Successful Weight Loss with Protein-Sparing Modified Fast in a Morbidly Obese Boy with Panhypopituitarism, Diabetes Insipidus, and Defective Thirst Regulation

Lee, Yann-Jinn; Backeljauw, Philippe; Kelly, Patricia; Verdi, P.; Redmond, Geoffrey P.

doi:10.1177/000992289203100409

Cited by 8 publications

(4 citation statements)

References 9 publications

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“…In individuals with both “common” and hypothalamic obesity, lifestyle modification alone can be insufficient to achieve weight loss, though can have many benefits for overall metabolic and mental health and are rightly part of any comprehensive weight loss intervention strategy ( 25 , 26 ). There is some anecdotal evidence for protein-sparing modified fasts and low-carbohydrate diets in individuals with hypothalamic obesity, which is consistent with reports in our cohort that lower-carbohydrate approaches tended to be helpful ( 27 ). Most likely, nutrition and exercise are effective in maximizing the extent of the weight loss response to pharmacologic interventions and ensuring the sustainability of that response.…”

Section: Discussionsupporting

confidence: 91%

A Survey of Patient-Relevant Outcomes in Pediatric Craniopharyngioma: Focus on Hypothalamic Obesity

et al. 2022

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ContextIndividuals treated for pediatric craniopharyngioma, a rare, grade 1 brain tumor, frequently develop hypothalamic obesity, a complication often recalcitrant to intervention. Although hypothalamic obesity is known to adversely impact quality of life, less is known about how caregivers and patients experience this condition.ObjectiveOur goal was to examine the approaches that families take towards weight management and the impact on social function in individuals with craniopharyngioma and obesity. Individuals with craniopharyngioma without obesity were included as a comparison.Subjects and MethodsAdult caregivers of children <18y with craniopharyngioma completed a web-based survey posted by a patient advocacy organization between February and July 2020. Questions related to the child’s diagnosis, medications, lifestyle modifications, and social function along with research priorities. Descriptive statistics were generated. Linear regression was used to assess the independent effects of obesity and other covariates on social function.ResultsOf 106 respondents, 60 (57%) reported their child had obesity at the time of survey completion. In contrast, only 6 (5.7%) had obesity prior to craniopharyngioma diagnosis. A majority (92%) of those with obesity had tried limiting calories or carbohydrates; 31% and 69% found these helpful, respectively. Thirty-eight percent had tried weight loss medications (stimulants, metformin, GLP1R-agonists, and topiramate) and 48% found at least one helpful. Both stimulant and anti-depressant use were reported more frequently with obesity. An index (T-score) reflecting social function was lower in the cohort than a population reference, 41 (SD 11) vs. 50 (SD 10), p<0.001. In a linear model, both older age and obesity were independently associated with greater social impairment. Ninety-four percent of respondents caring for a child with obesity (and 79% of all respondents) identified “improving treatments and prevention for hypothalamic obesity” as a key research priority.ConclusionsOnly a minority of individuals with hypothalamic obesity had trialed medication, even though many reported that lifestyle modification was inadequate. Furthermore, social function was significantly impaired overall in survivors compared to a reference cohort, and even more so in individuals with obesity. These findings highlight the opportunity to improve social functioning as an additional potential benefit of improved treatments for hypothalamic obesity.

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Section: Discussionsupporting

confidence: 91%

A Survey of Patient-Relevant Outcomes in Pediatric Craniopharyngioma: Focus on Hypothalamic Obesity

et al. 2022

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“…Due to disturbances in satiety regulation, central sympathetic output, and energy expenditure, CP patients often develop morbid HO that is mostly nonresponsive to conventional lifestyle modifications (diet and exercise) [55][56][57][58][59] (Table 1). Only 1 case report observed a clinical significant effect of a dietary intervention by a protein-sparing modified fasting program (PSMF) [60]; however, once it had been discontinued, weight gain occurred.…”

