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Psychiatric disorders in Prader-Willi syndrome: epidemiology and management.

Although people with intellectual disabilities are at increased risk for psychiatric disorders, the type and rate of these problems differ between those with different causes for their retardation. In this paper, we review behavioural and psychiatric problems in persons with Prader-Willi syndrome, a disorder caused by a paternally derived deletion at chromosome 15(q11-q13) in about 70% of affected patients, and by maternal uniparental disomy in the majority of the remaining patients. In addition to the syndrome's characteristic hyperphagia and food seeking, individuals with Prader-Willi syndrome also have increased risks of nonfood, compulsive behaviours. These include skin picking, which is highly prevalent, as well as more variable rates of hoarding, redoing and concerns with symmetry, exactness, cleanliness, ordering and arranging. Relative to others with mental retardation, persons with Prader-Willi syndrome are at a marked increased risk for developing full-blown, obsessive-compulsive disorder. In addition, many people with Prader-Willi syndrome show increased rates of tantrums, oppositionality and aggression. Recent findings suggest that they also have an increased risk of psychotic disorder or affective illness with a psychotic component, especially young adult patients and those with the maternal uniparental disomy as opposed to paternal deletion. Dietary approaches include a reduced-calorie diet and increased physical activity, as well as close supervision around food and keeping food locked away. To date, neither CNS stimulants nor anorectic agents have been effective in treating hyperphagia, in part because hyperphagia in Prader-Willi syndrome is attributed to decreased satiation as opposed to increased hunger. Treatment for compulsivity and maladaptive behaviours include: behavioural programming; a structured, predictable routine; extra help with transitions; family support; and pharmacotherapy. Although formal drug studies have yet to be conducted, SSRIs have been effective in reducing skin picking, compulsivity and aggressive episodes in some individuals with Prader-Willi syndrome. Atypical antipsychotics have also proven helpful in persons with psychotic features or extreme aggression and impulsivity. Largely on the basis of case studies, the risks and benefits of these and other drugs in Prader-Willi syndrome are reviewed. Drug trials that move beyond case studies and that assess the relative efficacy of behavioural treatments alone or in combination with pharmacotherapy are sorely needed.

Antipsychotic Agents↗

Demonstration of insulin transformation products in insulin vials by high-performance liquid chromatography.

OBJECTIVE: To assess the importance of temperature, light, agitation, and regular withdrawal of insulin on the rates of appearance of three groups of insulin transformation products (ITPs) in vials of human and beef soluble insulin. RESEARCH DESIGN AND METHODS: Twelve insulin-treated patients (6 receiving human soluble insulin, 6 receiving beef soluble insulin) participated in the study. Reverse-phase high-performance liquid chromatography was used to measure ITP in serial samples from vials stored in various laboratory environments or issued to patients. Vials in the laboratory were analyzed on nine occasions over 26 wk; those issued to patients were analyzed weekly. RESULTS: Results were expressed as rates of change (% total protein/wk). Except for human insulin stored at 4 degrees C, sampling did not accelerate transformation. Storage at higher temperatures caused more rapid transformation to all ITP groups, and human insulin was more susceptible than beef insulin. Exposure to light accelerated transformation mainly to group 3 ITP. Fibrillation occurred in beef but not human insulin carried in a shirt pocket. Results from patients' vials were consistent with reported storage conditions. CONCLUSIONS: All three ITP groups form in human and beef soluble insulin. Their rate of production is a function of temperature and light exposure. Differences between the two insulins were probably due to differences in formulation. Patients should be discouraged from hoarding vials and storing insulin in direct sunlight. One or more ITPs could contribute to the residual immunogenicity of modern insulin preparations.

Analysis of Variance↗

Sources of animal science research information: usefulness and reliability.

