risk of Relapse in Treating Young Patients With Acute Lymphoblastic Leukemia," protocol identification #NCT00430118 for BFM and #NCT00613457 for AIEOP.

  13. Radiation treatment of early vocal cord carcinoma. Eighteen years experience at "Instituto de Oncologia y Radioterapia de Mar del Plata".

    PubMed

    Castro Vita, H

    1990-01-01

    A detailed retrospective analysis of 260 patients with T 1 NO MO vocal cord carcinoma treated at "Instituto de Oncologia y Radioterapia de Mar del Plata" from 1967 to 1985 was performed. The majority of the patients were in the age range of 50 to 79 years, and 79% were males. The overall observed three year tumor free survival was 85.3%. When survival rate was adjusted for intercurrent disease and second primary tumor death, the 3 year tumor free survival was 92%. Sixty two percent of the patients (17/27) undergoing surgical salvage for recurrence, were controlled. Second primary tumors were seen in 18 patients (6.9%). It is emphasized the importance of close follow up to diagnose as early as possible both the vocal cord recurrence and the second primary tumors.

  14. Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP).

    PubMed

    Barone, Angelica; Lucarelli, Annunziata; Onofrillo, Daniela; Verzegnassi, Federico; Bonanomi, Sonia; Cesaro, Simone; Fioredda, Francesca; Iori, Anna Paola; Ladogana, Saverio; Locasciulli, Anna; Longoni, Daniela; Lanciotti, Marina; Macaluso, Alessandra; Mandaglio, Rosalba; Marra, Nicoletta; Martire, Baldo; Maruzzi, Matteo; Menna, Giuseppe; Notarangelo, Lucia Dora; Palazzi, Giovanni; Pillon, Marta; Ramenghi, Ugo; Russo, Giovanna; Svahn, Johanna; Timeus, Fabio; Tucci, Fabio; Cugno, Chiara; Zecca, Marco; Farruggia, Piero; Dufour, Carlo; Saracco, Paola

    2015-06-01

    Acquired aplastic anemia (AA) is a rare heterogeneous disease characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3/million inhabitants/year, in Europe, but higher in East Asia. Survival in severe aplastic anemia (SAA) has markedly improved in the past 2 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. In SAA hematopoietic stem cell transplant (HSCT) from a matched sibling donor (MSD) is the treatment of choice. If a MSD is not available, the options include immunosuppressive therapy (IST) or unrelated donor HSCT. The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary, evidence-based document issued by a group of pediatric hematologists was discussed, modified and approved during a series of "Consensus Conferences" according to procedures previously validated by the AIEOP Board. The guidelines highlight the importance of referring pediatric patients with AA to pediatric centers with long experience in diagnosis, differential diagnosis, management, supportive care and follow-up of AA.

  15. Outcome of children with high-risk acute myeloid leukemia given autologous or allogeneic hematopoietic cell transplantation in the aieop AML-2002/01 study.

    PubMed

    Locatelli, F; Masetti, R; Rondelli, R; Zecca, M; Fagioli, F; Rovelli, A; Messina, C; Lanino, E; Bertaina, A; Favre, C; Giorgiani, G; Ripaldi, M; Ziino, O; Palumbo, G; Pillon, M; Pession, A; Rutella, S; Prete, A

    2015-02-01

    We analyzed the outcome of 243 children with high-risk (HR) AML in first CR1 enrolled in the AIEOP-2002/01 protocol, who were given either allogeneic (ALLO; n=141) or autologous (AUTO; n=102) hematopoietic SCT (HSCT), depending on the availability of a HLA-compatible sibling. Infants, patients with AML-M7, or complex karyotype or those with FLT3-ITD, were eligible to be transplanted also from alternative donors. All patients received a myeloablative regimen combining busulfan, cyclophosphamide and melphalan; [corrected] AUTO-HSCT patients received BM cells in most cases, while in children given ALLO-HSCT stem cell source was BM in 96, peripheral blood in 19 and cord blood in 26. With a median follow-up of 57 months (range 12-130), the probability of disease-free survival (DFS) was 73% and 63% in patients given either ALLO- or AUTO-HSCT, respectively (P=NS). Although the cumulative incidence (CI) of relapse was lower in ALLO- than in AUTO-HSCT recipients (17% vs 28%, respectively; P=0.043), the CI of TRM was 7% in both groups. Patients transplanted with unrelated donor cord blood had a remarkable 92.3% 8-year DFS probability. Altogether, these data confirm that HSCT is a suitable option for preventing leukemia recurrence in HR children with CR1 AML.

  16. A hematology consensus agreement on antifungal strategies for neutropenic patients with hematological malignancies and stem cell transplant recipients. Gruppo Italiano Malattie Ematologiche dell'Adulto, Gruppo Italiano Trapianto di Midollo Osseo, Associazione Italiana Ematologia ed Oncologia Pediatrica, Invasive Fungal Infections Cooperative Group of the European Organization for Research and Treatment of Cancer and Sorveglianza Epidemiologica delle Infezioni Fungine nelle Emopatie Maligne.

    PubMed

    Girmenia, Corrado; Aversa, Franco; Busca, Alessandro; Candoni, Anna; Cesaro, Simone; Luppi, Mario; Pagano, Livio; Rossi, Giuseppe; Venditti, Adriano; Nosari, Anna Maria

    2013-09-01

    In the attempt to establish key therapy definitions and provide shared approaches to invasive fungal diseases in neutropenic patients, trials of empiric, preeemptive and targeted antifungal therapy (EAT, PAT and TAT) were reviewed, and a Consensus Development Conference Project was convened. The Expert-Panel concurred that all antifungal treatments, including EAT, should always follow an adequate diagnostic strategy and that the standard definition of PAT may be misleading: being PAT guided by the results of a diagnostic work-up, it should better be termed diagnostic-driven antifungal therapy (DDAT). The Expert-Panel agreed that radiological findings alone are insufficient for the choice of a TAT and that the identification of the etiologic pathogen is needed. The Consensus Agreement proceeded identifying which clinical and microbiological findings were sufficient to start a DDAT and which were not. Finally, an algorithm to rationalize the choice of antifungal drugs on the basis of clinical manifestations, antifungal prophylaxis, instrumental and laboratory findings was drawn up.

  17. Ewing Sarcoma of the Bone in Children under 6 Years of Age

    PubMed Central

    De Ioris, Maria Antonietta; Prete, Arcangelo; Cozza, Raffaele; Podda, Marta; Manzitti, Carla; Pession, Andrea; Schiavello, Elisabetta; Contoli, Benedetta; Balter, Rita; Fagioli, Franca; Bisogno, Gianni; Amoroso, Loredana

    2013-01-01

    Background Ewing Sarcoma Family Tumours (ESFT) are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. Methods The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) from 1990 to 2008 were reviewed. The Kaplan–Meier method was used for estimating overall and progression-free survival (OS, PFS) curves; multivariate analyses were performed using Cox proportional hazards regression model. Results This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23%) patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58–83%) and 72% (95% CI 57–83%) for patients with localized disease and 38% (95% CI 17–60%) and 21% (95% CI 5–45%) for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P<0.01), while patients treated in the last decade had better survival (P  = 0.002). In fact, the 5-year OS and PFS for patients diagnosed in the period 2000–2008 were 89% (95% CI 71–96%) and 86% (95% CI 66–94%), respectively. Conclusion The axial localization is the most common site of ESFT in pre-scholar children. Patients treated in the most recent period have an excellent outcome. PMID:23382839

  18. Feasibility and Outcome of Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplant High-Dose Cyclophosphamide for Children and Adolescents with Hematologic Malignancies: An AIEOP-GITMO Retrospective Multicenter Study.

    PubMed

    Berger, Massimo; Lanino, Edoardo; Cesaro, Simone; Zecca, Marco; Vassallo, Elena; Faraci, Maura; De Bortoli, Massimiliano; Barat, Veronica; Prete, Arcangelo; Fagioli, Franca

    2016-05-01

    Post-transplant high-dose cyclophosphamide (PTCy) is a novel approach to prevent graft-versus-host disease (GVHD) and rejection in patients given haploidentical hematopoietic stem cell transplantation (HSCT). Thirty-three patients with high-risk hematologic malignancies and lacking a match-related or -unrelated donor were treated with PTCy haploidentical HSCT in 5 Italian AIEOP centers. Nineteen patients had a nonmyeloablative preparative regimen (57%), and 14 patients received a full myeloablative conditioning regimen (43%). No patients received serotherapy; GVHD prophylaxis was based on PTCy (50 mg/kg on days +3 and +4) combined with mycophenolate plus tacrolimus or cyclosporine A. Neutrophil and platelet engraftment was achieved on days +17 (range, 14 to 37) and +27 (range, 16 to 71). One patient had autologous reconstitution for anti-HLA antibodies. Acute GVHD grades II to IV and III to IV and chronic GVHD developed in 22% (95% CI, 11 to 42), 3% (95% CI, 0 to 21), and 4% (95% CI, 0 to 27) of cases, respectively. The 1-year overall survival rate was 72% (95% CI, 56 to 88), progression-free survival rate was 61% (95% CI, 43 to 80), cumulative incidence of relapse was 24% (95% CI, 13 to 44), and transplant-related mortality was 9% (95% CI, 3 to 26). The univariate analysis for risk of relapse incidence showed how 3 significant variables, mother as donor (P = .02), donor gender as female (P = .04), and patient gender as female (P = .02), were significantly associated with a lower risk of relapse. Disease progression was the main cause of death. PTCy is a safe procedure also for children and adolescents who have already received several lines of chemotherapy. Among the different diseases, a trend for better 1-year rates of overall survival was obtained for nonacute leukemia patients.

  19. Recommendations for the use of long-term central venous catheter (CVC) in children with hemato-oncological disorders: management of CVC-related occlusion and CVC-related thrombosis. On behalf of the coagulation defects working group and the supportive therapy working group of the Italian Association of Pediatric Hematology and Oncology (AIEOP).

    PubMed

    Giordano, Paola; Saracco, Paola; Grassi, Massimo; Luciani, Matteo; Banov, Laura; Carraro, Francesca; Crocoli, Alessandro; Cesaro, Simone; Zanazzo, Giulio Andrea; Molinari, Angelo Claudio

    2015-11-01

    Central venous catheters (CVC), used for the management of children with hemato-oncological disorders, are burdened by a significant incidence of mechanical, infective, or thrombotic complications. These complications favor an increasing risk in prolongation of hospitalization, extra costs of care, and sometimes severe life-threatening events. No guidelines for the management of CVC-related occlusion and CVC-related thrombosis are available for children. To this aim, members of the coagulation defects working group and the supportive therapy working group of the Italian Association of Pediatric Hematology and Oncology (AIEOP) reviewed the pediatric and adult literature to propose the first recommendations for the management of CVC-related occlusion and CVC-related thrombosis in children with hemato-oncological disorders.

  20. Prognostic factors in advanced epithelial ovarian cancer. (Gruppo Interregionale Cooperativo di Oncologia Ginecologica (GICOG)).

    PubMed Central

    Marsoni, S.; Torri, V.; Valsecchi, M. G.; Belloni, C.; Bianchi, U.; Bolis, G.; Bonazzi, C.; Colombo, N.; Epis, A.; Favalli, G.

    1990-01-01

    The data on 914 patients enrolled in four randomised trials in advanced ovarian cancer, consecutively conducted by the same cooperative group between 1978 and 1986, were analysed with the aims of: (1) determining the impact of selected prognostic variables on survival; (2) finding, from the interaction of favourable prognostic factors and treatment, an approximate estimate of the magnitude of the survival advantage associated with the use of platinum-based combination chemotherapy. The overall 3-year survival in this series of patients is twice that reported historically (22%; 95% CL 18.7-25.4). The proportional hazard regression model was used to perform the analysis on survival. Residual tumour size, age, FIGO stage and cell type were all independent determinants of survival. Differences in survival from the various prognostic groups were impressive with 5-year survival rates ranging from 7 to 62%. However, these differences were not qualitative (i.e. the kinetics of survival were similar for the best and the worst groups) suggesting that current prognostic factors are of little use for selecting 'biologically' different sub-populations. Platinum-based regimens were associated to an overall prolonged median survival, but this benefit was not observable in the subgroup with most favourable prognosis (less than 2 cm residual tumour size). The implications of these observations for clinical research and ovarian cancer patients care are discussed. PMID:2119684

  1. Mutations of SETBP1 and JAK3 in juvenile myelomonocytic leukemia: a report from the Italian AIEOP study group.

    PubMed

    Bresolin, Silvia; De Filippi, Paola; Vendemini, Francesca; D'Alia, Mirko; Zecca, Marco; Meyer, Lueder H; Danesino, Cesare; Locatelli, Franco; Masetti, Riccardo; Basso, Giuseppe; Te Kronnie, Geertruy

    2016-05-17

    Juvenile myelomonocytic leukemia (JMML) is a rare aggressive disease of early childhood. Driver mutations in the Ras signaling pathways are a key feature of JMML patients. Mutations in SETBP1 and JAK3 were recently identified in a subset of JMML patients characterized by poor prognosis and progression of disease. In this study, we report the results of a screening for mutations in SETBP1 and JAK3 of a cohort of seventy Italian patients with JMML, identifying 11.4% of them harboring secondary mutations in these two genes and discovering two new mutations in the SKI domain of SETBP1.JMML xenotransplantation and colony assay provide an initial understanding of the secondary nature of these events occurring in early precursor cells and suggest a different propagating capacity of clones harboring particular mutations.

  2. A survey on hematology-oncology pediatric AIEOP centers: prophylaxis, empirical therapy and nursing prevention procedures of infectious complications.

    PubMed

    Livadiotti, Susanna; Milano, Giuseppe Maria; Serra, Annalisa; Folgori, Laura; Jenkner, Alessandro; Castagnola, Elio; Cesaro, Simone; Rossi, Mario R; Barone, Angelica; Zanazzo, Giulio; Nesi, Francesca; Licciardello, Maria; De Santis, Raffaella; Ziino, Ottavio; Cellini, Monica; Porta, Fulvio; Caselli, Desiree; Pontrelli, Giuseppe

    2012-01-01

    A nationwide questionnaire-based survey was designed to evaluate the management and prophylaxis of febrile neutropenia in pediatric patients admitted to hematology-oncology and hematopoietic stem cell transplant units. Of the 34 participating centers, 40 and 63%, respectively, continue to prescribe antibacterial and antimycotic prophylaxis in low-risk subjects and 78 and 94% in transplant patients. Approximately half of the centers prescribe a combination antibiotic regimen as first-line therapy in low-risk patients and up to 81% in high-risk patients. When initial empirical therapy fails after seven days, 63% of the centers add empirical antimycotic therapy in low-and 81% in high-risk patients. Overall management varies significantly across centers. Preventive nursing procedures are in accordance with international guidelines. This survey is the first to focus on prescribing practices in children with cancer and could help to implement practice guidelines.

  3. Characterization of children with FLT3-ITD acute myeloid leukemia: a report from the AIEOP AML-2002 study group.

    PubMed

    Manara, E; Basso, G; Zampini, M; Buldini, B; Tregnago, C; Rondelli, R; Masetti, R; Bisio, V; Frison, M; Polato, K; Cazzaniga, G; Menna, G; Fagioli, F; Merli, P; Biondi, A; Pession, A; Locatelli, F; Pigazzi, M

    2017-01-01

    Recurrent molecular markers have been routinely used in acute myeloid leukemia (AML) for risk assessment at diagnosis, whereas their post-induction monitoring still represents a debated issue. We evaluated the prognostic value and biological impact of minimal residual disease (MRD) and of the allelic ratio (AR) of FLT3-internal-tandem duplication (ITD) in childhood AML. We retrospectively screened 494 children with de novo AML for FLT3-ITD mutation, identifying 54 harboring the mutation; 51% of them presented high ITD-AR at diagnosis and had worse event-free survival (EFS, 19.2 versus 63.5% for low ITD-AR, <0.05). Forty-one percent of children with high levels of MRD after the 1st induction course, measured by a patient-specific real-time-PCR, had worse EFS (22.2 versus 59.4% in low-MRD patients, P<0.05). Next, we correlated these parameters with gene expression, showing that patients with high ITD-AR or persistent MRD had characteristic expression profiles with deregulated genes involved in methylation and acetylation. Moreover, patients with high CyclinA1 expression presented an unfavorable EFS (20.3 versus 51.2% in low CyclinA1 group, P<0.01). Our results suggest that ITD-AR levels and molecular MRD should be considered in planning clinical management of FLT3-ITD patients. Different transcriptional activation of epigenetic and oncogenic profiles may explain variability in outcome among these patients, for whom novel therapeutic approaches are desirable.

