Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 85
Filtrar
1.
Artigo em Inglês | MEDLINE | ID: mdl-31660778

RESUMO

Purpose: This study evaluated the long-term psychological impact of childhood cancer and also sought to identify the risk factors in the development of psychological issues. Methods: Young adult (18-38 years) survivors of a childhood cancer (except leukemia), diagnosed younger than 15 years between 1987 and 1999 in the Rhône-Alpes region of France, were invited to a semistandardized psychological interview after a medical follow-up consultation during two successive long-term follow-up studies in Oncology (SALTO-1 and -2). Psychiatric issues from the DSM-IV were diagnosed and compared with the general French population (GFP) through interviews based on the Mini-International Neuropsychiatric Interview (MINI). Results: Of the 288 childhood cancer survivors (CCSs) who attended the consultations, 247 completed the MINI interview. Fifty-five percent indicated they had suffered from psychiatric issues after their cancer compared to 31.9% of the GFP (p < 0.0001). These issues were generally anxiety problems (40.5%), mood disorders (28.7%), and substance dependency (10.5%; p < 0.0001). The risk of suicide was, however, less for the CCS group (8.9% vs. 13.6%, p = 0.03). Unemployment was a significant risk factor for mood disorders (p = 0.009). Men were 4.1 times more likely than women to be addicted during their lifetime (p = 0.0004), while adults cured of bone tumors were 14.3 times more likely to be at risk of drug dependence than adults cured of central nervous system tumors (p = 0.01). Conclusion: CCSs are particularly vulnerable to psychiatric disorders throughout their life. Systematic and long-term psychological monitoring of these patients will enable their psychiatric issues to be detected sooner.

2.
Neuro Oncol ; 2019 Aug 23.
Artigo em Inglês | MEDLINE | ID: mdl-31504825

RESUMO

BACKGROUND: Medulloblastomas may occur in a predisposition context, including familial adenomatosis polyposis. Medulloblastomas related to APC germline pathogenic variant remain rare and poorly described. Their similarities with sporadic WNT medulloblastomas still require to be described. METHODS: We performed a multicentric retrospective review of 12 patients treated between 1988 and 2018 for medulloblastoma with identified or highly suspected (personal of familial history) APC germline pathogenic variant. We report personal and familial history, APC gene pathogenic variant whenever available, clinical and histological characteristics of the medulloblastoma, treatments, and long-term outcome including second tumor and late sequelae. RESULTS: Medulloblastomas associated with APC pathogenic variant are mainly classic (11/11 patients, 1 NA), non-metastatic (10/12 patients) medulloblastomas, with nuclear immunoreactivity for ß-catenin (9/9 tested cases). 10/11 assessable patients are disease-free with a median follow-up of 10.7 years [1-28]. Secondary tumors included desmoid tumors in 7 patients (9 tumors), 1 thyroid carcinoma, 2 pilomatricomas, 1 osteoma, 1 vertebral hemangioma, and 1 malignant Triton in the radiation field which caused the only cancer-related death in our series. CONCLUSIONS: Medulloblastomas associated with APC pathogenic variant have an overall favorable outcome, even for metastatic tumors. Yet, long-term survival is clouded by second tumor occurrence; treatment may play some role in some of these second malignancies. Our findings raise the question of applying de-escalation therapeutic protocol to treat patients with APC germline pathogenic variant given the excellent outcome, and reduced intensity of craniospinal irradiation may be further evaluated.

3.
Nano Lett ; 19(10): 7043-7049, 2019 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-31468976

RESUMO

Circular-polarization-resolved magneto-infrared studies of multilayer epitaxial graphene (MEG) are performed using tunable quantum cascade lasers in high magnetic fields up to 17.5 T. Landau level (LL) transitions in the monolayer and bilayer graphene inclusions of MEG are resolved, and considerable electron-hole asymmetry is observed in the extracted electronic band structure. For monolayer graphene, a four-fold splitting of the n = 0 to n = 1 LL transition is evidenced and attributed to the lifting of the valley and spin degeneracy of the zeroth LL and the broken electron-hole symmetry. The magnetic field dependence of the splitting further reveals its possible mechanisms. The best fit to experimental data yields effective g-factors, gVS* = 6.7 and gZS* = 4.8, for the valley and Zeeman splittings, respectively.