Fifty-two animal scientists (8 private consultants, 22 feed industry representatives, 22 university personnel) were surveyed regarding frequency of use and reliability of information from 27 different publications and information sources. Among the information sources, these scientists (6 dairy specialists, 25 beef cattle specialists, 17 swine specialists, 4 dealing with multiple species) most frequently scanned Feedstuffs, the Journal of Animal Science, National Research Council (NRC) species bulletins, abstracts from regional and national meetings of either ASAS or ADSA, annual reports from experiment stations, and proceedings from state nutrition conferences. Differences among species specialties were detected: dairy specialists read the Journal of Dairy Science, abstracts from ADSA meetings, Dairy Herd Management, Hoard's Dairyman quite extensively; beef specialists read Beef and National Cattleman frequently; and swine specialists used the Pfizer Conference, National Hog Farmer, and Pork 95. Frequency of use rankings of publications were surprisingly similar for feed industry and university specialists; however, private consultants tended to use certain publications (Professional Animal Scientist, Feed Management, Beef Today) to a greater degree. For reliability, Journal of Animal Science, Journal of Dairy Science, NRC bulletins, the Professional Animal Scientist, Pfizer Report, and reports from ASAS and ADSA meetings received the highest rankings, and university workers ranked reliability of NRC publications and Animal Feed Science and Technology higher than feed industry personnel. Regarding timeliness of information, Feedstuffs, National Hog Farmer, Pork 95, and reports from state nutrition conferences ranked best, and NRC bulletins, Journal of Animal Science, and Journal of Dairy Science ranked lowest. Applicability of information was correlated with frequency of use (r = .38**) and presumed reliability (r = .59**). Asked whether some formal appraisal of articles appearing in the popular press by a panel of specialists would be desirable, 88% of the scientists, especially private consultants and university personnel, favored or were neutral toward pre- or postpublication appraisal. Mechanisms to institute such an appraisal system are outlined and ethical responsibilities of researchers, reviewers, administrators, and societies related to research information are discussed.

Animal Husbandry↗

Recognition and Management of Behavioral Disturbances in Dementia.

Behavioral disturbances are seen in most patients with dementia at some point in their course. They cause immense patient suffering and are responsible for caregiver stress, institutionalization, and hospitalization. Identification of predisposing and precipitating factors is very important. The approach to the management of behavioral disturbances in dementia patients should be structured and thorough. Ensuring the safety of the patient and others should be paramount. Addressing the causes of behavioral disturbances such as comorbid medical illnesses, polypharmacy, pain, personal need, environmental factors, etc. is critical to a successful outcome. Many behavioral disturbances such as wandering and hoarding are not amenable to pharmacotherapy. Nonpharmacologic interventions are the mainstay of managing behavioral disturbances. Success of pharmacologic interventions will depend on accurate identification of specific syndromes, e.g., depression-anxiety and psychosis and severity of symptoms. Response to pharmacologic interventions is usually modest and may be associated with significant symptom resolution. Many behavioral disturbances can be prevented by avoiding inappropriate medications and educating patient, family, caregivers, and health care providers. Hospitalization can be avoided and institutionalization delayed by early recognition and treatment of behavioral disturbances. Leadership from physicians to implement preventive measures is recommended. Research to clarify the biological underpinnings of behavioral disturbances and to address cost-effectiveness of currently identified interventions is needed.

Journal Article↗

Tobacco use among school children in Chennai city, India.

AIMS: The present study was done to build a database on prevalence of tobacco use among students of grade 8 to 10 in Chennai city, for the purpose of advocacy of tobacco control and planning tobacco control interventions and evaluation. MATERIALS AND METHODS: A two-stage stratified probability sample of students in grades 8-10 corresponding to 13 to 15 years of age were selected from private/government aided private schools and purely government aided corporation schools. Data was collected by a pretested, closed-ended self-administered questionnaire. RESULTS: A total of 1255 students participated in this survey. Among them 64.4 boys and 35.6% were girls. Ever tobacco use was reported by 37.6% of the students (41.6 males and 30.2% females). Current users of tobacco (any products) were reported by 41.1% of the students. Prevalence was more among boy students (46.3%) when compared to that of girl students (31.6%). There existed no significant difference between current users of tobacco based on the zones of the school. Tobacco users prevalence was found more in corporation schools when compared to that of private schools. Parental and friends tobacco use was reported more often by tobacco users compared to never users. Purchasing tobacco products in a store was reported by 82.5% and almost no one was refused because of age. Almost everyone reported watching a lot of cigarette advertisements on TV, whereas about half reported watching advertisements on other medias like outdoor hoardings (45.7%), newspapers (65.3%) and social events (67.4%). CONCLUSION: This study demonstrates that among the 13 to 15-year old school going children (corresponding to grades 8 to 10) in Chennai city, the current tobacco use is high.

