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Query: UMLS:C0028754 (obesity)
 124,988 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In patients with craniopharyngioma, pituitary failure and often lifelong hormone replacement therapy, persisting ophthalmological problems with impaired vision, cranial nerve palsies, and psychoneurological deficits will lead to a persisting impairment in quality of life and social competence. Some patients completely depend on assistance. To prevent this, a major goal is an early diagnosis and limitation of operative radicality to minimize postoperative complications. Surgery in centers with special expertise is mandatory. Radiotherapeutical strategies are relatively safe and improve the outcome and recurrence-free survival after incomplete surgical resection or after local recurrence. Multidisciplinary concepts and prospective data acquisition are desirable with regard to therapy and outcome in patients with craniopharyngioma. Problems in the follow-up period are the development of atherosclerotic complications and metabolic syndrome as a consequence of occasionally excessive obesity, which may impair the long-term survival of the patients. Cause and progression of these complications are not fully understood, therapeutical strategies for the morbid obesity are not available. Only interdisciplinary co-operation will help to develop and evaluate therapeutical concepts for the management of this rare disease.
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PMID:[CranioNet -- an interdisciplinary strategy for craniopharyngioma]. 1660 3

Craniopharyngiomas are benign suprasellar tumors; however, their tendency to recur after resection and the risks associated with aggressive tumor resection pose a surgical dilemma. We reviewed our series of operated craniopharyngiomas and selected pediatric cases operated as first-hand cases and followed in our institution. We studied 37 cases. Resection was total in 65%. No patient died in the operative period; three patients died 6 months to 8 years after surgery of endocrine-related cause or sudden death. Tumor progression occurred in 93% of cases after subtotal resection versus 43% after total resection. Among 20 operations for recurrence, total resection was achieved in 45%. Visual deficits were often stabilized or improved after surgery, but worsened in 30% because of surgical damage or tumor recurrence. Fifty-nine percent of patients follow a normal school curriculum. All patients have some degree of pituitary hormonal replacement and 48% have obesity. Hypothalamic damage was generally associated with intraventricular extension of the tumor recurrence and re-operations, especially through combined approaches. Craniopharyngiomas in children are particularly aggressive tumors. Although the best oncological results are obtained with total resection, in some cases the functional price of surgery may be too high and alternative techniques should be proposed. The life-long management of these patients requires that the best use is made of all potential therapeutic tools.
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PMID:Radical resection for craniopharyngiomas in children: surgical technique and clinical results. 1670 Mar 8

Prognosis in childhood craniopharyngioma survivors hinges upon late effects such as pituitary deficiency and obesity. Observations indicate that reduced physical activity and increased daytime sleepiness might be risk factors for obesity. We analyzed the degree of daytime sleepiness in 115 childhood craniopharyngioma patients (47% obese) using the Epworth Sleepiness Scale (ESS). Thirty-five (30%) displayed increased daytime sleepiness (ESS score > 10) of whom 14 were obese (26% of obese cohort). Polysomnography (PSG) and Multiple Sleep Latency Tests (MSLT) were conducted with 10 obese patients presenting increased daytime sleepiness, with only two craniopharyngioma patients revealing a sleep related breathing disorder. Four patients had repeated episodes of SOREM (sleep onset rapid eye movement), the classic PSG criterion for narcolepsy. Three patients displayed hypersomnia. All but one patient qualified as acutely obese. We speculate that secondary narcolepsy is an exacerbating condition of childhood craniopharyngioma obesity, supported by recent reports on orexin and narcolepsy which suggest hypothalamic failure in idiopathic narcolepsy.
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PMID:Secondary narcolepsy may be a causative factor of increased daytime sleepiness in obese childhood craniopharyngioma patients. 1670 Mar 20

