Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028754 (obesity)
 124,988 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of hypothalamic dysfunction in a girl with a twelve-year follow-up is reported. Onset occurred at the age of three with severe obesity, hypothermia, hypersomnia, and lethargy. Somatotropic, gonadotropic, and thyrotropic hormones were low, whereas prolactin was increased. Imaging techniques failed to disclose any lesion of the hypothalamus or pituitary. Clomipramine improved the vegetative disorders. The literature on clinical and hormonal disorders of hypothalamic dysfunction is reviewed.
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PMID:[Hypothalamic syndromes. Review of clinical and endocrinal semiology]. 823

A 9 1/2-year-old Taiwanese boy with Prader-Willi syndrome had the following characteristics: difficulties with sucking, feeding and hypotonia during infancy, a dysmorphic face (triangular mouth, high arched palate, almond-shaped eyes and large head circumference with a relatively narrow bifrontal diameter), borderline intelligence, hypogonadism, hyperphagia, skin picking and truncal obesity. The boy experienced two hypersomnia episodes, at age 8 and 9 years, with both episodes lasting for 10 days. During the two episodes, he was found to have an exacerbated case of hyperphagia, pica, poor emotional control, stereotyped speech and agitated behavior upon awakening. After each episode, the boy had complete remission. Our findings show that the two episodes are compatible with Kleine-Levin syndrome. The relationship between the two syndromes, the Prader-Willi syndrome and the Kleine-Levin syndrome, deserves further study.
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PMID:Kleine-Levin syndrome in a boy with Prader-Willi syndrome. 865 Apr 57

Excessive daytime sleepiness in the general community is a newly recognized problem about which there is little standardized information. Our aim was to measure the levels of daytime sleepiness and the prevalence of excessive daytime sleepiness in a sample of Australian workers and to relate that to their self-reported sleep habits at night and to their age, sex, and obesity. Sixty-five percent of all 507 employees working during the day for a branch of an Australian corporation answered a sleep questionnaire and the Epworth sleepiness scale (ESS) anonymously. Normal sleepers, without any evidence of a sleep disorder, had ESS scores between 0 and 10, with a mean of 4.6 +/- 2.8 (standard deviation). They were clearly separated from the "sleepy" patients suffering from narcolepsy or idiopathic hypersomnia whose ESS scores were in the range 12-24, as described previously. ESS scores > 10 were taken to represent excessive daytime sleepiness, the prevalence of which was 10.9%. This was not related significantly to age (22-59 years), sex, obesity, or the use of hypnotic drugs but was related significantly but weakly to sleep-disordered breathing (frequency of snoring and apneas), the presence of insomnia, and reduced time spent in bed (insufficient sleep).
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PMID:Daytime sleepiness and sleep habits of Australian workers. 941 43

Sleep disorders are very prevalent in the general population and are associated with significant medical, psychological, and social disturbances. Insomnia is the most common. When chronic, it usually reflects psychological/behavioral disturbances. Most insomniacs can be evaluated in an office setting, and a multidimensional approach is recommended, including sleep hygiene measures, psychotherapy, and medication. The parasomnias, including sleepwalking, night terrors, and nightmares, have benign implications in childhood but often reflect psychopathology or significant stress in adolescents and adults and organicity in the elderly. Excessive daytime sleepiness is typically the most frequent complaint and often reflects organic dysfunction. Narcolepsy and idiopathic hypersomnia are chronic brain disorders with an onset at a young age, whereas sleep apnea is more common in middle age and is associated with obesity and cardiovascular problems. Therapeutic naps, medications, and supportive therapy are recommended for narcolepsy and hypersomnia; continuous positive airway pressure, weight loss, surgery, and oral devices are the common treatments for sleep apnea.
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PMID:Sleep and its disorders. 1007 85

The obstructive sleep apnea syndrome (OSAS) is caused by an intermittent and repetitive obstruction of the upper respiratory tract during sleep, which leads to a complete (apnea) or partial (hypopnea) block of air flow. It is quite prevalent, being seen in 4-6% of males and 2% of females. Structural abnormalities present in the upper respiratory tract and obesity are the fundamental etiological factors. Clinical manifestations are due to sleep fragmentation and oxygen desaturation which cause the apnea. Day hypersomnia, snoring and episodes of apnea described by the spouse are the three basic symptoms. The diagnosis is based on polysomnography, which can be substituted for a night cardiorespiratory polygraphy. It has an important morbimortality rate, mainly due to traffic and labor accidents, ischemic heart disease and chronic respiratory failure. The treatment is multifactorial. First, eliminating alcohol and hypnotic drugs. Obesity, which is almost always present, must also be corrected. Structural abnormalities of the upper respiratory tract may require a surgical solution. The treatment preferred nowadays is the application of a nasal continuous positive airway pressure (CPAP) while the patient is asleep. It should be considered for those symptomatic patients with an apnea-hypopnea index over 30, or if the index is below 30, than when a respiratory insufficiency or cardiovascular risk factors are present. In some cases surgical procedures may be considered, such as uvulopalatopharyngoplasty.
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PMID:[Obstructive sleep apnea syndrome]. 1219 5