Adolescent↗

Shared information boosts competition in healthcare networks.

The time has come for healthcare providers to stop hoarding information. By building healthcare information networks, providers at all levels can gain a competitive edge not by "owning" information, but by better using richer, more complete, "shared" information.

Comprehensive Health Care↗

Which is the real Kaiser?

Fans of Kaiser Permanente, the nation's oldest and largest HMO, consider the company a cost-effective healthcare delivery system that supports its patients, caregivers and the practice of high-quality medicine. Its detractors see Kaiser as an evil HMO empire that hoards money, mistreats doctors and skimps on care. The dichotomy shows that Kaiser is like a giant mirror, reflecting the struggles and uncertainties of the evolving U.S. healthcare system.

Attitude to Health↗

Getting ready for professional-risk capitation.

California has long stood as the harbinger for things to come to the rest of the health care nation. Based on research in the state, Maurice Penner warns physician group managers to begin developing a geographically-diverse and credentialed network of physicians to attract the coming hoards of managed care plans. Compensation under capitation, he adds, is worth some advance planning.

California↗

Sports sponsorship by cigarette companies influences the adolescent children's mind and helps initiate smoking: results of a national study in India.

To estimate the effect of large scale tobacco sponsorship of cricket, a study was conducted on children's knowledge and perceptions about smoking and their impact on subsequent smoking uptake. Twelve nations played 36 matched in the Wills World Cup-1996 cricket series over one month during which Wills (a cigarette brand) was extensively advertised by live broadcast to a 2-billion viewers with WILLS logo on the players' T-shirts and playground, newspapers, magazines, and hoardings. An anonymous structured questionnaire including 4 knowledge based questions about tobacco, 4 about perceptions directly promoted by Wills and 10 questions related to wrong perceptions about smoking was administered by class teachers six months after the series. A total of 5822 children (65% boys and 35% girls) in Grade 10, aged 13-17 years (median 14) were selected. Smokers increased from 137 (2.4%) before the series to 649 (11.1%) after the series. The smoking initiation rate was 2.04% (13/636) in children with full knowledge and no wrong perceptions, 7.8% (48/618) among those with less knowledge and no wrong perceptions. Among those with less knowledge but believing in at least 2 of Wills related perceptions and 3 of the smoking related perceptions the rate for smoking initiation was 20.55% (127/618). The sponsorship appeared to have a similar effect on initiation rates in both sexes despite the strong social taboo against girls smoking in India. Wrong perceptions about smoking promoted by tobacco sponsorship increases smoking initiation amongst both boys and girls even when they are aware of the risks involved. The study suggests that education, without bans on advertisements is unlikely to stop initiation of smoking among children.

Adolescent↗

Adult service refusers in the greater Dublin area.

The reclusive behaviour and living conditions of people living in the community can be a source of concern to others, especially when statutory and voluntary support are refused. A cross-sectional observational study of service providers was carried out to profile 'service refusers' in the greater Dublin area and to describe the support offered. Two hundred and thirty three subjects were identified. Most were female (127, 54.5%) and 65 years of age or older (162, 69.5%). Approximately 50% (117, 50.2%) were living in private accommodation. The majority of subjects were considered to exhibit traits of poor personal hygiene (171, 73.4%), suspiciousness (160, 68.7%), secrecy (144, 61.8%) or hoarding (126, 54.1%). Forty-seven per cent of subjects (110) identified met the criteria for 'Diogenes Syndrome'. A wide variety supports had been offered including meals-on-wheels, home-helps and house cleaning. Respondents considered that 51.9% (121) of subjects had not been offered a service which proved successful. Despite the best efforts of agencies, service refusers can remain steadfast in not availing of the help that is on offer.

Adult↗

[Medicine and its museums].

