The relationship between IGF-I concentration, cognitive function and quality of life in adults with Prader-Willi syndrome.

Mental retardation is one of the clinical characteristics of Prader-Willi syndrome (PWS) and in part of the patients growth hormone deficiency is demonstrable. Cognitive function seems to be influenced by insulin-like growth factor I (IGF-I); however, little is known about cognitive function in relation to IGF-I levels in PWS adults. The aim of the present study was to evaluate cognitive function in adult PWS patients in comparison to healthy siblings and to investigate whether there is a correlation between cognitive function and IGF-I levels. Anthropometric measurements, IGF-I levels, quality of life (QoL), Appetite Assessment Score, IQ (GIT and Raven) and cognitive function (by four subtests of the Cambridge Neuropsychological Automated Testing Battery, CANTAB) were evaluated in PWS patients and their healthy siblings served as control group. PWS patients had significantly lower IGF-I levels, IQ and QoL when compared to controls. Reaction times were longer and performance was worse on CANTAB subtests in PWS adults. IGF-I on one hand and IQ, Appetite Assessment Score and cognitive performance on the other hand seem to be correlated in PWS patients. In conclusion, IGF-I levels, IQ and QoL are significantly lower in PWS subjects when compared to healthy siblings. In PWS adults, temporal as well as prefrontal cognitive functions are impaired. Higher IGF-I levels appear to be related to better intellectual skills and faster temporal memory processing in PWS patients. © 2011 Elsevier Inc

The GH/IGF-I axis and pituitary function and size in adults with Prader-Willi syndrome

Background: In adults with Prader-Willi syndrome (PWS), limited information is available about pituitary function, more specifically the prevalence of growth hormone deficiency (GHD). The aim of this study was to gain more insight into endocrine function in PWS adults, with emphasis on GH secretion. Methods: 15 randomly selected adult PWS individuals were included and 14 healthy brothers and sisters served as a control group. Main outcome measures were IGF-I, IGFBP-3 and peak GH level after a combined GHRH-arginine test. Other pituitary hormone deficits are diagnosed based on serum levels of the concerning hormones. The size of the pituitary gland was measured on MRI images. Results: In PWS adults, IGF-I levels were low and IGFBP-3 levels normal when compared to healthy controls. GHD was diagnosed in 8-38% of the PWS patients, depending on the criteria used. Hypogonadism was present in 87% of the patients. Hypothyroidism and adrenal insufficiency could also be demonstrated. Anterior pituitary size was lower in PWS individuals when compared to healthy controls. Conclusion: In this study, pituitary hormone deficiencies are demonstrated in a considerable number of adults with PWS, hypogonadism and GHD being most prominent. Furthermore, the anterior pituitary is smaller in comparison with healthy controls. Copyright © 2011 S. Karger AG, Basel

Explaining not-in-my-backyard responses to different social groups: the role of group characteristics and emotions

To examine why people are reluctant to engage in intergroup contact, the present study asked members of a nationwide online panel (N = 555) to imagine that they would get individuals of a particular social group as next-door neighbors. Respondents were randomly assigned to one of five different social groups hypothesized to differ in emotion-arousing potential: elderly people, people with mild or severe intellectual disability, economic refugees, and young offenders. It was found that differences in acceptance between these groups could be well explained by emotions aroused while anticipating contact yet less well by differences in previous contact with these groups. Furthermore, emotions appeared to be uniquely related to preferred interpersonal relationships. It is concluded that research on how to reduce prejudice through intergroup contact should be complemented with a better understanding of why people are reluctant to engage in such contact in the first place

The influence of group size and stigma severity on social acceptance: The case of people with intellectual disability moving into neighbourhoods

Dutch adults from a nationwide Internet panel (N=426) were asked to imagine that their next-door neighbours would move out and that people with intellectual disability would move in. Severity of disability and group size were varied to manipulate intergroup threat. These two factors independently influenced social acceptance and a variety of emotional and behavioural measures. In particular, it was found that a large group with severe disability aroused the strongest negative response, whereas a small group with mild disability aroused the weakest negative response. Small groups with a severe disability and large groups with a mild disability aroused similar and intermediate negative responses. Results are discussed in terms of theories of intergroup threat and stigmatisation. Practical implications for predicting the success of de-institutionalisation and social integration of groups with special needs are addressed. Copyright (C) 2011 John Wiley & Sons, Ltd

The influence of group size and stigma severity on social acceptance: The case of people with intellectual disability moving into neighbourhoods

