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  • Author: H M van Santen x
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Open access

Resting energy expenditure in children at risk of hypothalamic dysfunction

J Van Schaik, M Burghard, M H Lequin, E A van Maren, A M van Dijk, T Takken, L B Rehorst-Kleinlugtenbelt, B Bakker, L Meijer, E W Hoving, M Fiocco, A Y N Schouten-van Meeteren, W J E Tissing, and H M van Santen

Objective

Children with suprasellar brain damage are at risk of hypothalamic dysfunction (HD). HD may lead to decreased resting energy expenditure (REE). Decreased REE, however, is not present in all children with HD. Our aim was to assess which children suspect for HD have low REE, and its association with clinical severity of HD or radiological hypothalamic damage.

Patients and methods

A retrospective cohort study was performed. Measured REE (mREE) of children at risk of HD was compared to predicted REE (pREE). Low REE was defined as mREE <90% of predicted. The mREE/pREE quotient was associated to a clinical score for HD symptoms and to radiological hypothalamic damage.

Results

In total, 67 children at risk of HD (96% brain tumor diagnosis) with a mean BMI SDS of +2.3 ± 1.0 were included. Of these, 45 (67.2%) had low mREE. Children with severe HD had a significant lower mean mREE/pREE quotient compared to children with no, mild, or moderate HD. Mean mREE/pREE quotient of children with posterior hypothalamic damage was significantly lower compared to children with no or anterior damage. Tumor progression or tumor recurrence, severe clinical HD, and panhypopituitarism with diabetes insipidus (DI) were significant risk factors for reduced REE.

Conclusion

REE may be lowered in children with hypothalamic damage and is associated to the degree of clinical HD. REE is, however, not lowered in all children suspect for HD. For children with mild or moderate clinical HD symptoms, REE measurements may be useful to distinguish between those who may benefit from obesity treatment that increases REE from those who would be better helped using other obesity interventions.

Open access

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

S C Clement, W E Visser, C A Lebbink, D Albano, H L Claahsen-van der Grinten, A Czarniecka, R P Dias, M P Dierselhuis, I Dzivite-Krisane, R Elisei, A Garcia-Burillo, L Izatt, C Kanaka-Gantenbein, H Krude, L Lamartina, K Lorenz, M Luster, R Navardauskaitė, M Negre Busó, K Newbold, R P Peeters, G Pellegriti, A Piccardo, A L Priego, A Redlich, L de Sanctis, M Sobrinho-Simões, A S P van Trotsenburg, F A Verburg, M Vriens, T P Links, S F Ahmed, and H M van Santen

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.