Implementation of a molecular tumor board at a regional level to improve access to targeted therapy.

Background: With the development of precision oncology, Molecular Tumor Boards (MTB) are developing in many institutions. However, the implementation of MTB in routine clinical practice has still not been thoroughly studied.

Material And Methods: Since the first drugs approved for targeted therapies, patient tumor samples were centralized to genomic testing platforms.

Prognostic and Predictive Factors of Ebola Virus Disease Outcome in Elderly People during the 2014 Outbreak in Guinea.

Elderly people occupy a prominent position in African societies; however, their potential linkage to high case fatality rate (CFR) in Ebola virus disease (EVD) was often overlooked. We describe the predictive factors for EVD lethality in the elderly. A total of 2,004 adults and 309 elderly patients with confirmed EVD were included in the analysis.

Ebola virus disease in children during the 2014-2015 epidemic in Guinea: a nationwide cohort study.

Unlabelled: The most recent epidemic of Ebola virus disease (EVD) has resulted in more than 11,000 deaths in West Africa. It has threatened child health in the affected countries, including Guinea. This nationwide retrospective cohort study included all children under 20 years of age with laboratory-confirmed EVD in Guinea during the 2014-2015 Ebola outbreak for analysis.

Factors associated with treatment lag in infantile spasms.

Aim: the aim of this study was to evaluate the conditions in which infantile spasms are diagnosed and their possible impact on the course of the disease.

Method: we carried out a retrospective study of the reasons for delayed treatment of infantile spasms (treatment lag) in western France over the period 1990-2003. A total of 156 infants, 87 male (55%) and 69 female (45%), with infantile spasms were identified, in 45 (29%) of whom the spasms were symptomatic.

Impact of age, leukocyte count and day 21-bone marrow response to chemotherapy on the long-term outcome of children with philadelphia chromosome-positive acute lymphoblastic leukemia in the pre-imatinib era: results of the FRALLE 93 study.

Background: We explored the heterogeneity of philadelphia chromosome-positive acute lymphoblastic leukemia (Ph1-ALL) in a study of the effect of early features on prognosis in children. Here we report the long-term results of the FRALLE 93 study conducted in the era before the use of tyrosine kinase inhibitors.

Methods: Between 1993 and 1999, 36 children with Ph1-ALL were enrolled into the FRALLE 93 protocol.

Childhood cancer survival in France, 1990-1999.

The aim of this study was to describe the overall survival after childhood cancer in France using follow-up data from regional population-based registries. The survival of children (aged under 15 years) diagnosed with a cancer during 1990-1999 was analysed. For all cancers, the survivals were, respectively, 90.

Five distinct biological processes and 14 differentially expressed genes characterize TEL/AML1-positive leukemia.

Background: The t(12;21)(p13;q22) translocation is found in 20 to 25% of cases of childhood B-lineage acute lymphoblastic leukemia (B-ALL). This rearrangement results in the fusion of ETV6 (TEL) and RUNX1 (AML1) genes and defines a relatively uniform category, although only some patients suffer very late relapse. TEL/AML1-positive patients are thus an interesting subgroup to study, and such studies should elucidate the biological processes underlying TEL/AML1 pathogenesis.

Late ovarian relapse of TEL/AML1 positive ALL confirming that TEL deletion is a secondary event in leukemogenesis.

We describe here a late extramedullary ovarian relapse in an 18-year-old female who was diagnosed with hypotetraploid cell acute lymphoblastic leukaemia (cALL) at the age of 6. At both occurrences of the disease cells were analyzed by morphology, immunophenotyping, cytogenetics and molecular methods. TEL/AML1 was detected by RT-PCR and FISH analysis in both events.

Intrafamilial variable phenotypic expression of a CIAS1 mutation: from Muckle-Wells to chronic infantile neurological cutaneous and articular syndrome.

Among hereditary inflammatory disorders, Muckle-Wells syndrome, chronic infantile neurological cutaneous and articular syndrome (CINCA), and familial cold urticaria have recently been shown to be caused by dominantly inherited mutations in the CIAS1 gene. Reports suggest that these 3 diseases result from distinct missense mutations, with very few overlapping symptoms. We describe a French family presenting an intrafamilial overlapping clinical phenotype of CINCA and Muckle-Wells syndrome, caused by a mutation in CIAS1 gene.

Particular cutaneous side effects with etoposide-containing courses: is VP16 or etoposide phosphate responsible?

Purpose: Etoposide is commercially available in France in two different pharmaceutical forms: VP16 and its phosphate ester (etoposide phosphate, EP). EP shows better chemical and physical properties, is said to be less toxic but is five times more expensive than VP16. Some criteria were defined for the use of each form in the Paediatric Oncohaematology Department in Hopital Sud in Rennes.

Cancer incidence among children in France, 1990-1999.

Background: Cancer is the second most important cause of death for children aged less than 15 years in France, unintentional injuries being the leading cause. The aim of the present study was to estimate the incidence of childhood cancer from six Childhood Cancer Registries covering 32% of France.

Procedure: Incident cancer cases diagnosed between 1990 and 1999 in children (0-14 years) resident in the administrative areas covered by each Registry were included.

Successful endoscopic drainage of a pancreatic pseudocyst in a 17-month-old boy.

Unlabelled: Pancreatic pseudocysts (PPCs) develop in 10% to 30% of children with acute pancreatitis. PPCs that impinge on neighbouring structures, cause pain, or fail to undergo spontaneous resolution must be drained. Surgery and percutaneous aspiration are the most widely used techniques for draining PPCs.

Impact of Topoisomerase II alpha and spermine on the clinical outcome of children with acute lymphoblastic leukemia.

It has been reported in the literature that a leukemic cell may be (or become) resistant to anti-cancer treatment because many mechanisms, such as efflux membrane pump (multi-drug resistance, MDR-P170), intracellular transport (LRP, MRP), or different detoxification systems (glutathione transferases, methallothioneines) may be implicated. Topoisomerase II alpha (TopoII) are also reported as responsible for resistance since their main action is to repair DNA breakage. Polyamines are described as having a protective DNA action by stabilizing the double stranded DNA helix.

Central neurotoxicity of cyclosporine in two children with nephrotic syndrome.

The central neurotoxicity of cyclosporin A (CsA) has been abundantly documented in pediatric and adult recipients of bone marrow or organ transplants, with variations in the rate of occurrence from 0.5% to 35%. We report two cases of central neurotoxicity ascribable to CsA in children with nephrotic syndrome due to lipoid nephrosis.