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MANCINI J., BAUMSTARCK-BARRAU K., SIMEONI M.-C., GROB J.-J., MICHEL G., TARPIN C., LOUNDOU A.-D., LAMBERT A., CLÉMENT A. & AUQUIER P. (2010) European Journal of Cancer Care To establish the best approach to develop a quality of life (QoL) questionnaire for cancer-patient caregivers, this study attempts to identify primary domains of QoL in terms of their impact on a purposive sample of caregivers. Seventy-seven informal adult caregivers of cancer patients (breast cancer, paediatric haematological malignancies or melanoma) with different relationships with the patients (parents, children, spouses, siblings, and friends) were recruited at three specialised French centres and extensively interviewed. Caregivers' lives were altered in several domains: psychological well-being, leisure and everyday activities, relationships with institutional caregivers, occupation and finances, relationships with family and friends, physical well-being, and relationship with the patient. The relative importance of these domains varied mainly in association with the caregiver-patient relationship. Multiple correspondence analysis identified two isolated clusters: children, and, most significantly, friends and siblings. The latter groups emphasised the repercussions on their psychological well-being and their relationship with the patient, but were less willing to discuss the impact on their relationship with caregivers and on occupation, finances, leisure, and everyday activities. This study focuses on the caregiver's perspective and advocates the development of a short QoL core questionnaire. Additional modules should be cancer-specific or dedicated to specifics of the caregiver-patient relationship. [ABSTRACT FROM AUTHOR]

Abstract: In 2007, the French National Cancer Institute initiated a cancer communication campaign entitled “We are two million ordinary heroes”. Aims of this study were to investigate perceptions about this campaign, for 34 women treated for breast cancer. Triangulation of the reports from narratives and assessment of psychological variables ensures better validity of the data. Three psychological identification processes have been identified: identification with the hero character with a value of emotional repression; adherence to content “ordinary” with low-level of perceived self-efficacy; no identification in response to a double-bind message, with sense-making process about traumatic event. These findings underscore the need to promote communication health campaigns taking into account possible effects of media discourse on individuals and families struggling with cancer. [Copyright &y& Elsevier]

In the framework of the HyTunnel-CS European project sponsored by Clean Hydrogen Joint Undertaking (CH JU), a number of tests were conducted in a full-scale tunnel in France. These tests are devoted to safety of hydrogen-fuelled vehicles having a compressed gas storage. The goal of the study is to develop recommendations for Regulations, Codes and Standards (RCS) for inherently safer use of hydrogen vehicles in enclosed transportation systems. Two sets of tests have been performed, (a) five tests with compressed hydrogen tanks, (b) two tests with compressed helium tanks. The hydrogen gas pressure varied between 47 bar and 610 bar. The blast wave overpressures are recorded together with fireball characteristics. The obtained experimental data are compared to existing engineering correlations and it is confirmed that not only the mechanical energy of compressed gas but also a fraction of chemical energy contribute to the blast wave strength. • Tank rupture tests are conducted in a full-scale tunnel. • Compressed helium tanks as well as compressed hydrogen tanks are opened with detonation belts. • Blast wave evolution with time and distance is analyzed and compared to available correlations. • Fireball evolution inside tunnel cross-section is filmed and analyzed. [ABSTRACT FROM AUTHOR]

Abstract: During recent decades, videogame playing has become one of the main leisure activities in children and adolescents. However, studies on video game addiction are still very few. In order to evaluate the difficulties induced by an excessive “consumption” of video games, the team of Dr. Tejeiro developed a self-rating questionnaire: the Problem Videogame Playing questionnaire (PVP, Tejeiro, 2002). We translated this questionnaire with the agreement of the author and carried out, then, a “back-translation”. Population: The sample consisted of 50 children (29 children with an Attention Deficit Hyperactivity Disorder [ADHD] and 21 controls). All subjects were evaluated on their consumption of videogames (PVP scale) and on their levels of externalized symptomatology by the Conners Parent rating scale and the Child Behavior Checklist (CBCL). Results: None the controls scored above four on the PVP scale whereas 10 hyperactive children among the 29 answered affirmatively to five or more questions (P =0.02). We focused on PVP scores, and we distinguished two sub-groups among the hyperactive population: children having a score equal to or higher than 5 (n =10, group 1) and those having a score lower than 5 (group 2). The subjects of group 1 presented a higher index of hyperactivity on the Conners Parents rating scale than group 2 (P =0.02); and obtained higher scores on the Child Behavior Checklist, on the delinquent subscale (P =0.03), the aggressive subscale (P =0.02) and on externalizing problems (P =0.01). Conclusion: The hyperactive children present more problems associated with video games. Children with high PVP scores appear with a particular behavioral clinical profile. This tool appears useful, for the clinician, in the description of the behaviours of children and teenagers towards video games. Also, it is necessary to use this questionnaire, in its French version, on larger populations. [Copyright &y& Elsevier]

Abstract: Aim of the study: The purpose of this study was to report in acute childhood idiopathic thrombocytopenic purpura (ITP) the current practices of French paediatric hematologists and to compare them to recent publications of American and British teams. Method: A questionnaire was sent online to the members of the French Society of Pediatric Hematology/Immunology (SHIP). This questionnaire, adapted from a similar american study conducted in 2001, asked 16 questions based on the clinical presentation of a 5-year-old boy referred for an acute ITP. Results: 59/123 SHIP members responded to the survey. In response to question regarding initial treatment, 86% of physicians would be given active treatments and only 9% would rarely or never administer any drug. When asked which agent would be used in case of treatment, 68% would choose to prescribe intravenous immunoglobulins and 32% corticosteroids, nobody recommended the use of anti-D immunoglobulins. Furthermore, 83% would usually hospitalize such a child. Conclusion: Finally, this study allowed us to update the current French management of treating pediatric ITP which is almost comparable among this subset of pediatric hematologists, but showed some discrepancies comparatively to the American and British studies. [Copyright &y& Elsevier]