  4. Marriage and parenthood among childhood cancer survivors: a report from the Italian AIEOP Off-Therapy Registry

    PubMed Central

    Pivetta, Emanuele; Maule, Milena M.; Pisani, Paola; Zugna, Daniela; Haupt, Riccardo; Jankovic, Momcilo; Aricò, Maurizio; Casale, Fiorina; Clerico, Anna; Cordero di Montezemolo, Luca; Kiren, Valentina; Locatelli, Franco; Palumbo, Giovanna; Pession, Andrea; Pillon, Marta; Santoro, Nicola; Terenziani, Monica; Valsecchi, Maria Grazia; Dama, Elisa; Magnani, Corrado; Merletti, Franco; Pastore, Guido

    2011-01-01

    Background The aim of this study was to describe the patterns of marriage and parenthood in a cohort of childhood cancer survivors included in the Off-Therapy Registry maintained by the Italian Association of Pediatric Hematology and Oncology. Design and Methods We analyzed a cohort of 6,044 patients diagnosed with cancer between 1960 and 1998, while aged 0 to 14 years and who were 18 years old or older by December 2003. They were followed up through the regional vital statistics registers until death or the end of follow up (October 30, 2006), whichever occurred first, and their marital status and date of birth of their children were recorded. The cumulative probabilities of being married and having a first child were computed by gender and compared by tumor type within the cohort. Marriage and fertility rates (the latter defined as the number of live births per woman-year) were compared with those of the Italian population of the same age, gender, area of residence and calendar period by means of the observed to expected (O/E) ratios. Results During the follow-up period, 4,633 (77%) subjects had not married. The marriage O/E ratios were 0.56 (95% CI: 0.51–0.61) and 0.70 (95% CI: 0.65–0.76) among men and women, respectively. Overall, 263 men had 367 liveborn children, and 473 women had 697 liveborn children. The female fertility O/E ratio was 0.57 (95% CI: 0.53–0.62) overall, and 1.08 (95% CI: 0.99–1.17) when analyses were restricted to married/cohabiting women Conclusions Childhood cancer survivors are less likely to marry and to have children than the general population, confirming the life-long impact of their previous disease on their social behavior and choices. The inclusion of counseling in the strategies of management and long-term surveillance of childhood cancer patients could be beneficial to survivors as they approach adulthood. PMID:21228031

  5. Hodgkin's disease in children: seventeen years experience at the Instituto Português de Oncologia de Francisco Gentil.

    PubMed

    Patrício, M B; De Sousa, J V

    1981-10-01

    The experience of 52 children with Hodgkin's disease was reviewed. Compared with Hodgkin's disease in adults there was an increased incidence among boys. Mixed cellularity (MC) was the most common histologic type (60.5% in boys, and 64.4% in girls) as was also observed in adults (61.1% in men and 63.0% in women). The methods of therapy consisted of three main groups: extended-field radiotherapy (EFRT) + MOPP; involved-field radiotherapy (IVRT) + MOPP, 44.4% for IVRT + Monochemotherapy, and 80% for EFRT alone. The progressive improvement in results was associated with the change from IVRT to EFRT and the introduction of multiagent chemotherapy.

  6. Management and results of endometrial carcinoma treated at Instituto Português de Oncologia de Francisco Gentil.

    PubMed

    Tavares, M A; Patricio, M B; Vilhena, M; Da Silva, J N

    1977-02-01

    The experience of 260 patients with endometrial carcinoma was reviewed. The influence of factors such as age, stage of disease, grade and degree of myometrial penetration on the survival was presented, showing that survival decreases in elderly patients, in patients with advanced stage of disease, when the tumor is undifferentiated, and when the tumor deeply penetrates the myometrium. The methods of therapy, fall into three main groups: surgery, radiotherapy, and combined therapy, the latter yielding the best 5-year survival rate, in all stages. The incidence of vaginal recurrences was low, probably due to the fact that 68.8% of the patients were treated by a combined therapeutic modality.

  7. Hematopoietic stem cell transplantation for children with high-risk acute lymphoblastic leukemia in first complete remission: a report from the AIEOP registry.

    PubMed

    Fagioli, Franca; Quarello, Paola; Zecca, Marco; Lanino, Edoardo; Rognoni, Carla; Balduzzi, Adriana; Messina, Chiara; Favre, Claudio; Foà, Roberto; Ripaldi, Mimmo; Rutella, Sergio; Basso, Giuseppe; Prete, Arcangelo; Locatelli, Franco

    2013-08-01

    Children with high-risk acute lymphoblastic leukemia in first complete remission can benefit from allogeneic hematopoietic stem cell transplantation. We analyzed the outcome of 211 children with high-risk acute lymphoblastic leukemia in first complete remission who were given an allogeneic transplant between 1990 and 2008; the outcome of patients who, despite having an indication for transplantation and a suitable donor, did not receive the allograft for different reasons in the same time period was not analyzed. Sixty-nine patients (33%) were transplanted between 1990 and 1999, 58 (27%) between 2000 and 2005, and 84 (40%) between 2005 and 2008. A matched family donor was employed in 138 patients (65%) and an unrelated donor in 73 (35%). The 10-year probabilities of overall and disease-free survival were 63.4% and 61%, respectively. The 10-year cumulative incidences of transplantation-related mortality and relapse were 15% and 24%, respectively. After 1999, no differences in either disease-free survival or transplant-related mortality were observed in patients transplanted from unrelated or matched family donors. In multivariate analysis, grade IV acute graft-versus-host disease was an independent factor associated with worse disease-free survival. By contrast, grade I acute graft-versus-host disease and age at diagnosis between 1 and 9 years were favorable prognostic variables. Our study, not intended to evaluate whether transplantation is superior to chemotherapy for children with acute lymphoblastic leukemia in first complete remission and high-risk features, shows that the allograft cured more than 60% of these patients; in the most recent period, the outcome of recipients of grafts from matched family and unrelated donors was comparable.

  8. Hematopoietic stem cell transplantation for children with high-risk acute lymphoblastic leukemia in first complete remission: a report from the AIEOP registry

    PubMed Central

    Fagioli, Franca; Quarello, Paola; Zecca, Marco; Lanino, Edoardo; Rognoni, Carla; Balduzzi, Adriana; Messina, Chiara; Favre, Claudio; Foà, Roberto; Ripaldi, Mimmo; Rutella, Sergio; Basso, Giuseppe; Prete, Arcangelo; Locatelli, Franco

    2013-01-01

    Children with high-risk acute lymphoblastic leukemia in first complete remission can benefit from allogeneic hematopoietic stem cell transplantation. We analyzed the outcome of 211 children with high-risk acute lymphoblastic leukemia in first complete remission who were given an allogeneic transplant between 1990 and 2008; the outcome of patients who, despite having an indication for transplantation and a suitable donor, did not receive the allograft for different reasons in the same time period was not analyzed. Sixty-nine patients (33%) were transplanted between 1990 and 1999, 58 (27%) between 2000 and 2005, and 84 (40%) between 2005 and 2008. A matched family donor was employed in 138 patients (65%) and an unrelated donor in 73 (35%). The 10-year probabilities of overall and disease-free survival were 63.4% and 61%, respectively. The 10-year cumulative incidences of transplantation-related mortality and relapse were 15% and 24%, respectively. After 1999, no differences in either disease-free survival or transplant-related mortality were observed in patients transplanted from unrelated or matched family donors. In multivariate analysis, grade IV acute graft-versus-host disease was an independent factor associated with worse disease-free survival. By contrast, grade I acute graft-versus-host disease and age at diagnosis between 1 and 9 years were favorable prognostic variables. Our study, not intended to evaluate whether transplantation is superior to chemotherapy for children with acute lymphoblastic leukemia in first complete remission and high-risk features, shows that the allograft cured more than 60% of these patients; in the most recent period, the outcome of recipients of grafts from matched family and unrelated donors was comparable. PMID:23445874

  9. [Reversible cortical atrophy secondary to anti-NMDA receptor antibody encephalitis].

    PubMed

    Bravo-Oro, Antonio; Acosta-Yebra, Danae; Grimaldo-Zapata, Ilse P; Reyes-Vaca, Guillermo

    2015-05-16

    Introduccion. La encefalitis por anticuerpos antirreceptor de N-metil-D-aspartato (NMDA) inicialmente se describio como un sindrome paraneoplasico asociado a teratoma de ovario, pero cada vez con mas frecuencia se han ido publicando casos en mujeres jovenes y niños como un cuadro encefalopatico autoinmune secundario en el 40-50% de los casos a un proceso viral. Clinicamente, se caracteriza por un cuadro progresivo de manifestaciones psiquiatricas, crisis convulsivas, discinesias y disautonomias. Un hallazgo neurorradiologico poco comunicado es la atrofia cortical reversible, de la cual se desconoce su mecanismo. Caso clinico. Niña que a los 6 años comenzo con crisis convulsivas focales, con electroencefalograma epileptogeno y tomografia de craneo inicial normal. Se inicio tratamiento anticonvulsionante. A las tres semanas aparecieron nuevas crisis convulsivas, manifestaciones psiquiatricas y alteraciones en el ciclo de sueño-vigilia. Ante la sospecha de encefalitis por anticuerpos antirreceptor de NMDA, estos se determinaron en el suero y el liquido cefalorraquideo con resultado positivo. Resonancia magnetica durante el ingreso con atrofia cortical generalizada. Oncologia Pediatrica descarto asociacion a tumores. A los dos años del cuadro, con la paciente libre de crisis convulsivas, una valoracion neuropsicologica mostro la afectacion de funciones ejecutivas y una resonancia magnetica de control evidencio la recuperacion de la atrofia cortical. Conclusion. El mecanismo de la atrofia cortical reversible se desconoce, pero en pacientes con encefalitis por anticuerpos antirreceptor de NMDA podria ser directamente proporcional a la cantidad de anticuerpos circulantes y el tiempo de exposicion a estos en la corteza cerebral. Es muy importante el diagnostico temprano y el inicio de inmunomodulacion.

  10. [Is attention deficit hyperactivity disorder associated with other prevalent pathologies of early childhood?].

    PubMed

    Cardo, Esther; Amengual-Gual, Marta

    2015-02-25

    Objetivo. Revisar si el trastorno por deficit de atencion/hiperactividad (TDAH) se asocia con otras patologias medicas prevalentes de la edad pediatrica. Desarrollo. Se han seleccionado varias patologias pediatricas con el objetivo de revisar su asociacion con TDAH: en neumologia pediatrica, asma y otros procesos alergicos; en neurologia pediatrica, cefalea y convulsion febril; en gastroenterologia pediatrica, diarrea, estreñimiento, dolor abdominal, reflujo gastroesofagico e infeccion por Helicobacter pylori; en nefrologia pediatrica, enuresis; en cardiologia pediatrica, soplos y cardiopatias congenitas; en endocrinologia pediatrica, alteraciones tiroideas y obesidad, y en oftalmologia pediatrica, ametropia y estrabismo. Conclusion. Se han encontrado varios estudios que relacionan el TDAH con procesos alergicos, sobrepeso/obesidad, resistencia periferica a la hormona tiroidea, enuresis, convulsion febril, cefalea, cardiopatias congenitas, alteraciones oftalmologicas y caries, con algunas controversias y detalles por definir. Se puede concluir que son necesarios mas estudios interdisciplinarios para esclarecer las asociaciones y los mecanismos subyacentes implicados, con la finalidad de conocer mejor la compleja entidad TDAH y plantearse intervenciones preventivas, diagnosticas y terapeuticas en cuanto a sus comorbilidades se refiere.

  11. American Indial Educational Opportunities Program at Hampton University

    NASA Technical Reports Server (NTRS)

    Molin, Paulette F.

    1999-01-01

    The American Indian Educational Opportunities Program (AIEOP) at Hampton University was formed to provide scholarship and other support to eligible students from state and federally recognized tribal groups on campus. During the reporting period, AIEOP worked to enhance American Indian participation at Hampton through a variety of means, including recruitment and retention of students, outreach activities, curatorial efforts, course instruction, and sponsorship of educational programs. Dr. Paulette F. Molin, a member of the Minnesota Chippewa Tribe, served as the program's director.

  12. [Hand-foot-mouth disease: its course in the city of Milan (1980-86). Epidemiologic study gathered from the records of the community and scholastic ambulatory services of the Istituto di Clinica Dermatologic I and Dermatologia Pediatrica of the University].

    PubMed

    Sala, F; Mansi, M; Greppi, F; Ferrari, L; Perotta, E; Andreani, B

    1989-03-01

    Hand-foot-mouth disease: the Authors describe the epidemiological observations in a period of seven years made in a big urban area of Milan. They point out the periodic course of hand-foot and mouth disease, which is present in particular during May and November and particularly in children under six years. The Authors point out the difference between males and females and they try to correlate their dates with those gathered from the study of the literature.

  13. [Reversible porencephalic cyst related to shunt dysfunction].

    PubMed

    Santín-Amo, José M; Rico-Cotelo, María; Serramito-García, Ramón; Gelabert-González, Miguel; Allut, Alfredo G

    2014-03-16

    Introduccion. El tratamiento quirurgico de la hidrocefalia es uno de los procedimientos quirurgicos mas habituales en la neurocirugia pediatrica, y las derivaciones ventriculoperitoneales constituyen una herramienta fundamental en el tratamiento de la hidrocefalia tanto infantil como del adulto. Las complicaciones de las valvulas son relativamente frecuentes, sobre todo en la poblacion pediatrica, y, entre estas, las mas habituales incluyen: las obstrucciones, las desconexiones, el hiperdrenaje y las infecciones. Caso clinico. Niña de 7,5 años, portadora de una valvula ventriculoperitoneal, que presentaba cefalea intermitente. Un estudio con tomografia computarizada demostro una lesion quistica temporal derecha. Tras la revision valvular, la tomografia computarizada evidencio la reduccion del quiste. Conclusiones. La formacion de una cavidad porencefalica es una complicacion poco frecuente. Se relaciona con problemas en el cateter distal en pacientes con ventriculos dilatados y de las que existen escasas referencias en la bibliografia.

  14. [Current situation of the demand for health care in neuropaediatrics. Characteristics of consultations and comparison with other paediatric specialties].

    PubMed

    Blanco-Lago, R; Garcia-Ron, A; Granizo-Martinez, J J; Ruibal, J L

    2014-11-01

    Introduccion. En los ultimos años parecemos asistir a una creciente demanda asistencial en neuropediatria. Los estudios epidemiologicos son necesarios para dar a conocer dicha demanda y asi favorecer una adecuada gestion de los recursos sanitarios. Objetivo. Conocer el peso proporcional de las consultas de neuropediatria en el global de las consultas pediatricas en nuestro hospital, la tasa anual de consulta en neurologia infantil por cada 1.000 habitantes menores de 14 años y las caracteristicas de dicha consulta (datos demograficos, motivos de consulta y otras). Pacientes y metodos. Estudio retrospectivo, descriptivo, sobre la actividad asistencial de consultas pediatricas y neuropediatricas en un hospital publico de nivel II en el sur de Madrid, durante el periodo 2008-2012. Resultados. Desde la apertura de nuestro centro, las consultas de pediatria han experimentado un marcado crecimiento, siendo las de neuropediatria las mas demandadas, ya que en el año 2012 atendieron a un total de 2.129 pacientes (718 primeras consultas), con un indice de sucesiva/primera consulta de 1,96. En neuropediatria, se atendieron el 23,49% de todas las consultas pediatricas realizadas en el hospital. La tasa media de primeras consultas en el periodo de estudio fue de 72,86/1.000 niños. Los principales motivos de consulta fueron los problemas de aprendizaje/trastornos de conducta (24,1%), seguidos de cefalea (21,9%), episodios paroxisticos (14,8%) y retraso del desarrollo psicomotor (9%). Conclusiones. El incremento en la demanda asistencial de la neuropediatria ha resultado claramente superior al de las otras especialidades pediatricas que llevan en funcionamiento el mismo periodo. En los cinco años de estudio, la tasa de primeras visitas se ha triplicado. Esta sobrecarga asistencial podria condicionar la atencion a los pacientes con patologia neurologica grave. Serian necesarios estudios similares en diferentes regiones para conocer la realidad de la neuropediatria española.

  15. [Neuronal communication and synaptic metabolism in childhood epilepsy].

    PubMed

    García-Cazorla, Àngels; Cortès-Saladelafont, Elisenda; Duarte, Sofia

    2015-03-01

    Introduccion. Los conocimientos que la neurociencia basica y el neurometabolismo estan aportando en epilepsia pediatrica, y en concreto en mecanismos de comunicacion sinaptica, crecen rapidamente. Existe, no obstante, una desconexion entre estos avances y una vision que los integre de manera global y en la practica clinica y terapeutica. Objetivos. Ofrecer una vision integradora de los diferentes mecanismos moleculares y metabolicos que se conocen y postulan en epilepsia pediatrica, y sugerir conceptos como el de 'metabolismo sinaptico' y 'fenotipos sinapticos' como herramientas utiles para desarrollar este enfoque. Desarrollo. Se revisan los estudios mas destacados que intentan explicar las caracteristicas esenciales de la comunicacion sinaptica en el cerebro en desarrollo, a traves de diferentes moleculas, basicamente proteinas sinapticas, canales ionicos (cotransportadores de cloro, sodio y potasio), la compartimentalizacion pre y postsinaptica, y los principales actores metabolicos (neurotransmisores, metabolismo energetico, factores de crecimiento y lipidos). A partir de esta combinacion de mecanismos biologicos se sugieren ejemplos de 'fenotipos sinapticos' en dos casos concretos de epilepsia genetica (SCN1A) y metabolica (epilepsia con respuesta a la piridoxina). Conclusiones. Una perspectiva holistica, entendiendo la diversidad de elementos relacionados y que suceden en determinados momentos del neurodesarrollo, puede ayudar a delinear fenotipos, vias de metabolismo sinaptico y conectividad cerebral, que faciliten no solo la comprension de la fisiopatologia, sino nuevas aproximaciones terapeuticas en epilepsia pediatrica.