There are described in this article the historical patrimonies belonging to five museums of Cordoba city, Argentina: the Museo de anatomia, which was named after Pedro Ara, notable Spanish Anatomist; the Museo de Anatomia Patologica; the Museo de Historia de la Medicina, created by Prof. Enrique P. Aznarez; the Museo "Obispo Salguero" of the Hospital San Roque; and the Museo Historico del Hospital Nacional de Clinicas, declared national historic monument. All these museums have a rich historic hoard, reflecting one of the important cultural aspects of this province.

Argentina↗

[Cyclothymic obsessive-compulsive disorder. Clinical characteristics of a neglected and under-recognized entity].

OBJECTIVE: Clinical research is largely focused on depressive comorbidity in obsessional compulsive disorder (OCD). However some recent publications have suggested that bipolar comorbidity occurs in authentic OCD and its presence has a differential impact on the clinical picture and course of OCD. METHOD: Recent data from the collaborative survey conducted with AFTOC (French Association of patients suffering from OCD) have revealed a high rate of bipolar comorbidity in OCD: 30% for hypomania and 50% for cyclothymia. RESULTS: The present paper presents further comparative analyses between OCD with (n = 302) versus without cyclothymia (n = 272). The sub-group "Cyclothymic OCD" is characterized by a different clinical picture (higher frequency of aggressive, impulsive, religious and sexual obsessions, and compulsions of control, hoarding, repetition), episodic course, higher rate of major depressive episodes (with more intensity and recurrence) associated with higher rates of suicide attempts and psychiatric admissions, and less favorable response to anti-OCD treatments. CONCLUSION: These data suggested that cyclothymic OCD could represent a specific distinct variant form of OCD. More vigilance is needed toward this entity which is largely under-recognized in clinical practice.

Adolescent↗

[A comparison of the behavioral response of the field mouse (Apodemus sylvaticus L.) to a 24 hour fast as measured by actograph or in a cage].

1. The "general activity" of Apodemus Sylvaticus L. has been recorded and analysed using two techniques: a) in an actograph, several components of the "general activity" have been recorded and quantified over 24 hours, including wheel running, locomotion in various areas of the enclosure, nest occupancy, drinking, eating and hoarding; b) in a breeding cage, ten times smaller than the actograph and where the possibilities of locomotion are considerably reduced, the wheel running only has been recorded. In these two situations, we have compared the effects of a food deprivation for 24 hours. 2. In the actograph, starvation increases the general locomotion in the enclosure without detectable changes in wheel running. On the other hand, in the breeding cage, wheel running is somewhat increased. 3. Refeeding results in decreased wheel running under both experimental conditions, and restores general locomotion in the actograph to the predeprivation level. 4. These results are discussed in view of the current literature. The apparent disagreement between our results and those of other workers is attributed to the fact that the latter used experimental conditions where the measured response was predetermined by the lack of choice in expressed responses which were offered to the animal. Consequently, we suggest that the interpretation of such experiments can be improved by allowing a choice of possible behavioural response and that each of them should be recorded separately.

Animals↗

Stress and distress.