4.
J Neuroradiol ; 46(3): 207-213, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30731145

RESUMO

INTRODUCTION: Medulloblastoma is the most common malignant cerebral tumor during childhood, arising in the posterior fossa. Children treated for medulloblastoma often experience working memory (WM) deficits, affecting their quality of life and school performance. The aim of the present study undertaken to describe the cerebellar involvement in WM deficits observed in these children. MATERIAL AND METHODS: 23 healthy children and 11 children treated for medulloblastoma were included into study. All subjects performed a detailed neuropsychological examination, an anatomical and functional MRI. Stimuli were presented to the participants with alternating sensory modality and nature of communication in a block design during functional magnetic resonance imaging acquisitions. Non-parametric tests were used for analyzing neuropsychological and behavioral data. SPM8 and SUIT (Spatially Unbiased Atlas Template) were used for anatomical and functional MRI data analyses. RESULTS: Patients had cerebellar resections mainly located in the left posterior lobe. Patients had significantly reduced intelligence quotient, central executive and visuospatial WM. In healthy children group, fMRI showed activations for non-verbal and visuospatial WM in the left posterior cerebellar lobe. CONCLUSION: This study provides further evidence that left posterior cerebellar lobe plays a critical role in WM. Indeed, lesions of left posterior cerebellar lobe were associated with WM impairment in children treated for cerebellar medulloblastoma. Additionally, fMRI using WM tasks showed activation in the left posterior cerebellar lobe in healthy children. Taken together, these findings may help for improving treatment and rehabilitation of children referred for cerebellar tumor.


Assuntos
Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/psicologia , Doenças Cerebelares/diagnóstico por imagem , Doenças Cerebelares/psicologia , Imagem por Ressonância Magnética/métodos , Meduloblastoma/diagnóstico por imagem , Meduloblastoma/psicologia , Memória de Curto Prazo , Adolescente , Mapeamento Encefálico , Neoplasias Encefálicas/cirurgia , Criança , Feminino , França , Humanos , Deficiência Intelectual/etiologia , Masculino , Meduloblastoma/cirurgia , Testes Neuropsicológicos , Procedimentos Neurocirúrgicos , Qualidade de Vida , Fatores de Risco
5.
Haematologica ; 104(8): 1554-1564, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30655378

RESUMO

We describe the clinical, hematologic and genetic characteristics of a retrospective series of 126 subjects from 64 families with hereditary xerocytosis. Twelve patients from six families carried a KCNN4 mutation, five had the recurrent p.Arg352His mutation and one had a new deletion at the exon 7-intron 7 junction. Forty-nine families carried a PIEZO1 mutation, which was a known recurrent mutation in only one-third of the cases and private sequence variation in others; 12 new probably pathogenic missense mutations were identified. The two dominant features leading to diagnosis were hemolysis that persisted after splenectomy and hyperferritinemia, with an inconstant correlation with liver iron content assessed by magnetic resonance imaging. PIEZO1-hereditary xerocytosis was characterized by compensated hemolysis in most cases, perinatal edema of heterogeneous severity in more than 20% of families and a major risk of post-splenectomy thrombotic events, including a high frequency of portal thrombosis. In KCNN4-related disease, the main symptoms were more severe anemia, hemolysis and iron overload, with no clear sign of red cell dehydration; therefore, this disorder would be better described as a 'Gardos channelopathy'. These data on the largest series to date indicate that PIEZO1-hereditary xerocytosis and Gardos channelopathy are not the same disease although they share hemolysis, a high rate of iron overload and inefficient splenectomy. They demonstrate the high variability in clinical expression as well as genetic bases of PIEZO1-hereditary xerocytosis. These results will help to improve the diagnosis of hereditary xerocytosis and to provide recommendations on the clinical management in terms of splenectomy, iron overload and pregnancy follow-up.