Individuals with Prader-Willi syndrome (PWS) generally survive into adulthood. Common causes of death are obesity related cor pulmonale and respiratory failure. We report on a case series of eight children and two adults with unexpected death or critical illness. Our data show age-specific characteristics of PWS patients with fatal or life-threatening illnesses. Under the age of 2 years, childhood illnesses in general were associated with high fever and rapid demise or near-demise. Hypothalamic dysfunction likely plays a role in exaggerated fever response, but also perhaps in central regulation of adrenal function. Below average sized adrenal glands were found in three children, which raises the possibility of unrecognized adrenal insufficiency in a subset of individuals with PWS and emphasizes the vital role of autopsy. The tub drowning death of an adult patient could be related to central hypersomnia, which has been reported in PWS. We suggest that increased risk for critical illness be considered in the discussion of anticipatory guidance for the care of infants with PWS. Since a number of children died while hospitalized, particularly close observation of PWS children who are ill enough to warrant hospital admission is recommended.
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PMID:Unexpected death and critical illness in Prader-Willi syndrome: report of ten individuals. 1469 14

Prader-Willi Syndrome (PWS) is a genetic disorder characterized by hypotonia, mental retardation or learning disability, hyperphagia and compulsive eating due to hypothalamic dysfunction. Obesity is a major cause of increased morbidity and mortality among patients with PWS. Gastric restrictive surgery has been associated with partial breakdown of the staple-line in PWS. We report two patients with PWS associated with morbid obesity and obstructive sleep apnea who underwent biliopancreatic diversion (BPD). A 27-year-old male with BMI 52 kg/m(2) and a 20 year-old female with BMI 64 kg/m(2) underwent BPD. No perioperative complications were observed. After BPD, the male's BMI was 36.7 kg/m(2) at 12 months and the female's BMI was 48.4 kg/m(2) at 28 months, with excess weight loss 58% and 48%, respectively. They developed loose stools associated with eating. These patients have shown a considerable improvement in hypersomnia and respiratory difficulties. BPD proved to be an effective approach to weight loss in PWS, resulting in improvement of sleep apnea, behavior problems and quality of life.
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PMID:Results of biliopancreatic diversion in two patients with Prader-Willi syndrome. 1597 69

Histamine H(3) receptor antagonists are potential therapeutic agents for cognitive dysfunction, epilepsy, hypersomnia and obesity. GT-2331 (4-[(R,R)-2-(5,5-dimethyl-1-hexynyl)cyclopropyl]-1H-imidazole) was originally identified as a potent histamine H(3) receptor antagonist. However, recent reports demonstrated a complex pharmacology for GT-2331. To further understand the pharmacological profile of GT-2331, we characterized GT-2331 using various in vitro and in vivo assays. In vitro, GT-2331 behaved as a full agonist on adenylyl cyclase inhibition and as a partial agonist on [(35)S]GTPgammaS binding at the recombinant rat histamine H(3) receptor. In contrast, in vivo, GT-2331 had no effect on brain histamine turnover while the histamine H(3) receptor agonist R-alpha-methylhistamine significantly decreased histamine turnover. Furthermore, GT-2331 completely blocked R-alpha-methylhistamine-induced water intake, suggesting that GT-2331 behaves as a full antagonist. Thus, GT-2331 displayed the spectrum of pharmacological activities from full agonism to full antagonism, these observations suggest that histamine H(3) receptor ligands need to be carefully evaluated in various paradigms.
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PMID:Detailed pharmacological characterization of GT-2331 for the rat histamine H3 receptor. 1631 45

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

Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder characterized by mental retardation and distinct physical, behavioral, and psychiatric features. Based on parents' questionnaires, we examined the prevalence of behavioral and psychiatric disorders of 165 persons with PWS aged 2-31 years in Japan. The data were analyzed comparing four different age groups with PWS: group 1, 2-5 years (n=34); group 2, 6-11 years (n=57); group 3, 12-17 years (n=45); and group 4, 18-31 years (n=29). Further, we compared the results of our PWS group 4 with those of 42 age-, gender-, and intelligence level-matched intellectual disability (ID) individuals without PWS. Our results showed that repetitive speech and stubbornness were prominent from early childhood and other behavioral problems such as hyperphagia, stealing food, temper tantrums, lying, and emotional lability tended to be more frequent with age among persons with PWS. Moreover, young adults with PWS have significantly higher rates of behavioral and psychiatric disorders than IDs without PWS, such as stubbornness, hyperphagia, temper tantrums, self-injurious behavior (skin picking), hypersomnia, inactivity, and delusion. Degree of obesity was not necessarily related to behavioral and psychiatric features associated with PWS. Our findings revealed that persons with PWS are more vulnerable to behavioral and psychiatric disorders particularly in young adulthood compared to those with ID from other etiologies in Japan.
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PMID:Behavioral and psychiatric disorders in Prader-Willi syndrome: a population study in Japan. 1731 21


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