  16. American Indian Education Opportunities Program. Supplement 9

    NASA Technical Reports Server (NTRS)

    Molin, Paulette F.

    1997-01-01

    Activities of the American Indian Educational Opportunities Program (AIEOP) at Hampton University for this reporting period included the establishment of a student chapter of the American Indian Science & Engineering Society (AISES), a move to new office space, hosting events on campus for visiting students from the American Indian Education Program of Oxon Hill, Maryland and Onondaga Community College in Syracuse, New York, collaboration with the Multicultural Leadership Team at NASA Langley Research Center for a Native American elder to serve as a speaker, participation in Native American conferences and other events, and continuing efforts to recruit and retain American Indian students.

  17. Foreign children with cancer in Italy

    PubMed Central

    2011-01-01

    Background There has been a noticeable annual increase in the number of children coming to Italy for medical treatment, just like it has happened in the rest of the European Union. In Italy, the assistance to children suffering from cancer is assured by the current network of 54 centres members of the Italian Association of Paediatric Haematology and Oncology (AIEOP), which has kept records of all demographic and clinical data in the database of Mod.1.01 Registry since 1989. Methods We used the information stored in the already mentioned database to assess the impact of immigration of foreign children with cancer on centres' activity, with the scope of drawing a map of the assistance to these cases. Results Out of 14,738 cases recorded by all centres in the period from 1999 to 2008, 92.2% were born and resident in Italy, 4.1% (608) were born abroad and living abroad and 3.7% (538) were born abroad and living in Italy. Foreign children cases have increased over the years from 2.5% in 1999 to. 8.1% in 2008. Most immigrant children came from Europe (65.7%), whereas patients who came from America, Asia and Oceania amounted to 13.2%, 10.1%, 0.2%, respectively. The immigrant survival rate was lower compared to that of children who were born in Italy. This is especially true for acute lymphoblastic leukaemia patients entered an AIEOP protocol, who showed a 10-years survival rate of 71.0% vs. 80.7% (p < 0.001) for immigrants and patients born in Italy, respectively. Conclusions Children and adolescents are an increasingly important part of the immigration phenomenon, which occurs in many parts of the world. In Italy the vast majority of children affected by malignancies are treated in AIEOP centres. Since immigrant children are predominantly treated in northern Italy, these centres have developed a special expertise in treating immigrant patients, which is certainly very useful for the entire AIEOP network. PMID:21923939

  18. [Biphasic pulmonary blastoma with germ cell differentiation: a challenge in diagnosis and treatment].

    PubMed

    Teixeira, Alexandra; Vieira, Claúdia; Sousa, Nuno; Begonha, Rosa; Afonso, Mariana; Amaro, Teresina; Maurício, Joaquina

    2011-12-01

    Serviço de Oncologia Médica. Instituto Português de Oncologia Francisco Gentil. Porto. Portugal. A 27-year-old man, smoker, presented with three months history of fever. A left pulmonary mass inseparable from the heart was identified and serum alpha-fetoprotein was 4160 ng/ml. The morphologic aspects and immunohistochemistry of the biopsy specimen, in conjunction with the clinical findings were compatible with a diagnosis of pulmonary blastoma with germ cell differentiation. The tumour was considered unresectable. The patient was submitted to two cycles of primary chemotherapy with bleomycin, etoposide and cisplatin. Despite a reduction in serum alpha-fetoprotein, the tumor did not regress. Second line chemotherapy (with paclitaxel, ifosfamide and cisplatin) was instituted, but progressive disease was identified after 2 cycles. Six months after the diagnosis cerebral metastases were found and the patient died. This case illustrates a rare situation of difficult diagnosis and treatment.

  19. Preventing transmission of infectious agents in the pediatric in-patients hematology-oncology setting: what is the role for non-pharmacological prophylaxis?

    PubMed Central

    Caselli, Désirée; Cesaro, Simone; Livadiotti, Susanna; Ziino, Ottavio; Paolicchi, Olivia; Zanazzo, Giulio; Milano, Giuseppe M.; Licciardello, Maria; Barone, Angelica; Cellini, Monica; Raffaella, De Santis; Giacchino, Mareva; Rossi, Mario Renato; Aricò, Maurizio; Castagnola, Elio

    2011-01-01

    The most intensive chemotherapy regimens were used in the past for leukemia patients who were the main focus of trials on infections; today there are increasing numbers of children with solid cancer and considerable risk of infection who do receive intensive standard-dose chemotherapy. Despite a continuous will to protect the immune-compromised child from infections, evidence-based indications for intervention by non-pharmacological tools is still lacking in the pediatric hematology-oncology literature. Guidelines on standard precautions as well as precautions to avoid transmission of specific infectious agents are available. As a result of a consensus discussion, the Italian Association for Pediatric Hematology-Oncology (AIEOP) Cooperative Group centers agree that for children treated with chemotherapy both of these approaches should be implemented and vigorously enforced, while additional policies, including strict environmental isolation, should be restricted to patients with selected clinical conditions or complications. We present here a study by the working group on infectious diseases of AIEOP. PMID:21647282

  20. Management of diabetes at summer camps.

    PubMed

    Ciambra, Roberta; Locatelli, Chiara; Suprani, Tosca; Pocecco, Mauro

    2005-01-01

    We report our experience in the organization of diabetic children summer-camps since 1973. Guidelines for organization have been recently reported by the SIEDP (Società Italiana di Endocrinologia e Diabetologia Pediatrica). Our attention is focused on diabetes management at camp, organization and planning, medical staff composition and staff training, treatment of diabetes-related emergencies, written camp management plan, diabetes education and psychological issues at camp, prevention of possible risks, assessment of effectiveness of education in summer camps and research at camp.

  1. [The contribution of the Italian association of cancer registries (AIRTUM)].

    PubMed

    Crocetti, Emanuele; Buzzoni, Carlotta

    2016-01-01

    The study of cluster requires the ability to identify, with accuracy and completeness, the health events of interest and their geographical location and time of occurrence. For rare and complex diseases, such as childhood cancers, it is possible to observe a significant health migration from the place of residence, which makes the detection even more complex. The best tool to identify these rare diseases is represented by cancer registries (CRs). In fact, CRs collect, through many sources, information related to tumours that arise in the population resident in their areas of activity. The number of the sources of information has increased thanks to the computerization of health services. The availability of multiple sources of information increases the completeness of data collection overcoming the limits of a single source, and makes it possible to describe the diagnostic-therapeutic course and the outcome of the cases. Among all data sources, for childhood cancers the model 1.01, which summarize the clinical information of the cases treated in one of the Italian Association of paediatric haematology and oncology (AIEOP) centres, is relevant. Moreover, CRs produce reliable and comparable data due to the use of international rules and classifications for the definition of the topography and morphology of cancer, for the date of diagnosis, and for quality checks. In Italy, the Italian association of cancer registries (AIRTUM) coordinates the activities of 45 population CRs, both general and specialized (by age or tumour type). AIRTUM involves a population of over 6.7 million citizens under the age of 20 years, approximately 60% of the total resident population. AIRTUM plays a role of coordination, support, and harmonization for Italian CRs through training, accreditation, and a shared database, it promotes and participates in national and international collaboration involving scientific societies (AIEOP, Italian Association of medical oncology - AIOM, Italian

  2. [Optic neuritis in childhood. A pediatric series, literature review and treatment approach].

    PubMed

    Lopez-Martin, D; Martinez-Anton, J

    2016-08-01

    Introduccion. En la edad pediatrica, la forma mas frecuente de neuritis optica se presenta generalmente despues de un cuadro infeccioso, con edema de papila, que suele ser bilateral y tiene buen pronostico. La conversion a esclerosis multiple es infrecuente. Objetivo. Presentar las caracteristicas clinicas y de laboratorio de una serie pediatrica de neuritis optica. Pacientes y metodos. Se analiza una serie de 17 casos de neuritis optica en niños y jovenes de 4 a 14 años, referidos entre los años 2000 y 2015. Resultados. La edad mediana de la serie fue de 11 años. Predominaron los pacientes de sexo femenino y el antecedente infeccioso fue poco frecuente; en cinco pacientes, la afectacion fue bilateral, y cuatro casos se presentaron como neuritis optica retrobulbar. La resonancia magnetica mostro hiperintensidad en T2 en los nervios opticos afectados en cinco pacientes. El estudio del liquido cefalorraquideo y bandas oligoclonales fue normal en todos los casos. Los pacientes, tratados con metilprednisolona intravenosa, tuvieron buena recuperacion. Solo en tres casos se comprobo una evolucion posterior a esclerosis multiple. Conclusiones. En esta serie, los casos que evolucionaron a esclerosis multiple no mostraron diferencias clinicas, aunque si presentaron mayor cantidad de lesiones hiperintensas en la resonancia magnetica. Este hecho, descrito en trabajos previos, apoya nuestro esquema diagnostico y terapeutico en un intento por acercarnos al manejo optimo de esta patologia.

  3. [Adolescents with cancer: the "Youth Project" at the Istituto Nazionale Tumori in Milan].

    PubMed

    Ferrari, Andrea; Veneroni, Laura; Clerici, Carlo Alfredo; Spreafico, Filippo; Terenziani, Monica; Massimino, Maura; Luksch, Roberto; Casanova, Michela; Meazza, Cristina; Polastri, Daniela; Gandola, Lorenza

    2013-01-01

    Adolescents with cancer are a particular group of patients who are less likely to gain access to optimal cancer services at comprehensive cancer Centers: many studies suggest adolescents fare less well than children with the same disease. The paper describes the key issues of the "Youth Project" of the Pediatric Oncology Unit IRCCS Fondazione Istituto Nazionale Tumori in Milan, dedicated to adolescents (over 15 years old) and young adults (up to 25 years old) with solid tumors. This project is a possible clinical and organizational model to address the unique needs of patients in this age group and for bridge the gap in access to care and in recruitment in clinical trials, in clinical and psycho-social management and in curves of healing. The paper also describes the activity of the Adolescent Commission established by the Italian Pediatric Hematology Oncology (AIEOP).

  4. [1st National Meeting of Multidisciplinary Work in Oncogeriatrics: expert consensus document].

    PubMed

    Antonio, Maite; Saldaña, Juana; Formiga, Francesc; Lozano, Alicia; González-Barboteo, Jesús; Fernández, Paz; Arias, Fernando; Arribas, Lorena; Barbero, Elisabeth; Bescós, María del Mar; Boya, Maria Jesús; Bueso, Pilar; Casas, Ana; Dotor, Emma; Fort, Eduard; García-Alfonso, Pilar; Herruzo, Ismael; Llonch, Mireia; Morlans, Germà; Murillo, Maria Teresa; Ossola, Gustavo; Peiró, Inma; Saiz, Fabiola; Sanz, Javier; Serra, José Antonio; Trelis, Jordi; Yuste, Antonio

    2012-01-01

    On 2nd of June 2011 the Institut Català d' Oncologia l'Hospitalet--Hospital Duran i Reynals hosted the first Meeting of Multidisciplinary Work in Oncogeriatrics. The reason for the meeting, which follows on from an initiative of the Medical Societies of Radiotherapy, Oncology, Geriatrics and Gerontology and Palliative Care and Medical Oncology, was to initiate a joint line of work among the different specialties that generally take part in the handling of the elderly patient suffering from oncologic pathologies. This document summarises the different subjects covered during the Meeting.

  5. [SEIP-SERPE-SEOP Consensus document on the treatment of uncomplicated acute osteomyelitis and septic arthritis].

    PubMed

    Saavedra-Lozano, J; Calvo, C; Huguet Carol, R; Rodrigo, C; Núñez, E; Obando, I; Rojo, P; Merino, R; Pérez, C; Downey, F J; Colino, E; García, J J; Cilleruelo, M J; Torner, F; García, L

    2015-04-01

    This is a Consensus Document of the Spanish Society of Paediatric Infectious Diseases (Sociedad Española de Infectología Pediatrica), Spanish Society of Paediatric Rheumatology (Sociedad Española de Reumatología Pediátrica) and the Spanish Society of Paediatric Orthopaedics (Sociedad Española de Ortopedia Pediátrica), on the treatment of uncomplicated acute osteomyelitis and septic arthritis. A review is presented on the medical and surgical treatment of acute osteoarticular infection, defined as a process with less than 14 days of symptomatology, uncomplicated and community-acquired. The different possible options are evaluated based on the best available scientific knowledge, and a number of evidence-based recommendations for clinical practice are provided.

  6. Case report: Inflammatory myofibroblastic tumor of pancreatic origin in a patient with down syndrome: The role of diagnostic ultrasound.

    PubMed

    Colangelo, M; Di Renzo, D; Persico, A; Chiesa, P Lelli

    2011-03-01

    Sommario INTRODUZIONE: Il tumore miofibroblastico infiammatorio (TIM) è un tumore solido benigno, di incerta eziologia. MATERIALI E METODI: Riportiamo un case-report di un bambino (4 anni, sindrome di Down), con massa pancreatica identificata all’ultrasonografia (US) e confermata da TC. RISULTATI: Il monitoraggio del TIM è stato effettuato con US seriate. Il follow-up a 4 anni dalla diagnosi è senza recidive. DISCUSSIONE: Il paziente, data l’impossibilità dell’exeresi chirurgica radicale, è stato trattato con successo con terapia medica (FANS). Abbiamo deciso di monitorizzare la lesione con ultrasonografie seriate, riducendo il numero di esami TC ai quali il paziente avrebbe dovuto sottoporsi, evitando così l’esposizione a radiazioni ionizzanti. La letteratura, infatti, riporta ampiamente come l’esecuzione di ripetuti esami TC si accompagni ad una importante radioesposizione con conseguente rischio cancerogeno, dato assolutamente non trascurabile in età pediatrica.

  7. [Hirayama disease in paediatrics: a clinical case report and review of the literature].

    PubMed

    Martínez-Cayuelas, Elena; Martínez-Salcedo, Eduardo; Alarcón-Martínez, Helena; Puche-Mira, Alberto; Domingo-Jiménez, Rosario; de Mingo-Casado, Pedro; Serrano-García, Cristina

    2015-04-01

    Introduccion. La enfermedad de Hirayama es una rara atrofia muscular juvenil que afecta a varones jovenes de origen asiatico, con atrofia muscular habitualmente de una de las extremidades superiores de progresion lenta con estabilizacion posterior. Se diagnostica por estudios electromiograficos/electroneurograficos con velocidad de conduccion (EMG/ENG-VC), y por resonancia magnetica (RM) medular en posicion neutra y en flexion cervical. El tratamiento se basa en el collarin cervical y cirugia (casos graves). Son muy pocos los estudios realizados en edad pediatrica. Caso clinico. Niña de 7 años, con atrofia de la musculatura de la mano y el antebrazo izquierdos, de dos años de evolucion. En EMG/ENG-VC presenta signos de denervacion cronica muy grave en los miotomos correspondientes a C7, C8 y D1 izquierdos, con conservacion de amplitudes de potenciales sensitivos evocados, congruentes con mielopatia cervical. La RM medular cervical en posicion neutra muestra un resultado normal en ese nivel. Posteriormente, por la sospecha dirigida de enfermedad de Hirayama, se realiza una nueva RM medular cervical en posicion neutra y en flexion, que muestra asimetria en el tamaño y morfologia de los cordones anteriores medulares en C6/C7, hiperseñal en el asta anterior homolateral e ingurgitacion del plexo venoso epidural posterior. Con el diagnostico de enfermedad de Hirayama se inicia tratamiento con collarin cervical para evitar la progresion del daño. Conclusiones. Se presenta un caso de enfermedad de Hirayama peculiar por las caracteristicas epidemiologicas, con la finalidad de difundir esta entidad en nuestro medio, cuyo diagnostico precoz permite un tratamiento eficaz, y se revisan los estudios realizados en edad pediatrica.

  8. [Characterisation of the paediatric population of Costa Rica with tuberous sclerosis and a description of the behaviour of the associated epilepsy].

    PubMed

    Ulate-Campos, Adriana; Benavides-Lara, Adriana; Hernández, Laura

    2013-12-01

    Introduccion. La esclerosis tuberosa (ET) es una patologia autosomica dominante debida a un trastorno en la diferenciacion y proliferacion celular que produce hamartomas en diferentes organos. Afecta de forma variable el sistema nervioso central, los riñones, la piel y otros organos. No existen estudios de su prevalencia ni de su comportamiento en la poblacion pediatrica de Costa Rica. Objetivos. Caracterizar la poblacion pediatrica costarricense con ET y describir el comportamiento de su epilepsia. Pacientes y metodos. Estudio de prevalencias basado en la revision de expedientes clinicos de todos los pacientes menores de 18 años con diagnostico de ET seguidos en el Hospital Nacional de Niños durante el periodo 2000-2010. Resultados. Durante el periodo de estudio se incluyeron 37 pacientes para una prevalencia de 3,09 por 100.000 nacidos vivos (intervalo de confianza al 95% = 1,88-4,31). No se presentaron diferencias significativas por sexo. La mediana de edad al diagnostico fue de 14 meses. Los criterios mayores mas frecuentes fueron manchas hipomelanoticas (97,3%), angiofibromas faciales (56%) y tuberomas corticales (54,1%). Treinta y cinco pacientes presentaron convulsiones (95%). Los tratamientos que lograron reduccion de al menos un 50% de las crisis convulsivas fueron la vigabatrina (16,2%) y la cirugia de epilepsia (16,2%). Conclusiones. En todo paciente con epilepsia debe realizarse una valoracion minuciosa de la piel, ya que las lesiones en la piel son un hallazgo muy frecuente en la ET. La epilepsia en la ET es farmacorresistente en un elevado numero de pacientes y la vigabatrina debe valorarse como tratamiento farmacologico de primera linea.

