Tuberous sclerosis complex (TSC) (OMIM191100) is a genetic disorder with multi-system involvement including neurodevelopmental manifestations. There is great interest in understanding the pathogenetic mechanisms underlying these neurobehavioral and…
We investigated the prevalence and phenomenology of repetitive behavior in genetic syndromes to detail profiles of behavior. The Repetitive Behaviour Questionnaire (RBQ) provides fine-grained identification of repetitive behaviors. The RBQ was…
Background Self-injurious behaviour is frequently identified as part of the behavioural phenotype of Cornelia de Lange syndrome (CdLS). We conducted a case-control study of the prevalence and phenomenology of self-injurious behaviour (SIB) in CdLS.…
Background Self-injurious behaviour is commonly seen in Cornelia de Lange syndrome (CdLS). However, there has been limited research into the aetiology of self-injury in CdLS and whether environmental factors influence the behaviour. Methods We…
OBJECTIVE: Somatization of emotions accounts for excess emergency department (ED) visits in the form of medically unexplained symptoms (MUS). Intensive short-term dynamic psychotherapy (ISTDP) has been used to diagnose and manage somatization. We…
Discovering the hormonal and neural mechanisms that promote affiliative social behavior is a high priority in behavioral neuroscience. Although studies with standard laboratory rodents have afforded many important insights, exciting advances are also…
OBJECTIVE: The aim of this study was to investigate the primary concerns of terminally ill cancer patients in a Short-Term Life Review among Japanese, Koreans, and Americans to develop intervention programs to be tailored to patients in other…
Costello syndrome is a rare rasopathy caused by germline mutations in the oncogene HRAS resulting in increased signal transduction through the Ras/mitogen-activated protein kinase pathway. In contrast to the more common rasopathies, such as…
In trying to improve clinician communication skills, we have often heard clinicians at every level admonished to "use silence," as if refraining from talking will improve dialogue. Yet we have also noticed that this "just do it," behavior-focused…
BACKGROUND: End-of-life care (EOLC) discussions and decisions are common in pediatric oncology. Interracial differences have been identified in adult EOLC preferences, but the relation of race to EOLC in pediatric oncology has not been reported. We…
BACKGROUND: End-of-life care (EOLC) discussions and decisions are common in pediatric oncology. Interracial differences have been identified in adult EOLC preferences, but the relation of race to EOLC in pediatric oncology has not been reported. We…
CONTEXT: There are few randomized controlled trials on the effectiveness of palliative care interventions to improve the care of patients with advanced cancer. OBJECTIVE: To determine the effect of a nursing-led intervention on quality of life,…
PURPOSE: To determine whether spiritual care from the medical team impacts medical care received and quality of life (QoL) at the end of life (EoL) and to examine these relationships according to patient religious coping. PATIENTS AND METHODS:…
OBJECT: Deepening sedation is often needed in patients with intracranial hypertension. All widely used sedative and anesthetic agents (opioids, benzodiazepines, propofol, and barbiturates) decrease blood pressure and may therefore decrease cerebral…
BACKGROUND: The purpose of the current study was to investigate longitudinally children's behavioral and social competence outcomes up to 2 years after pediatric stem cell transplantation (SCT) and related factors. METHODS: Ninety-nine mothers and 24…
Videotelephony (real-time audio-visual communication) has been used successfully in adult palliative home care. This paper describes two attempts to complete an RCT (both of which were abandoned following difficulties with family recruitment),…
OBJECTIVES: To assess neonatology fellow training in guiding family decision making for high-risk newborns and in several critical communication skills for physicians in these scenarios. DESIGN: A Web-based national survey. SETTING:…
The purpose of this study was to examine gender differences in spousal caregiving at the end of life. The primary research question was to determine gender differences in caregiver strain among spousal caregivers. The study was conducted over a…
OBJECTIVE: The primary objective of this study was to examine how the comprehensive nature of the Stress Process Model could elucidate on the stressors associated with caring for a palliative cancer patient. Method: A qualitative research strategy…
To test the validity and reliability of the scale for the assessment and rating of ataxia (SARA) in Friedreich ataxia (FRDA). SARA is limited to eight items and can be performed rapidly. Ninety-six patients with a molecular genetic diagnosis of FRDA…
INTRODUCTION: The outcomes of different types of transitions of young people with chronic diseases have been poorly investigated. OBJECTIVE: To evaluate and compare a structured transition from the paediatric diabetes services (PDS) into the adult…
Sleep and behavioural difficulties are common in children with developmental disabilities. Mothers often wake and tend to their child when their child is having sleep difficulties. Therefore, mothers of children with developmental disabilities can…
Previously overlooked factors in elders' depressive symptomatology were examined, including death fear, sibling death, and sibling closeness. Participants were 150 elders (61 men, 89 women) aged 65-97 years with at least one sibling. Measures were…
This article explores ways in which siblings and child friends are represented as being involved in death and loss experiences. These representations are taken from 51 selected examples of death-related literature designed to be read by or with…
BACKGROUND AND PURPOSE: The purpose of this study was to describe gait parameters in children and adolescents with a diagnosis of Friedreich ataxia (FA) and examine the relationship between disease severity, measured by the Friedreich Ataxia Rating…