Severe obesity is a major problem in patients suffering from craniopharyngioma (CP), a benign tumor located in pituitary and hypothalamic regions. In this study, the hypothesis that hypothalamic damage leads to a reduction in overall sympathetic tone was tested. Catecholamines, as well as their metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA), markers of catecholamine turnover, were measured in morning voided urine of 109 patients participating in a German pediatric CP study, and their physical activity was analyzed using a questionnaire. HVA and VMA results were compared with age-matched HVA and VMA in urine of patients proven to not have a catecholamine-secreting tumor. Patients with the most severe obesity displayed the lowest urine HVA and VMA values. Patients with hypothalamic CP had 3.2-fold higher BMI values (p<0.0001), lower HVA (0.72-fold, p<0.001), and VMA (0.84-fold, p<0.01) values, and significantly lower activity scores than those without hypothalamic involvement, but their epinephrine- and norepinephrine/creatinine ratios were not significantly different, possibly due to low levels. The low HVA and VMA values suggest decreased sympathetic outflow contributing to reduced physical activity and severe obesity, especially in patients with a hypothalamic tumor. In further studies investigating treatment options for hypothalamic obesity, disturbed sympathetic tone should be considered.
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PMID:Reduced sympathetic metabolites in urine of obese patients with craniopharyngioma. 1751 78

The neuropsychological functioning of patients who had undergone surgical removal of craniopharyngiomas was compared to that of an endocrine control group composed of patients with nontumor hypopituitarism, an obese control group, and a normal control group. Neuropsychological assessments consisting of measures of intelligence, memory, attention, and executive functioning were carried out. The craniopharyngioma group had lower Performance IQ than did the normal control group, but their Performance IQ was comparable to that of the hypopituitarism and obese control groups. The craniopharyngioma patients did not differ in Verbal or Full Scale IQs from the remaining groups. There were no group differences on measures of verbal or nonverbal memory, ability to sustain attention, or executive functioning including measures of verbal or figural fluency, nonverbal problem solving, ability to copy a complex geometric figure, and visual motor and visual sequencing skills. The group mean scores on the measures of intelligence and neuropsychological abilities for the craniopharyngioma patients were in the low-average to average range. While craniopharyngioma patients can have significant morbidity including endocrine and visual deficits as well as obesity resulting from hyperphagia, neuropsychological deficits are not always present. Their neuropsychological outcome is more benign than some previous studies have suggested.
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PMID:Neuropsychological functioning following craniopharyngioma removal. 1860 90

Obesity is a common complication of treatment for some childhood cancers, particularly acute lymphoblastic leukaemia (ALL) and craniopharyngioma. Evidence-based guidance is available for the general paediatric population on the diagnosis, aetiology, consequences, prevention and treatment of obesity, and this should be considered as the starting point for considering such issues in patients with malignancy. In ALL, a high proportion of patients show rapid and excessive weight gain soon after diagnosis which originates partly in lifestyle, in particular via markedly reduced levels of physical activity. Good evidence on risk factors for obesity in ALL is available, and the natural history and aetiology of obesity in ALL are now fairly well understood, while for craniopharyngioma the natural history is reasonably well understood. Understanding the natural history and aetiology of obesity should facilitate preventive interventions in the future. Evidence on preventive interventions is required urgently, and it should focus on promotion of a reduction in sedentary behaviour and increases in physical activity. Such interventions should be helpful in obesity prevention, but could also have a wide range of additional benefits in the prevention or amelioration of other late effects of treatment.
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PMID:Obesity during and after Treatment for Childhood Cancer. 1929 3