  9. A Trust-Based Pact in Research Biobanks. From Theory to Practice.

    PubMed

    Sanchini, Virginia; Bonizzi, Giuseppina; Disalvatore, Davide; Monturano, Massimo; Pece, Salvatore; Viale, Giuseppe; Di Fiore, Pier Paolo; Boniolo, Giovanni

    2016-05-01

    Traditional Informed Consent is becoming increasingly inadequate, especially in the context of research biobanks. How much information is needed by patients for their consent to be truly informed? How does the quality of the information they receive match up to the quality of the information they ought to receive? How can information be conveyed fairly about future, non-predictable lines of research? To circumvent these difficulties, some scholars have proposed that current consent guidelines should be reassessed, with trust being used as a guiding principle instead of information. Here, we analyse one of these proposals, based on a Participation Pact, which is already being offered to patients at the Istituto Europeo di Oncologia, a comprehensive cancer hospital in Milan, Italy.

  10. Minimal residual disease (MRD) analysis in the non-MRD-based ALL IC-BFM 2002 protocol for childhood ALL: is it possible to avoid MRD testing?

    PubMed

    Fronkova, E; Mejstrikova, E; Avigad, S; Chik, K W; Castillo, L; Manor, S; Reznickova, L; Valova, T; Zdrahalova, K; Hrusak, O; Jabali, Y; Schrappe, M; Conter, V; Izraeli, S; Li, C K; Stark, B; Stary, J; Trka, J

    2008-05-01

    The ALL IC-BFM 2002 protocol was created as an alternative to the MRD-based AIEOP-BFM ALL 2000 study, to integrate early response criteria into risk-group stratification in countries not performing routine PCR-based MRD testing. ALL IC stratification comprises the response to prednisone, bone marrow (BM) morphology at days 15 and 33, age, WBC and BCR/ABL or MLL/AF4 presence. Here, we compared this stratification to the MRD-based criteria using MRD evaluation in 163 patients from four ALL IC member countries at days 8, 15 and 33 and week 12. MRD negativity at day 33 was associated with an age of 1-5 years, WBC<20,000 microl(-1), non-T immunophenotype, good prednisone response and non-M3 morphology at day 15. There were no significant associations with gender or hyperdiploidy in the study group, or with TEL/AML1 fusion within BCP-ALL. Patients with M1/2 BM at day 8 tended to be MRD negative at week 12. Patients stratified into the standard-risk group had a better response than intermediate-risk group patients. However, 34% of them were MRD positive at day 33 and/or week 12. Our findings revealed that morphology-based ALL IC risk-group stratification allows the identification of most MRD high-risk patients, but fails to discriminate the MRD low-risk group assigned to therapy reduction.

  11. Guidelines on Vaccinations in Paediatric Haematology and Oncology Patients

    PubMed Central

    Cesaro, Simone; Giacchino, Mareva; Fioredda, Francesca; Barone, Angelica; Battisti, Laura; Bezzio, Stefania; Frenos, Stefano; De Santis, Raffaella; Livadiotti, Susanna; Marinello, Serena; Zanazzo, Andrea Giulio; Caselli, Désirée

    2014-01-01

    Objective. Vaccinations are the most important tool to prevent infectious diseases. Chemotherapy-induced immune depression may impact the efficacy of vaccinations in children. Patients and Methods. A panel of experts of the supportive care working group of the Italian Association Paediatric Haematology Oncology (AIEOP) addressed this issue by guidelines on vaccinations in paediatric cancer patients. The literature published between 1980 and 2013 was reviewed. Results and Conclusion. During intensive chemotherapy, vaccination turned out to be effective for hepatitis A and B, whilst vaccinations with toxoid, protein subunits, or bacterial antigens should be postponed to the less intensive phases, to achieve an adequate immune response. Apart from varicella, the administration of live-attenuated-virus vaccines is not recommended during this phase. Family members should remain on recommended vaccination schedules, including toxoid, inactivated vaccine (also poliomyelitis), and live-attenuated vaccines (varicella, measles, mumps, and rubella). By the time of completion of chemotherapy, insufficient serum antibody levels for vaccine-preventable diseases have been reported, while immunological memory appears to be preserved. Once immunological recovery is completed, usually after 6 months, response to booster or vaccination is generally good and allows patients to be protected and also to contribute to herd immunity. PMID:24868544

  12. [Use of fish oil lipid emulsions in hospitalized patients under 18 years old with abnormal results in liver tests associated with total parental nutrition].

    PubMed

    Giraldo Villa, Adriana; Henao Roldan, Catherine; García Loboguerrero, Fanny; Martínez Volkmar, María Isabel; Contreras Ramírez, Mónica María; Ruiz Navas, Patricia

    2014-04-01

    Introducción: La Nutricion Parental Total (NPT) prolongada esta asociada con complicaciones potencialmente mortales en la poblacion pediatrica como lo es la colestasis. Dentro del soporte nutricional, la fuente de acidos grasos, el contenido de fitoesteroles y la dosis de lipidos se han relacionado con el desarrollo de esta complicacion. Objetivo: Describir el comportamiento de las pruebas hepaticas en pacientes pediatricos con NPT en quienes se uso lipidos a base de acidos grasos omega 3 (omegavenR). Métodos: Estudio retrospectivo, en menores de 18 anos con tratamiento intravenoso por minimo 8 dias con acidos grasos omega 3. Los pacientes fueron inicialmente clasificados en dos grupos: colestasis y alteracion de pruebas hepaticas. Se evaluo el comportamiento de la transaminasa glutamico oxalacetica (TGO), transaminasa glutamico piruvica (TGP), bilirrubina total (BT), bilirrubina directa, gama glutamil transferasa (GGT) y fosfatasa alcalina (FA) antes y despues del tratamiento con omegaven R. Resultados: 33 pacientes cumplieron los criterios de inclusion. Al finalizar la administracion de omegavenR, 82,4% de los pacientes que presentaron inicialmente colestasis mostraron resolucion o mejoria. En el grupo de pacientes con alteracion de pruebas hepaticas 18,8% progresaron a colestasis. Conclusiones: Nuestro estudio sugiere que el uso de omegaven R en pacientes pediatricos con NPT y BD ≥ 2 mg/dL, parece revertir o mejorar la colestasis, mientras que en pacientes con alteracion de pruebas hepaticas aun no se tiene claro su efecto.

  13. [Neurofibromatosis type 2 in childhood: a clinical characterization].

    PubMed

    Hinojosa-Mateo, C M; Reche-Sainz, J A; Hernandez-Nunez, A; Ramos-Lopez, M; Arpa-Fernandez, A; Natera-de Benito, D

    2017-02-01

    Introduccion. La neurofibromatosis de tipo 2 (NF2) es un trastorno neuroectodermico con patron de herencia autosomico dominante que condiciona una predisposicion para desarrollar tumores de varios tipos en el sistema nervioso central y periferico. Se asocia tambien con alteraciones oculares y cutaneas. Caso clinico. Varon de 12 años con diagnostico de NF2 de acuerdo con los criterios de Baser et al e inicio en la infancia. Se realiza una revision bibliografica sobre la evolucion de los criterios diagnosticos en los niños. Conclusiones. El modo de presentacion de la NF2 en la infancia difiere de la presentacion en los adultos. Las manifestaciones iniciales de NF2 en los niños son las alteraciones oculares y cutaneas, no las auditivas. La clinica de inicio mas frecuente en la edad pediatrica es la triada de cataratas subcapsulares posteriores, lesiones intracutaneas en forma de placa o tumores nodulares subcutaneos, y sintomas neurologicos secundarios a la afectacion de pares craneales distintos al VIII par, tronco encefalico o medula espinal. Debido a que los criterios diagnosticos de NF2 son menos sensibles en los pacientes pediatricos, los niños con cataratas congenitas o de aparicion precoz y manifestaciones cutaneas tipicas de NF2 deben ser seguidos estrechamente.

  14. [Anti-NMDA receptor encephalitis: two paediatric cases].

    PubMed

    González-Toro, M Cristina; Jadraque-Rodríguez, Rocío; Sempere-Pérez, Ángela; Martínez-Pastor, Pedro; Jover-Cerdá, Jenaro; Gómez-Gosálvez, Francisco

    2013-12-01

    Introduccion. La encefalitis asociada a anticuerpos antirreceptores de N-metil-D-aspartato (NMDA) es una patologia neurologica autoinmune documentada en la poblacion pediatrica de manera creciente en los ultimos años. Se presentan dos casos de nuestra experiencia con clinica similar. Casos clinicos. Caso 1: niña de 5 años que inicia un cuadro de convulsiones y alteracion de conciencia, asociando trastornos del movimiento y regresion de habilidades previamente adquiridas que evoluciona a autismo. Caso 2: niña de 13 años que presenta hemiparesia izquierda, movimientos anomalos, trastorno de conducta y disautonomia. En ambos casos se obtienen anticuerpos antirreceptores de NMDA positivos en el liquido cefalorraquideo y se diagnostican de encefalitis antirreceptor de NMDA. En el primer caso se inicia el tratamiento con perfusion intravenosa de corticoides e inmunoglobulinas y es necesario asociar rituximab. En el segundo, corticoides e inmunoglobulinas. La evolucion fue favorable en ambas pacientes, con una leve alteracion del lenguaje como secuela en el primer caso y una recaida en el segundo caso, con resolucion completa. Conclusion. La encefalitis antirreceptor de NMDA es un trastorno tratable y es importante el diagnostico y tratamiento precoz, ya que mejora el pronostico y disminuye las recaidas.

  15. [Acute optic neuropathy: differential diagnoses].

    PubMed

    Buompadre, María Celeste

    2013-09-06

    Introduccion. La alteracion funcional del nervio optico se caracteriza por un deficit en la agudeza visual, en la vision cromatica y en el campo visual, defecto pupilar aferente y, en algunos casos, edema del nervio o atrofia y palidez. Objetivo. Describir el espectro de neuropatias opticas agudas, su clinica, diagnostico y tratamiento, con mayor interes en aquellas de presentacion en la edad pediatrica. Desarrollo. La neuritis optica puede ser monofasica, recurrente o el componente de un cuadro desmielinizante polisintomatico. El objetivo del tratamiento es reducir el numero y la gravedad de los ataques y prevenir discapacidad. La infecciosa es secundaria a diferentes microorganismos (bacterias, virus, hongos y protozoos). El tratamiento depende de la etiologia. La isquemica anterior no arteritica o idiopatica es la forma mas frecuente y es secundaria a enfermedad de pequeños vasos (ciliares posteriores). La neuropatia optica hereditaria o de Leber representa una causa importante de afectacion visual cronica y se caracteriza por la afectacion selectiva de las celulas ganglionares de la retina. Hasta el momento, la terapia solo es de apoyo. En el papiledema asociado a hipertension endocraneal, la agudeza visual generalmente se conserva pero existe aumento de la mancha ciega. El tratamiento se basa en disminuir la hipertension y el factor etiologico si existe. Conclusiones. Las neuropatias opticas agudas constituyen un amplio grupo de entidades, de etiologia diversa y con un pronostico visual variable. La presencia de signos del examen neurologico, fondo de ojo y neuroimagenes pueden orientar hacia el diagnostico y tratamiento oportuno.

  16. [Reversible neuropsychological deterioration associated to zonisamide in a paediatric patient with tuberous sclerosis].

    PubMed

    Fournier-Del Castillo, M Concepción; Melero-Llorente, Javier; Blanco-Beregana, Miriam; Robles-Bermejo, Fernando; Budke, Marcelo; Garcia-Fernandez, Marta; Garcia-Penas, Juan José; Perez-Jimenez, M Ángeles

    2015-01-16

    Objetivo. Documentar el deterioro cognitivo reversible asociado a altas dosis de zonisamida, utilizando indices de cambio fiable para controlar los efectos de practica derivados de evaluaciones neuropsicologicas repetidas. Caso clinico. Niño de 11 años con complejo esclerosis tuberosa y epilepsia refractaria del lobulo frontal izquierdo, evaluado en el contexto de un programa de cirugia de la epilepsia pediatrica. La zona epileptogena se relaciono con un tuber epileptogeno localizado en el giro frontal inferior del hemisferio izquierdo. Los efectos de altas dosis de zonisamida mimetizaron una afectacion de la corteza elocuente en la zona epileptogena y un impacto de las crisis no controladas en el funcionamiento cognitivo asociado al hemisferio dominante para el lenguaje. La retirada del farmaco mejoro significativamente, mas alla de los efectos de practica, el cociente intelectual total, el indice intelectual de comprension verbal y habilidades cognitivas especificas sustentadas en el lenguaje. Conclusiones. La diferenciacion entre los efectos cognitivos de los farmacos y la existencia de un deficit funcional por afectacion de la corteza elocuente en el area epileptogena puede ser crucial para la toma de decisiones en cirugia de la epilepsia.

  17. [Consensus document on the aetiology, diagnosis and treatment of acute otitis media].

    PubMed

    Del Castillo Martín, F; Baquero Artigao, F; de la Calle Cabrera, T; López Robles, M V; Ruiz Canela, J; Alfayate Miguelez, S; Moraga Llop, F; Cilleruelo Ortega, M J; Calvo Rey, C

    2012-11-01

    This is the consensus document on acute otitis media (AOM) of the Sociedad Española de Infectología Pediatrica (SEIP), Sociedad Española de Pediatría Extrahospitalaria y Atención Primaria (SEPEAP), Sociedad Española de Urgencias Pediátricas (SEUP) and Asociación Española de Pediatría de Atención Primaria (AEPAP). It discusses the aetiology of the disease and its potential changes after the introduction of the pneumococcal 7-valent, 10-valent and 13-valent vaccines. A proposal is made based on diagnostic classification of otitis media as either confirmed or likely. AOM is considered confirmed if 3 criteria are fulfilled: acute onset, signs of occupation of the middle ear (or otorrhea) and inflammatory signs or symptoms, such as otalgia or severe tympanic hyperaemia. Oral amoxicillin is the antibiotic treatment of choice (80mg/kg/day divided every 8hours). Amoxicillin-clavulanate (80mg/kg/day) is indicated in the following cases: when the child is under 6 months, in infants with severe clinical manifestations (fever>39°C or severe pain), there is family history of AOM sequels, and after amoxiciline treatment failure.

  18. [Cluster headache in pediatric population: four case reports and review of the literature].

    PubMed

    Alfaro-Iznaola, C; Natera-de Benito, D; Rodriguez-Diaz, R; Taboas-Pereira, M A; Cuadrado-Martin, M; Ballesteros-Garcia, M M

    2016-07-16

    Introduccion. La cefalea en racimos es una cefalea primaria de origen trigeminoautonomico cuyo inicio en la infancia es infrecuente. Se presentan cuatro casos en los que el inicio de la sintomatologia se produjo entre los 2 y los 13 años. Casos clinicos. Se incluyen tres varones y una niña con inicio a los 2, 7, 13 y 12 años, respectivamente. Los cuatro pacientes cumplen los criterios propuestos por la tercera edicion de la Clasificacion Internacional de las Cefaleas ICHD-III (beta). Conclusiones. A pesar de ser poco frecuente durante la edad pediatrica, la cefalea en racimos debe formar parte del diagnostico diferencial de un niño que consulta por cefalea. Subrayamos la importancia de conocer sus criterios diagnosticos para evitar el retraso diagnostico que se ha descrito con frecuencia. En nuestros pacientes, el tratamiento con verapamilo resulto mas eficaz que el tratamiento con flunaricina. Los tratamientos con mejor respuesta en fase aguda fueron la oxigenoterapia y los triptanes.

  19. Small sizes and indolent evolutionary dynamics challenge the potential role of P2RY8-CRLF2-harboring clones as main relapse-driving force in childhood ALL.