Introduction: Muscle dysmorphia, a theme mainly investigated by Anglo-Saxon research, is a specific body dysmorphic disorder that characterizes individuals who think they are insufficiently muscular and lean. Understudied in francophone countries, tools translated and validated in French to measure the symptomatology of muscle dysmorphia remain rare., Objectives: The aim of this study was the translation into French and the psychometric validation of a tool evaluating muscle dysmorphia, the Muscle Dysmorphic Disorder Inventory (MDDI-Fr), through 13 items divided into three dimensions: the "Drive For Size" (DFS), the "Appearance Intolerance" (AI) and the "Functional Impairment" (FI)., Methodology: A first translation step was conducted, followed by two cross-sectional studies conducted in France. In both of the latter studies, a sociodemographic questionnaire and the Muscle Dysmorphic Disorder Inventory - French version (MDDI-Fr) were administered. The first study included 342 university students (Mage=20.9; SDage=2.9), and 1822 athletes for the second study (Mage=23.9; SDage=5.9). Validation was performed using internal consistency measures and confirmatory factor analysis., Results: The correlation matrices showed good significant results between items and dimensions. The three dimensions appeared distinct from each other with significant intra-dimension correlations. The internal consistency of the tool and its three dimensions show valid Cronbach's alphas (study 1: DFS=0.79, AI=0.74, FI=0.75, MDDI=0.74; study 2: DFS=0.72, AI=0.75, FI=0.83, MDDI=0.75). In addition, the tri-factor analysis shows significant and promising scores for the tool in three dimensions and 13 items for study 1 (χ 2 /df=4.67, CFI=0.85, TLI=0.83, RMSEA=0.10, SRMR=0.00), as for study 2 (χ 2 /df=16.08, CFI=0.87, TLI=0.84, RMSEA=0.09, SRMR=.06). Gender analyses were conducted on study 2, which showed that the DFS subscale (items 1, 4, 5) and the AI subscale (items 6 and 9) presented the greatest differences in factor loadings between genders., Conclusion: The translation, as well as the validation of the MDDI-Fr structured with 13 items and three dimensions, showed significantly satisfying results for its adaptation in French in a population of French men and women practicing weight training. However, the "Appearance Intolerance" dimension shows moderate links with the global tool, which should be investigated in future studies. Although the psychometric properties of the MDDI-Fr are promising, additional research is needed to gain a better understanding of gender differences in the results, especially for the DFS dimension., (Copyright © 2023 L'Encéphale, Paris. Published by Elsevier Masson SAS. All rights reserved.)

The prevalence of asymptomatic leishmaniasis in dogs and their owners in the main endemic areas of France has not been studied to date. The objective of this study was to quantify asymptomatic Leishmania infantum infection in southeast France in healthy people and their dogs using molecular and serological screening techniques. We examined the presence of parasitic DNA using specific PCR targeting kinetoplast DNA (kDNA) and specific antibodies by serology (ELISA for dogs and Western blot for humans) among immunocompetent residents and their dogs in the Alpes-Maritimes. Results from 343 humans and 607 dogs were included. 46.9% (n = 161/343) of humans and 18.3% (n = 111/607) of dogs were PCR positive; 40.2% of humans (n = 138/343) and 9.9% of dogs (n = 60/607) were serology positive. Altogether, 66.2% of humans (n = 227) and 25.7% of dogs (n = 156) had positive serologies and/or positive PCR test results. Short-haired dogs were more frequently infected (71.8%, n = 112) than long-haired dogs (12.2%, n = 19) (p = 0.043). Dogs seemed to be more susceptible to asymptomatic infection according to their breed types (higher infection rates in scenthounds, gun dogs and herding dogs) (p = 0.04). The highest proportion of dogs and human asymptomatic infections was found in the Vence Region, corresponding to 28.2% (n = 20/71) of dogs and 70.5% (n = 31/44) of humans (4.5/100,000 people). In conclusion, the percentage of infections in asymptomatic humans is higher than in asymptomatic dogs in the studied endemic area. It is questionable whether asymptomatic infection in humans constitutes a risk factor for dogs., (© M. Hide et al., published by EDP Sciences, 2024.)

Since the emergence of the SARS-CoV-2 infection, many recommendations have been made. However, the very specific nature of acute lymphoblastic leukemias and their treatment in children and adolescents led the Leukemia Committee of the French Society for the fight against Cancers and leukemias in children and adolescents (SFCE) to propose more specific recommendations. Here is the second version of these recommendations updated according to the evolution of knowledge on COVID19., (Copyright © 2021 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Our objectives were to assess the quality of life (QoL) of parents of childhood leukemia survivors compared with population norms and to identify the determinants of parents' long-term QoL., Methods: Parents of minors who had survived childhood leukemia participating in the French LEA cohort (Leucémie de l'Enfant et de l'Adolescent-French Childhood Cancer Survivor Study for Leukemia) were asked to complete the French version of the WHOQOL-BREF. Results were compared with age- and sex-matched values from a French reference population. Parents' and survivors' characteristics likely to be associated with QoL, long after the child's leukemia diagnosis, were explored using multivariate analysis., Results: We included 487 parents (mean age 42.9 ± 6.0 years, mean follow-up time from diagnosis 7.3 ± 3.3 years). Compared with the reference population, scores for physical health and social relationships for parents of childhood leukemia survivors were significantly lower (P < 0.001, effect size = 0.24 and P < 0.001, effect size = 0.29, respectively) contrary to scores for psychological health which were significantly higher (P < 0.001, effect size = 0.29). Even if health- and cancer-related characteristics were associated with parents' QoL in some dimensions, the only factor associated with each of the three dimensions (social relationships, physical health, and psychological) in the multivariate analysis was the parent's financial situation., Conclusions: Long after leukemia diagnosis, the parents reported lower scores in the physical health and social relationship domains. Despite the difficulties of actually influencing socioeconomic characteristics, it is important to consider the social situation of each family in the long-term care of survivors and their families., (© 2020 Wiley Periodicals, Inc.)

Childhood chronic immune thrombocytopenic purpura (cITP) is a rare disease. In severe cases, there is no evidence for the optimal therapeutic strategy. Our aim was to describe the real-life management of non-selected children with cITP at diagnosis. Since 2004, patients less than 18 years old with cITP have been enrolled in the national prospective cohort, OBS'CEREVANCE. From 1990 to 2014, in 29 centres, 392 children were diagnosed with cITP. With a median follow-up of six years (2·0-25), 45% did not need second-line therapy, and 55% (n = 217) received one or more second lines, mainly splenectomy (n = 108), hydroxychloroquine (n = 61), rituximab (n = 61) or azathioprine (n = 40). The overall five-year further second-line treatment-free survival was 56% [95% CI 49·5-64.1]. The use of splenectomy significantly decreased over time. Hydroxychloroquine was administered to children with positive antinuclear antibodies, more frequently older and girls, and reached 55% efficacy. None of the patients died. Ten years after the initial diagnosis, 55% of the 56 followed children had achieved complete remission. Children with cITP do not need second-line treatments in 45% of cases. Basing the treatment decision on the pathophysiological pathways is challenging, as illustrated by ITP patients with positive antinuclear antibodies treated with hydroxychloroquine., (© 2020 British Society for Haematology and John Wiley & Sons Ltd.)

Background: Hepatitis-associated aplastic anemia (AA) is a rare syndrome combining acute hepatitis of variable severity and AA. Hepatitis may be severe enough to require urgent liver transplantation (LT). Herein, we describe clinical presentation and management of a cohort of pediatric patients diagnosed with AA after undergoing LT for nonviral hepatitis., Methods: To describe this rare clinical situation, we performed a national survey and identified nine children treated for AA following LT during the last 10 years in France., Results: All patients were treated first for hepatic failure with urgent LT. AA was diagnosed with a median delay of 34 days [21-200] from the diagnosis of hepatitis. Seven children were treated with antithymocyte globulin/cyclosporine, one with CSA alone and one received bone marrow transplantation. At the last visit (median follow-up: 4 years), outcomes were excellent: all patients were alive and in hematological remission (complete remission: 7; partial remission: 2). Immunosuppressive therapy was pursued in all patients due to the liver transplant. No unusual toxicities were reported., Conclusion: AA after LT is considered a therapeutic challenge. Nevertheless, hematological outcome is good using a standard immunosuppressive approach., (© 2020 Wiley Periodicals, Inc.)