Craniopharyngioma is a rare, benign, suprasellar brain tumor associated with a significant number of endocrine and metabolic impairments. Growth hormone deficiency, caused by the tumor itself or by its subsequent surgical treatment, is the most common hormone deficiency in these patients and replacement is frequently necessary. Hypothalamic obesity observed after surgery treatment, whether combined with radiotherapy or not, presents with increased abdominal fat and altered lipid profiles and is likely caused by both disruption of the mechanisms controlling satiety, hunger and energy balance and impairment of sensitivity to leptin, insulin and ghrelin axis. It is well known that hyperlipemia is associated with acute pancreatitis, both as a precipitant and as an epiphenomenon. Moreover, the increased incidence of acute pancreatitis during growth hormone therapy is possibly due to increased enzyme production (e.g., amylase, lipase and elastase). We report the case of a 13-year-old girl affected by craniopharyngioma on growth hormone replacement treatment who developed acute pancreatitis. We suggest including routine evaluation of lipid profile during follow-up of all children on growth hormone treatment, especially those affected by hypopituitarism secondary to craniopharyngioma, given pancreatic adverse effects of growth hormone replacement therapy and associated metabolic impairment due to hypothalamic obesity.
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PMID:Acute pancreatitis in a girl with panhypopituitarism due to craniopharyngioma on growth hormone treatment. A combination of risk factors. 1950 96

Relationships of blood circulating melanocortins to childhood obesity are not well established. We evaluated serum alpha-melanocyte-stimulating hormone (alpha-MSH) in lean children and different study groups of childhood obesity. We examined serum alpha-MSH in 52 otherwise healthy children with childhood obesity (Ob; mean age, 11 years; 32 girls/20 boys), 27 normal-weight children of same age, 7 additional obese patients with reduced melanocortin-4 receptor function (MC4Rmut), and 22 patients with craniopharyngioma (CP). Fasting serum alpha-MSH and leptin were measured by radioimmunoassay. Serum alpha-MSH was also evaluated 1 hour after 500-kcal liquid meal (CP and Ob) and at the end of 1-year lifestyle intervention in 24 Ob patients. The alpha-MSH levels were similar in obese vs lean children but significantly lower in CP (P < .001) and significantly higher (P < .05) in MC4Rmut patients compared with Ob. One hour after liquid meal, alpha-MSH increased in patients with Ob but not with CP. After 1 year, alpha-MSH levels increased significantly in the successful weight reduction Ob subgroup despite unchanged cortisol levels. The alpha-MSH changes correlated to weight status changes (r = 0.67, P = .0003) but not to changes of cortisol, insulin, or homeostasis model assessment of insulin resistance index. Persistently low alpha-MSH levels in CP patients are suspected to be due to pituitary or hypothalamic damage. High peripheral levels in MC4Rmut carriers indicate up-regulation of alpha-MSH. Changes of weight status are associated with changes of peripheral alpha-MSH.
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PMID:Changes of peripheral alpha-melanocyte-stimulating hormone in childhood obesity. 1976 64

Treatment of craniopharyngioma (CP) in childhood can lead to severe, debilitating obesity with devastating medical and psychological outcomes. Despite sustained nutritional and exercise-oriented interventions, no efficacious medical option is available for hypothalamic obesity. We describe two adolescents who developed morbid obesity and significant comorbidities following diagnosis and treatment of CP, in whom bariatric surgery was achieved, illustrating a novel approach for symptomatic hypothalamic obesity, as well as positive and negative outcomes.
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PMID:Outcome after bariatric surgery in two adolescents with hypothalamic obesity following treatment of craniopharyngioma. 1996 Aug 98

Craniopharyngiomas are embryogenic malformations of the sellar region of low histological malignancy, thought to be derived from Rathke's pouch epithelium. With an overall incidence of 0.5-2.0 new cases per million of the population per year, 30-50% of all cases occur in childhood. Overall survival rates are high. However, quality of survival is substantially reduced in many survivors, owing to long-term sequelae, such as extreme obesity caused by hypothalamic tumor involvement, which occurs in up to 40% of all cases. The treatment of craniopharyngioma remains controversial. Radical surgical strategies are associated with poor outcome in craniopharyngioma with hypothalamic involvement.
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PMID:Childhood craniopharyngioma: current controversies on management in diagnostics, treatment and follow-up. 2036 5


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