Body mass index at diagnosis of a childhood brain tumor; a reflection of hypothalamic-pituitary dysfunction or lifestyle?

Purpose: Childhood brain tumor survivors (CBTS) are at risk of becoming overweight, which has been shown to be associated with hypothalamic-pituitary (HP) dysfunction during follow-up. Body mass index (BMI) at diagnosis is related to BMI at follow-up. It is uncertain, however, whether aberrant BMI at brain tumor diagnosis reflects early hypothalamic dysfunction or rather reflects genetic and sociodemographic characteristics. We aimed to examine whether BMI at childhood brain tumor diagnosis is associated with HP dysfunction at diagnosis or its development during follow-up. Methods: The association of BMI at diagnosis of a childhood brain tumor to HP dysfunction at diagnosis or during follow-up was examined in a Dutch cohort of 685 CBTS, excluding children with craniopharyngioma or a pituitary tumor. Individual patient data were retrospectively extracted from patient charts. Results: Of 685 CTBS, 4.7% were underweight, 14.2% were overweight, and 3.8% were obese at diagnosis. Being overweight or obese at diagnosis was not associated with anterior pituitary deficiency or diabetes insipidus at diagnosis or during follow-up. In children with suprasellar tumors, being obese at diagnosis was associated with central precocious puberty. Conclusion: Overweight or obesity at diagnosis of a childhood brain tumor seems not to be associated with pituitary deficiencies. These results suggest that genetics and lifestyle may be more important etiologic factors for higher BMI at diagnosis in these children than hypothalamic dysfunction. To improve the long-term outcome of CBTS with regards to overweight and obesity, more attention should be given to lifestyle already at the time of brain tumor treatment

Neonatale screening op congenitale hypothyreoïdie : ruim 30 jaar ervaring in Nederland [Neonatal screening for congenital hypothyroidism: more than 30 years of experience in the Netherlands]

Doel Beschrijving van de Nederlandse neonatale screening op congenitale hypothyreoïdie (CH). Opzet Descriptief onderzoek. Methode We verzamelden gegevens over de neonatale screening op CH in de periode 1 januari 1981-31 december 2011 van de Dienst Vaccinvoorziening en Preventieprogramma’s van het RIVM, van laboratoria en van kinderartsen naar wie pasgeborenen met een afwijkende uitslag werden verwezen. De screeningsprocedure werd verscheidene keren bijgesteld. In de periode 1981-1994 werden bijvoorbeeld alleen de waarden van T4 en TSH bepaald uit het hielprikkaartje. Vanaf 1995 werd ook de waarde van thyroxinebindend globuline (TBG) meegenomen. Resultaten De deelname was 99,7%. Tot 1995 was de sensitiviteit 94%, de specificiteit 99,51% en de positief voorspellende waarde 6%; vanaf 1995 was dit respectievelijk 98%, 99,85% en 21%. De totale prevalentie van CH was 1:2670 (prevalentie CH van thyreoïdale oorsprong: 1:3100 en centrale CH: 1:21.600). De percentages kinderen met ernstige CH bij wie in de perioden 1981-1990, 1991-2000 en 2001-2011 vóór de 15e levensdag met thyroxinebehandeling werd gestart, waren respectievelijk 24 (63/263), 63 (170/269) en 96 (176/184). Conclusie De sensitiviteit en specificiteit van het screeningsprogramma is vanaf 1995 aanzienlijk hoger dan vóór 1995. In de recente jaren worden patiënten met ernstige CH aanzienlijk vroeger behandeld dan in de beginjaren. De neonatale screening op CH kan als een belangrijk succes van de openbare gezondheidszorg worden beschouwd

Spatial and temporal expression of immunoglobulin superfamily member 1 in the rat

Diabetes mellitus: pathophysiological changes and therap

Management and consequences of postoperative fluctuations in plasma sodium concentration after pediatric brain tumor surgery in the sellar region : a national cohort analysis

Purpose: Severe fluctuations in plasma sodium concentration and plasma osmolarity, including central diabetes insipidus (CDI), may have significant influence on postoperative morbidity and mortality after pediatric brain tumor surgery.The aim of this study was to describe the frequency, severity and neurological consequences of these fluctuations in pediatric brain tumor survivors. Methods: A retrospective, multi-institutional chart review was conducted among all children who underwent brain tumor surgery in the sellar or suprasellar region in seven university hospitals in the Netherlands between January 2004 and December 2013. Results: Postoperative CDI was observed in 67.5% of 120 included children. Fluctuations of plasma sodium concentration ≥ 10 mmol/L/24 h during the first ten postoperative days were seen in 75.3% of patients with CDI, with a maximum delta of 46 mmol/L/24 h. When compared to patients without CDI, altered mental status occurred more frequently in patients with postoperative CDI (5.1 vs. 23.5% respectively, p = 0.009). Low plasma sodium concentration was related to altered mental status and the occurrence of seizures. Frequency and severity of fluctuations in plasma sodium concentration during the first ten postoperative days were significantly higher in patients with permanent CDI at last follow-up than in patients with transient CDI or without CDI (p = 0.007). Conclusion: Postoperative CDI is a common complication after pediatric brain tumor surgery in the sellar or suprasellar region. Extreme plasma sodium concentrations and large intra-day fluctuations still occur and seem to influence the postoperative neurological course. These results illustrate the need for intensive monitoring in a highly experienced center

Development of a pediatric differentiated thyroid carcinoma registry within the EuRRECa project:rationale and protocol

Background: Although differentiated thyroid carcinoma (DTC) is the most frequent endocrine pediatric cancer, it is rare in childhood and adolescence. While tumor persistence and recurrence are not uncommon, mortality remains extremely low. Complications of treatment are however reported in up to 48% of the survivors. Due to the rarity of the disease, current treatment guidelines are predominantly based on the results of small observational retrospective studies and extrapolations from results in adult patients. In order to develop more personalized treatment and follow-up strategies (aiming to reduce complication rates), there is an unmet need for uniform international prospective data collection and clinical trials. Methods and analysis: The European pediatric thyroid carcinoma registry aims to collect clinical data for all patients ≤18 years of age with a confirmed diagnosis of DTC who have been diagnosed, assessed, or treated at a participating site. This registry will be a component of the wider European Registries for Rare Endocrine Conditions project which has close links to Endo-ERN, the European Reference Network for Rare Endocrine Conditions. A multidisciplinary expert working group was formed to develop a minimal dataset comprising information regarding demographic data, diagnosis, treatment, and outcome. We constructed an umbrella-type registry, with a detailed basic dataset. In the future, this may provide the opportunity for research teams to integrate clinical research questions. Ethics and dissemination: Written informed consent will be obtained from all participants and/or their parents/guardians. Summaries and descriptive analyses of the registry will be disseminated via conference presentations and peer-reviewed publications.</p