Purpose. The improvement of the management of childhood cancer has led to a strong increase of the overall survival rate about 80%. Radiation therapy, element still required for the management in some cancers, is associated with long-term side effects. The aim of this study was to analyze late effects of childhood cancer survivors whose treatment included radiation therapy.Methods. SALTO's study, based on childhood cancer registers of Rhone-Alpes-Auvergne, concerned children with solid cancer or lymphoma before the age of 15 years, diagnosed between 1987 and 1992. These patients, at least 18 years old, attended to a clinic consultation with a pediatric oncologist and adult internist between 2011 and 2014. Adults treated during childhood cancer with radiation therapy, associated or not with chemotherapy or surgery, have been included and compared with those treated only with chemotherapy and/or surgery. Late effects have been classified as moderate or severe according to the CTCAE.Results. Fifty-nine patients with radiation therapy attended these consultations and 91 without radiation therapy. Fifty-eight patients with radiation therapy (98.3%) had at least one sequela. The main late effects all ranks were orthopedic (37%), endocrine (32%), thyroid (31%) dysfunction and obesity (31%). The main late effects of grade 3-4 were endocrine, renal, obesity and secondary cancers. The proportion of endocrine, thyroid, orthopedic, skin late effects, hearing loss and secondary cancers were significantly higher in irradiated patients compared to non-irradiated patients.Conclusion. These results are important for the current implementation of long term follow up of childhood cancer survivors, focused on these late effects to greater prevention or early treatment. (C) 2016 Elsevier Masson SAS. All rights reserved.

Background. - The population of survivors of childhood cancer is currently growing. Studies from other countries have shown an increased risk of late mortality. In order to measure this risk within a French cohort, the mortality of children who had survived five years from a cancer diagnosis were compared to the mortality of the general population, according to follow-up interval and cancer and treatment characteristics.Methods. - The study population consisted of 635 children diagnosed with cancer before the age of 15 who had survived at least five years, and were registered in the Rhone-Alpes region cancer registry from 1987 to 1992. Mortality was compared with general population rates of the Rhone-Alpes region to assess age and sex standardized mortality ratio (SMR) and absolute excess risk of death.Results. - The median follow-up of children was 14.0 years. Among the 42 observed deaths, 71.4% were attributed to a recurrence of the original cancer, 9.5% to a second cancer. The 15-year cumulative risk of death, all causes, was 7.1%. The overall mortality of the cohort was 20.7 fold greater than the general population (95% CI: 14.9-27.9), and the absolute excess risk of 6.9 per 1000 persons-years. The long term excess-mortality was higher in case of recurrence of original cancer (SMR = 99.9, 95% CI: 67.9-141.9, absolute excess risk 35.4 per 1000 persons-years); it was raised during the five to nine years follow-up interval after diagnosis (SMR = 33.8, 95% CI: 23.2-47.3) mainly due to the primary malignancy, and decreased after (10-14 years follow-up interval SMR = 6.5, 95% IC 2.4-14.2).Conclusion. - The late mortality of childhood cancer is significantly increased during the five to nine years following diagnosis and decreases after, but the cohort follow-up has to be extended in order to assess outcome beyond 15 years after diagnosis. (C) 2008 Elsevier Masson SAS. Tous droits reserves.

UNLABELLED:Cancer is rare in children, and pediatric malignancies represent only 1% of all cancers.OBJECTIVES:The cure rate is high and increasing, and ongoing data collection is therefore warranted.MATERIALS AND METHODS:Here we report the incidence and survival rates of childhood cancers between 1987 and 1999 in the Rhône-Alpes region of France.RESULTS:A total of 1945 cases were recorded during the study period, with an average of 149.6 new cases per year. The approximate incidence rate was 134.1/10(6) per year and the age-standardized incidence rate was 139.2/10(6) per year. The histological distribution and 5-year survival rates were respectively 30.2 and 73% for leukemia, 12.3 and 91.6% for lymphoma, 24.7 and 60.1% for CNS tumors, 9.1 and 71.1% for neuroblastoma, 2.5 and 94.1% for retinoblastoma, 5.8% and 89.9% for renal tumors, 1 and 75% for liver tumors, 6.1 and 60.9% for bone tumors, 4.1 and 58.6% for soft-tissue tumors, 1.1 and 71% for germ cell tumors, and 2.4 and 85.1% for carcinomas.CONCLUSION:The overall survival rate was 75%. Long-term treatment complications warrant further studies of children who survive into adulthood.