Introduction: In France, the management of pediatric cancers is carried out in reference hospitals that can delegate care to local health centres (LHC), forming "care networks". There is no LHC in Corsica, forcing children and their families to leave the island for all care in the reference centre. The aim of this study was to describe the situation in Corsica and to consider this organisation., Methods: This is a descriptive preliminary study based on databases from the National Childhood Cancer Registry, "RHeOP" network and a patient questionnaire. We included over a period of 10 years all children with onco-hematological disease who resided in Corsica., Results: The incidence of pediatric cancers since 2005 ranged from 5 to 12 new cases per year. The hospital centre of Timone (Marseille) was the reference centre for 73% of patients, followed by Nice University Hospital for 14%. Almost all the parents interviewed (90%) were in favour of creating an LHC and all of them highlighted many difficulties that, according to them, could be improved by the presence of a LHC in Corsica (organisation of travel, delay and distance from home…). However, there currently appears to be a lack of training for medical and paramedical staff to provide quality second-line care., Conclusion: The rates of pediatric onco-hematological diseases in Corsica may warrant the creation of an LHC on the island. Additional cost studies on the feasibility of an LHC in Corsica are needed to optimise the care and quality of life of these children and their families., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Binge drinking is a major public health problem associated with various negative short-term and long-term clinical and social outcomes. If there is evidence to suggest a relationship between ADHD and alcohol use in college students, no study has investigated the role of ADHD symptomatology in binge drinking. Thus, this research was designed to explore the relative contributions of inattention and hyperactivity/impulsivity symptoms to binge drinking in a sample of French college students while controlling for effects of perceived stress. Participants (N = 7011; mean age = 20.9; 74.9% of females) completed self-report surveys assessing ADHD symptoms, perceived stress, sociodemographic characteristics, and binge drinking frequency. Multinomial logistic regression revealed significant associations between higher levels of ADHD symptoms in general, but not perceived stress, and increasing frequency of binge drinking. Moreover, higher levels of inattention and hyperactivity/impulsivity symptoms were independently associated with greater frequency of binge drinking. The association was stronger between high rates of binge drinking and inattention than for hyperactivity/impulsivity. These findings, which remained statistically significant after adjusting for a range of potential confounders (including perceived stress), suggest that the presence of ADHD symptoms may be an important factor related to binge drinking., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Published by Elsevier B.V.)

The inauguration of a fitness path at the Carémeau University Hospital Campus was an opportunity for teams at the Nîmes University Hospital Centre to participate in an inter-service Nordic walking challenge. It was an opportunity to discover a particular sporting discipline, promote team cohesion in services and enjoy physical activity in a friendly atmosphere., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Purpose: Patients with primary immunodeficiency (PID) are at risk of serious complications. However, data on the incidence and causes of emergency hospital admissions are scarce. The primary objective of the present study was to describe emergency hospital admissions among patients with PID, with a view to identifying "at-risk" patient profiles., Methods: We performed a prospective observational 12-month multicenter study in France via the CEREDIH network of regional PID reference centers from November 2010 to October 2011. All patients with PIDs requiring emergency hospital admission were included., Results: A total of 200 admissions concerned 137 patients (73 adults and 64 children, 53% of whom had antibody deficiencies). Thirty admissions were reported for 16 hematopoietic stem cell transplantation recipients. When considering the 170 admissions of non-transplant patients, 149 (85%) were related to acute infections (respiratory tract infections and gastrointestinal tract infections in 72 (36%) and 34 (17%) of cases, respectively). Seventy-seven percent of the admissions occurred during winter or spring (December to May). The in-hospital mortality rate was 8.8% (12 patients); death was related to a severe infection in 11 cases (8%) and Epstein-Barr virus-induced lymphoma in 1 case. Patients with a central venous catheter (n = 19, 13.9%) were significantly more hospitalized for an infection (94.7%) than for a non-infectious reason (5.3%) (p = 0.04)., Conclusion: Our data showed that the annual incidence of emergency hospital admission among patients with PID is 3.4%. The leading cause of emergency hospital admission was an acute infection, and having a central venous catheter was associated with a significantly greater risk of admission for an infectious episode.

Background: Efforts to improve the quality of end-of-life (EOL) care depend on better knowledge of the care that children, adolescents, and young adults with cancer receive, including high-intensity EOL (HI-EOL) care. The objective was to assess the rates of HI-EOL care in this population and to determine patient- and hospital-related predictors of HI-EOL from the French national hospital database., Methods: This was a population-based, retrospective study of a cohort of patients aged 0 to 25 years at the time of death who died at hospital as a result of cancer in France between 2014 and 2016. The primary outcome was HI-EOL care, defined as the occurrence of ≥1 chemotherapy session <14 days from death, receiving care in an intensive care unit ≥1 time, >1 emergency room admission, and >1 hospitalization in an acute care unit in the last 30 days of life., Results: The study included 1899 individuals from 345 hospitals; 61.4% experienced HI-EOL care. HI-EOL was increased with social disadvantage (adjusted odds ratio [AOR], 1.30; 95% confidence interval [CI], 1.03-1.65; P = .028), hematological malignancies (AOR, 2.09; 95% CI, 1.57-2.77; P < .001), complex chronic conditions (AOR, 1.60; 95% CI, 1.23-2.09; P = .001) and care delivered in a specialty center (AOR, 1.70; 95% CI, 1.22-2.36; P = .001). HI-EOL was reduced in cases of palliative care (AOR, 0.31; 95% CI, 0.24-0.41; P < .001)., Conclusion: A majority of children, adolescents, and young adults experience HI-EOL care. Several features (eg, social disadvantage, cancer diagnosis, complex chronic conditions, and specialty center care) were associated with HI-EOL care. These findings should now be discussed with patients, families, and professionals to define the optimal EOL., (© 2019 American Cancer Society.)

