1/17. pseudohypoparathyroidism--another monogenic obesity syndrome.obesity is a common feature of pseudohypoparathyroidism (PHP) type 1a, but is usually associated with short stature. We describe two children referred because of hyperphagia and excessive weight gain from early infancy. Tall stature in both children initially confounded the diagnosis of PHP, but on follow-up both children developed the typical hormonal abnormalities and Case 2 developed typical skeletal features of Albright hereditary osteodystrophy. PHP type 1a is caused by germline loss of function mutations in the alpha subunit of GS, the ubiquitously expressed G protein that couples many hormone receptors to the adenylate cyclase second messenger system. Recent evidence suggest that the hypothalamic GS protein coupled melanocortin-4 receptor (MC4R) may mediate the central effects of leptin on inhibition of satiety. Similar patterns of infancy onset hyperphagia, excessive weight gain and tall stature are seen in subjects with congenital leptin deficiency and in subjects with MC4R mutations. We suggest that the genetic mutations in GSalpha which underlie PHP type 1a may also directly result in severe obesity. This diagnosis should be considered in any child with a history of hyperphagia and early onset morbid obesity.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
2/17. A boy with normal growth in spite of growth hormone deficiency after resection of a suprasellar teratoma.We reported a boy with panhypopituitarism after removal of a suprasellar teratoma and pituitary stalk transection at the age of 3 months. His growth was accelerated after 5 years of age without growth hormone (GH) therapy, although he had poor height growth until age 4 under treatment with hydrocortisone, levothyroxine sodium, and desamino-D-arginine vasopressin (DDAVP). hyperphagia and obesity developed after surgery. Endocrinological examination revealed no GH response to glucagon, low serum levels of insulin-like growth factor (IGF)-1 and IGF binding protein-3 (IGFBP-3). serum prolactin was normal, but serum insulin was high. Some patients who received an operation for craniopharyngioma were reported to achieve normal growth without GH secretion, but the mechanism is still unknown. High serum levels of prolactin or insulin can be associated with normal IGF in GH deficient patients. This patient had obesity and high serum insulin, which may be related to growth without GH secretion.- - - - - - - - - - ranking = 0.33333333333333keywords = obesity (Clic here for more details about this article) |
3/17. Morbid obesity and hyperphagia in the wagr syndrome.A 33-year-old man with wagr syndrome is described with morbid obesity associated with hyperphagia and an apparent lack of satiety. It is possible that a gene associated with satiety is present at 11p13 although it is premature to conclude that obesity is a specific feature of wagr syndrome.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
4/17. hyperphagia, severe obesity, impaired cognitive function, and hyperactivity associated with functional loss of one copy of the brain-derived neurotrophic factor (BDNF) gene.The neurotrophin brain-derived neurotrophic factor (BDNF) inhibits food intake, and rodent models of BDNF disruption all exhibit increased food intake and obesity, as well as hyperactivity. We report an 8-year-old girl with hyperphagia and severe obesity, impaired cognitive function, and hyperactivity who harbored a de novo chromosomal inversion, 46,XX,inv(11)(p13p15.3), a region encompassing the BDNF gene. We have identified the proximal inversion breakpoint that lies 850 kb telomeric of the 5' end of the BDNF gene. The patient's genomic dna was heterozygous for a common coding polymorphism in BDNF, but monoallelic expression was seen in peripheral lymphocytes. serum concentration of BDNF protein was reduced compared with age- and BMI-matched subjects. haploinsufficiency for BDNF was associated with increased ad libitum food intake, severe early-onset obesity, hyperactivity, and cognitive impairment. These findings provide direct evidence for the role of the neurotrophin BDNF in human energy homeostasis, as well as in cognitive function, memory, and behavior.- - - - - - - - - - ranking = 1.1666666666667keywords = obesity (Clic here for more details about this article) |
5/17. Hypothalamic or central obesity is associated with an early rise in plasma insulin concentration.insulin levels in a 7-year-old boy with hyperphagia and obesity following an episode of meningoencephalitis were studied sequentially during the course of progressive weight gain. High fasting insulin levels (1183 pmol/L) and strikingly high insulin release in response to glucose (7892 pmol/L) were found within weeks of the onset of the illness. The abnormality in insulin secretion occurred prior to the marked weight gain. Hyperinsulinemia was not accompanied by hypoglycemia. Early hyperinsulinemia may be a primary event in the development of hyperphagia and obesity following hypothalamic injury.- - - - - - - - - - ranking = 1keywords = obesity (Clic here for more details about this article) |