Cancer is rare in children, and pediatric malignancies represent only 1% of all cancers.Objectives. - The cure rate is high and increasing, and ongoing data collection is therefore warranted.Materials and methods. - Here we report the incidence and Survival rates of childhood cancers between 1987 and 1999 in the Rhone-Alpes region of France.Results. - A total of 1945 cases were recorded during the study period, with an average of 149.6 new cases per year. The approximate incidence rate was 134.1/10(6) per year and the age-standardized incidence rate was 139.2/10(6) per year. The histological distribution and 5-year survival rates were respectively 30.2 and 73% for leukemia, 12.3 and 91.6% for lymphoma, 24.7 and 60.1% for CNS tumors, 9.1 and 71.1% for neuroblastoma, 2.5 and 94.1% for retinoblastoma, 5.8% and 89.9% for renal tumors, 1 and 75% for liver tumors, 6.1 and 60.9% for bone tumors, 4.1 and 58.6% for soft-tissue tumors, 1.1 and 71% for germ cell tumors, and 2.4 and 85.1% for carcinomas.Conclusion. - The overall survival rate was 75%. Long-term treatment complications warrant further studies of children who survive into adulthood. (c) 2005 Elsevier SAS. Tous droits reserves.

Although systematic vitamin D supplementation in adolescents remains debated, rickets is nevertheless a well recognized pathology in this age group. Adolescence is an at-risk period because of rapid growth, insufficient calcium intake and/or vitamin D status. Surveys have shown that calcium intake is insufficient (< 1000 mg a day) in 45% of boys and 71% of girls and that vitamin D status is deficient (25-OH-D < 10 ng/ml). The aims of the study carried out by the Calcium Group of the Societe Francaise de Pediatrie, were to evaluate the frequency of rickets, and to define the criteria for the adolescent population at risk. Forty-one adolescents with rickets were hospitalized between 1985 and 2000. Most of the cases were from the Northern France: 20 from Paris and suburbs, eight from the North-West, four from the North, four from the North-East; five were from the Center of France. The mean age was 13 years and two months for the 28 girls, and 14 years and four months for the 13 boys. Eighty per cent of the adolescents were from immigrant families (33/41): 15 were from sub-Saharan Africa, ten from North Africa, six from Pakistan and two from Turkey. Two thirds of the adolescents were hospitalized in the 2nd quarter of the year. Some adolescents suffered from lower limb pain, 16 had deformations of lower limbs, particularly genu valgum, associated with pain; seven others had either muscle spasms (4), tetany (3). Serum calcium level was low (average 1.84 mmol/l: [1.1-2.5]), and serum 25-OH D level was extremely low. Radiographic characteristics observed were metaphyseal strips on the knees, with condensed edges at times, with the presence of bone demineralization. The treatment combined calcium and vitamin D, and was often administered intravenously when a hypocalcemia was detected. Rickets is not frequent in adolescents, but nonetheless this pathology is not exceptional, and the number of cases is probably underestimated. Rickets affects immigrant adolescents in particular but nevertheless could also present a certain risk period for the general population. (C) 2004 Elsevier SAS. Tous droits reserves.

Although systematic vitamin D supplementation in adolescents remains debated, rickets is nevertheless a well recognized pathology in this age group. Adolescence is an at-risk period because of rapid growth, insufficient calcium intake and/or vitamin D status. Surveys have shown that calcium intake is insufficient (< 1000 mg a day) in 45% of boys and 71% of girls and that vitamin D status is deficient (25-OH-D < 10 ng/ml). The aims of the study carried out by the Calcium Group of the Société Française de Pédiatrie, were to evaluate the frequency of rickets, and to define the criteria for the adolescent population at risk. Forty-one adolescents with rickets were hospitalized between 1985 and 2000. Most of the cases were from the Northern France: 20 from Paris and suburbs, eight from the North-West, four from the North, four from the North-East; five were from the Center of France. The mean age was 13 years and two months for the 28 girls, and 14 years and four months for the 13 boys. Eighty per cent of the adolescents were from immigrant families (33/41): 15 were from sub-Saharan Africa, ten from North Africa, six from Pakistan and two from Turkey. Two thirds of the adolescents were hospitalized in the 2nd quarter of the year. Some adolescents suffered from lower limb pain, 16 had deformations of lower limbs, particularly genu valgum, associated with pain; seven others had either muscle spasms (4), tetany (3). Serum calcium level was low (average 1.84 mmol/l: [1.1-2.5]), and serum 25-OH D level was extremely low. Radiographic characteristics observed were metaphyseal strips on the knees, with condensed edges at times, with the presence of bone demineralization. The treatment combined calcium and vitamin D, and was often administered intravenously when a hypocalcemia was detected. Rickets is not frequent in adolescents, but nonetheless this pathology is not exceptional, and the number of cases is probably under-estimated. Rickets affects immigrant adolescents in particular but nevertheless could also present a certain risk period for the general population.