6.
Eur J Cancer ; 103: 227-237, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30273888

RESUMO

AIMS: Survival after cancer diagnosed during childhood or adolescence continues to improve with new treatments and supportive therapies. Optimal long-term care requires that risks to vulnerable organs are clearly defined and translated into guidelines that are implemented into practice. PanCareLIFE is a pan-European consortium that addresses survivorship issues comprising fertility, hearing impairment and quality of life. This article describes the scientific basis of PanCareLIFE's studies. METHODS: PanCareLIFE involves 17 partner institutions from eight European countries, with additional 11 data providers from five other countries. Study designs and methods include molecular genetic, cohort and case-control studies, a longitudinal study and an intervention study. Ethics and data protection issues have been taken into account from the beginning. RESULTS: PanCareLIFE will investigate the way that treatment impairs female fertility, by evaluating anti-Müllerian hormone levels and the underlying genetic susceptibility to loss of fertility. For our fertility studies, more than 6000 survivors have completed questionnaires, more than 1500 provided serum samples and more than 400 case-control triads have been identified. Fertility preservation guidelines for boys and girls will be developed. More than 2000 survivors have contributed audiograms for the ototoxicity study. Almost 1000 samples were sent for genetic analysis related to ototoxicity and gonadal reserve. The SF-36 questionnaire will measure quality of life in more than 10,000 survivors. CONCLUSIONS: The large number of subjects enrolled in PanCareLIFE and the detailed information accumulated will allow in-depth evaluation of important outcomes. Fertility preservation guidelines will help patients and their families make informed decisions and contribute to their long-term well-being.

7.
BMC Cancer ; 18(1): 930, 2018 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-30257669

RESUMO

BACKGROUND: Improved risk stratification, more effective therapy and better supportive care have resulted in survival rates after childhood cancer of around 80% in developed countries. Treatment however can be harsh, and three in every four childhood cancer survivors (CCS) develop at least one late effect, such as gonadal impairment. Gonadal impairment can cause involuntary childlessness, with serious consequences for the well-being of CCS. In addition, early menopause increases the risk of comorbidities such as cardiovascular disease and osteoporosis. Inter-individual variability in susceptibility to therapy related gonadal impairment suggests a role for genetic variation. Currently, only one candidate gene study investigated genetic determinants in relation to gonadal impairment in female CCS; it yielded one single nucleotide polymorphism (SNP) that was previously linked with the predicted age at menopause in the general population of women, now associated with gonadal impairment in CCS. Additionally, one genome wide association study (GWAS) evaluated an association with premature menopause, but no GWAS has been performed using endocrine measurements for gonadal impairment  as the primary outcome in CCS. METHODS: As part of the PanCareLIFE study, the genetic variability of chemotherapy induced gonadal impairment among CCS will be addressed. Gonadal impairment will be determined by anti-Müllerian hormone (AMH) levels or alternatively by fertility and reproductive medical history retrieved by questionnaire. Clinical and genetic data from 837 non-brain or non-bilateral gonadal irradiated long-term CCS will result in the largest clinical European cohort assembled for this late-effect study to date. A candidate gene study will examine SNPs that have already been associated with age at natural menopause and DNA maintenance in the general population. In addition, a GWAS will be performed to identify novel allelic variants. The results will be validated in an independent CCS cohort. DISCUSSION: This international collaboration aims to enhance knowledge of genetic variation which may be included in risk prediction models for gonadal impairment in CCS.

8.
JMIR Res Protoc ; 7(9): e10824, 2018 Sep 14.
Artigo em Inglês | MEDLINE | ID: mdl-30215599

RESUMO

BACKGROUND: Despite a significant number of studies on female fertility following childhood, adolescent, and young adult (CAYA) cancer, studies establishing precise (dose-related) estimates of treatment-related risks are still scarce. Previous studies have been underpowered, did not include detailed treatment information, or were based on self-report only without any hormonal assessments. More precise assessments of who is at risk for sub- or infertility are needed. OBJECTIVE: The objective of our study is to describe the design and methods of 2 studies on female fertility (a cohort study and a nested case-control study) among female survivors of CAYA cancer performed within the European PanCareLIFE project. METHODS: For the cohort study, which aims to evaluate the overall risk of fertility impairment, as well as the risk for specific subgroups of female CAYA cancer survivors, 13 institutions from 9 countries provide data on fertility impairment. Survivors are defined as being fertility impaired if they meet at least one of 8 different criteria based on self-reported and hormonal data. For the nested case-control study, which aims to identify specific treatment-related risk factors associated with fertility impairment in addition to possible dose-response relationships, cases (fertility impaired survivors) are selected from the cohort study and matched to controls (survivors without fertility impairment) on a 1:2 basis. RESULTS: Of the 10,964 survivors invited for the cohort study, data are available from 6619 survivors, either questionnaire-based only (n=4979), hormonal-based only (n=72), or both (n=1568). For the nested case-control study, a total of 450 cases and 882 controls are identified. CONCLUSIONS: Results of both PanCareLIFE fertility studies will provide detailed insight into the risk of fertility impairment following CAYA cancer and diagnostic- or treatment-related factors associated with an increased risk. This will help clinicians to adequately counsel both girls and young women, who are about to start anticancer treatment, as well as adult female CAYA cancer survivors, concerning future parenthood and to timely refer them for fertility preservation. Ultimately, we aim to empower patients and survivors and improve their quality of life. REGISTERED REPORT IDENTIFIER: RR1-10.2196/10824.

