Resting energy expenditure in children at risk of hypothalamic dysfunction

Schaik, Jiska van; Burghard, Marcella; Lequin, Maarten H.; Maren, Ea van; Dijk, Am van; Takken, Tim; Rehorst, Lb; Bakker, Boudewijn; Meijer, Lisethe; Hoving, EW; Fiocco, Marta; Meeteren, Antoinette Y. N. Schouten–van; Tissing, Wim J. E.; Santen, Hanneke M van

doi:10.1530/ec-22-0276

Cited by 4 publications

(2 citation statements)

References 60 publications

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“…Recent studies have been attached increasingly importance to decreased energy expenditure in the development of HyOb. Previous studies have reported a decreased energy expenditure in patients with hypothalamic damage regardless of body composition, and this lowered energy expenditure was related with severe clinical HD ( 40 – 42 ). These findings help explain HyOb in our patients without excessive appetite.…”

Section: Discussionmentioning

confidence: 84%

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Metabolic syndrome as a common comorbidity in adults with hypothalamic dysfunction

Yuan

et al. 2022

Front. Endocrinol.

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ObjectiveHypothalamic dysfunction (HD) results in various endocrine disorders and is associated with an increased risk of metabolic comorbidities. This study aimed to analyze the clinical characteristics and metabolic abnormalities of adults with HD of various causes.MethodsThis study retrospectively reviewed adults with HD treated at our center between August 1989 and October 2020. Metabolic characteristics of patients were compared to those of age-, sex-matched lean, and body mass index (BMI)-matched controls.ResultsTemperature dysregulation (61.0%) was the most common hypothalamic physiological dysfunction. At least one anterior pituitary hormone deficiency was observed in 50 patients (84.7%), with hypogonadotropic hypogonadism being the most frequently observed. Metabolic syndrome was confirmed in 31 patients (52.5%) and was significantly more prevalent in those with panhypopituitarism or overweight/obesity. Metabolic syndrome (MetS) was significantly more common in patients with HD than in both lean and BMI-matched controls (P < 0.001 and P = 0.030, respectively). Considering the components of MetS, elevated fasting glucose levels were significantly more common in patients with HD than in BMI-matched controls (P = 0.029). Overweight/obesity and panhypopituitarism were significant risk factors for MetS in the multivariate analysis on patients with HD. Moreover, in the multivariate analysis on patients and BMI-matched control, HD was a significant risk factor of MetS (P=0.035, OR 2.919) after adjusted for age, sex and BMI.ConclusionsTemperature dysregulation and hypogonadotropic hypogonadism are the most common physiological and endocrine dysfunctions, respectively. MetS and unfavorable metabolic profiles were prevalent in adults with HD. HD was a significant risk factor of MetS after adjusted for BMI.

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Section: Discussionmentioning

confidence: 84%

“…These findings help explain HyOb in our patients without excessive appetite. Measurement of resting energy expenditure can be useful for these patients by helping to determine whether they can benefit from therapy targeting at energy expenditure, such as dexamphetamines ( 2 , 40 ).…”

Section: Discussionmentioning

confidence: 99%

Metabolic syndrome as a common comorbidity in adults with hypothalamic dysfunction

Yuan

et al. 2022

Front. Endocrinol.

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Treatment and outcome of the Dutch Childhood Craniopharyngioma Cohort study: First results after centralization of care

et al. 2023

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Introduction Childhood craniopharyngioma (cCP) has excellent survival, but quality of life may be severely hampered by hypothalamic dysfunction. We aimed to evaluate treatment and hypothalamic outcomes of a Dutch cCP cohort, and evaluate the effect of centralization of care. Methods A retrospective cohort study was performed, including cCP patients diagnosed between 2004-2021. Treatment characteristics and hypothalamic outcomes were evaluated and compared before and since centralization of care in May 2018. Results We included 87 cCP patients. Cyst drainage/fenestration was performed in 29.9%, limited resection in 27.6%, near total resection in 16.1%, and gross total resection (GTR) in 25.4%. Radiotherapy was given in 46.0%. After a median follow-up of 6.5 years, hypothalamic obesity (HO) was present in 24.7% and panhypopituitarism with diabetes insipidus in 71.3%. Higher BMI SDS at diagnosis and Muller grade II at last MRI of follow-up were associated to overweight/obesity. No association was found between extensiveness of resection and overweight/obesity at last follow-up. When comparing before and after centralization of care, rates of GTR remained similar, but BMI outcomes changed; mean ΔBMI SDS 1 year after diagnosis from 1.12 (SD 1.15) to 0.81 (SD 1.24), and HO after 1 year decreased from 33.3% to 12.0% (p = 0.067), and after 2 years from 28.6% to 6.7% (p = NS). Conclusion In our nationwide cohort, GTR was performed in a relatively low percentage of patients and extensiveness of resection was no longer associated to HO at follow-up. A trend towards improvement of BMI is observed since centralization of care, which needs further exploration.

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Outcome measures in Angelman syndrome

Hagenaar,

Bindels-de Heus,

van Gils

et al. 2024

J Neurodevelop Disord

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Background Angelman syndrome (AS) is a rare neurodevelopmental disorder characterized by severe intellectual disability, little to no expressive speech, visual and motor problems, emotional/behavioral challenges, and a tendency towards hyperphagia and weight gain. The characteristics of AS make it difficult to measure these children’s functioning with standard clinical tests. Feasible outcome measures are needed to measure current functioning and change over time, in clinical practice and clinical trials. Aim Our first aim is to assess the feasibility of several functional tests. We target domains of neurocognitive functioning and physical growth using the following measurement methods: eye-tracking, functional Near-Infrared Spectroscopy (fNIRS), indirect calorimetry, bio-impedance analysis (BIA), and BOD POD (air-displacement plethysmography). Our second aim is to explore the results of the above measures, in order to better understand the AS phenotype. Methods The study sample consisted of 28 children with AS aged 2–18 years. We defined an outcome measure as feasible when (1) at least 70% of participants successfully finished the measurement and (2) at least 60% of those participants had acceptable data quality. Adaptations to the test procedure and reasons for early termination were noted. Parents rated acceptability and importance and were invited to make recommendations to increase feasibility. The results of the measures were explored. Results Outcome measures obtained with eye-tracking and BOD POD met the definition of feasibility, while fNIRS, indirect calorimetry, and BIA did not. The most important reasons for early termination of measurements were showing signs of protest, inability to sit still and poor/no calibration (eye-tracking specific). Post-calibration was often applied to obtain valid eye-tracking results. Parents rated the BOD POD als most acceptable and fNIRS as least acceptable for their child. All outcome measures were rated to be important. Exploratory results indicated longer reaction times to high salient visual stimuli (eye-tracking) as well as high body fat percentage (BOD POD). Conclusions Eye-tracking and BOD POD are feasible measurement methods for children with AS. Eye-tracking was successfully used to assess visual orienting functions in the current study and (with some practical adaptations) can potentially be used to assess other outcomes as well. BOD POD was successfully used to examine body composition. Trial registration Registered d.d. 23-04-2020 under number ‘NL8550’ in the Dutch Trial Register: https://onderzoekmetmensen.nl/en/trial/23075

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Resting energy expenditure in children at risk of hypothalamic dysfunction

Cited by 4 publications

References 60 publications

Metabolic syndrome as a common comorbidity in adults with hypothalamic dysfunction

Metabolic syndrome as a common comorbidity in adults with hypothalamic dysfunction

Treatment and outcome of the Dutch Childhood Craniopharyngioma Cohort study: First results after centralization of care

Outcome measures in Angelman syndrome

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