I must ask the reader's indulgence for this article's concern with applications of the stress concept, which are distinct from, although related to clinical medicine. It has not been my object to deal with the way physicians have been aided by stress research in the practice of medicine--that information is already widely available. Rather, I have attempted to sketch briefly the history of the stress theory and to demonstrate how this information can help anyone, physician or layman, lead a more complete and satisfying life. The applications of the stress theory have been dealt with at length elsewhere. I believe that we can find within scientifically verified observations the basis of a code of behavior suited to our century. The great laws of nature that regulate the defenses of living beings against stress of any kind are essentially the same at all levels of life, from individual cells to entire complex human organisms and societies. It helps a great deal to understand the fundamental advantages and disadvantages of catatoxic and syntoxic attitudes by studying the biologic basis of self-preservation as reflected in syntoxic and catatoxic chemical mechanisms. When applied to everyday problems, this understanding should lead to choices most likely to provide us the pleasant eustress (from the Greek eu meaning good, as in euphoria) involved in achieving fulfillment and victory, thereby avoiding the self-destructive distress of frustration and failure. So the translation of the laws governing resistance of cells and organs to a code of behavior comes down to three basic precepts: 1. Find your own natural stress level. People differ with regard to the amount and kind of work they consider worth doing to meet the exigencies of daily life and to assure their future security and happiness. In this respect, all of us are influenced by hereditary predispositions and the expectations of our society. Only through planned self-analysis can we establish what we really want; too many people suffer all their lives because they are too conservative to risk a radical change by breaking with hiabits or traditions. 2. Altruistic egoism. The selfish (i.e., self-interested) hoarding of the goodwill, respect, esteem, support, and love of our neighbors is the most efficient way to give vent to our pent-up energy and to create a more enjoyable, beautiful, or useful environment.3. Earn thy neighbor's love. This motto--which is merely a rewording of the command to "love thy neighbor as thyself"--is compatible with man's natural structure, and although it is based on altruistic egoism, it could hardly be attacked as unethical. Who would blame the man who wants to assure his own homeostasis and happiness only by accumulating the treasure of other poeple's benevolence and love? Yet this makes him virtually unassailable, for nobody wants to attack and destroy those upon whom he depends.

Adaptation, Physiological↗

Interrelationships between mortality and fertility in Germany: rural and urban Prussia and modern Germany.

"The paper investigates the interrelationship between fertility and infant mortality and its economic determinants by time-series methods for historical and modern Germany. It is studied whether the causal effects of infant mortality on fertility have to be considered as hoarding or replacement, and whether the costs of nutrition have an influence on family decision making about demographic variables. Results show that there are indications for replacement motives, and that economic factors matter." (SUMMARY IN FRE AND ITA)

Demography↗

Suicide among nursing home residents: a population-based study.

OBJECTIVE: The rate of suicide is highest among the oldest old and is associated with chronic medical illness and functional impairment. These risk factors are prevalent among nursing home (NH) residents, yet little has been published on suicide in this population. METHODS: We performed a population-based, retrospective cohort study to identify clinical features of NH residents attempting or completing suicide. A computerized data-base search was conducted to identify cases of completed suicide in Olmsted County and surrounding communities (846 NH beds) between 1981 and 1997. Subjects' medical records were reviewed to identify clinical details. RESULTS: Five cases of completed suicide and three suicide attempts were identified, including six men and two women. Deaths were the result of drowning, hanging, or medication overdose (the latter following a period of intentional hoarding). Those who died ranged in age from 69 to 87 years. Most had been NH residents for less than 6 months. No deaths occurred in patients with severe cognitive impairment. CONCLUSIONS: Suicide and suicide attempts in the NH seem to be uncommon but are likely underrecognized and underreported. Suicide risk among NH residents seems to be highest among men and those recently admitted to facilities. In addition, suicide methods differ among NH residents compared with community-dwelling older people, likely reflecting environmental restrictions and physical limitations. Suicide and suicide attempts are important resident health issues and risk management concerns for staff in long-term-care facilities.

Journal Article↗

Pharmacologic treatment of severe skin-picking behaviors in Prader-Willi syndrome. Two case reports.

BACKGROUND: Prader-Willi syndrome (PWS) is characterized by hypotonia at birth, hypogonadism, early childhood obesity, and mental deficiency. Other behavioral symptoms that become prominent during adolescence and adulthood include temper outbursts, stealing and hoarding food, and skin picking. The self-excoriating skin picking behavior observed in individuals with PWS is quite common and can lead to persistent sores and infections, even requiring hospitalization. OBSERVATION: Two patients with PWS who displayed repetitive, self-mutilatory behavior of skin picking are described. They were both treated successfully with different doses of fluoxetine, a selective serotonin reuptake inhibitor. CONCLUSIONS: The skin-picking behavior in patients with PWS may be a variant of the spectrum of obsessive-compulsive disorders. Obsessive-compulsive disorders have been successfully treated with serotonin reuptake inhibitors such as fluoxetine. Thus, fluoxetine may be considered an option in the management of skin-picking behavior in patients with PWS.

Adult↗