Objectives: The aim of the study was to estimate the association between self-esteem and subsequent self-rated health during college years, taking into account a wide range of potential confounders., Design: Prospective longitudinal study., Setting: The French i-Share cohort., Participants: The sample consisted of 1011 college students., Primary and Secondary Outcome Measures: The association between self-esteem and later self-rated health was evaluated using multivariate modelling.Data regarding self-rated health, global self-esteem and demographic, educational, social, behavioural, environmental and financial characteristics were collected through an internet-based questionnaire., Results: The 1011 participants had a median age of 21.9 years and 79% (795/1011) were females. Self-rated health was assessed a median of 8 months after the self-esteem measurement. Twenty per cent of the students declared average to very poor health (203/1011). Students with higher levels of self-esteem were more likely to declare good or very good self-rated health (adjusted OR=1.40, 95% CI 1.15 to 1.72, p value=0.001). Other factors associated with good or very good self-rated health were low body mass index, a comfortable financial situation during childhood and three personality traits (low persistence and harm avoidance and high cooperativeness)., Conclusions: This study offers novel findings on the impact of self-esteem on self-rated health among college students. Interventions targeting self-esteem should be experimented during university years in order to improve health outcomes., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Binge eating disorder (BED) is associated with higher psychopathology, including emotional and personality disorders, in the adult population, whether or not they are obese; although few data are available on adolescents, particularly among obese adolescents., Objective: To explore the association of both emotional disorders and personality dimensions with BED in obese adolescents., Methods: The sample consisted of 115 French adolescents enrolled at a clinical unit for the multidisciplinary care of their overweight or obesity. BED was defined using the Binge Eating Scale (BES). Emotional disorders and personality dimensions were assessed using the following tools: Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Junior Temperament and Character Inventory (JTCI); impulsivity was determined by the Barratt Impulsiveness Scale (BIS) and the Toronto Alexithymia Scale (TAS). Associations between emotional disorders (BDI/BAI) and personality dimensions (TAS/BIS/JTCI), considered first separately and then jointly with BED were determined with multivariate analysis., Results: More severe depression (β=0.27, CI [0.06; 0.48], P=0.011), a higher level of excess weight (β=1.91, CI [0.22; 3.59], P=0.027), older age (β=1.28, CI [0.43; 2.14], P=0.003), and greater cooperativeness (β=0.36, CI [0.07; 0.66], P=0.017) were independently significantly associated with the presence of BED., Conclusions: This cross-sectional study underlines the co-occurrence of emotional and personality disorders with BED. This points out the importance of a multidisciplinary approach and the relevance of a joint diagnosis of binge eating, emotional disorders, and personality dimensions in obese adolescents, for better prevention and treatment of pediatric obesity., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

The present review lays out the main principles governing outpatient management in the French health system in 2018, and more specifically in plastic and reconstructive head and neck surgery. The historical regulatory aspects and the changes of the last few years are explained, along with the trends and health authorities' expectations for the years to come. The main limitations to implementing outpatient procedures are the common to all surgical specialities, plastic and reconstructive head and neck surgery being no exception. Apart from purely technical aspects concerning surgical procedure and anesthesia, there are issues concerning institutional approval, the organization and continuity of health care, and the patient's environment. The French General Inspectorate of Social Affairs (IGAS), in its 2012 report on the assessment and pricing of hospital care and medical acts, stated that outpatient surgery was becoming standard practice and conventional admission a fall-back, with the aim of meeting the requirement to provide more care without more expenditure. Outpatient plastic and reconstructive head and neck surgery may be available for most patients, but still presupposes certain conditions., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Objective: To evaluate the association between medical and social environmental factors and the risk of repeating a grade in childhood leukemia survivors., Study Design: A cross-sectional study of childhood leukemia survivors, recruited through the LEA cohort (Leucémie de l'Enfant et de l'Adolescent [French Childhood Cancer Survivor Study for Leukemia]) in 2014. An adjusted logistic regression model was used to identify variables linked to repeating a grade after the diagnosis among the survivors, and the rates of repeating a grade were compared between the survivors and their siblings using a multilevel logistic regression model., Results: The mean age at inclusion of the 855 participants was 16.2 ± 7.0 years, and the mean duration of follow-up from diagnosis to evaluation was 10.2 ± 6.2 years. After disease onset, 244 patients (28.5%) repeated a grade, with a median interval of 4 years (IQR, 2-8 years). Independent factors associated with repeating a grade were male sex (OR, 1.78; 95% CI, 1.21-2.60), adolescence (OR, 2.70; 95% CI, 1.63-4.48), educational support during the treatment period (OR, 3.79; 95% CI, 2.45-5.88), low parental education level (OR, 2.493; 95% CI, 1.657-3.750), and household financial difficulties (OR, 2.62; 95% CI, 1.607-4.28). Compared with siblings, survivors were at greater risk of repeating a grade (OR, 1.87; 95% CI, 1.48-2.35)., Conclusions: The most vulnerable patients seemed to be adolescents and those with parents of low socioeconomic status. Improving the schooling career of leukemia survivors will require that the medical community more carefully consider the social status of patients., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Previous studies on the putative role of allergy in the aetiology of childhood leukaemia have reported contradictory results. The present study aimed to analyse the relation between a medical history of asthma or eczema and childhood acute lymphoid leukaemia (ALL) in light of potential candidate gene-environment interactions., Methods: Analyses were based on a subset of 434 cases of ALL and 442 controls successfully genotyped and of European ancestry children enrolled in a French population-based case-control study conducted in 2003-2004. Information about medical history was obtained during a standardized interview with the mothers. Candidate polymorphisms in genes of the Th2 cytokines IL4, IL10, IL13 and IL4-receptor, were genotyped or imputed., Results: None of the variant alleles were directly associated with childhood acute lymphoid leukaemia. A medical history of asthma or eczema was reported more often in the control group (OR = 0.7 [0.5-1.0]). This association was mostly seen in the group of children not carrying the IL13-rs20541 variant allele (Interaction Odds Ratio IOR 1.9, p-interaction = 0.07) and in those carrying the IL10 triple variant haplotype (IOR 0.5, p-interaction = 0.04). No interaction was observed with the candidate polymorphisms in IL4 and IL4R., Conclusion: This study provides a new insight into the relationship between allergic symptoms and childhood acute lymphoid leukaemia, by suggesting this inverse association could be limited to children carrying certain genetic polymorphisms. If confirmed, these results could help better understand the biological mechanisms involved in the development of childhood acute lymphoid leukaemia., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

In this retrospective study, we evaluate long-term complications in nearly all β-thalassemia-major patients who successfully received allogeneic hematopoietic stem cell transplantation in France. Ninety-nine patients were analyzed with a median age of 5.9 years at transplantation. The median duration of clinical follow up was 12 years. All conditioning regimens were myeloablative, most were based on busulfan combined with cyclophosphamide, and more than 90% of patients underwent a transplant from a matched sibling donor. After transplantation, 11% of patients developed thyroid dysfunction, 5% diabetes, and 2% heart failure. Hypogonadism was present in 56% of females and 14% of males. Female patients who went on to normal puberty after transplant were significantly younger at transplantation than those who experienced delayed puberty (median age 2.5 vs 8.7 years). Fertility was preserved in 9 of 27 females aged 20 years or older and 2 other patients became pregnant following oocyte donation. In addition to patient's age and higher serum ferritin levels at transplantation, time elapsed since transplant was significantly associated with decreased height growth in multivariate analysis. Weight growth increased after transplantation particularly in females, 36% of adults being overweight at last evaluation. A comprehensive long-term monitoring, especially of endocrine late effects, is required after hematopoietic stem cell transplantation for thalassemia., (Copyright© 2018 Ferrata Storti Foundation.)