Section: Lifestyle and Dietary Interventionsmentioning

confidence: 99%

The Diagnosis and Treatment of Craniopharyngioma

Müller

2019

Neuroendocrinology

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Craniopharyngioma (CP) is a rare embryonic malformation of the sellar/parasellar region with a low histological grade. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. Recent insight into the molecular pathogenesis of CP opens new perspectives on targeted therapy in papillary CP harboring BRAF-V600E mutations. Further research to elucidate pathogenic mechanisms and hopefully prevent hypothalamic involvement of CP is warranted. If the tumor is favorably localized, the therapy of choice is complete resection, with care taken to preserve the optical and hypothalamic functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), the recommended therapy is a limited hypothalamus-sparing surgical strategy followed by local irradiation. Surgical treatment strategies should be based on a multidisciplinary approach involving experienced teams. Centralizing the treatment of CP in experienced "centers of excellence" and multicenter-based networks for reference assessments should be considered to assure a high standard of treatment quality. CP recurrence and progression are frequent. Irradiation has proven effective in reducing recurrences and progression. Proton beam therapy, available in a wider range in the near future, will help to avoid radiooncological side effects. Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious sequelae that compromise quality of life (QoL), such as hypothalamic obesity and psychopathological symptoms. Novel insights into neuropsychological sequelae after CP occurrence should be the basis for the development of therapeutic neuropsychological interventions. CP should be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' clinical and QoL consequences by experienced multidisciplinary teams.

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“…In addition, unsuccessful weight loss through caloric restriction and exercise is believed to be due in part to the marked decline in the resting energy expenditure from the perceived “starvation response” of increased vagal tone and decreased leptin sensitivity. Even in those patients who are motivated to reduce their energy intake and increase their activity, dietary modifications are only sporadically beneficial [27]. This may be related to other factors, such as a low energy expenditure, the home environment, and behavioral problems.…”

Section: Etiologymentioning

confidence: 99%

“…Since then, there have been few scattered case studies [27] showing the benefits of protein-sparing modified fasting or limitation of carbohydrates in individuals suffering from HO. Buoyed by these reports, some centers encourage low-carbohydrate diets for the treatment of HO with varying degrees of anecdotal success, especially at a level of 50 g of carbohydrates per day.…”

Section: Treatmentmentioning

confidence: 99%

Hypothalamic Obesity: Prologue and Promise

Abuzzahab

Roth

2019

Horm Res Paediatr

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Hypothalamic obesity (HO) frequently occurs following damage to the medial hypothalamic region, encompassing the arcuate nucleus, the paraventricular nucleus, the ventromedial nucleus, the dorsomedial nucleus, and the dorsal hypothalamic area, which are critically involved in the regulation of satiety and energy balance through neural and humoral connections. HO is most commonly described in the context of craniopharyngioma and its treatment, but it can also occur following other suprasellar tumors, radiation, trauma, or a surgical insult to the hypothalamus. A constellation of loss of satiety and a reduction of the metabolic rate, thermogenesis, and physical activity as well as increased vagal tone and hyperinsulinism with insulin and leptin resistance results in rapid weight gain due to a decreased energy expenditure and increased energy storage in adipose cells. To date, no viable long-term solution for HO has been found, due either to the requirement of intact hypothalamic pathways or to significant side effects. Newer therapeutic modalities focused on the unique pathophysiology of this condition offer potential for successful treatment. In this review, we describe the etiology of HO as well as past/current treatment approaches in the categories of hyperinsulinism, surgical approaches, and targeting energy expenditure/anorectic drugs. We conclude by providing an overview of the clinical trials currently underway.

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Successful Weight Loss with Protein-Sparing Modified Fast in a Morbidly Obese Boy with Panhypopituitarism, Diabetes Insipidus, and Defective Thirst Regulation

Cited by 8 publications

References 9 publications

A Survey of Patient-Relevant Outcomes in Pediatric Craniopharyngioma: Focus on Hypothalamic Obesity

A Survey of Patient-Relevant Outcomes in Pediatric Craniopharyngioma: Focus on Hypothalamic Obesity

The Diagnosis and Treatment of Craniopharyngioma

Hypothalamic Obesity: Prologue and Promise

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