    PubMed

    Morak, Maria; Attarbaschi, Andishe; Fischer, Susanna; Nassimbeni, Christine; Grausenburger, Reinhard; Bastelberger, Stephan; Krentz, Stefanie; Cario, Gunnar; Kasper, David; Schmitt, Klaus; Russell, Lisa J; Pötschger, Ulrike; Stanulla, Martin; Eckert, Conny; Mann, Georg; Haas, Oskar A; Panzer-Grümayer, Renate

    2012-12-20

    The P2RY8-CRLF2 fusion defines a particular relapse-prone subset of childhood acute lymphoblastic leukemia (ALL) in Italian Association of Pediatric Hematology and Oncology Berlin-Frankfurt-Münster (AIEOP-BFM) 2000 protocols. To investigate whether and to what extent different clone sizes influence disease and relapse development, we quantified the genomic P2RY8-CRLF2 fusion product and correlated it with the corresponding CRLF2 expression levels in patients enrolled in the BFM-ALL 2000 protocol in Austria. Of 268 cases without recurrent chromosomal translocations and high hyperdiploidy, representing approximately 50% of all cases, 67 (25%) were P2RY8-CRLF2 positive. The respective clone sizes were ≥ 20% in 27% and < 20% in 73% of them. The cumulative incidence of relapse of the entire fusion-positive group was clone size independent and significantly higher than that of the fusion-negative group (35% ± 8% vs 13% ± 3%, P = .008) and primarily confined to the non-high-risk group. Of 22 P2RY8-CRLF2-positive diagnosis/relapse pairs, only 4/8 had the fusion-positive dominant clone conserved at relapse, whereas none of the original 14 fusion-positive small clones reappeared as the dominant relapse clone. We conclude that the majority of P2RY8-CRLF2-positive clones are small at diagnosis and virtually never generate a dominant relapse clone. Our findings therefore suggest that P2RY8-CRLF2-positive clones do not have the necessary proliferative or selective advantage to evolve into a disease-relevant relapse clone.

  20. Low PKCα expression within the MRD-HR stratum defines a new subgroup of childhood T-ALL with very poor outcome.

    PubMed

    Milani, Gloria; Rebora, Paola; Accordi, Benedetta; Galla, Luisa; Bresolin, Silvia; Cazzaniga, Gianni; Buldini, Barbara; Mura, Rossella; Ladogana, Saverio; Giraldi, Eugenia; Conter, Valentino; Te Kronnie, Geertruy; Valsecchi, Maria Grazia; Basso, Giuseppe

    2014-07-30

    Pediatric T-cell Acute Lymphoblastic Leukemia (T-ALL) outcome has improved in the last decades, yet one patient in every four still relapses. Except treatment response and immunophenotype, few markers are reliably prognostic in pediatric T-ALL patients. Aiming to improve T-ALL risk stratification, we investigated a new candidate biomarker with potential prognostic relevance. A phosphoproteomic screening of 98 pediatric T-ALL samples at diagnosis had been performed using the high-throughput Reverse Phase Protein Arrays technique, which led to the identification of PKCαS657 as an activated protein with a broad variation among T-ALL samples. To evaluate PKCα potential as a prognostic biomarker, PKCα expression was analyzed using RQ-PCR in a cohort of 173 patients, representative of ALL2000-ALLR2006 AIEOP study. A threshold of PKCα expression with the highest discrimination for incidence of relapse was identified. Patients with PKCα down-regulation, compared to patients with PKCα levels above the threshold, presented a markedly increased cumulative incidence of relapse (43.8% vs. 10.9%, P<0.001), as well as a worse 4-year overall survival (66% vs. 87.9%, P=0.002) and event-free survival (53.1% vs. 85.2%, P=0.002). In particular, low PKCα expression identified cases with extremely poor outcome within the high-risk minimal residual disease (MRD) stratum, their incidence of relapse being of 69% vs. 15% in the high PKCα levels group. In a multivariate analysis adjusting for main prognostic features, PKCα proved to be an independent prognostic factor related to incidence of relapse. Very high risk patients within the high-risk MRD stratum, identified by PKCα expression, could be proposed for experimental therapeutic protocols.

  1. Biological, Functional and Genetic Characterization of Bone Marrow-Derived Mesenchymal Stromal Cells from Pediatric Patients Affected by Acute Lymphoblastic Leukemia

    PubMed Central

    Conforti, Antonella; Biagini, Simone; Del Bufalo, Francesca; Sirleto, Pietro; Angioni, Adriano; Starc, Nadia; Li Pira, Giuseppina; Moretta, Francesca; Proia, Alessandra; Contoli, Benedetta; Genovese, Silvia; Ciardi, Claudia; Avanzini, Maria Antonietta; Rosti, Vittorio; Lo-Coco, Francesco; Locatelli, Franco; Bernardo, Maria Ester

    2013-01-01

    Alterations in hematopoietic microenvironment of acute lymphoblastic leukemia patients have been claimed to occur, but little is known about the components of marrow stroma in these patients. In this study, we characterized mesenchymal stromal cells (MSCs) isolated from bone marrow (BM) of 45 pediatric patients with acute lymphoblastic leukemia (ALL-MSCs) at diagnosis (day+0) and during chemotherapy treatment (days: +15; +33; +78), the time points being chosen according to the schedule of BM aspirates required by the AIEOP-BFM ALL 2009 treatment protocol. Morphology, proliferative capacity, immunophenotype, differentiation potential, immunomodulatory properties and ability to support long-term hematopoiesis of ALL-MSCs were analysed and compared with those from 41 healthy donors (HD-MSCs). ALL-MSCs were also genetically characterized through array-CGH, conventional karyotyping and FISH analysis. Moreover, we compared ALL-MSCs generated at day+0 with those isolated during chemotherapy. Morphology, immunophenotype, differentiation potential and in vitro life-span did not differ between ALL-MSCs and HD-MSCs. ALL-MSCs showed significantly lower proliferative capacity (p<0.001) and ability to support in vitro hematopoiesis (p = 0.04) as compared with HD-MSCs, while they had similar capacity to inhibit in vitro mitogen-induced T-cell proliferation (p = N.S.). ALL-MSCs showed neither the typical translocations carried by the leukemic clone (when present), nor other genetic abnormalities acquired during ex vivo culture. Our findings indicate that ALL-MSCs display reduced ability to proliferate and to support long-term hematopoiesis in vitro. ALL-MSCs isolated at diagnosis do not differ from those obtained during treatment. PMID:24244271

  2. The opinion of clinical staff regarding painfulness of procedures in pediatric hematology-oncology: an Italian survey

    PubMed Central

    2011-01-01

    Background Beliefs of caregivers about patient's pain have been shown to influence assessment and treatment of children's pain, now considered an essential part of cancer treatment. Painful procedures in hematology-oncology are frequently referred by children as the most painful experiences during illness. Aim of this study was to evaluate professionals' beliefs about painfulness of invasive procedures repeatedly performed in Pediatric Hemato-Oncology Units. Methods Physicians, nurses, psychologists and directors working in Hemato-Oncology Units of the Italian Association of Pediatric Hematology-Oncology (AIEOP) were involved in a wide-nation survey. The survey was based on an anonymous questionnaire investigating beliefs of operators about painfulness of invasive procedures (lumbar puncture, bone marrow aspirate and bone marrow biopsy) and level of pain management. Results Twenty-four directors, 120 physicians, 248 nurses and 22 psychologists responded to the questionnaire. The score assigned to the procedural pain on a 0-10 scale was higher than 5 in 77% of the operators for lumbar puncture, 97.5% for bone marrow aspiration, and 99.5% for bone marrow biopsy. The scores assigned by nurses differed statistically from those of the physicians and directors for the pain caused by lumbar puncture and bone marrow aspiration. Measures adopted for procedural pain control were generally considered good. Conclusions Invasive diagnostic-therapeutic procedures performed in Italian Pediatric Hemato-Oncology Units are considered painful by all the caregivers involved. Pain management is generally considered good. Aprioristically opinions about pain depend on invasiveness of the procedure and on the professional role. PMID:21663631

  3. CRLF2 over-expression is a poor prognostic marker in children with high risk T-cell acute lymphoblastic leukemia

    PubMed Central

    Palmi, Chiara; Savino, Angela M.; Silvestri, Daniela; Bronzini, Ilaria; Cario, Gunnar; Paganin, Maddalena; Buldini, Barbara; Galbiati, Marta; Muckenthaler, Martina U.; Bugarin, Cristina; Mina, Pamela Della; Nagel, Stefan; Barisone, Elena; Casale, Fiorina; Locatelli, Franco; Nigro, Luca Lo; Micalizzi, Concetta; Parasole, Rosanna; Pession, Andrea; Putti, Maria C.; Santoro, Nicola; Testi, Anna M.; Ziino, Ottavio; Kulozik, Andreas E.; Zimmermann, Martin; Schrappe, Martin; Villa, Antonello; Gaipa, Giuseppe; Basso, Giuseppe; Biondi, Andrea; Valsecchi, Maria G.; Stanulla, Martin; Conter, Valentino; te Kronnie, Geertruy; Cazzaniga, Giovanni

    2016-01-01

    Pediatric T-ALL patients have a worse outcome compared to BCP-ALL patients and they could benefit from new prognostic marker identification. Alteration of CRLF2 gene, a hallmark correlated with poor outcome in BCP-ALL, has not been reported in T-ALL. We analyzed CRLF2 expression in 212 T-ALL pediatric patients enrolled in AIEOP-BFM ALL2000 study in Italian and German centers. Seventeen out of 120 (14.2%) Italian patients presented CRLF2 mRNA expression 5 times higher than the median (CRLF2-high); they had a significantly inferior event-free survival (41.2%±11.9 vs. 68.9%±4.6, p=0.006) and overall survival (47.1%±12.1 vs. 73.8%±4.3, p=0.009) and an increased cumulative incidence of relapse/resistance (52.9%±12.1 vs. 26.2%±4.3, p=0.007) compared to CRLF2-low patients. The prognostic value of CRLF2 over-expression was validated in the German cohort. Of note, CRLF2 over-expression was associated with poor prognosis in the high risk (HR) subgroup where CRLF2-high patients were more frequently allocated. Interestingly, although in T-ALL CRLF2 protein was localized mainly in the cytoplasm, in CRLF2-high blasts we found a trend towards a stronger TSLP-induced pSTAT5 response, sensitive to the JAK inhibitor Ruxolitinib. In conclusion, CRLF2 over-expression is a poor prognostic marker identifying a subset of HR T-ALL patients that could benefit from alternative therapy, potentially targeting the CRLF2 pathway. PMID:27449287

  4. Is there a standard for surgical therapy of hepatocellular carcinoma in healthy and cirrhotic liver? A comparison of eight guidelines

    PubMed Central

    Manzini, Giulia; Henne-Bruns, Doris; Porzsolt, Franz; Kremer, Michael

    2017-01-01

    Background and aims Liver resection (LR) and transplantation are the most reliable treatments for hepatocellular carcinoma (HCC). Aim was to compare different guidelines regarding indication for resection and transplantation because of HCC with and without underlying cirrhosis. Methods We compared the following guidelines published after 1 January 2010: American (American Association for the Study of Liver Diseases (AASLD)), Spanish (Sociedad Espanola de Oncologia Medica (SEOM)), European (European Association for the study of liver-European Organization for Research and Treatment of Cancer (EASL-EORTC) and European Society for Medical Oncology-European Society of Digestive Oncology (ESMO-ESDO)), Asian (Asian Pacific Association for the Study of Liver (APASL)), Japanese (Japan Society of Hepatology (JSH)), Italian (Associazione Italiana Oncologia Medica (AIOM)) and German (S3) guidelines. Results All guidelines recommend resection as therapy of choice in healthy liver. Guidelines based on the Barcelona Clinic Liver Cancer staging system recommend resection for single HCC<2 cm and Child-Pugh A cirrhosis and for HCC≤5 cm with normal bilirubin and portal pressure, whereas transplantation is recommended for multiple tumours between Milan criteria and for single tumours ≤5 cm and advanced liver dysfunction. Patients with HCC and Child-Pugh C cirrhosis are not candidates for transplantation. JSH guidelines recommend LR for patients with Child-Pugh A/B with HCC without tumour size restriction; APASL guidelines in general exclude patients with Child-Pugh A from transplantation. In patients with Child-Pugh B, transplantation is the second-line therapy, if resection is not possible for patients within Milan criteria. German and Italian guidelines recommend transplantation for all patients within Milan criteria. Conclusions Whereas resection is the standard therapy of HCC in healthy liver, a standard regarding the indication for LR and transplantation for HCC in

  5. CYR61 and TAZ Upregulation and Focal Epithelial to Mesenchymal Transition May Be Early Predictors of Barrett's Esophagus Malignant Progression.

    PubMed

    Cardoso, Joana; Mesquita, Marta; Dias Pereira, António; Bettencourt-Dias, Mónica; Chaves, Paula; Pereira-Leal, José B

    2016-01-01

    Barrett's esophagus is the major risk factor for esophageal adenocarcinoma. It has a low but non-neglectable risk, high surveillance costs and no reliable risk stratification markers. We sought to identify early biomarkers, predictive of Barrett's malignant progression, using a meta-analysis approach on gene expression data. This in silico strategy was followed by experimental validation in a cohort of patients with extended follow up from the Instituto Português de Oncologia de Lisboa de Francisco Gentil EPE (Portugal). Bioinformatics and systems biology approaches singled out two candidate predictive markers for Barrett's progression, CYR61 and TAZ. Although previously implicated in other malignancies and in epithelial-to-mesenchymal transition phenotypes, our experimental validation shows for the first time that CYR61 and TAZ have the potential to be predictive biomarkers for cancer progression. Experimental validation by reverse transcriptase quantitative PCR and immunohistochemistry confirmed the up-regulation of both genes in Barrett's samples associated with high-grade dysplasia/adenocarcinoma. In our cohort CYR61 and TAZ up-regulation ranged from one to ten years prior to progression to adenocarcinoma in Barrett's esophagus index samples. Finally, we found that CYR61 and TAZ over-expression is correlated with early focal signs of epithelial to mesenchymal transition. Our results highlight both CYR61 and TAZ genes as potential predictive biomarkers for stratification of the risk for development of adenocarcinoma and suggest a potential mechanistic route for Barrett's esophagus neoplastic progression.

  6. Dysphagia in Head and Neck Cancer Patients: Pretreatment Evaluation, Predictive Factors, and Assessment during Radio-Chemotherapy, Recommendations

    PubMed Central

    Merlano, Marco C.; Russi, Elvio G.

    2013-01-01

    Progress in head and neck cancer (HNC) therapies has improved tumor response, loco-regional control, and survival. However, treatment intensification also increases early and late toxicities. Dysphagia is an underestimated symptom in HNC patients. Impairment of swallowing process could cause malnutrition, dehydration, aspiration, and pneumonia. A comprehensive literature review finalized in May 2012 included searches of electronic databases (Medline, Embase, and CAB abstracts) and scientific societies meetings materials (American Society of Clinical Oncology, Associazione Italiana Radioterapia Oncologica, Associazione Italiana di Oncologia Cervico-Cefalica, American Head and Neck Society, and European Society for Medical Oncology). Hand-searches of HNC journals and reference lists were carried out. Approximately one-third of dysphagia patients developed pneumonia requiring treatment. Aspiration pneumonia associated mortality ranged from 20% to 65%. Unidentified dysphagia caused significant morbidity, increased mortality, and decreased the quality of life. In this review we underline definition, causes, predictive factors of dysphagia and report on pretreatment and on-treatment evaluation, suggesting some key points to avoid underestimation. A multi-parameter assessment of swallowing problems may allow an earlier diagnosis. An appropriate evaluation might lead to a better treatment of both symptoms and cancer. PMID:24069513

  7. [Retrospective analysis of the effect of a vagus nerve stimulator implanted in paediatric patients with refractory epilepsy].

    PubMed

    Fuentes-Pita, P; Gomez-Lado, C; Dacruz, D; Eiris-Punal, J; Prieto-Gonzalez, A; Castro-Gago, M

    2016-07-01

    Introduccion. El estimulador vagal es una alternativa terapeutica en los pacientes con epilepsia refractaria al tratamiento con farmacos antiepilepticos que no son candidatos a cirugia de reseccion. Objetivo. Analizar la eficacia del estimulador vagal en los pacientes pediatricos de nuestro centro. Pacientes y metodos. Conjunto de 13 pacientes implantados entre los años 2008 y 2013. Se registro la frecuencia de crisis previa a la implantacion, al año, a los dos años y al final del seguimiento. Asimismo, se recogio el numero de farmacos antiepilepticos utilizados, de forma cualitativa la mejoria conductual y el cambio en la intensidad de las crisis, asi como la aparicion de efectos secundarios y la retirada o no del dispositivo. Resultados. Al año, a los dos años y al final del seguimiento se habia producido una reduccion en el numero de crisis del 61%, 66,7% y 69%, respectivamente, y uno de los pacientes se encontro libre de crisis a los dos años. Al final del seguimiento, un 23% de los que habian disminuido sus crisis habia experimentado una reduccion superior al 90%. De forma independiente al efecto sobre el numero de crisis, el 77% de los pacientes presento una mejoria en la intensidad y duracion de las crisis, y ese mismo porcentaje mostro una mejoria conductual. Los efectos secundarios aparecieron en un 30,7% de los pacientes y fueron de intensidad leve. Conclusiones. A pesar del pequeño tamaño de la muestra, nuestros resultados indican que el estimulador vagal tiene una eficacia relevante en la poblacion pediatrica farmacorresistente, tanto sobre la frecuencia e intensidad de las crisis como sobre la conducta.

  8. [The use of the ketogenic diet as treatment for refractory epilepsy in the paediatric age].