Purpose: To identify spectral-domain optical coherence tomography (SD-OCT) predictive morphological features for the outcome of Ranibizumab therapy for neovascular age-related macular degeneration (AMD)., Methods: This is a retrospective multicentric study that involved 64 eyes with naïve AMD. Patients who received three monthly intravitreal injections of Ranibizumab were stratified into (1) "responders" [≥ 5 letters gain on Early Treatment Diabetic Retinopathy Study (ETDRS) scale] and (2) "nonresponders" (< 5 letters gain). Best-corrected visual acuity (BCVA) and SD-OCT morphological features were compared at baseline and one month after three consecutive injections of Ranibizumab. Univariate and multivariate analyses were carried out to correlate these morphological features with the change in BCVA., Results: Among the 64 patients enrolled, 40 (62.5%) were "responders" and 24 (37.5%) "nonresponders". Age, sex, and BCVA were comparable between both groups. A multivariate correlational analysis found that subfoveal choroidal thickness (SFCT) and the presence of pigment epithelial detachment (PED) > 250 μ m at baseline were two independent prognostic indicators of final BCVA. No other SD-OCT morphological studied features seem to affect final BCVA after Ranibizumab treatment., Conclusion: SFCT and the presence of PED > 250 μ m are two significant biomarkers that may predict improvement after Ranibizumab therapy for AMD. These markers may guide ophthalmologists' treatment decision under financial constraints and limited time.

The prevalence of the metabolic syndrome among adults from the French LEA childhood acute leukemia survivors' cohort was prospectively evaluated considering the type of anti-leukemic treatment received, and compared with that of controls. The metabolic profile of these patients was compared with that of controls. A total of 3203 patients from a French volunteer cohort were age- and sex-matched 3:1 to 1025 leukemia survivors (in both cohorts, mean age: 24.4 years; females: 51%). Metabolic syndrome was defined according to the National Cholesterol Education Program's Adult Treatment Panel III criteria. Metabolic syndrome was found in 10.3% of patients (mean follow-up duration: 16.3±0.2 years) and 4.5% of controls, (OR=2.49; P <0.001). Patients transplanted with total body irradiation presented the highest risk (OR=6.26; P <0.001); the other treatment groups also showed a higher risk than controls, including patients treated with chemotherapy only. Odd Ratios were 1.68 ( P =0.005) after chemotherapy only, 2.32 ( P =0.002) after chemotherapy and cranial irradiation, and 2.18 ( P =0.057) in patients transplanted without irradiation. Total body irradiation recipients with metabolic syndrome displayed a unique profile compared with controls: smaller waist circumference (91 vs 99.6 cm; P =0.01), and increased triglyceride levels (3.99 vs 1.5 mmol/L; P <0.001), fasting glucose levels (6.2 vs 5.6 mmol/L; P =0.049), and systolic blood pressure (137.9 vs 132.8 mmHg; P =0.005). By contrast, cranial irradiation recipients with metabolic syndrome had a larger waist circumference (109 vs 99.6 cm; P =0.007) than controls. Regardless of the anti-leukemic treatment, metabolic syndrome risk was higher among childhood leukemia survivors. Its presentation differed depending on the treatment type, thus suggesting a divergent pathophysiology. This study is registered at clinicaltrials.gov identifier: 01756599 ., (Copyright© 2018 Ferrata Storti Foundation.)

Objective: To gain insight into how primary immunodeficiencies (PIDs) affect children's health status and quality of life., Study Design: The French Reference Center for PIDs conducted a prospective multicenter cohort that enrolled participants who met all criteria: patients included in the French Reference Center for PIDs registry, children younger than18 years, and living in France. Participants were asked to complete both a health questionnaire and a health-related quality of life (HR-QoL) questionnaire. A severity score was assigned to each health condition: grade 1 (mild) to grade 4 (life-threatening). HR-QoL in children was compared with age- and sex-matched French norms., Results: Among 1047 eligible children, 656 were included in the study, and 117 had undergone hematopoietic stem cell transplantation; 40% experienced at least one grade 4 condition, and 83% experienced at least one grade 3 or 4 condition. Compared with the French norms, children with PID scored significantly lower for most HR-QoL domains. Low HR-QoL scores were associated strongly with burden of poor conditions., Conclusions: Our results quantify the magnitude of conditions in children with PID and demonstrate that the deleterious health effects borne by patients already are evident in childhood. These results emphasize the need to closely monitor this vulnerable population and establish multidisciplinary healthcare teams from childhood., Trial Registration: ClinicalTrials.gov: NCT02868333 and EudraCT 2012-A0033-35., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Although survival from childhood cancer has increased, little is known on the long-term impact of treatment late effects on occupational attainment or work ability., Methods: A total of 3512 five-year survivors treated before the age of 19 years in 10 French cancer centres between 1948 and 2000 were identified. Educational level, employment status and occupational class of survivors were assessed by a self-reported questionnaire. These outcome measures were compared with sex-age rates recorded in the French population, using indirect standardisation. Paternal occupational class was also considered to control for the role of survivors' socioeconomic background on their achievement. Multivariable analyses were conducted to explore clinical characteristics associated with the outcomes., Results: A total of 2406 survivors responded to the questionnaire and survivors aged below 25 years were included in the current analysis. Compared with national statistics adjusted on age and sex, male survivors were more likely to be college graduates (39.2% vs 30.9% expected; P<0.001). This higher achievement was not observed either for leukaemia or central nervous system (CNS) tumour survivors. Health-related unemployment was higher for survivors of CNS tumour (28.1% vs 4.3%; P<0.001) but not for survivors of other diagnoses. Survivors of non-CNS childhood cancer had a similar or a higher occupational class than expected., Conclusions: Survivors treated for CNS tumour or leukaemia, especially when treatment included cranial irradiation, might need support throughout their lifespan.

In the south of France, Leishmania infantum is responsible for numerous cases of canine leishmaniasis (CanL), sporadic cases of human visceral leishmaniasis (VL) and rare cases of cutaneous and muco-cutaneous leishmaniasis (CL and MCL, respectively). Several endemic areas have been clearly identified in the south of France including the Pyrénées-Orientales, Cévennes (CE), Provence (P), Alpes-Maritimes (AM) and Corsica (CO). Within these endemic areas, the two cities of Nice (AM) and Marseille (P), which are located 150 km apart, and their surroundings, concentrate the greatest number of French autochthonous leishmaniasis cases. In this study, 270 L. infantum isolates from an extended time period (1978-2011) from four endemic areas, AM, P, CE and CO, were assessed using Multi-Locus Microsatellite Typing (MLMT). MLMT revealed a total of 121 different genotypes with 91 unique genotypes and 30 repeated genotypes. Substantial genetic diversity was found with a strong genetic differentiation between the Leishmania populations from AM and P. However, exchanges were observed between these two endemic areas in which it seems that strains spread from AM to P. The genetic differentiations in these areas suggest strong epidemiological structuring. A model-based analysis using STRUCTURE revealed two main populations: population A (consisting of samples primarily from the P and AM endemic areas with MON-1 and non-MON-1 strains) and population B consisting of only MON-1 strains essentially from the AM endemic area. For four patients, we observed several isolates from different biological samples which provided insight into disease relapse and re-infection. These findings shed light on the transmission dynamics of parasites in humans. However, further data are required to confirm this hypothesis based on a limited sample set. This study represents the most extensive population analysis of L. infantum strains using MLMT conducted in France.