6/17. Hypothalamic obesity due to hydrocephalus caused by aqueductal stenosis.A case is presented of 14 year old female with hypothalamic obesity due to hydrocephalus caused by aqueductal stenosis. Evidence of hypothalamic obesity included 1) acute hyperphagia and weight gain, 2) neuroradiology showed hydrocephalus with focal enlargement of the third ventricle, 3) endocrinological studies revealed hyperinsulinaemia and impaired growth hormone (GH) response to arginine, but normal GH response to growth hormone-releasing factor (GRF) and 4) Torkildsen's ventriculo-cisternal shunting resulted in improvement in hyperphagia and obesity.- - - - - - - - - - ranking = 1.1666666666667keywords = obesity (Clic here for more details about this article) |
7/17. Abnormal food-seeking behavior after surgery for craniopharyngioma.Three patients are described in whom surgical removal of a craniopharyngioma was followed by extreme hyperphagia resulting in obesity and abnormal food-seeking behavior, including foraging for food, stealing food or stealing money for food. These behaviors resemble those seen in the prader-willi syndrome but contrast with those noted in bulimia. This deviant behavior was a major factor in the poor outcome of surgery. Attempts at rehabilitation were unsuccessful.- - - - - - - - - - ranking = 0.16666666666667keywords = obesity (Clic here for more details about this article) |
8/17. hyperphagia and obesity. Relationship to medial hypothalamic lesions.Progressive left hemiparesis followed by face and trunk cutaneous vasodilation and hyperphagia developed in a 28-year-old man. He began eating five to six meals a day and gained 16 kg in 60 days. Computed tomography disclosed a neoplastic lesion involving the midline via the hypothalamus and reaching the contralateral lenticular nucleus. Findings from endocrine studies, including thyroid-stimulating hormone, growth hormone, prolactin, and cortisol serum levels, were normal. hyperphagia and consequent obesity were associated with bilateral destruction of the ventromedial hypothalamic area; cutaneous vasodilation was related to involvement of the preoptic area.- - - - - - - - - - ranking = 0.83333333333333keywords = obesity (Clic here for more details about this article) |
9/17. Reduction of food theft and long-term maintenance of weight loss in a Prader-Willi adult.Behavioral interventions have had limited success in managing the chronic hyperphagia and obesity that are of presumed organic etiology in prader-willi syndrome. Thus, frequent foraging for food and covert consumption continue to be health-threatening problems for many Prader-Willi individuals. This case study was designed to replicate methods for assessment and treatment of food theft. A token program based on differential-reinforcement-of-other-behavior and response-cost eliminated theft in three hospital settings. Prior to discharge, the program was expanded to include contingencies on exercise behavior and weight loss, and staff from the subject's group home residence were trained to implement a modified program in the natural environment. Reduced food theft and continued weight loss were maintained in the group home and an apartment-living arrangement. A total of 81 lb (37 kg) was lost during a 2-yr period.- - - - - - - - - - ranking = 0.16666666666667keywords = obesity (Clic here for more details about this article) |
10/17. bulimia: diagnostic clues.bulimia is an eating disorder characterized by the ingestion of large amounts of food, usually followed by self-induced vomiting or laxative abuse. Although sometimes a symptom of obesity or anorexia nervosa, bulimia is often associated with borderline weight and nutritional status and thus may be difficult to detect. Since secrecy and shame accompany this syndrome, patients are reluctant to seek treatment. We present ten diagnostic clues for identifying bulimic patients: (1) preoccupation with weight, (2) gastrointestinal complaints, (3) dental and oropharyngeal changes, (4) salivary gland enlargement, (5) edema and bloating, (6) amenorrhea, (7) dermatologic complaints, (8) substance abuse, (9) laboratory changes, and (10) serious consequences. A case study illustrates the major features of the disorder and its treatment.- - - - - - - - - - ranking = 0.16666666666667keywords = obesity (Clic here for more details about this article) |
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