Background. Cancer is the second most important cause of death for children aged less than 15 years in France, unintentional injuries being the leading cause. The aim of the present study was to estimate the incidence of childhood cancer from six Childhood Cancer Registries covering 32% of France. Procedure. Incident cancer cases diagnosed between 1990 and 1999 in children (0-14 years) resident in the administrative areas covered by each Registry were included. Annual age-standardized rates (ASRs) were adjusted by the world population. The estimated annual percent change (EAPC) Was Used to measure trend towards changes in the annual age-standardized incidence rate. Results. With 4234 registered cases, the ASRs per million children were 137.5 for all cancers combined, 42.3 for leukemia, 29.1 for central-nervous-system tumors, 15.6 for lymphomas, 14.1 for sympathetic-nervous-system tumors, and 9.1 for renal tumors. The ASR of all cancers combined was slightly higher in males (145.8 per million children) than in females (128.7 per million children) with an M/F ratio of 1.2. No significant incidence trend was observed, with an EAPC of +0.2% [IC 95% (-2.5; +3.0); P=0.891. Conclusions. The estimated incidence rates are similar to those reported in previous studies in European and North American countries. These results will contribute to the development of National Registration of Childhood Cancer in France and support the national research prograrn on childhood cancer. (C) 2004 Wiley-Liss, Inc.

INTRODUCTION:Some necrotizing vasculitis may be associated with familial Mediterranean fever (FMF). We report a new case of polyarteritis nodosa (PAN) that preceded the diagnosis of FMF. OBSERVATION:A young woman of Turkish origin had a long childhood history of inflammatory arthralgia and myalgia, leading to the provisional diagnosis of chronic juvenile arthritis, then, after a confirmative muscle biopsy, to the diagnosis of PAN, whose outcome remained benign. At the age of 19, she was diagnosed as having FMF on clinical and genetic grounds, and colchicine led to the regression of most symptoms. DISCUSSION:As with Henoch-Schönlein's purpura, PAN seems significantly associated with FMF. Its characteristics are a younger age at onset, more frequent peri-renal hematoma, overlap between classical PAN and micropolyangeitis, and overall better prognosis. In its muscular form, PAN is difficult to distinguish from protracted febrile myalgia, a recently described manifestation of FMF, in which pathological findings are poorly documented.

We present the case of a breast-fed 5-month-old infant who presented with pancytopenia, secondary to intense myelofibrosis during the winter months because of undiagnosed rickets. The patient responded to oral vitamin D with rapid resolution of symptoms. Secondary hyperparathyroidism was the probable cause of the myelofibrosis.Conclusion Although nutritional rickets remains a problem in developing countries, children in northern climates in industrialized countries may also be at risk. Rickets must be considered when assessing myelofibrosis in a very young child.

Thirty-two children with Henoch–Schönlein purpura with or without renal symptoms were studied to characterize the IgA hyperglobulinemia observed in the serum of most patients. It was shown that only the IgA1 subclass concentration was increased. Secretory IgA and IgA to gliadin levels were frequently increased in serum, with a good correlation between them. Circulating IgA immune complexes were detected often and contained high activity to gliadin. In contrast, IgA activity to tetanus toxoid did not change. We failed to show any differences in renal involvement. These data suggest that elevation of serum IgA in Henoch–Schönlein purpura is due in part to a disturbance of the gut mucosal immune system, and the presence of circulating IgA immune complexes with dietary antigens can be postulated but cannot explain the occurrence of urinary symptoms.

BACKGROUND:The syndrome of generalized resistance to thyroid hormones is more frequent than was thought.CASE REPORTS:A 13-year old girl was examined for her short stature. Evaluation of her thyroid function showed increased levels of IT3 and IT4 and normal value of TSH; she also had mosaic Turner's syndrome. Her cousin was rapidly diagnosed as suffering from the same syndrome because of moderately high thyrotropic levels found during neonatal screening; this syndrome was confirmed by molecular biology tests. Five generations of this family were identified as being affected with a pattern indicating autosomal dominant inheritance.CONCLUSION:The clinical manifestations of familial generalized resistance to thyroid hormones vary but this syndrome is easy to biologically confirm. The importance of diagnosing affected children as early as possible should be emphasized, as in such cases their development must be closely monitored particularly where their growth and neurodevelopment are concerned.

Le syndrome de résistance généralisée aux hormones thyroïdien res paraît plus fréquent qu'on ne le pensait. On doit l’évoquer devant toute hyperhormonémic thyroïdienne avec un taux de TSH non effondré, le diagnostic est parfois établi dès la période néonatale grâce au dépistage.

Background. - The syndrome of generalized resistance to thyroid hormones is more frequent than was thought.Case reports. - A 13-year old girl was examined for her short stature. Evaluation of her thyroid function showed increased levels of IT3 and IT4 and normal value of TSH; she also had mosaic Turner's syndrome. Her cousin was rapidly diagnosed as suffering from the same syndrome because of modertely high thyrotropic levels found during neonatal screening; this syndrome was confirmed by molecular biology rests. Five generations of this family were identified as being affected with a pattern indicating autosomal dominant inheritance.Conclusion. - The clinical manifestations of familial generalized resistance to thyroid hormones vary but this syndrome is easy to biologically confirm. The importance of diagnosing affected children as early as possible should be emphasized, as in such cases their development must be closely monitored particularly where their growth and neurodevelopment are concerned.