Pediatric Endocrine Disorders of Energy Balance

Lustig, Robert H.

doi:10.1007/s11154-005-6183-1

Cited by 11 publications

(14 citation statements)

References 181 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Hypothetically, the insulin-resistant state could represent a sign of the disruption of the hypothalamic-pituitary signalling pathways, as already described in craniopharyngioma patients [34-36]. Only 1 patient developed type 2 diabetes, and it is possible that in this patient, endogenous excessive GH production and acute pancreatitis contributed to the development of diabetes.…”

Section: Discussionmentioning

confidence: 83%

Endocrine Long-Term Follow-Up of Children with Neurofibromatosis Type 1 and Optic Pathway Glioma

Sani

Albanese

2017

Horm Res Paediatr

View full text Add to dashboard Cite

Background/Aims: Children with optic pathway glioma (OPG) face sequelae related to tumour location and treatment modalities. We aimed to assess the prevalence of hypothalamic-pituitary dysfunctions in children with neurofibromatosis type 1 (NF1) and OPG who did not receive radiotherapy or surgical resection. The causative role of tumour location on endocrinopathy development is investigated. Methods: A retrospective follow-up study of 40 children with NF1 and OPG evaluated between August 1996 and May 2015 was undertaken. Patients who underwent radiotherapy or surgical resection were excluded and 36 patients were studied. Tumour location was classified according to the Dodge criteria: stage I, optic nerve alone; stage II, optic chiasm with or without optic nerve involvement; and stage III, involvement of the hypothalamus or other adjacent structures. Results: Endocrinopathies were diagnosed in 20/36 (55.6%) children during a mean follow-up of 9.1 (0.2–13.6) years: 0/4 OPGs were Dodge stage I, 12/21 (57.1%) stage II, and 8/11 (72.7%) stage III. The first endocrinopathy was found at a mean age of 7.4 (5.0–13.2) years, 2.4 (0–6.7) years after tumour diagnosis. We found growth hormone deficiency (GHD; 36.1%), central precocious puberty (33.3%), obesity with insulin resistance/impaired glucose tolerance (11.1%), early puberty (5.5%), GH excess (5.5%), ACTH deficiency (5.5%), hypogonadotropic hypogonadism (2.7%), and thyrotropin deficiency (2.7%). GHD was transient in all of those who were retested. Conclusion: This population is at high risk of endocrinopathies due to tumour location. Lifelong endocrine follow-up is recommended.

show abstract

Section: Discussionmentioning

confidence: 83%

Endocrine Long-Term Follow-Up of Children with Neurofibromatosis Type 1 and Optic Pathway Glioma

Sani

Albanese

2017

Horm Res Paediatr

View full text Add to dashboard Cite

show abstract

“…Damage to satiety centres with increased food intake and subsequent leptin and insulin resistance may play a role in the rapid weight gain 4,5 . In addition, hypersecretion of insulin secondary to hypothalamic‐damage‐induced vagal disinhibition has been proposed as an important mechanism leading to obesity in this population 6 …”

Section: Introductionmentioning

confidence: 99%

Insulin sensitivity and secretion in children and adolescents with hypothalamic obesity following treatment for craniopharyngioma

Simoneau-Roy

O’Gorman

Pencharz

et al. 2010

Clinical Endocrinology

View full text Add to dashboard Cite

SummaryBackground Craniopharyngioma (CP), a tumour occurring in the hypothalamic-pituitary area, results in morbid obesity in 25-60% of affected children. It has been suggested that abnormalities of insulin secretion and/or insulin action due to hypothalamic injury may be associated with weight gain and the metabolic syndrome in this population. Aim To evaluate: (i) insulin secretion (IS) and insulin sensitivity (Si); (ii) features of the metabolic syndrome (MS) and (iii) factors involved in risk for diabetes and heart disease in obese youth treated for CP. Methods Obese subjects treated for CP were compared to BMImatched control subjects. All subjects underwent anthropometric, blood pressure, resting energy expenditure and body fat assessment. Cholesterol and inflammatory markers, oral glucose tolerance test (OGTT) and frequent sampling intravenous glucose tolerance test (FSIGT), with calculation of IS and Si, were performed. Results Fifteen CP subjects and 15 controls (C) were studied. There were no differences between CP and C for age, gender, BMI or pubertal status. MS was present in 10/15 CP and 3/15 C (P = 0AE03), including impaired glucose tolerance (IGT) in 6/15 CP and 0/15 C (P = 0AE02). Measures of IS, including first and second phase IS, and insulin area-under-the-curve (AUC ins ) during OGTT, were significantly higher in CP. Si, measured by frequent sampled intravenous glucose tolerance test (Si-FSIGT), was significantly lower in CP subjects (0AE96 ± 0AE34 vs. 1AE67 ± 0AE7; P = 0AE01). AUC ins was negatively correlated with Si-FSIGT (r = )0AE62; P = 0AE003).

show abstract

“…Inability to store triglycerides in adipose tissue causes its deposition in liver and skeletal muscle causing fatty liver, insulin resistance and eventually type 2 diabetes [7]. Lack of adipose tissue also results in a decrease in the secretion of adipocytokines which leads to disturbance in food intake and energy expenditure [8]. …”

Section: Discussionmentioning

confidence: 99%

Severe Resistance to Weight Gain, Lack of Stored Triglycerides in Adipose Tissue, Hypoglycaemia, and Increased Energy Expenditure: A Novel Disorder of Energy Homeostasis

et al. 2012

View full text Add to dashboard Cite

Background: Growth during childhood is a consequence of the equilibrium of energy balance. Obesity results from a shift of the equilibrium towards increased energy intake over expenditure. A clinical description of extreme leanness and failure to thrive secondary to a shift of the equilibrium towards increased energy expenditure over energy intake has not been previously described in the medical literature. Subjects and Methods: We report the case of a female child born premature with a birth weight of 1.1 kg who presented with extreme failure to thrive, persistent hypoglycaemia, paucity of fat in the adipose tissue with increased brown fat and increased resting energy expenditure. Results: Complete cessation of weight and height was noted between 3 months to 3.5 years of age. Hypoglycaemia was secondary to depleted energy stores and increased insulin sensitivity. Increased resting energy expenditure was demonstrated on indirect calorimetric assessment. Biopsy of adipose tissue demonstrated paucity of stored fat with increase in brown fat. No gain in weight and height was demonstrated despite high calorie intake of enteral and parenteral feeds. Conclusion: We describe a unique case of extreme failure to thrive with increased energy expenditure and severe hypoglycaemia. Unravelling the molecular basis of this novel disorder has the potential to provide insights into the prevention of obesity.

show abstract

Pediatric Endocrine Disorders of Energy Balance

Cited by 11 publications

References 181 publications

Endocrine Long-Term Follow-Up of Children with Neurofibromatosis Type 1 and Optic Pathway Glioma

Endocrine Long-Term Follow-Up of Children with Neurofibromatosis Type 1 and Optic Pathway Glioma

Insulin sensitivity and secretion in children and adolescents with hypothalamic obesity following treatment for craniopharyngioma

Severe Resistance to Weight Gain, Lack of Stored Triglycerides in Adipose Tissue, Hypoglycaemia, and Increased Energy Expenditure: A Novel Disorder of Energy Homeostasis

Contact Info

Product

Resources

About