Symbolisations affectives évoquées par la maladie du diabète. Étude multicas concernant des patients pédiatriques et leur mère = Affective symbolizations evoked by the disease of diabetes. Multiple-case study on pediatric patients and their caregiver

Les auteurs ont réalisé une enquête sur les symbolisations affectives évoquées par le diabète, en tant que maladie ainsi que les modalités de partage et de gestion de ces symbolisations dans la relation mère-enfant. Les enfants participants à cette enquête sont atteints du diabète de type 1. Une étude multicas a été effectuée auprès de cinq duos mère-enfant, selon plusieurs méthodes qualitatives tant pour la collecte de données (interview semi-structurée, dessins, associations libres) que pour l’analyse des données (analyse emotionnelle du texte, activité référentielle, échelle du soi réflexif). Les résultats de cette étude font apparaître que les enfants partagent avec leur propre mère la plupart des symbolisations affectives liées à la maladie, ainsi que les principales modalités de gestion des émotions. Il émerge ainsi une correspondance entre les capacités de la mère et de l’enfant à réfléchir sur les émotions évoquées par la maladie. En outre, les duos qui disposent d’une plus grande capacité réflexive sont caractérisés par des meilleures dynamiques d’élaboration et d’intégration de la maladie dans le chemin de vie, par de moindres surinvestissements affectifs ainsi que par des mécanismes de défense plus évolués. La capacité de réfléchir sur les émotions évoquées par la maladie semble donc être un facteur de protection pour une bonne adaptation psychologique de l’enfant diabétique et de sa mère.The authors inquired about the affective experience of some diabetic children and their caregiver, through a qualitative methodology, psychodynamic models and instruments that allow listening to narrative's “deep level”. A multi-case study was conducted on five mother-child duos by several methods of data collection (semi-structured interview, drawing, free associations) and of data analysis (Text Emotional Analysis, Reflective Self-Scale, Referential Activity, analysis of children's drawing, analysis of free associations, analysis of single cases). Results show, a decided correspondence, inside of the duos, between the level of reflectivity and referential score of the mother and that of the child besides the sharing of the main strategies used in order to manage the emotions and the defense mechanisms. Moreover, the duos with greater reflective and referential ability are characterized by the greater dynamics of illness elaboration and integration in their life history, lower affective super-investments and less defense mechanism. The reflective ability seems, therefore, to be a protecting factor for the psychological adaptation of the mother and the child. Regarding the affective symbolizations evoked from the disease in the patients of this research, in addition to a particularly marked collusion in the single duos, some symbolizations emerge that are shared with all the participants even they appear at various degree

Ifosfamide in pediatric solid tumors.

Phase II studies conducted in Europe and the USA on pediatric solid tumors have shown that ifosfamide, as a single agent, is an active drug against a variety of neoplasms - rhabdomyosarcoma (RMS), some non-RMS soft tissue sarcomas, Wilms' tumor, bone sarcomas and neuroblastoma. Furthermore, an increase in tumor response rate has been observed when ifosfamide has been used in combination with other drugs. The usual dose of ifosfamide varies from 1.8 to 3 g/m(2)/day for 2-5 days according to the different regimens. Some controversies still exist on the modality of drug administration and more precisely on the time of infusion, however in pediatric practice, short infusion (e.g. 3 h) is usually preferred because of the reduced neurotoxicity in comparison to lengthier administration (e.g. 24 h). Ifosfamide is currently included in the standard therapy of pediatric bone and soft tissue sarcomas. It is also used in a selected high-risk group of patients with Wilms' tumor, neuroblastoma and germ cell tumors. Copyright 2003 S. Karger AG, Base

Dapsone treatment in a girl with severe chronic thrombocytopenic pupura does it work? Don\u2019t touch it!

Dapsone has been shown to be effective in treating immune thrombocytopenic purpura (ITP) in adults. In children the experience is limitated. We describe our experience using dapsone in a female with refractory, symptomatic ITP who suffered intracranic haemorrhage and massive gastric bleeding. After treatment platelet counts was more than 100 x 103/\u3bcL, and reached 1000x103/\u3bcL. Discontinuation resulted in a rapid decrease in platelet counts, with severe intracranial haemorrhage (ICH). The recovery of dapsone led the platelets count to lower values. We suggest that treatment should not be discontinued in responders, at least in children with symptomatic ITP. Additional studies of dapsone in children are warranted

Prevalence and significance of BRAF alterations in a pediatric population with low-grade gliomas

Background Low-grade gliomas (LGG) are the most common brain tumours in children, represented by heterogeneous pathological entities. BRAF gene alterations have been recently identified as responsible of constitutive activation of the mitogen-activated protein kinase pathway (MAPK) and hence involved in the development of LGG in children. There is evidence that the BRAF V600E mutation is more common in supratentorial LGG while the KIAA1549:BRAF fusion in posterior fossa pilocytic astrocytoma (PA). Objective This case series describes the prevalence of BRAF alterations in LGG patients, trying to relate them to outcome. Methods Children aged 0\u201314 years, with a diagnosis of LGG, referred to a single neuro-oncologic centre, were retrospectively reviewed to analyze clinical and histopathological features related to BRAF alterations. Results A total of 35 patients were included (16 males, median age 85.5 \ub1 81.3 months). BRAF mutations were searched on 7/35 children (20 %) resulting positive in 5/7 (71 %). Two of them (40 %) showed the KIAA1549:BRAF fusion. They were both pilocytic astrocytomas located in posterior fossa: 1/2 (50 %) was totally resected, showing stable disease (SD) 1 year after surgey; the other was not surgically treatable and showed a progressive disease during chemotherapy. Three out of five (60 %) presented V600E mutation. All of them were supratentorial: 1 pilomyxoid astrocytoma, treated with two partial resections and subse- quent chemotherapy, was in SD 5 years after diagnosis; 1 diffuse pleomorphic xanthoastrocytoma underwent three partial resections and showed SD after 5 years of vemurafenib; 1 glioneuronal tumor was in SD after complete resection and radiotherapy. Conclusion Our data support the evidence that specific mutations of the BRAF pathway are related to site and histological subtype of brain tumors. This sample is too small to help supporting the hypothesis that these alterations have a prognostic impact. Extent of surgery and localization seem to be the most important prognostic factors