9.
Artigo em Inglês | MEDLINE | ID: mdl-30136876

RESUMO

PURPOSE: Survival rate of childhood cancers is now reaching 80% overall. However, early or late complications related to surgery, chemotherapy, and radiotherapy remain at a high rate and greatly increase the risk of late mortality. The objective of this study is to assess the autonomic nervous system (ANS) activity, measured through heart rate variability indices in childhood cancer survivors compared with healthy controls. METHODS: This prospective study included 51 long-term childhood cancer survivors diagnosed before 15 years of age between 1987 and 1992 and controlled for age and sex with healthy volunteers. RESULTS: We observed a significant increase in spontaneous heart rate (beats per minute) (67 ± 10 vs. 60 ± 10, p = 0.001), and all the studied parameters showed a significantly altered ANS activity in cases compared with healthy controls. In both groups, the main cofactors of dysautonomia (tobacco, drugs, cannabis, estro-progestative pills, alcohol, limited physical activity) were analyzed without any significant difference. The effect of cancer treatments received was not analyzed due to the small number of participants. CONCLUSION: The results showed a significant ANS dysfunction in childhood cancer survivors compared with healthy controls and suggested the value of autonomic screening to underscore and possibly quantify the effect of the cancer treatments in a larger cohort. This evaluation could lead to the recommendation to increase physical activity, the most efficient way known to improve ANS activity, as already shown in other pathologies (breast cancer).

10.
BMJ Open ; 8(7): e022409, 2018 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-30049701

RESUMO

INTRODUCTION: Severe haemophilia is a rare disease characterised by spontaneous bleeding from early childhood, which may lead to various complications, especially in joints. It is nowadays possible to avoid these complications thanks to substitutive therapies for which the issue of adherence is major. The transition from adolescence to adulthood in young people with severe haemophilia is a critical period as it is associated with a high risk of lack of adherence to healthcare, which might have serious consequences on daily activities and on quality of life. METHODS AND ANALYSIS: We present the protocol for a cross-sectional, observational, multicentric study to assess the differences between adolescents and young adults with severe haemophilia in France through the transition process, especially on adherence to healthcare. This study is based on a mixed methods design, with two complementary and consecutive phases, comparing data from a group of adolescents (aged 14-17 years) with those from a group of young adults (aged 20-29 years). The quantitative phase focuses on the determinants (medical, organisational, sociodemographic and social and psychosocial and behavioural factors) of adherence to healthcare (considered as a marker of the success of transition). The qualitative phase explores participants' views in more depth to explain and refine the results from the quantitative phase. Eligible patients are contacted by the various Haemophilia Treatment Centres participating in the French national registry FranceCoag. ETHICS AND DISSEMINATION: The study was approved by the French Ethics Committee and by the French National Agency for Medicines and Health Products Safety (number: 2016-A01034-47). Study findings will be disseminated to the scientific and medical community in peer-reviewed journals and presented at scientific meetings. Results will be popularised to be communicated via the French association for people with haemophilia to participants and to the general public. TRIAL REGISTRATION NUMBER: NCT02866526; Pre-results.