Objective: To determine presenting features, management and prognosis in extranodal non-Hodgkin lymphoma of the sinonasal tract., Material and Methods: A retrospective study between 2004 and 2013 in the University Hospital Center of Nantes (France) recruited patients with lymphoma discovered by sinonasal involvement. Epidemiologic, diagnostic, clinical and prognostic criteria were analyzed, with survival studied on the Kaplan-Meier estimator and Log-rank test., Results: Twenty-two patients were included: 14 male, 7 female, with a mean age of 65 years at diagnosis. All had non-Hodgkin lymphoma, with strong predominance of diffuse large B-cell lymphoma (77%). Seven patients had risk factors for lymphoma (infection by HIV, EBV or chronic lymphocytic leukemia). A majority (68%) had advanced tumor at diagnosis (stage IV on the Ann Arbor classification). Most were located in the craniofacial bones (68%), mainly involving the maxillary or ethmoidal sinuses. The most frequent presenting symptoms were unilateral nasal obstruction, mucopurulent rhinorrhea, recurrent epistaxis or diplopia. Treatment consisted in chemotherapy, in some cases associated to radiotherapy. Overall survival was 82% at 12 months and 73% at 36 months. Recurrence-free survival was 76% at 12 months and 64% at 36 months., Conclusion: Lymphoma is an aggressive pathology; revelation by sinonasal involvement is rare. Recommended treatment is chemotherapy, possibly associated to radiotherapy. Prognosis depends on histologic type, Ann Arbor stage at diagnosis and the therapeutic options available for the individual patient., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Introduction: The objectives of the present study are to describe gender differences in factors associated with moderate risk and problem gambling., Method: Data were extracted from the 2010 Health Barometer, a large survey on a representative sample of the general population aged 15-85 years living in France (n=27,653), carried out by the National Institute for Health Promotion and Health Education. Data were collected between October 2009 and July 2010. A computer-assisted telephone interview system was used., Results: The findings indicate that men are three times more likely to experience problems with gambling. Men and women have different patterns of gambling activities. Men were more involved with Rapido, internet gambling, sports and racetrack betting, poker, and casino tables, whereas women gambled more often on scratch games. Both men and women engaging in immediate reward games were significantly more likely to experience difficulties with gambling. This association, however, was stronger in women. Furthermore, suicidal ideation and behaviors were more likely to be associated with gambling problems in women as compared to men., Conclusions: The study underscores the importance of considering gender-related differences in the study of gambling behaviors., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Background: Factors related to early stimulation of the immune system (breastfeeding, proxies for exposure to infectious agents, normal delivery, and exposure to animals in early life) have been suggested to decrease the risk of childhood acute lymphoblastic leukaemia (ALL)., Methods: The national registry-based case-control study, ESTELLE, was carried out in France in 2010-2011. Population controls were frequency matched with cases on age and gender. The participation rates were 93% for cases and 86% for controls. Data were obtained from structured telephone questionnaires administered to mothers. Odds ratios (OR) were estimated using unconditional regression models adjusted for age, gender, and potential confounders., Results: In all, 617 ALL and 1225 controls aged ⩾1 year were included. Inverse associations between ALL and early common infections (OR=0.8, 95% confidence interval (CI): 0.6, 1.0), non-first born (⩾3 vs 1; OR=0.7, 95% CI: 0.5, 1.0), attendance of a day-care centre before age 1 year (OR=0.7, 95% CI: 0.5, 1.0), breastfeeding (OR=0.8, 95% CI: 0.7, 1.0), and regular contact with pets (OR=0.8, 95% CI: 0.7, 1.0) in infancy were observed., Conclusions: The results support the hypothesis that conditions promoting the maturation of the immune system in infancy have a protective role with respect to ALL.

In this report, we address the issue of late-effects after allogeneic stem cell transplantation in children. In an effort to harmonize clinical practices between different French transplantation centers, the French Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC) set up the fourth annual series of workshops which brought together practitioners from all member centers and took place in September 2013 in Lille., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Allogeneic hematopoietic SCT (HSCT) appears to be an efficient tool to cure high-risk AML in first CR but the choice between BU-based or TBI-based conditioning regimens still remains controversial. In order to analyze the impact of conditioning regimen on long-term survival, we conducted a retrospective analysis from French registry data including all consecutive patients under 18 years old (n=226) from 1980 to 2004 transplanted for AML in CR1 from sibling (n=142) or matched unrelated donors and given either TBI-1200 cGy and CY 120 mg/kg (TBI-Cy, n=84) or BU 16 mg/kg and CY 200 mg/kg (BuCy200, n=142). Patient subgroups were comparable for all criteria except for median age at diagnosis and HSCT and for donor type. Both 5-year OS and disease-free survival (DFS) were significantly better in BuCy200 group (P=0.02 and 0.005, respectively). In multivariate analysis, both HLA matching and BuCy200 appeared as good prognostic factors for treatment-related mortality and DFS. Grade 2-4 acute GvHD and chronic GvHD rates were statistically higher in TBI-Cy group than in Bu-Cy200 one with a RR at 2 (P=0.002). In total, Bu-Cy200 conditioning regimen gives better outcome compared with TBI-Cy irrespective of the stem cell source and the donor type.

Objectives: To estimate the incidence of suicide attempts during imprisonment in a French prison and to determine factors associated with them., Methods: All male inmates of a prison for prisoners awaiting trial or serving a short sentence (Bordeaux, France) were eligible for this cross-sectional study. They were face-to-face interviewed by an experienced psychologist (n = 369; mean age = 36 years). Socio-demographic data, imprisonment conditions, health status, healthcare utilization, mental health, impulsiveness, inmate-to-inmate violence (victim and perpetrator status) and suicidal behaviours were documented., Results: The incidence of suicidal attempts during imprisonment was 13.4 per 100 person-years. Having made at least one suicide attempt during imprisonment was associated with: being a victim of physical or sexual violence without perpetrating it (adjusted OR = 5.4; 95%CI [2.4-12]), suffering from depressive and anxious symptoms (adjusted OR = 3.3; 95%CI [1.5-7.7]), having children (adjusted OR = 3.0; 95%CI [1.2-7.7]) and having a poor perceived health status (OR = 2.5; 95%CI [1.1-5.4]). A history of suicide attempt before imprisonment was not associated with the risk of suicide attempts while imprisoned., Conclusion: Our results suggest that violence plays a major role in the suicidal process in prison. The inclusion of inmate-to-inmate violence in the screening checklists of inmate suicide risk should be explored., (Copyright © 2013 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.)

