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Angelman syndrome (AS) is a complex, heterogeneous, and life-long neurodevelopmental disorder. Despite the considerable impact on individuals and caregivers, no disease-modifying treatments are available. To support holistic clinical management and the development of AS-specific outcome measures for clinical studies, we conducted primary and secondary research identifying the impact of symptoms on individuals with AS and their unmet need. This qualitative research adopted a rigorous step-wise approach, aggregating information from published literature, then evaluating it via disease concept elicitation interviews with clinical experts and caregivers. We found that the AS-defining concepts most relevant for treatment included: impaired expressive communication, seizures, maladaptive behavior, cognitive impairment, motor function difficulties, sleep disturbance, and limited self-care abilities. We highlight the relevance of age in experiencing these key AS concepts, and the difference between the perceptions of clinicians and caregivers towards the syndrome. Finally, we outline the impact of AS on individuals, caregivers, and families.

Develops a descriptive model utilizing grounded theory to classify sex offenders' cognitions concerning their offending behavior. The model consists of four categories that were tested to determine their content validity and reliability. Results suggest that the model has provisional validity and adequate interrater reliability. Discusses the theoretical and clinical implications of the model. (Author/MKA)

Distorted or maladaptive thinking is widely acknowledged as an important variable in both the initiation and maintenance of sexual offending. The aim of this study was to develop a descriptive model to classify sex offenders' cognitions concerning their offending behavior. Offense descriptions were obtained from 20 incarcerated male child sex offenders undergoing assessment for a sex offender treatment program A data-driven approach to model development -- grounded theory -- was taken in the qualitative analysis of these offense accounts. The resulting model consisted of four categories: offense chain, cognitive operations, cognitive content, and metavariates. To determine its content validity and reliability, the model was applied to the offense descriptions of a sample of 25 incarcerated child molesters also undergoing pretreatment assessment. Results suggest that the model has provisional validity and adequate interrater reliability. Finally, the authors discuss the theoretical and clinical implications of the model, The study of cognitive factors in sexual offending has been hampered by the lack of a framework to guide model building and empirical research. There have been a number of [...]

Abstract Purpose To examine the psychometric properties of the Injection Pen Assessment Questionnaire (IPAQ) including the following: 1) item and scale characteristics (e.g., frequencies, item distributions, and factor structure), 2) reliability, and 3) validity. Methods Focus groups and one-on-one dyad interviews guided the development of the IPAQ. The IPAQ was subsequently tested in 136 parent–child dyads in a Phase 3, 2-month, open-label, multicenter trial for a new Genotropin® disposable pen. Factor analysis was performed to inform the development of a scoring algorithm, and reliability and validity of the IPAQ were evaluated using the data from this two months study. Psychometric analyses were conducted separately for each injection pen. Results Confirmatory factor analysis provides evidence supporting a second order factor solution for four subscales and a total IPAQ score. These factor analysis results support the conceptual framework developed from previous qualitative research in patient dyads using the reusable pen. However, the IPAQ subscales did not consistently meet acceptable internal consistency reliability for some group level comparisons. Cronbach’s alphas for the total IPAQ score for both pens were 0.85, exceeding acceptable levels of reliability for group comparisons. Conclusions The total IPAQ score is a useful measure for evaluating ease of use and preference for injection pens in clinical trials among patient dyads receiving hGH. The psychometric properties of the individual subscales, mainly the lower internal consistency reliability of some of the subscales and the predictive validity findings, do not support the use of subscale scores alone as a primary endpoint.

In recent years, the development of models of the offense process, relapse prevention in particular, have provided frameworks for the implementation of treatment. However, relapse prevention suffers from some conceptual problems, and therefore we have developed a descriptive model that attempts to provide a more satisfactory account of the offense process. This model contains a number of distinct points of individual differences, which create the possibility of different pathways. The current study examines these pathways, evident in offense transcripts from 86 sexual offenders. The majority of offense profiles were classifiable as belonging to three major pathways. Approximately one third reflected an appetitive, positive affect pathway. One quarter of the sample reflected the traditional covert planning, negative affect, restraint pathway. The third major pathway reflected a negative restraining process but with explicit planning. The clinical significance of these pathways is described and some suggestions for future research are made.

Objective: The factor structure of the eating disorder examination (EDE) has never been tested in a clinical pediatric sample, and no normative data exist. Method: The factor structure of an adapted EDE was examined in a clinical sample of 665 females aged 9-17 years with anorexia nervosa spectrum (70%), bulimia nervosa spectrum (12%), purging disorder (3%), and unspecified feeding and eating disorders (15%). Results: The original four-factor model was a good fit in a confirmatory factor analysis as well a higher order model with three dimensions of restraint, eating concern, and combined weight concern/ shape concern. Normative data are reported for clinicians to identify the percentiles in which their patients' score. Discussion: The findings support dimensions of restraint, eating concern, weight concern, and shape concern in a clinical pediatric sample. This supports the factorial validity of the EDE, and the norms may assist clinicians to evaluate symptoms in females under 18 years. [ABSTRACT FROM AUTHOR]

Background: Young males with eating disorders are a neglected study population in eating disorders. The aim of this study was to provide knowledge about the clinical presentation of eating disorders in young males. Methods: The data source was the Helping to Outline Paediatric Eating Disorders (HOPE) Project (N ~ 1000), a prospective, ongoing registry comprising consecutive paediatric (<18 years) tertiary eating disorder referrals. Young males with DSM-5 eating disorders (n = 53) were compared with young females with eating disorders (n = 704). Results: There was no significant difference in the prevalence of diagnosis of bulimia nervosa (2 % vs 11 %, p = 0.26) among sexes. Males had comparable duration of illness (9 months; p = 0.28) and a significantly earlier age of onset (M = 12 years; p <0.001). Shape concern (2.39 vs 3.57, p <0.001) and weight concern (1.97 vs 3.09, p <0.001) were lower in males, and body mass index z score (-1.61 vs -1.42, p = 0.29) and medical compromise (odds ratio [OR] = 0.64, 95 % CI: 0.36, 1.12) were comparable. Males had a two-folder higher odds of being diagnosed with unspecified feeding or eating disorders (40 % vs 22 % for females, p = 0.004). Driven exercise to control weight and shape was common and comparable in prevalence among males and females (51 % vs 47 %, p = 0.79) and males were less likely to present with self-induced vomiting (OR = 0.23, 95 % CI: 0.09, 0.59). Conclusion: Boys with eating disorders are an understudied group with similarities and differences in clinical presentation from girls with eating disorders. Parents and physicians are encouraged to consider changes in weight, disturbed vital signs, and driven, frequent exercise for the purposes of controlling weight or shape, as possible signs of eating disorders among male children. Diagnostic classification, assessment instruments, conceptualisation, and treatment methods need to be refined to improve application to young males. [ABSTRACT FROM AUTHOR]