12.
Blood ; 132(12): 1318-1331, 2018 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-29914977

RESUMO

Congenital neutropenias (CNs) are rare heterogeneous genetic disorders, with about 25% of patients without known genetic defects. Using whole-exome sequencing, we identified a heterozygous mutation in the SRP54 gene, encoding the signal recognition particle (SRP) 54 GTPase protein, in 3 sporadic cases and 1 autosomal dominant family. We subsequently sequenced the SRP54 gene in 66 probands from the French CN registry. In total, we identified 23 mutated cases (16 sporadic, 7 familial) with 7 distinct germ line SRP54 mutations including a recurrent in-frame deletion (Thr117del) in 14 cases. In nearly all patients, neutropenia was chronic and profound with promyelocytic maturation arrest, occurring within the first months of life, and required long-term granulocyte colony-stimulating factor therapy with a poor response. Neutropenia was sometimes associated with a severe neurodevelopmental delay (n = 5) and/or an exocrine pancreatic insufficiency requiring enzyme supplementation (n = 3). The SRP54 protein is a key component of the ribonucleoprotein complex that mediates the co-translational targeting of secretory and membrane proteins to the endoplasmic reticulum (ER). We showed that SRP54 was specifically upregulated during the in vitro granulocytic differentiation, and that SRP54 mutations or knockdown led to a drastically reduced proliferation of granulocytic cells associated with an enhanced P53-dependent apoptosis. Bone marrow examination of SRP54-mutated patients revealed a major dysgranulopoiesis and features of cellular ER stress and autophagy that were confirmed using SRP54-mutated primary cells and SRP54 knockdown cells. In conclusion, we characterized a pathological pathway, which represents the second most common cause of CN with maturation arrest in the French CN registry.

13.
J Adolesc Young Adult Oncol ; 7(4): 415-423, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29851372

RESUMO

PURPOSE: To describe fecundity in female survivors of childhood cancer and consider the correlation with quality of life (QOL). MATERIALS AND METHODS: Of 1744 women treated for childhood cancer before the age of 15 years at one of eight French cancer treatment centers between 1948 and 1992, 1187 who were alive in 2005 were sent a self-administered questionnaire, including questions about health status, QOL (MOS SF-36), and fecundity. A standardized fecundity ratio (SFR) was calculated (SFR: observed/expected number of children) for each individual based on a national reference. RESULTS: Of the 972 individuals (82%) who responded, 53% had at least 1 child. The overall SFR, 0.65, was dependent upon the initial diagnosis, more decreased in Central Nervous System tumors (0.24; p < 10-3) than in Germ cell (0.46; p = 0.03) or Sympathetic Nervous System tumors (0.79; p = 0.02). The average QOL motor score was 72.5 ± 19.5, and the average mental score was 61.4 ± 16.7. After adjusting for age, pathology, and self-reported sequelae in the questionnaires, it was determined that SF-36 mental (p = 0.002) and motor (p < 0.0002) scores correlated positively with fecundity, and SF-36 scores correlated negatively with locomotor late effects (p < 0.0001), growth insufficiency (p = 0.002), and psychological disorders (p < 0.001). Gonadal insufficiency was correlated with neither motor nor mental scores. CONCLUSION: Women treated for childhood cancer demonstrated impaired fecundity that correlated with poor QOL, as registered by the SF-36. Patients should be warned of the risk of impaired fecundity early during the follow-up. If possible, preservation of fertility should be prioritized at initiation of therapy.

14.
Nat Nanotechnol ; 13(9): 870, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29784966

RESUMO

In the version of this Article originally published, the second affiliation for Walter A. de Heer had not been included; it should be 'TICNN, Tianjin University, Tianjin, China'. This has now been added and the numbering of subsequent affiliations amended accordingly in all versions of the Article.

15.
Haematologica ; 103(7): 1143-1149, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29599204

RESUMO

In this retrospective study, we evaluate long-term complications in nearly all ß-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.