Dutch adults from a nationwide Internet panel (N=426) were asked to imagine that their next-door neighbours would move out and that people with intellectual disability would move in. Severity of disability and group size were varied to manipulate intergroup threat. These two factors independently influenced social acceptance and a variety of emotional and behavioural measures. In particular, it was found that a large group with severe disability aroused the strongest negative response, whereas a small group with mild disability aroused the weakest negative response. Small groups with a severe disability and large groups with a mild disability aroused similar and intermediate negative responses. Results are discussed in terms of theories of intergroup threat and stigmatisation. Practical implications for predicting the success of de-institutionalisation and social integration of groups with special needs are addressed. Copyright (C) 2011 John Wiley & Sons, Ltd

Applicability of induced sputum for molecular dosimetry of exposure to inhalatory carcinogens: P-32-postlabeling of lipophilic DNA adducts in smokers and nonsmokers

The lung is a major target organ for smoking-associated cancer. We examined the applicability of induced sputum for molecular dosimetry of exposure to tobacco smoke-related carcinogens. Sputum induction was performed by inhalation of 4.5% saline delivered from an ultrasonic nebulizer for a period of up to 21 min in a group of smoking (n = 20) and nonsmoking (n = 24) healthy individuals. Samples were analyzed for total and differential cell counts and cell viability, Subsequently, DNA contents of the samples were isolated, and measurement of lipophilic DNA adducts was done by the P-32-postlabeling assay using nuclease P1 (NP1) and butanol enrichment methods. All subjects tolerated the induction procedure without experiencing any troublesome symptoms, and 90% of smokers (18 of 20) and 88% of nonsmokers (21 of 24) succeeded in producing sufficient amounts of sputum. Total cell counts and percentages of viable cells in smokers were higher than those in nonsmokers (6.7 +/- 6.0 versus 4.7 +/- 6.0 x 10(6), P = 0.40 and 80 +/- 15 versus 63 +/- 17, P = 0.01. respectively). In cell differentials, smokers had lower percentages of bronchoalveolar macrophages and higher percentages of neutrophils (69 +/- 24 versus 92 +/- 5, P = 0.002 and 26 +/- 26 versus 4 +/- 4, P = 0.008, respectively). Using the NP1 digestion method, all smokers and only one nonsmoker showed a diagonal radioactive zone in their adduct maps; adduct levels in smokers were higher than those in nonsmokers (3.1 +/- 1.4 versus 0.6 +/- 0.8/10(8) nucleotides; P = 0.0007), and also, adduct levels were significantly related to smoking indices. Applying the butanol extraction method, however, only half of the smokers and three nonsmokers showed the diagonal radioactive zone in their adduct maps; adduct levels in smokers were higher than those in nonsmokers (4.6 +/- 3.7 versus 1.0 +/- 1.9/10(8) nucleotides; P = 0.02), and the levels of adducts were significantly related to the smoking indices. There was a correlation between the levels of adducts determined by the two enrichment methods (r = 0.7; P = 0.02). Paired comparison showed no differences between the levels of adducts measured by the two methods (P = 0.55). We conclude that induced sputum can serve for molecular dosimetry of inhalatory exposure to carcinogens and that the NP1 version of the P-32-postlabeling assay is a choice of preference for studying smoking-induced DNA adducts in the lower respiratory tract

Prevalence of psychopathology in children and adolescents with and without intellectual disability

Background: The main objective of this study was to assess and compare the prevalence of a wide range of emotional and behavioral problems in children with and without intellectual disability (ID). Methods: We studied 1,041 non-residential children randomly selected from special schools for educable (IQ 60 to 80) and trainable (IQ 30 to 60) children without severe additional physical or sensory impairments, and compared them to 1,855 children randomly selected from the general population (both ages 6 to 18). Parents completed the Child Behavior Checklist (CBCL), and teachers the Teacher's Report Form (TRF). Results: Controlling for sex, age, and socioeconomic status, we found that both educable and trainable children had significantly higher mean scores on all CBCL and TRF scales than children without ID, except for trainable children on the scales Anxious/Depressed and Somatic Complaints. Almost 50% of children with ID had a Total Problem score in the deviant range compared to about 18% in children without ID. Compared to children without ID, the most prominent problem behaviors of educable children were Social Problems, Attention Problems, and Aggressive Behavior, and trainable children had an increased risk for Social Problems, Attention Problems, Withdrawn and Thought Problems. Conclusions: Elevated scale scores reflected differences between children with and without ID over a broad range of items, and not solely on items more likely to be related to developmental delay. Therefore, problem areas covered by the items in these scales deserve special attention in the mental health care of children with ID