    PubMed

    Pablos-Sánchez, Tamara; Oliveros-Leal, Liliana; Núñez-Enamorado, Noemí; Camacho-Salas, Ana; Moreno-Villares, José Manuel; Simón-De las Heras, Rogelio

    2014-01-16

    Introduccion. El 23-25% de los niños epilepticos son refractarios a farmacos antiepilepticos. El interes por la dieta cetogenica como tratamiento en estos pacientes no candidatos a otras opciones terapeuticas ha resurgido ultimamente. Objetivo. Valorar la eficacia y seguridad del tratamiento con dieta cetogenica en un importante numero de pacientes pediatricos con epilepsia refractaria en nuestro centro y determinar si los resultados obtenidos corroboran otros de publicacion reciente. Pacientes y metodos. Se revisaron retrospectivamente las historias clinicas de 41 niños con epilepsia refractaria que fueron tratados con dieta cetogenica entre 1998 y 2011, la mayoria con dieta tipo Radcliffe II. La mediana de edad al inicio de la dieta fue de 3,92 años. Resultados. A los seis meses del inicio de la dieta se redujeron las crisis en al menos un 50% en un 36,84% de la muestra (el 10,53% de los niños alcanzo mas de un 90% de reduccion y un 5,26% quedo sin crisis). Aproximadamente un 50% por grupo de edad en los mas pequeños respondio de manera positiva. Un 58,54% de los pacientes presento algun efecto secundario, tolerable y transitorio, principalmente elevacion de los niveles de colesterol y estreñimiento, sin observarse variacion en los parametros antropometricos. Conclusiones. La dieta cetogenica supone una buena alternativa terapeutica en los casos de epilepsia refractaria en la edad pediatrica, con mayor probabilidad de beneficio cuanto menor sea la edad del niño al inicio de la dieta. En general, es bien tolerada. Son de gran importancia en estos pacientes las revisiones periodicas con control nutricional.

  9. [Epilepsy surgery in children with focal cortical dysplasias].

    PubMed

    Villarejo-Ortega, Francisco J; Álvarez-Linera Prado, Juan; Pérez-Jiménez, M Ángeles

    2013-09-06

    Introduccion. Las displasias corticales focales (DCF) son la primera etiologia de cirugia de la epilepsia pediatrica. La evaluacion prequirurgica en DCF a menudo es compleja, ya que son lesiones que pueden ser altamente epileptogenas y, a la vez, conservar funcion neurologica, y no visualizarse en la resonancia magnetica. El exito de la cirugia depende, en gran medida, de la adecuada identificacion de la lesion y de la posibilidad de realizar una reseccion completa del tejido displasico. En este trabajo se revisa la bibliografia relacionada con el tema, en relacion con la experiencia de los autores. Desarrollo. Se revisan algunos avances relacionados con la evaluacion prequirurgica y el abordaje neuroquirurgico de la epilepsia en niños con DCF; se comentan los resultados de las series quirurgicas en relacion con las clasificaciones de DCF y los factores de pronostico posquirurgico; se describen algunos fenotipos anatomoelectroclinicos distintivos en niños con DCF y su abordaje quirurgico; y se comentan brevemente los actuales retos y el futuro del tratamiento quirurgico de la epilepsia en DCF. Conclusiones. El avance los metodos de diagnostico prequirurgico y de procedimientos quirurgicos esta permitiendo ofrecer un tratamiento exitoso en edades tempranas a pacientes con DCF previamente considerados 'no lesionales' y a pacientes con lesiones localizadas en la 'corteza elocuente'. La identificacion de fenotipos anatomoelectroclinicos de DCF permite establecer abordajes quirurgicos y expectativas de pronostico posquirurgico adecuadas a cada situacion, mejor en las DCF IIb transmantle y en las displasias de fondo de surco que en las DCF multilobares, en su mayoria DCF I.

  10. Meningococco B: controllo di due focolai epidemici mediante vaccinazione

    PubMed Central

    2014-01-01

    Riassunto La problematica di un efficace approccio vaccinale nei confronti del Meningococco B (MenB) è stata superata identificando con la metodica della "reverse vaccinology" alcuni antigeni capaci di indurre una risposta verso la maggior parte dei ceppi di MenB circolanti nel mondo. Il nuovo vaccino MenB a 4 componenti (4CMenB) è stato autorizzato in Europa, Australia e Canada, ed è entrato nei calendari di immunizzazione pediatrica internazionali: Australia, Canada, UK. In Italia, le prime regioni che hanno raccomandato la vaccinazione contro il MenB sono state Basilicata e Puglia. La gestione di epidemie/focolai epidemici richiede la messa in atto di una risposta rapida da parte delle autorità sanitarie nei confronti di una emergenza sanitaria ad elevato impatto, anche emotivo, sulla popolazione, come recentemente dimostrato in due università americane. Alla dichiarazione di focolaio epidemico in atto, in entrambi i contesti si è attivata una procedura per l'uso del vaccino 4CMenB non ancora autorizzato negli USA. È stato così possibile organizzare gli interventi di profilassi attiva nei due campus universitari, adottando il primo impiego su larga scala del nuovo vaccino 4CMenB e conseguendo, in tempi relativamente brevi, elevati tassi di copertura vaccinale. A fronte di circa 14000 studenti immunizzati con almeno una dose, non è stata segnalata alcuna problematica di eventi avversi conseguenti all'immunizzazione; ad oggi non si sono verificati casi nei soggetti che hanno ricevuto il vaccino. Come conseguenza dei due focolai descritti, è oggi in corso la valutazione da parte dell'FDA per l'estensione dell'uso del vaccino 4CMenB negli Stati Uniti negli adolescenti e giovani adulti. PMID:25916017

  11. [Learning disorders in neurofibromatosis type 1].

    PubMed

    Garcia-Penas, J J

    2017-02-24

    Introduccion. Los deficits neurocognitivos y las dificultades de aprendizaje representan las complicaciones neurologicas mas frecuentes de la neurofibromatosis tipo 1 (NF1) en la edad pediatrica y son responsables de una importante morbilidad evolutiva. Los niños con NF1 muestran alteraciones en atencion, percepcion visual, lenguaje, funciones ejecutivas, logros academicos y conducta. Los estudios en modelos animales sugieren que las alteraciones de aprendizaje en la NF1 se relacionan con una potenciacion de la actividad Ras que conduce a un incremento de la inhibicion mediada por el acido gamma-aminobutirico (GABA) y a una disminucion de la potenciacion sinaptica a largo plazo. Objetivo. Describir la frecuencia, gravedad, tipologia y evolucion natural de los deficits neurocognitivos especificos de la NF1. Desarrollo. Los trastornos neurocognitivos y conductuales afectan al 50-80% de los niños con NF1. Se pueden definir tres subtipos de perfiles cognitivos en la NF1, incluyendo trastorno de aprendizaje global, trastorno especifico de aprendizaje y trastorno por deficit de atencion/hiperactividad aislado. Los deficits cognitivos mas frecuentes se relacionan con la alteracion visuoespacial, aunque tambien son importantes las alteraciones de la memoria de trabajo y de la funcion ejecutiva asociadas con la disfuncion de la corteza prefrontal. Conclusiones. Existe una gran frecuencia global de problemas cognitivos en la NF1, lo cual implica que la disfuncion neurocognitiva sea la mayor complicacion medica que afecta la calidad de vida de estos pacientes. El diagnostico y el tratamiento precoces de los trastornos de aprendizaje en estos niños son basicos para conseguir un mejor desempeño academico.

  12. [Aicardi syndrome: retrospective study of a series of seven case reports].

    PubMed

    Fernández-Ramos, Joaquín Alejandro; López-Laso, Eduardo; Simón-De Las Heras, Rogelio; Camino-León, Rafael; Guerra-García, Pilar; Camacho-Salas, Ana; Aguilar-Quintero, María; Núñez-Enamorado, Noemí

    2013-12-01

    Introduccion. El sindrome de Aicardi es un trastorno presumiblemente dominante ligado al cromosoma X, que afecta en exclusiva a mujeres, clasicamente definido por la triada de agenesia del cuerpo calloso, lagunas coriorretinianas y espasmos infantiles, letal en varones en la vida intrauterina. Pacientes y metodos. Estudio descriptivo retrospectivo de pacientes diagnosticadas y seguidas hasta el final de la edad pediatrica de sindrome de Aicardi en dos hospitales universitarios durante un periodo de 29 años. Resultados. Encontramos siete niñas, todas desarrollaron espasmos infantiles antes de los 6 meses de edad. La evolucion fue a espasmos mas alla de la infancia (n = 2), a epilepsia parcial farmacorresistente (n = 3) y a epilepsia parcial bien controlada (n = 1). Seis casos presentaron retraso mental grave-profundo, y uno, moderado-grave. Fallecieron dos niñas a los 2 y 6 años. En todas, los estudios de neuroimagen mostraron agenesia del cuerpo calloso, quistes intracraneales y malformaciones del desarrollo cortical cerebral, ademas de lesiones oftalmologicas: lagunas coriorretinianas (n = 7), anoftalmia/microftalmia (n = 4) y coloboma del nervio optico (n = 3). Otros hallazgos fueron cardiopatia congenita, anomalias costovertebrales, linfangioma cervical e hipertricosis focal. Conclusiones. El sindrome de Aicardi debe sospecharse en niñas con espasmos infantiles y agenesia del cuerpo calloso. Deben descartarse en estas pacientes las alteraciones oftalmologicas, las anomalias de la migracion y organizacion neuronal y los quistes intracraneales. El pronostico es grave por su elevada morbimortalidad y por la frecuente evolucion a epilepsia refractaria y retraso mental grave.

  13. [A descriptive study of non-symptomatic epilepsy according to age at onset at a Neuropediatric Section of regional reference].

    PubMed

    Ochoa-Gomez, L; Lopez-Pison, J; Fernando-Martinez, R; Fuertes-Rodrigo, C; Samper-Villagrasa, P; Monge-Galindo, L; Pena-Segura, J L

    2016-11-16

    Objetivo. Estudio descriptivo de las epilepsias no sintomaticas (idiopaticas y criptogenicas), segun la edad de inicio, controladas en una unidad de neuropediatria de referencia regional durante tres años. Pacientes y metodos. Revision de historias de niños con epilepsia no sintomatica de la base de datos de neuropediatria controlados del 1 de enero de 2008 al 31 de diciembre de 2010. Resultados. De 4.595 niños atendidos en el periodo, se diagnosticaron de epilepsia 605 (13,17%), de las cuales 156 (25,79%) fueron idiopaticas, y 172 (28,43%), criptogenicas. La edad media de inicio del total fue de 4,78 años; 6,31 años en las idiopaticas y 5,43 años en las criptogenicas. El 26,12% del total de epilepsias se inicio en el primer año. Las epilepsias idiopaticas predominan en el grupo de inicio de 6-10 años, y las criptogenicas, en el de 3-6 años. La epilepsia de ausencias y la epilepsia benigna de la infancia con paroxismos centrotemporales son los sindromes epilepticos idiopaticos mas prevalentes. Conclusiones. Existen muchas diferencias de datos epidemiologicos publicados sobre epilepsia infantil por la dificultad que entraña un diagnostico sindromico en la edad pediatrica, debido a la variabilidad clinica y electroencefalografica. La ausencia de una clasificacion universalmente aceptada de los sindromes epilepticos dificulta comparaciones entre series. Todas las epilepsias son sintomaticas, puesto que tienen causa, sea genetica o adquirida. Una clasificacion util es la etiologica, con dos grupos: un gran grupo con las etiologias establecidas o sindromes geneticos muy probables y otro de casos sin causa establecida. La edad de inicio orienta a determinadas etiologias.

  14. [Current state of the attention deficit hyperactivity disorder approach in neuropediatrics].

    PubMed

    Cardo, E; Ros-Cervera, G; Eiris-Punal, J; Escofet-Soteras, C; Fernandez-Jaen, A; Mulas, F; Sanchez-Carpintero, R; Sans, A; Grupo de Trabajo de Tdah de la Sociedad Espanola de Neurologia Pediatrica, Grupo de Trabajo de Tdah de la Sociedad Espanola de Neurologia Pediatrica

    2017-02-24

    Objetivo. Conocer el estado actual del enfoque del trastorno por deficit de atencion/hiperactividad (TDAH) entre los neuropediatras. Sujetos y metodos. Se realizo una encuesta telematica que recogia informacion preliminar sobre el interes, las dificultades en el manejo y el tratamiento del TDAH a los 437 socios de la Sociedad Española de Neurologia Pediatrica. Resultados. Respondio un 32,49% de los cuestionarios enviados, con una importante variabilidad geografica. El 97,89% afirmo que el 50% de sus consultas eran niños con trastornos de aprendizaje y TDAH. Respecto a quien iniciaba el tratamiento para el TDAH en su area, la mayoria contesto que el neuropediatra (57,97%), seguido del psiquiatra infantil (34,78%) y del pediatra de atencion primaria (5,31%). Respecto a las mayores dificultades para la valoracion inicial de los niños con sospecha de TDAH, se citaron la falta de un estudio psicopedagogico por parte de la escuela (49,79%), seguido de la falta de tiempo en la consulta (29,11%). Sobre las dificultades en el seguimiento, la mayor queja se produjo por la falta de coordinacion entre los profesionales, la escuela y los padres. Respecto a la medicacion, la mayoria de los pacientes se encontraba en tratamiento con algun tipo de metilfenidato de liberacion prolongada, un porcentaje estable utilizaba metilfenidato de liberacion inmediata como tratamiento unico o combinado, y se observo en un rango inferior el uso de clonidina y atomoxetina, y un incipiente uso de lisdexanfetamina. La adhesion al tratamiento farmacologico al año fue alrededor del 80%. Conclusiones. Es necesario avanzar en la capacitacion y educacion continua de nuestros especialistas neuropediatricos en el manejo del TDAH, y en homogeneizar la practica clinica y la coordinacion con educacion en el territorio español.

  15. [Neurological complications in the population of children with leukaemia].

    PubMed

    Martínez-Cayuelas, Elena; Domingo-Jiménez, Rosario; Pascual-Gázquez, Juan F; Martínez-Salcedo, Eduardo; Alarcón-Martínez, Helena; Bermúdez-Cortés, Mar; Fuster-Soler, José L; Pérez-Fernández, Virginia

    2015-02-01

    Introduccion. La leucemia es el cancer mas frecuente en edad pediatrica. Su tasa de curacion es del 80% con quimioterapia intensiva, que mejora la supervivencia, pero que tambien aumenta la frecuencia de efectos adversos, incluyendo los neurologicos. Objetivos. Describir la frecuencia y caracteristicas de las complicaciones neurologicas (CN) en pacientes con leucemia aguda linfoide (LAL) y leucemia aguda mieloide (LAM), e identificar los factores asociados a su presencia, la tasa de morbilidad neurologica y la supervivencia. Pacientes y metodos. Estudio retrospectivo de las CN presentes durante el tratamiento y seguimiento de los pacientes con LAL y LAM entre 1997 y 2012 por la unidad de oncohematologia infantil. Variables analizadas: datos demograficos, diagnostico oncologico, tratamiento y CN. Resultados. Se incluyo un total de 157 pacientes, 145 sin infiltracion de sistema nervioso central al diagnostico y ocho con infiltracion (tasa de CN del 14 y 12%, respectivamente). Las CN mas frecuentes fueron: neuropatias (31%), alteracion del nivel de conciencia (27%), convulsiones (22%) y cefalea (12%). Un 40% de los pacientes con CN ha presentado secuelas, pero ninguno ha fallecido como consecuencia de la CN. Se han detectado mas CN en el grupo de edad menor de 6 años con LAL de alto grado, en niveles de gravedad mas altos y en pacientes que habian recibido trasplante de precursores hematopoyeticos, todas ellas con diferencias estadisticamente significativas. Conclusiones. Las complicaciones neurologicas son frecuentes en los pacientes con leucemia aguda, en especial en aquellos con estadio de riesgo alto (sobre todo si son menores de 6 años) y trasplante de precursores hematopoyeticos. La mortalidad asociada es baja.

  16. Experimental characterization of the Clear-PEM scanner spectrometric performance

    NASA Astrophysics Data System (ADS)

    Bugalho, R.; Carriço, B.; Ferreira, C. S.; Frade, M.; Ferreira, M.; Moura, R.; Ortigão, C.; Pinheiro, J. F.; Rodrigues, P.; Rolo, I.; Silva, J. C.; Trindade, A.; Varela, J.

    2009-10-01

    In the framework of the Clear-PEM project for the construction of a high-resolution and high-specificity scanner for breast cancer imaging, a Positron Emission Mammography tomograph has been developed and installed at the Instituto Português de Oncologia do Porto hospital. The Clear-PEM scanner is mainly composed by two planar detector heads attached to a robotic arm, trigger/data acquisition electronics system and computing servers. The detector heads hold crystal matrices built from 2 × 2 × 20 mm3 LYSO:Ce crystals readout by Hamamatsu S8550 APD arrays. The APDs are optically coupled to both ends of the 6144 crystals in order to extract the DOI information for each detected event. Each one of 12288 APD's pixels is read and controlled by Application Specific Integrated Circuits water-cooled by an external cooling unit. The Clear-PEM frontend boards innovative design results in a unprecedented integration of the crystal matrices, APDs and ASICs, making Clear-PEM the PET scanner with the highest number of APD pixels ever integrated so far. In this paper, the scanner's main technical characteristics, calibration strategies and the first spectrometric performance evaluation in a clinical environment are presented. The first commissioning results show 99.7% active channels, which, after calibration, have inter-pixel and absolute gain distributions with dispersions of, respectively, 12.2% and 15.3%, demonstrating that despite the large number of channels, the system is uniform. The mean energy resolution at 511 keV is of 15.9%, with a 8.8% dispersion, and the mean CDOI-1 is 5.9%/mm, with a 7.8% dispersion. The coincidence time resolution, at 511 keV, for a energy window between 400 and 600 keV, is 5.2 ns FWHM.

  17. Profile of thrombin generation in children with acute lymphoblastic leukemia treated by Berlin-Frankfurt-Münster (BFM) protocols.

    PubMed

    Lejhancova-Tousovska, Katerina; Zapletal, Ondrej; Vytiskova, Sona; Strbackova, Petra; Sterba, Jaroslav

    2012-03-01

    Treatment with L-asparaginase is associated with coagulation disturbances with deep venous thrombosis being the most common clinical consequence. Use of the calibrated automated thrombogram allows precise estimation of thrombin generated in vitro. We show the first data on thrombin generation, measured by calibrated automated thrombography (CAT), in children with acute lymphoblastic leukemia treated with L-asparaginase. Thrombin generation was measured by means of CAT in 23 children treated for acute lymphoblastic leukemia. Samples were obtained at predefined time points during the induction and reinduction phase of acute lymphoblastic leukemia-intercontinental Berlin-Frankfurt-Münster (BFM) 2000 or Associazione Italiana Ematologica Oncologia Pedaitrica Interim BFM 2000 protocols. Antihrombin and fibrinogen were measured on the same sample. Twenty-eight sets of thrombin generation measurements were collected from 23 patients. We observed no significant effect of antithrombin deficiency and/or hypofibrinogenemia on thrombin generation. Endogenous thrombin generation and peak thrombin were significantly higher during induction than in the reinduction phase (P < 0.001). Four patients with severe infection experienced an increase in thrombin generation, reaching maximum in a median of 7.5 days after the onset of infection. Two of those patients developed deep venous thrombosis at the time of peaked endogenous thrombin generation. Thrombin generation in children with acute lymphoblastic leukemia treated according to BFM protocols is significantly higher during the induction phase compared with reinduction and is not substantially affected by hypofibrinogenemia and/or antithrombin deficiency. Severe infection during the induction phase enhances thrombin generation with subsequent risk of thrombosis.

  18. Diffuse malignant biphasic peritoneal mesothelioma with cystic areas.

    PubMed

    Cabibi, Daniela; Tutino, Roberta; Salamone, Giuseppe; Cocorullo, Gianfranco; Agrusa, Antonino; Gulotta, Gaspare

    2016-06-20

    età avanzata è stato riferito al servizio di oncologia per il prosieguo delle cure. E’ stato sottoposto a chemioterapia con Pemetrexed e Cisplatino e dopo sei mesi presenta soddisfacenti condizioni cliniche generali e la TC di controllo non ha mostrato progressione di malattia.

  19. P17.52ANALYSIS OF RISK AND PROGNOSIS FACTORS IN GLIOBLASTOMA - OUR EXPERIENCE IN 104 PATIENTS

    PubMed Central

    Zaruma, C. Llumiguano; Arregui, E.; Cano, J.M.; Gonzalez, J.; Amo-Salas, M.; Lopez, C.; Moreno, J.M.Borrás

    2014-01-01

    Comite de Neuro-Oncologia, Hospital General Universitario de Ciudad Real, Spain OBJECTIVE: Glioblastoma multiforme (GBM), is the most common and most aggressive malignant primary brain tumor in humans. These tumors are typically associated with a dismal prognosis and poor quality of life. Our objective is analyze both the overall survival (OS) and progression-free survival (PFS) based on different potentially prognostic and risk factors. MATERIALS AND METHODS: We have analyzed 104 patients diagnosed with GBM in our center between 2006 and 2013. The standard scheme that we used was the STUPP scheme, of which 59 patients have benefited. Variables used include age, the diameter of the tumor at onset and the presence of necrosis, and have been evaluated different treatment schemes. RESULTS: In terms of age, it was statistically significant for both the OS (p = 0.0026) and the PFS (p = 0.002). We did not see differences nor in the size of tumor at the diagnosis nor in the presence of necrosis. No differences were seen in OS or PFS with the degree of surgical resection according to STUPP scheme. Patients who have made a complete treatment with surgery, radiotherapy and chemotherapy, have presented an OS of 17.2 months, while PFS was 10.9 months. If we compare these patients with those who have only been operated, survival is statistically significant (p = 0.00015 and p = 0.0013 respectively). CONCLUSIONS: After analyzing our series, we see that the only prognostic factor is age, with strong statistical significance. In our 104 patients, there are no significant differences in regard to the size and the presence of necrosis at diagnosis. Regarding the treatment, the OS nearly 18 months that present our patients treated with STUPP scheme is superior than in other series. We need to expand our database to improve both diagnosis and treatment and the subsequent impact on OS and PFS.

  20. Medullary Carcinoma of the Penis: A Distinctive HPV-related Neoplasm: A Report of 12 Cases.

    PubMed

    Cañete-Portillo, Sofía; Clavero, Omar; Sanchez, Diego F; Silvero, Arturo; Abed, Francisco; Rodriguez, Ingrid M; Ayala, Gustavo; Alemany, Laia; Munoz, Nubia; de Sanjose, Silvia; Quint, Wim; Bosch, Francesc X; Cubilla, Antonio L

    2017-04-01

    A third to half of penile invasive squamous cell carcinomas are human papillomavirus (HPV) related. Warty (condylomatous), warty-basaloid, and basaloid carcinomas are the most common subtypes associated with HPV. Less frequent are clear cell and lymphoepithelioma-like carcinomas. Here we report a novel penile tumor associated with HPV. Twelve cases were selected from 1010 penile carcinomas, part of an international HPV detection study conducted at the Institut Català d'Oncologia, Barcelona, Spain. Immunostaining with p16 was performed on all cases, and HPV-mRNA detection was also performed. En bloc full tumor staining was the utilized criteria for positivity of p16. For HPV-DNA detection, whole-tissue section polymerase chain reaction analysis was performed by SPF10-DEIA-LiPA25 (version 1). The patients' ages ranged from 42 to 92 years (average, 71 y). The tumor was most commonly located in the glans. A characteristic microscopic finding was the presence of a moderate to dense tumor-associated inflammatory cell infiltrate composed of neutrophils, lymphocytes, plasma cells, or eosinophils. Tumors grew in large solid sheets, nests, or had a trabecular pattern. Cells were large and poorly differentiated or anaplastic. Keratinization was minimal or absent. Nuclei were large with prominent nucleoli. Mitoses were numerous. Tumor necrosis was common. Deep invasion of the corpora cavernosa was frequent. p16 and HPV-DNA were positive in all cases, whereas mRNA detection was positive in 9 cases only. The prevalent genotype was HPV16 (9 cases, 75%). Other genotypes were HPVs 58, 33, and 66. Medullary carcinomas of the penis are morphologically distinctive HPV-related high-grade neoplasms affecting older individuals. More studies are necessary to delineate the epidemiological, clinical, and molecular features of this unusual penile neoplasm.

  1. CYR61 and TAZ Upregulation and Focal Epithelial to Mesenchymal Transition May Be Early Predictors of Barrett’s Esophagus Malignant Progression

    PubMed Central

    Mesquita, Marta; Dias Pereira, António; Bettencourt-Dias, Mónica; Chaves, Paula; Pereira-Leal, José B.

    2016-01-01

    Barrett’s esophagus is the major risk factor for esophageal adenocarcinoma. It has a low but non-neglectable risk, high surveillance costs and no reliable risk stratification markers. We sought to identify early biomarkers, predictive of Barrett’s malignant progression, using a meta-analysis approach on gene expression data. This in silico strategy was followed by experimental validation in a cohort of patients with extended follow up from the Instituto Português de Oncologia de Lisboa de Francisco Gentil EPE (Portugal). Bioinformatics and systems biology approaches singled out two candidate predictive markers for Barrett’s progression, CYR61 and TAZ. Although previously implicated in other malignancies and in epithelial-to-mesenchymal transition phenotypes, our experimental validation shows for the first time that CYR61 and TAZ have the potential to be predictive biomarkers for cancer progression. Experimental validation by reverse transcriptase quantitative PCR and immunohistochemistry confirmed the up-regulation of both genes in Barrett’s samples associated with high-grade dysplasia/adenocarcinoma. In our cohort CYR61 and TAZ up-regulation ranged from one to ten years prior to progression to adenocarcinoma in Barrett’s esophagus index samples. Finally, we found that CYR61 and TAZ over-expression is correlated with early focal signs of epithelial to mesenchymal transition. Our results highlight both CYR61 and TAZ genes as potential predictive biomarkers for stratification of the risk for development of adenocarcinoma and suggest a potential mechanistic route for Barrett’s esophagus neoplastic progression. PMID:27583562

  2. Impact of nutrition since early life on cardiovascular prevention

    PubMed Central

    2012-01-01

    The cardiovascular disease represents the leading cause of morbidity and mortality in Western countries and it is related to the atherosclerotic process. Cardiovascular disease risk factors, such as dyslipidemia, hypertension, insulin resistance, obesity, accelerate the atherosclerotic process which begins in childhood and progresses throughout the life span. The cardiovascular disease risk factor detection and management through prevention delays the atherosclerotic progression towards clinical cardiovascular disease. Dietary habits, from prenatal nutrition, breastfeeding, complementary feeding to childhood and adolescence nutrition play a basic role for this topic. The metabolic and neuroendocrine environment of the fetus is fundamental in the body’s “metabolic programming”. Further several studies have demonstrated the beneficial effects of breastfeeding on cardiovascular risk factors reduction. Moreover the introduction of complementary foods represents another important step, with particular regard to protein intake. An adequate distribution between macronutrients (lipids, proteins and carbohydrates) is required for correct growth development from infancy throughout adolescence and for prevention of several cardiovascular disease risk determinants in adulthood. The purpose of this review is to examine the impact of nutrition since early life on disease. La malattia cardiovascolare rappresenta la principale causa di morbilità e mortalità dei paesi occidentali ed è correlata a degenerazione vascolare aterosclerotica. I fattori di rischio cardiovascolari quali dislipidemia, ipertensione, insulino resistenza e obesità accelerano tale processo il cui esordio è noto sin dell’età pediatrica ed evolve nel corso della vita. L’individuazione e la cura dei fattori di rischio cardiovascolari mediante la prevenzione dei fattori causali ritardano la progressione dell’aterosclerosi e l’insorgenza dei sintomi cardiovascolari. La nutrizione svolge un ruolo

  3. Pediatric allergy and immunology in Italy.

    PubMed

    Tozzi, Alberto E; Armenio, Lucio; Bernardini, Roberto; Boner, Attilio; Calvani, Mauro; Cardinale, Fabio; Cavagni, Giovanni; Dondi, Arianna; Duse, Marzia; Fiocchi, Alessandro; Marseglia, Gian L; del Giudice, Michele Miraglia; Muraro, Antonella; Pajno, Giovanni B; Paravati, Francesco; Peroni, Diego; Tripodi, Salvatore; Ugazio, Alberto G; Indinnimeo, Luciana

    2011-05-01

    In Italy, according to the International Study on Asthma and Allergies in Childhood study, the prevalence of current asthma, allergic rhinoconjunctivitis, and atopic eczema in 2006 was 7.9%, 6.5%, and 10.1% among children aged 6-7 and 8.4%, 15.5%, and 7.75% among children aged 13-14 yr. University education in this field is provided by the Postgraduate Schools of Pediatrics and those of Allergology and Clinical Immunology, as well as several annual Master courses. The Italian Society of Pediatric Allergology and Immunology (SIAIP) was founded in 1996 and counts about 1000 members. SIAIP promotes evidence-based management of allergic children and disseminates information to patients and their families through a quite innovative website and the National Journal 'Rivista Italiana di Allergologia Pediatrica'. In the last decade, four major regional, inter-regional, and national web-based networks have been created to link pediatric allergy centers and to share their clinical protocols and epidemiologic data. In addition, National Registers of Primary Immune-deficiencies and on Pediatric HIV link all clinical excellence centers. Research projects in the field of pediatric allergy and immunology are founded by the Italian Ministry of Education, University and Research (MIUR) and by the National Research Council (CNR), but the overall investments in this research area are quite low. Only a handful Italian excellence centers participate in European Projects on Pediatric Allergy and Immunology within the 7th Framework Program. The European Academy of Allergy and Clinical Immunology currently hosts two Italians in its Executive Committee (EC) and one in the EC of the Pediatric Section; moreover, major European Academy of Allergy and Clinical Immunology meetings and courses in the area of pediatrics (e.g., PAAM, Venice, 2009) have been held in Italy in the last 3 yr. Italian hallmarks in the management of allergic diseases in childhood are a quite alive and spread interest in

  4. [Primary and secondary outcomes in oncology clinical trials: definitions and uses].

    PubMed

    Vaz-Carneiro, António; Luz, Ricardo; Borges, Margarida; Costa, João

    2014-01-01

    Introdução: A prova de eficácia de uma intervenção terapêutica em oncologia consegue-se através de ensaios clínicos rigorosamente conduzidos. Um dos factores metodológicos mais importantes é a selecção de indicadores clínicos de eficácia (outcomes), necessários ao cálculo das chamadas medidas de associação que permitem a definição de eficácia terapêutica. Material e Métodos: Foi feita uma revisão narrativa baseada em alguns dos documentos de agências reguladoras internacionais, assim como documentos de consenso entre as sociedades científicas oncológicas, procurando listar e avaliar criticamente cada um dos indicadores utilizados em ensaios clínicos oncológicos. Resultados: Identificaram-se como indicadores mais importantes a sobrevivência global, a sobrevivência livre de progressão/sobrevivência livre de doença, a toxicidade/qualidade de vida e taxa objectiva de resposta tumoral. Discussão: A selecção do outcome primário deve basear-se no conceito de eficácia terapêutica, mas também na toxicidade relativa da terapêutica experimental, na sobrevivência esperada após progressão da doença, na existência de fármacos alternativos já estudados com indicações idênticas e até a prevalência da patologia em causa. Conclusão: A selecção de indicadores em ensaios clínicos oncológicos reveste-se de especial importância e a sua selecção deve ser bem fundamentada, dependendo da doença, dos doentes e do fármaco em estudo.

  5. [Hodgkin's Lymphoma and Autoimmunity: Is There a Relationship?].

    PubMed

    Jerónimo, Mónica; Silva, Sónia; Benedito, Manuela; Brito, Manuel João

    2015-01-01

    Introdução: A relação entre linfomas e doenças autoimunes é descrita na literatura como bidirecional, existindo poucos dados em idade pediátrica. Este trabalho tem como objetivo avaliar a prevalência de doenças autoimunes em crianças e adolescentes com linfoma de Hodgkin seguidos num Serviço de Oncologia Pediátrica. Material e Métodos: Ao rever a casuística do Serviço de linfomas de Hodgkin nos últimos 16 anos (dados colhidos prospetivamente), constatou-se uma incidência aparentemente elevada de doenças autoimunes nas raparigas pelo que se realizou um estudo retrospetivo, com atualização do seguimento fora de tratamento, relativamente à existência de doenças autoimunes. Avaliaram-se: idade, sexo, tipo de doença autoimune, relação temporal com o linfoma, estádio e grupo histológico do linfoma e terapêutica efetuada. Resultados: Incluíram-se 52 casos de linfoma de Hodgkin, dos quais sete (13,5%), todos do sexo feminino, tiveram uma doença autoimune diagnosticada previamente, em simult'neo ou posteriormente ao linfoma. As doenças autoimunes encontradas foram: artrite idiopática juvenil, doença inflamatória intestinal, doença de Behçet, hepatite autoimune, lúpus eritematoso sistémico, tiroidite de Hashimoto e púrpura trombocitopénica idiopática. Em quatro doentes o diagnóstico foi posterior ao linfoma, em dois, prévio, e num simult'neo. Todos os casos, exceto o diagnóstico simult'neo, estão fora de tratamento e sem recidiva da doença oncológica. Não se verificaram óbitos. Discussão: Verificou-se uma importante prevalência de doenças autoimunes nas raparigas com linfoma de Hodgkin. Apresentamos os dados e discutimos possíveis causas desta relação com base numa revisão bibliográfica. Conclusões: Esta associação deve ser evocada, sendo necessário mais estudos, sobretudo em idade pediátrica.

  6. How changes in nutrition have influenced the development of allergic diseases in childhood

    PubMed Central

    2012-01-01

    The increasing prevalence of allergic diseases in childhood in the last decades could be linked to concomitant dietary changes, especially with the modified and lower consumption of fruit, vegetables and minerals. The consumption of these foods by pregnant women and children in the first years of life seems to be associated with a reduced risk of asthma and related symptoms. Foods that can prevent the development of wheezing through their antioxidant effects contain vitamin C and selenium; blood levels of these elements correlate negatively with the risk of wheezing. Intake of vitamin E during pregnancy also appears to be correlated with a reduced risk of wheezing for the unborn child. Similarly, low intake of zinc and carotenoids by pregnant women is associated with an increased risk of wheezing and asthma in childhood. Fiber also has anti-inflammatory properties and protective effects against allergic diseases such as atopic dermatitis and asthma. The consumption of fat influences the development of the airways. Populations in Western countries have increased their consumption of n-6 PUFAs and, in parallel, reduced n-3 PUFAs. This has led to decreased production of PGE2, which is believed to have a protective effect against inflammation of the airways. Conflicting hypotheses also concern vitamin D; both an excess and a deficiency of vitamin D, in fact, have been associated with an increased risk of asthma. Further studies on the role of these substances are necessary before any conclusions can be drawn on a clinical level. Astratto La crescente prevalenza negli ultimi decenni delle malattie allergiche in età pediatrica potrebbe essere legata a concomitanti cambiamenti nella dieta, in particolare alla minore e modificata introduzione di frutta, verdura e minerali. Il consumo di questi alimenti da parte delle donne in gravidanza e dei bambini nei primi anni di vita sembra essere associato ad un ridotto rischio di asma e di sintomi correlati. Gli alimenti che

  7. Bundle Approach to Reduce Bloodstream Infections in Neutropenic Hematologic Patients with a Long-Term Central Venous Catheter.

    PubMed

    Martinez, Jose Manuel; Leite, Luís; França, Daniela; Capela, Rita; Viterbo, Luísa; Varajão, Natalina; Martins, Ângelo; Oliveira, Isabel; Domingues, Nélson; Moreira, Ilídia; Santo, Ana; Trigo, Filipe; Mariz, Jose

    2015-01-01

    Introdução: O objetivo deste estudo foi reduzir através de um pacote de medidas as infeções sistémicas e as taxas de infeções com origem no cateter venoso central nos doentes hematológicos em neutropenia com cateter venoso central de longa duração. Material e Métodos: Estudo prospetivo não randomizado realizado na unidade onco-hematológica do Instituto Português de Oncologia do Porto no período compreendido entre 1 de agosto de 2010 até 31 de janeiro de 2012. Durante este período foi introduzido um pacote de medidas (grupo estudo) e comparados os resultados nos 6 meses anteriores à sua implementação (grupo de controlo). As medidas consistiram na utilização de conectores de pressão neutra em detrimento dos conectores de pressão positiva, na sua troca mais frequente e numa solução anti-séptica mais eficaz. Foram incluídos neste estudo 116 doentes hematológicos com cateter venoso central de longa duração inserido por um período superior a 72 h. Foram contabilizados 8 867 dias de cateter (6 756 dias de cateter venoso central no grupo estudo e 2 111 dias de cateter venoso central no grupo de controlo). Resultados: Obteve-se uma redução significativa nas taxas de infeções sistémicas e infeções com origem no cateter venoso central. As taxas de infeções sistémicas: [32,69 (grupo de controlo) vs. 9,43 (grupo estudo)], com uma redução de incidência de 71% [risco relativo 0,2886, CI 95% (0,1793 - 0,4647), p < 0,001] e taxas de infeções com origem no cateter venoso central: [17,53 (grupo de controlo) vs. 4,73 (grupo estudo)], com redução de incidência de 71% [risco relativo 0,2936, CI 95% (0,1793 - 0,5615), p < 0,014]. Não foi encontrada diferença significativa (p > 0,05) na contagem de neutrófilos à data da colheita das amostras de hemoculturas entre ambos os grupos: 69% (< 500 neutrófilos/mm3) [71% (grupo estudo) vs. 68% (grupo de controlo)]. Conclusões: A introdução deste pacote de medidas baseado nas variáveis do

  8. [Epidemiological and Demographic Characteristics of Patients with Head and Neck Tumours in the Northern Portugal: Impact on Survival].

    PubMed

    Estêvão, Roberto; Santos, Tiago; Ferreira, Ana; Machado, Anabela; Fernandes, João; Monteiro, Eurico

    2016-10-01

    Introdução: Em Portugal não existem, ainda, estudos epidemiológicos e demográficos realizados em doentes portadores de tumores da cabeça e pescoço. Os objectivos desta análise são descrever os aspectos epidemiológicos e demográficos dos doentes portadores de tumores malignos da cabeça e do pescoço, referenciados ao serviço de Otorrinolaringologia de um centro oncológico do norte de Portugal e avaliar o impacto destas características na sobrevivência. Material e Métodos: Estudo retrospectivo descritivo, dos doentes referenciados entre janeiro de 2011 e dezembro de 2013 ao Serviço de Otorrinolaringologia do Instituto Português de Oncologia do Porto. Foram incluídos 566 doentes. As variáveis analisadas foram a localização anatómica dos tumores, o estadiamento, o género e a idade dos doentes, o distrito de proveniência, os hábitos etílicos e tabágicos, o nível educacional, a profissão, o estado civil e a estrutura familiar.Resultados: Os 566 doentes estudados correspondem a 498 homens e 68 mulheres, com idade média de 58,1 ± 12,2 anos. A maioria (80,5%) foi referenciada com doença em estadio avançado (III e IV). Verificamos hábitos etílicos moderados a excessivos em 78% dos doentes e 69% eram fumadores. A grande maioria dos doentes (82,3%) tinha apenas o ensino básico. Os doentes com tumores da hipofaringe e com hábitos etílicos excessivos foram referenciados em estadios mais avançados e apresentaram pior sobrevivência (p < 0,001). Os doentes casados foram referenciados em estadios mais precoces (p = 0,020) e os doentes sem apoio familiar apresentaram pior sobrevivência (p = 0,030). Discussão: Os dados epidemiológicos encontrados estão de acordo com a literatura internacional. A elevada taxa de doentes referenciados em estádio avançado pode ser atribuída ao atraso na procura de cuidados médicos ou a factores inerentes ao sistema de referenciação. A vigilância e o suporte familiar têm um papel

  9. Thyroid carcinoma in children and young adults: retrospective review of 19 cases.

    PubMed

    Saraiva, Joana; Ribeiro, Cristina; Melo, Miguel; Gomes, Leonor; Costa, Gracinda; Carrilho, Francisco

    2013-01-01

    Introdução: O carcinoma da tiróide é raro em crianças e em adultos jovens. A maior parte das orientações clínicas baseia-se em dados obtidos na população adulta. Persistem diversas controvérsias no que se refere à agressividade da apresentação clínica e da abordagem terapêutica.Objectivo: Avaliar todos os doentes com carcinoma da tiróide com menos de 20 anos no momento da apresentação, reflectindo a experiência da nossa unidade relativamente ao diagnóstico, tratamento e seguimento desta entidade.Material e Métodos: Trata-se de um estudo de revisão retrospectiva dos registos clínicos de todas as crianças e adultos jovens seguidos na Consulta de Oncologia do Serviço de Endocrinologia do Centro Hospitalar e Universitário de Coimbra entre 1996 e 2012 .Resultados: Foram estudados 19 doentes, com uma média etária de 16 anos, sendo 13 do sexo feminino. Nenhum dos doentes fora previamente submetido a irradiação da região do pescoço. A queixa de apresentação era a presença de uma massa cervical palpável em 84,6% dos casos. Foi realizada citologia aspirativa em 15 doentes, que foi diagnóstica ou suspeita de neoplasia em 71,4% dos casos. A tiroidectomia total foi levada a cabo em 18 casos (94,7%). Em todos estes foi identificada a presença de um carcinoma papilar. A invasão vascular e multicêntrica ocorreu em 21,1% dos casos. A dimensão tumoral média foi de 2,5 cm. O envolvimento ganglionar cervical foi diagnosticado em 31,6% dos casos e a presença de metástases à distância foi identificada em 5,2% dos casos. Na maior parte dos doentes (18 em 19), o quadro foi classificado como doença em estadio I. Todos os doentes fizeram terapêutica supressora com tiroxina e 84,2% dos doentes foram submetidos a ablação pós-operatória com iodo radioactivo (dose média de 85,7mCi). Durante um seguimento médio de cerca de 6 anos, 16 doentes permaneceram em remissão.Conclusão: Na nossa série, as taxas de metastização ganglionar cervical

  10. [Treatment of Gastroenteropancreatic Neuroendocrine Tumors with 177Lu-DOTA-TATE: Experience of the Portuguese Institute of Oncology in Porto].

    PubMed

    Sampaio, Inês Lucena; Luiz, Henrique Vara; Violante, Liliana Sobral; Santos, Ana Paula; Antunes, Luís; Torres, Isabel; Sanches, Cristina; Azevedo, Isabel; Duarte, Hugo

    2016-11-01

    Introdução: O objetivo deste artigo é rever a experiência do Instituto Português de Oncologia do Porto na terapêutica de tumores neuroendócrinos gastroenteropancreáticos com 177Lu-DOTA-TATE, tendo como principais pontos de análise a segurança e eficáciaterapêutica. Material e Métodos: Foi realizada uma análise retrospetiva dos processos clínicos de doentes com tumores neuroendócrinos gastroenteropancreáticos, submetidos a terapêutica com 177Lu-DOTA-TATE entre abril de 2011 e novembro de 2013. Resultados: Dos 36 casos revistos, 30 completaram os três ciclos de 177Lu-DOTA-TATE (83,3%). Nesses doentes foram registados: efeitos colaterais agudos em 8,9% dos ciclos; toxicidade hepática grau 3 CTCAE em 13,3% dos doentes (todos com alterações prévias da função hepática); ausência de toxicidade renal ou hematológica significativa; melhoria sintomática em 71,4% dos doentes; tempo mediano global desde o início da terapêutica até progressão de doença de 25,6 meses; tempo mediano global de sobrevivência desde o diagnóstico de 121,7 meses. Verificou-se um maior tempo livre de progressão de doença e de sobrevivência nos doentes com expressão elevada de recetores da somatostatina (p < 0,05). Discussão: A peptide receptor radionuclide therapy com 177Lu-DOTA-TATE apresenta respostas clínicas favoráveis com segurança e boa tolerabilidade terapêutica, conforme evidenciado no nosso estudo pelos seguintes achados: melhoria dos sintomas na maioria dos doentes e aumento significativo do tempo livre de progressão de doença e da sobrevivência (sobretudo nos doentes com expressão elevada de sstr), com efeitos colaterais agudos e subagudos/crónicos significativos numa minoria de doentes. Conclusão: A peptide receptor radionuclide therapy com 177Lu-DOTA-TATE é uma terapêutica promissora, com benefícios reais em termos de eficácia e segurança nos doentes com tumores neuroendócrinos gastroenteropancreáticos.

  11. [Hypofractionation in locally advanced breast cancer: "flash" scheme].

    PubMed

    Padilha, Marisa; Gonçalves, Sara; Fardilha, Carlos; Melo, Gilberto; Miranda, Cristina; Alves, Paula

    2013-01-01

    Introdução: O carcinoma da mama é uma das principais causas de morte no nosso país. No Serviço de Radioterapia do Instituto Português de Oncologia de Coimbra de Coimbra utilizamos, desde há mais de 30 anos, um esquema de hipofraccionamento de radioterapia, denominado “Flash”, como opção terapêutica em doentes idosos ou com baixo Performance Status, portadores de carcinoma da mama localmente avançado ou com estádios IIb ou IV, com intenção neoadjuvante ou paliativa. Objectivos: Avaliar a resposta ao tratamento, nomeadamente sobrevivência global aos três anos, resposta local e toxicidades aguda e crónica, no grupo de doentes seleccionados submetidos a esquema de hipofraccionamento, em estudo retrospectivo. Metodologia: Entre Janeiro de 2006 e Dezembro de 2008, um total de 83 doentes com diagnóstico de Carcinoma da Mama Localmente Avançado ou com estádios IIb ou IV, foi submetido a “Flash” mamário. A dose de radioterapia prescrita foi de 13Gy / 2Fr / 3 dias (em 23 doentes - 27,7%) e 26Gy / 4Fr / 2,5 semanas (em 60 doentes - 72,3%), com fotões de 4 MV, sobre a mama afectada. Foi avaliada sobrevivência global segundo o método de Kaplan-Meier. A análise estatística foi efectuada através da aplicação SPSS, versão 17.0 e os testes estatísticos foram avaliados ao nível de significância de 5%. Resultados: 80 doentes (96,4%) que efectuaram “Flash” mamário eram do género feminino, com idades compreendidas entre os 59 e os 93 anos (idade média 80,72 + 5,87 anos) e Performance Status (Karnosfsky: 0 - 100) entre 90 e 50%. Em 72 doentes (86,7%) o diagnóstico histológico foi Carcinoma Ductal Invasivo. A cirurgia após a realização do “Flash” Mamário foi realizada em 44 doentes (53%) após evidência de resposta local à radioterapia, sendo a Mastectomia Radical Modificada a técnica cirúrgica mais frequente. Efectuou-se o diagnóstico de metastização óssea em 10 doentes (12%), sendo que a taxa de sobrevivência global foi

  12. Geographical epidemiology of antibacterials in the preschool age

    PubMed Central

    2012-01-01

    activities. Moreover, the competent authorities should increasing their efforts to limit unnecessary prescriptions and increase appropriateness of prescribing. Riassunto Introduzione Le mappe tematiche consentono una più rapida ed immediata lettura delle differenze geografiche nella distribuzione di dati riferiti ad un territorio specifico. Lo scopo dello studio è mostrare, per la prima volta, l’applicazione di alcuni strumenti statistici e cartografici, nell’analisi dell’uso dei farmaci nella popolazione pediatrica di una regione italiana e valutare le differenze intra-regionali. Metodi Per valutare il tipo di distribuzione geografica delle prescrizioni, sono stati calcolati i tassi di prevalenza standardizzati (punteggi-z) a livello di ASL, Distretti Sanitari e Comuni. Per valutare la correlazione con le ospedalizzazioni è stato usato il coefficiente di correlazione di Pearson; per valutare l’esistenza di autocorrelazione spaziale è stato usato l’indice I di Moran. Tramite l’uso della statistica G di Getis-Ord sono stati identificati cluster di aree ad alto e basso livello di prevalenza. Infine con un modello di regressione logistica è stata stimata la probabilità di ricevere almeno una prescrizione nel corso dell’anno per tutti i pazienti inclusi nello studio. Risultati Con l’uso delle mappe è possibile vedere che le prescrizioni non sono correlate con lo stato di salute della popolazione, ma sono correlate con l’attitudine prescrittiva del pediatra. Questo è confermato anche dal modello di regressione logistica costruito per stimare la probabilità di ricevere almeno una prescrizione considerando come variabili indipendenti l’età, il sesso, la prevalenza di ricoveri nel distretto di residenza, l’attitudine prescrittiva del pediatra, la classe di età del pediatra e la durata della convenzione del pediatra con l’Azienda Sanitaria Locale (ASL). Conclusioni Gli interventi primari per razionalizzare l’uso degli antibiotici in et

  13. [Secondary hypothyroidism after cervical irradiation: systematic evaluation of thyroid function in follow-up].

    PubMed

    Gonçalves, Sara Monteiro; Ferreira, Brígida Costa; Guardado, Maria João; Marques, Rui; Serra, Tânia; Serra, Maria João; Roda, Domingos; Brandão, Joana; Melo, Gilberto; Lopes, Maria Carmo; Khouri, Leila

    2014-01-01

    Introdução: A disfunção tiroideia constitui uma consequência, por vezes subestimada, da exposição à radiação. O mecanismo subjacente não está claramente esclarecido, mas terá uma origem multifatorial. Os fatores de risco específicos para o desenvolvimento de hipotiroidismo secundário à radioterapia permanecem indeterminados. A irradiação direta da glândula tiroideia pode resultar, com maior frequência, em hipotiroidismo. Este é uma condição irreversível, requerendo monitorização e tratamento permanente. Objetivos: Avaliar a incidência de hipotiroidismo nos doentes portadores de neoplasias da cabeça e pescoço submetidos a irradiação cervical, a título intensivo ou adjuvante, bem como determinar se é justificável a integração no protocolo de seguimento Institucional, de parâmetros laboratoriais (TSH, T3 Livre e T4 Livre) para avaliação da função tiroideia neste grupo de doentes e qual a sua periodicidade. Material e Métodos: Este é um estudo observacional, descritivo, retrospetivo, que engloba um grupo de 376 doentes portadores de neoplasias da cabeça e pescoço, submetidos a tratamento de radioterapia, a título adjuvante ou intensivo, entre os anos de 2007 e 2012, no Instituto Português de Oncologia de Coimbra Francisco Gentil E.P.E. Cumpriram todos os critérios de inclusão 145 doentes. Foram utilizadas escalas de avaliação padronizadas para a definição de hipotiroidismo – LENT-SOMA scales (Late Effects Normal Tissues Subjective Objective Management Analysis). Foi considerada como complicação o hipotiroidismo Grau 1 ou superior. Resultados: Procedeu-se à análise de um grupo de 145 doentes. A localização tumoral mais frequente foi a Laringe (26,9%). Trinta e dois doentes efetuaram radioterapia adjuvante e 113 efetuaram esquemas intensivos. A técnica de radioterapia mais utilizada foi a intensidade modulada (IMRT), efetuada em 86,2% doentes. A taxa de incidência global de hipotiroidismo aos 12 meses foi de