16.
PLoS One ; 13(1): e0191885, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29377922

RESUMO

BACKGROUND: Filling the lung with dense liquid perfluorocarbons during total liquid ventilation (TLV) might compress the myocardium, a plausible explanation for the instability occasionally reported with this technique. Our objective is to assess the impacts of TLV on the cardiovascular system, particularly left ventricular diastolic function, in an ovine model of neonatal respiratory distress syndrome. METHOD: Eight newborns lambs, 3.0 ± 0.4 days (3.2 ± 0.3kg) were used in this crossover experimental study. Animals were intubated, anesthetized and paralyzed. Catheters were inserted in the femoral and pulmonary arteries. A high-fidelity pressure catheter was inserted into the left ventricle. Surfactant deficiency was induced by repeated lung lavages with normal saline. TLV was then conducted for 2 hours using a liquid ventilator prototype. Thoracic echocardiography and cardiac output assessment by thermodilution were performed before and during TLV. RESULTS: Left ventricular end diastolic pressure (LVEDP) (9.3 ± 2.1 vs. 9.2 ± 2.4mmHg, p = 0.89) and dimension (1.90 ± 0.09 vs. 1.86 ± 0.12cm, p = 0.72), negative dP/dt (-2589 ± 691 vs. -3115 ± 866mmHg/s, p = 0.50) and cardiac output (436 ± 28 vs. 481 ± 59ml/kg/min, p = 0.26) were not affected by TLV initiation. Left ventricular relaxation time constant (tau) slightly increased from 21.5 ± 3.3 to 24.9 ± 3.7ms (p = 0.03). Mean arterial systemic (48 ± 6 vs. 53 ± 7mmHg, p = 0.38) and pulmonary pressures (31.3 ± 2.5 vs. 30.4 ± 2.3mmHg, p = 0.61) were stable. As expected, the inspiratory phase of liquid cycling exhibited a small but significant effect on most variables (i.e. central venous pressure +2.6 ± 0.5mmHg, p = 0.001; LVEDP +1.18 ± 0.12mmHg, p<0.001). CONCLUSIONS: TLV was well tolerated in our neonatal lamb model of severe respiratory distress syndrome and had limited impact on left ventricle diastolic function when compared to conventional mechanical ventilation.


Assuntos
Diástole , Modelos Animais de Doenças , Ventilação Líquida/métodos , Síndrome do Desconforto Respiratório do Recém-Nascido/terapia , Função Ventricular Esquerda , Animais , Animais Recém-Nascidos , Fluorcarbonetos/farmacocinética , Síndrome do Desconforto Respiratório do Recém-Nascido/fisiopatologia , Ovinos
17.
Br J Radiol ; 91(1084): 20170819, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29293375

RESUMO

OBJECTIVE: The survival rate of children treated for cancer is currently about 80% at 5 years and we estimate that about 50,000 adults in France have survived childhood cancer. In 2011, there was a call for projects relating to long-term follow-up (LTFU), which led to several studies being conducted. Five years later, we sent a questionnaire to present LTFU in France and describe its strengths and weaknesses and to establish appropriate steps that should be taken. METHODS: A questionnaire was sent by email to all the members of the French Society of Childhood Cancers in spring 2016. The study involved 44 centres/hospitals with a Paediatric Oncology Department. RESULTS: 54 answers were analysed, provided by 31/44 (70%) centres working together with the French Society of Childhood Cancers. Screening is the main objective of LTFU care (90%). The main difficulties that arose were: lack of sufficient time to devote to this activity (57%), difficulties contacting adult childhood cancer survivors (aCCSs) (26%), aCCSs who ultimately did not show up to the consultation (19%), cost (15%), and lack of organization (13%). Seven LTFU programmes were identified: two regional organizations (Rhône Alpes and Grand Ouest), four centre-size organizations, and one national study (involving 15 Haematology Centres) relating to Child and Adolescent Leukaemia. CONCLUSION: LTFU is a major concern for French centres specialized in paediatric oncology. Organization is not well defined and difficulties still arise (Who are the best care providers? What frequency of care is most appropriate? etc.). Advances in knowledge: LTFU focused on health problems (physical, psychological, social, economic issues) that affect CCSs is needed to ensure that these patients regain the most optimal physical and emotional health possible. Practitioners suggest different ways to improve LTFU, such as national co-operation with Epidemiology Registers to promote homogenous LTFU care.