We report French prospective experience with reduced-intensity conditioning-allo-SCT in 46 patients (median age: 15.5 years, 4.8-20.2) presenting high-risk AL (n=11), Hodgkin's lymphoma (n=15) or solid tumors (n=20). Graft sources were BM (n=21), PBSC (n=20) and cord blood (CB; n=5) from related (n=20) or unrelated (n=26) donors. For CB grafts, only one patient out of five achieved sustained engraftment. For PBSC/BM grafts, engraftment rate was 95%, hematopoietic recovery times were not significantly different between BM, PBSC, sibling or unrelated grafts, day+100. Full donor chimerism was achieved in 94% of patients, and incidences of primary acute GVHD and chronic GVHD were 49% and 14%, respectively. Underlying disease was fatal in 39% of patients. TRM was 6.9%. Three-year OS was 49.15%. OS and EFS were not significantly different between patients transplanted with different grafts and with or without primary GVHD. Patients with solid tumor or measurable disease at transplant had poorer outcomes. Three-year EFS: 33.3% for ALL, 75.0% for AML, 51.8% for Hodgkin's lymphoma, 28.6% for neuroblastoma and 22.2% for sarcoma patients. This multicentre study concluded that Bu/fludarabine/anti-thymocyte globulin conditioning with PB or BM, related or unrelated grafts in patients with various malignancies at high-risk for transplantation toxicity results in high engraftment rates, low TRM and acceptable survival.

In the attempt to harmonize clinical practices between different French transplantation centers, the French Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC) set up the third annual series of workshops which brought together practitioners from all member centers and took place in October 2012 in Lille. Here we report our results and recommendations regarding the choice of optimal unrelated cord blood unit in terms of cell dose, HLA-matching and other characteristics., (Copyright © 2013. Published by Elsevier SAS.)

The late effects and quality of life (QoL) in childhood acute leukemia survivors were compared between hematopoietic stem cell transplantation (HSCT) recipients and patients who underwent conventional therapy. The study included 943 patients, 256 of whom underwent HSCT (27.1%). Medical visits were conducted to detect the occurrence of physical late effects. Based on patient age, different questionnaires were used to assess QoL. To evaluate the association between HSCT and each type of late effect or QoL dimension, the appropriate multivariate regressions were performed. QoL mean scores were compared with those obtained for age- and sex-matched French control subjects. Of all the survivors, 674 (71.5%) had at least 1 late effect, with the risk being 5.0 CI95 (3.0-8.6) times higher for transplantation survivors. For child survivors, scoring of QoL showed no significant differences between the treatment groups. The adult HSCT survivors reported lower physical dimension QoL scores than chemotherapy survivors. Compared with French norms, the survivor group reported a significantly lower mental composite score; however, the physical composite score showed no significant difference. Thus, transplanted survivors have a high risk of developing late effects, resulting in a decreased physical well-being in adulthood. However, long after treatment completion, childhood leukemia survivors report that effects on psychological well-being are more important than they are in physical QoL dimensions., (Copyright © 2013 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.)

Purpose: This study aimed to analyze the associations between childhood acute leukemia (AL) and maternal caffeinated beverage consumption during pregnancy, and to explore interactions between caffeinated and alcoholic beverage consumption and polymorphisms of enzymes involved in caffeine and ethanol metabolisms., Methods: The data were generated by the French ESCALE study, which included 764 AL cases and 1,681 controls in 2003-2004. The case and control mothers were interviewed on their consumption habits during pregnancy using a standardized questionnaire. Genotypes of the candidate alleles (NAT2*5 rs1801280, ADH1C*2 rs698 and rs1693482, CYP2E1*5 rs2031920 and rs3813867) were obtained using high-throughput genotyping and imputation data for 493 AL cases and 549 controls with at least two grandparents born in Europe., Results: Maternal regular coffee consumption during pregnancy was associated with childhood AL (OR = 1.2 [1.0-1.5], p = 0.02); the odds ratios increased linearly with daily intake (p for trend <0.001; >2 cups per day vs. no or less than 1 cup per week: AL: OR = 1.6 [1.2-2.1], lymphoblastic AL: OR = 1.5 [1.1-2.0], myeloblastic AL: OR = 2.4 [1.3-4.3]). The association was slightly more marked for children born to non-smoking mothers. Lymphoblastic AL was also associated with cola soda drinking (OR = 1.3 [1.0-1.5], p = 0.02). No significant gene-environment interactions with coffee, tea, cola soda, or alcohol drinking were observed., Conclusion: This study provides additional evidence that maternal coffee consumption during pregnancy may be associated with childhood AL. Coffee consumption is a prevalent habit and its potential involvement in childhood AL needs to be considered further.

Although hematopoietic stem cell transplantation (HSCT) offers curative potential for beta-thalassemia major (beta-TM), it is associated with a variable but significant incidence of graft rejection. We studied the French national experience for improvement over time and the potential benefit of antithymocyte globulin (ATG). Between December 1985 and December 2007, 108 patients with beta-TM underwent HSCT in 21 different French transplantation centers. The majority of patients received a matched sibling transplant (n = 96) and a busulfan- and cyclophosphamide-based conditioning regimen (n = 95), also with ATG in 57 cases. Ninety-five of the 108 patients survived, with a median follow-up of 12 years. Probabilities of 15-year survival and thalassemia-free survival after first HSCT were 86.8% and 69.4%, respectively. Graft failure occurred in 24 patients, 11 of whom underwent a second HSCT. The use of ATG was associated with a decrease in rejection rate from 35% to 10%. Thalassemia-free survival improved significantly with time, reaching 83% in the 54 patients undergoing HSCT after 1994 (median time of HSCT). In view of the increased risk of graft rejection after matched sibling HSCT, current French national guidelines recommend, for all children at risk for beta-TM, the systematic addition of ATG to the myeloablative conditioning regimen and special attention to optimize transfusion and chelation therapy in the pretransplantation period., (Copyright © 2013 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Patients with the Shwachman-Diamond syndrome often develop hematologic complications. No risk factors for these complications have so far been identified. The aim of this study was to classify the hematologic complications occurring in patients with Shwachman-Diamond syndrome and to investigate the risk factors for these complications., Design and Methods: One hundred and two patients with Shwachman-Diamond syndrome, with a median follow-up of 11.6 years, were studied. Major hematologic complications were considered in the case of definitive severe cytopenia (i.e. anemia <7 g/dL or thrombocytopenia <20 × 10(9)/L), classified as malignant (myelodysplasia/leukemia) according to the 2008 World Health Organization classification or as non-malignant., Results: Severe cytopenia was observed in 21 patients and classified as malignant severe cytopenia (n=9), non-malignant severe cytopenia (n=9) and malignant severe cytopenia preceded by non-malignant severe cytopenia (n=3). The 20-year cumulative risk of severe cytopenia was 24.3% (95% confidence interval: 15.3%-38.5%). Young age at first symptoms (<3 months) and low hematologic parameters both at diagnosis of the disease and during the follow-up were associated with severe hematologic complications (P<0.001). Fifteen novel SBDS mutations were identified. Genotype analysis showed no discernible prognostic value. CONCLUSIONS Patients with Shwachman-Diamond syndrome with very early symptoms or cytopenia at diagnosis (even mild anemia or thrombocytopenia) should be considered at a high risk of severe hematologic complications, malignant or non-malignant. Transient severe cytopenia or an indolent cytogenetic clone had no deleterious value.

There is little data available regarding children and adolescents with Hodgkin lymphoma (HL) who relapse after combined-modality treatment, even though they have a substantial chance of cure. The purpose of this national retrospective study was to evaluate the outcome of patients with recurrent/refractory HL and determine adverse prognostic factors. From 1990 to 2006, 70 patients (median age 13·9 years) with refractory (n = 31) or first relapse (n = 39) HL were identified. Median time from end of treatment to relapse was 6 months (3-56). Relapses occurred in irradiated areas in 43/70 patients. Salvage therapy consisted of chemotherapy and 50 patients received high-dose chemotherapy with autologous stem cell transplantation. Radiotherapy was performed in 29 cases, tandem autologous transplantation in five and allograft in three. With a median follow-up of 40 months (2-140), significant prognostic factors were time to progression/relapse and response to therapy before autograft. Event-free survival and overall survival in patients with refractory disease, early relapse and late relapse were 35 ± 9%, 67 ± 11%, 76 ± 10% and 48 ± 11%, 89 ± 7% and 80 ± 10%, respectively. As progression <3 months was a major adverse prognostic factor, novel therapeutic approaches are needed for this group of patients. By contrast, patients have substantial chance of long term second remission in case of relapse >3 months., (© 2012 Blackwell Publishing Ltd.)

Background: Attention-deficit hyperactivity disorder (ADHD) has been associated with socioeconomic difficulties later in life. Little research in this area has been based on longitudinal and community studies., Aims: To examine the relationship between childhood attention problems and socioeconomic status 18 years later., Method: Using a French community sample of 1103 youths followed from 1991 to 2009, we tested associations between childhood attention problems and socioeconomic status between ages 22 and 35 years, adjusting for potential childhood and family confounders., Results: Individuals with high levels of childhood attention problems were three times more likely to experience subsequent socioeconomic disadvantage than those with low levels of attention problems (odds ratio 3.44, 95% CI 1.72-6.92). This association remained statistically significant even after adjusting for childhood externalising problems, low family income, parental divorce and parental alcohol problems., Conclusions: This longitudinal community-based study shows an association between childhood attention problems and socioeconomic disadvantage in adulthood. Taking into account ADHD and associated difficulties could help reduce the long-term socioeconomic burden of the disorder.

Since several years, the use of intravenous immunoglobulins (IVIg) has increased. This growth has encouraged some countries to publish guidelines. In parallel, some countries have conducted audits to know how IVIg are used in clinical practice in the light of the available guidelines. The objective of this study was to assess IVIg use in three French university hospitals in 2006. All IVIg administrations were evaluated during 6 months (12 September 2005-12 March 2006) in French university hospitals of Marseille. Different data were recorded for each administration: patient characteristics, indication, formulation and quantity. During the study period, 2802 administrations of IVIg (corresponding to a total quantity of 76 780 g) have been recorded. Four hundred and thirty-five patients received at least one of these administrations. The five most reported indications were multifocal motor neuropathy (11.0% of total quantity), chronic inflammatory demyelinating polyradiculoneuropathy (10.2%), corticoresistant dermatomyositis (10.2%), immune thrombocytopaenia (9.9%) and primary immune deficiency (9.1%). According to available French recommendations, 70% of the IVIg use was for 'acknowledged indications', 9% for 'indications to be assessed' and 18% for 'unwarranted indications'. The 10 most reported indications were 'acknowledged indications' according to available recommendations of the French expert group. Nevertheless, the two most reported indications were not approved by the French Health Products Agency (AFSSAPS) at the time of the study and were approved since., (2011 The Authors Fundamental and Clinical Pharmacology. 2011 Société Française de Pharmacologie et de Thérapeutique.)

Background: Autoimmune hemolytic anemia is a rare condition in children. Little is known about its initial presentation and the subsequent progression of the disease., Design and Methods: Since 2004, a national observational study has been aiming to thoroughly describe cases and identify prognostic factors. Patients from all French hematologic pediatric units have been included if they had a hemoglobin concentration less than 11 g/dL, a positive direct antiglobulin test and hemolysis. Evans' syndrome was defined by the association of autoimmune hemolytic anemia and immunological thrombocytopenic purpura. Data from patients' medical records were registered from birth to last follow-up. Autoimmune hemolytic anemia was classified as primary or secondary. Remission criteria, qualifying the status of anemia at last follow-up, were used with the aim of identifying a subgroup with a favorable prognosis in continuous complete remission., Results: The first 265 patients had a median age of 3.8 years at diagnosis. In 74% of cases the direct antiglobulin test was IgG/IgG+C3d. Consanguinity was reported in 8% of cases and first degree familial immunological diseases in 15% of cases. Evans' syndrome was diagnosed in 37% of cases. Autoimmune hemolytic anemia was post-infectious in 10%, immunological in 53% and primary in 37% of cases. After a median follow-up of 3 years, 4% of children had died, 28% were still treatment-dependent and 39% were in continuous complete remission. In multivariate analysis, IgG and IgG+C3d direct antiglobulin tests were associated with a lower rate of survival with continuous complete remission (adjusted hazard ratio, 0.43; 95% confidence interval, 0.21-0.86)., Conclusions: This nationwide French cohort is the largest reported study of childhood autoimmune hemolytic anemia. The rarity of this condition is confirmed. Subgroups with genetic predisposition and underlying immune disorders were identified.

Purpose: To examine the link between symptoms of hyperactivity-inattention and conduct disorder in childhood, and the initiation of tobacco and cannabis use, controlling for other behavioral symptoms, temperament and environmental risk factors., Method: The sample (N=1107 participants, aged 4 to 18 years at baseline) was recruited from the population-based longitudinal Gazel Youth study with a follow-up assessment 8 years later. Psychopathology, temperament, environmental variables, and initiation of tobacco and cannabis use were self-reported. Event time analyses were performed to assess the effects of childhood disruptive symptoms on age at first use of tobacco and cannabis., Results: Proportional hazard models revealed that participants with high levels of childhood symptoms of both hyperactivity-inattention and conduct disorder were at highest risk of early tobacco initiation (in males: hazard ratio [HR]=2.05; confidence interval [CI]: 1.24-3.38; in females: HR=2.01; CI: 1.31-3.09), and, in males, of early cannabis initiation (HR=1.95; CI: 1.04-3.64). Temperament, through activity in both males and females and negative emotionality in females, was also associated to early substance use initiation., Conclusions: Children who simultaneously have high levels of symptoms of hyperactivity-inattention and conduct disorder are at increased risk for early substance initiation. These associations may guide childhood health professionals to consider the liability for early substance initiation in high-risk groups., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)