Assuntos
Neoplasias/epidemiologia , Neoplasias/terapia , Sobreviventes , Adolescente , Adulto , Criança , Feminino , Seguimentos , França/epidemiologia , Humanos , Masculino , Inquéritos e Questionários , Taxa de Sobrevida
18.
Eur J Cancer ; 91: 30-37, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29331749

RESUMO

METHODS: French patients (≤18years) treated for dysgerminoma between 1985 and 2005 in TGM-85, 90, 95 protocols were included. Treatment was based on primary unilateral oophorectomy followed by prophylactic lymph node irradiation (1985-1998) or a wait-and-see strategy (1998-2005) for localised completely resected tumours (pS1) or by platinum-based chemotherapy for advanced diseases. RESULTS: Forty-eight patients (median age 12.8 years) were included. Six patients had gonadal dysgenesis. Two had bilateral dysgerminoma. Twenty-eight patients had loco-regional dissemination, seven with para-aortic lymph nodes. None had distant metastases. Primary surgery was performed in 47/48 patients. Among the 15 patients with pS1 tumour: seven did not receive adjuvant treatment, six had lymph node irradiation and two received chemotherapy. Among the 32 patients with advanced tumour, 31 received cisplatinum-based (n = 25) or carboplatin-based (n = 8) regimen with lymph node irradiation for one of them and one did not receive adjuvant treatment. With a median follow-up of 14 years, all patients are alive in complete remission. Five events occurred: 2 contralateral dysgerminomas, 1 peritoneal relapse and 2 second neoplasms (teratoma and melanoma). Bilateral oophorectomy was necessary for 12 patients. Desire of pregnancy was expressed for 17/36 patients with unilateral oophorectomy, which succeeded in 13 cases (5 medically assisted). 2/17 had ovarian failure. The renal function was normal in 24/25 evaluated patients treated with platinum, ifosfamide or irradiation. The hearing function was evaluated on 17/36 patients treated with platinum: 12 Brock grade-0, 3 brock grade-1 and 2 grade-4. CONCLUSION: Dysgerminoma has an excellent prognosis even in advanced cases with conservative surgery and platinum-based chemotherapy. However the disease and/or treatment resulted in a high rate of bilateral oophorectomies and a significant impact on future fertility.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Sobreviventes de Câncer , Carboplatina/administração & dosagem , Cisplatino/administração & dosagem , Disgerminoma/terapia , Neoplasias Ovarianas/terapia , Ovariectomia , Adolescente , Idade de Início , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Carboplatina/efeitos adversos , Quimioterapia Adjuvante , Criança , Cisplatino/efeitos adversos , Intervalo Livre de Doença , Disgerminoma/epidemiologia , Disgerminoma/secundário , Feminino , França/epidemiologia , Humanos , Infertilidade Feminina/epidemiologia , Infertilidade Feminina/terapia , Metástase Linfática , Recidiva Local de Neoplasia , Segunda Neoplasia Primária , Neoplasias Ovarianas/epidemiologia , Neoplasias Ovarianas/patologia , Ovariectomia/efeitos adversos , Radioterapia Adjuvante , Técnicas de Reprodução Assistida , Fatores de Tempo , Resultado do Tratamento
19.
Nat Nanotechnol ; 13(2): 133-138, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29255290

RESUMO

Atomically thin graphene exhibits fascinating mechanical properties, although its hardness and transverse stiffness are inferior to those of diamond. So far, there has been no practical demonstration of the transformation of multilayer graphene into diamond-like ultrahard structures. Here we show that at room temperature and after nano-indentation, two-layer graphene on SiC(0001) exhibits a transverse stiffness and hardness comparable to diamond, is resistant to perforation with a diamond indenter and shows a reversible drop in electrical conductivity upon indentation. Density functional theory calculations suggest that, upon compression, the two-layer graphene film transforms into a diamond-like film, producing both elastic deformations and sp 2 to sp 3 chemical changes. Experiments and calculations show that this reversible phase change is not observed for a single buffer layer on SiC or graphene films thicker than three to five layers. Indeed, calculations show that whereas in two-layer graphene layer-stacking configuration controls the conformation of the diamond-like film, in a multilayer film it hinders the phase transformation.

20.
Phys Rev Lett ; 119(6): 067405, 2017 Aug 11.
Artigo em Inglês | MEDLINE | ID: mdl-28949645

RESUMO

Recent pump-probe experiments performed on graphene in a perpendicular magnetic field have revealed carrier relaxation times ranging from picoseconds to nanoseconds depending on the quality of the sample. To explain this surprising behavior, we propose a novel symmetry-breaking defect-assisted relaxation channel. This enables scattering of electrons with single out-of-plane phonons, which drastically accelerate the carrier scattering time in low-quality samples. The gained insights provide a strategy for tuning the carrier relaxation time in graphene and related materials by orders of magnitude.

SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA