Browse Items (127 total)

Background Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with…

Prior studies have suggested that sleep disturbance is common in Cornelia de Lange Syndrome (CdLS); however, the nature of this sleep disturbance has not been well characterized. In this study, we evaluate the prevalence of sleep disordered breathing…

We examined the psychometric properties of one part of the Sleep Questionnaire developed by Simonds and Parraga (SQ-SP; 1982), a questionnaire that is frequently used to explore sleep problems and behaviors related to sleep in individuals with…

Rett syndrome is a rare but severe neurological disorder typically associated with a mutation in the MECP2 gene. We describe change in gross motor function over 3 to 4 years for 70 subjects participating in the Australian Rett Syndrome Database.…

Rett Syndrome (RS) is a neuro-developmental disorder, predominantly affecting females, resulting in severe mental retardation and neuro-behavioral disability. Some RS theoreticians hypothesize that behaviors that are neurologically driven are not…

Loss of hand function is a core feature of Rett syndrome. This article describes longitudinal hand function at 3 time points for 72 subjects participating in the Australian Rett Syndrome Database. Approximately 40% of subjects with some grasping…

Previous studies demonstrated the efficacy and safety of prolonged-release melatonin in children and adolescents with neurodevelopmental and behavioral disorders. The long-term effectiveness and safety of prolonged-release melatonin treatment were…

In the current study, the authors examined whether the fatigue level of children diagnosed with cri du chat syndrome was associated with the expression of autistic symptoms. Sixty-nine children with cri du chat syndrome were compared with 47 children…

Lesch-Nyhan disease (LND) is characterized by dystonia, cognitive abnormalities, and self-injurious behavior. No effective therapies are available. LND is associated with a presynaptic dopaminergic deficit, but the reported effects of dopamine…

Aim of the study: To assess the efficacy and safety of endoscopic balloon dilatation of esophageal strictures in children. Material and methods: Design: retrospective case series; population: 49 patients under 18 years of age referred to our center…

This paper describes Mexican American family members' descriptions of perceived discrimination by pediatric health care providers (HCPs) and the families' reactions to the HCPs' discriminatory conduct. A retrospective, grounded theory design guided…

This secondary analysis of data examined the psychosocial outcomes of meaning in caregiving, self-esteem, optimism, burden, depression, spirituality, and posttraumatic growth in 273 parents caring for children with life-limiting illnesses to (a)…

CONTEXT: The rate of prescription opioid-related overdose death increased substantially in the United States over the past decade. Patterns of opioid prescribing may be related to risk of overdose mortality. OBJECTIVE: To examine the association of…

To identify causes of death (COD) in propositi with Cornelia de Lange syndrome (CdLS) at various ages, and to develop guidelines to improve management and avoid morbidity and mortality, we retrospectively reviewed a total of 426 propositi with…

Adolescents and young adults (AYA) with advanced or terminal cancer have distinctive medical and psychosocial needs that may not have been adequately provided by either pediatric or adult palliative care services. A discussion group, as part of a…

INTRODUCTION: Palliative care services that address physical pain and emotional, psychosocial, and spiritual needs may benefit individuals with Duchenne muscular dystrophy (DMD). METHODS: The objective of this study was to describe the palliative…

BACKGROUND: The use of CAM by the relapsed pediatric oncology population has largely gone unstudied. The main objective of this study was to describe the prevalence of and change in CAM use in oncology patients for whom frontline therapy had failed.…

AIM: To identify the nature of services for children and young people with progressive neuromuscular disorders (NMD) provided by Children's Hospices in the UK. METHODS: A questionnaire requesting aggregate data on the number of patients with a…

OBJECTIVES: The burden of suffering among patients with end-stage chronic diseases may be greater than those of cancer patients, as a result of longer duration of illness trajectory and high prevalence of symptoms, yet they may be less likely to…

OBJECTIVES: The burden of suffering among patients with end-stage chronic diseases may be greater than those of cancer patients, as a result of longer duration of illness trajectory and high prevalence of symptoms, yet they may be less likely to…

Neonatal nosocomial infection (NNI) is a major complication of neonatal care, increasing mortality, morbidity and the costs of healthcare. Management of NNI involves attention to many details of care, creating a culture of change within a neonatal…

It has been two decades since advance directives have become an integral part of health care. Impediments to their optimal usage are common and multifactorial. Decisions commonly have to be made when patients are unable to do so or choose not to…

Pain coping is thought to be the most significant behavioural contribution to the adjustment to pain. Little is known about how those with intellectual and developmental disabilities (IDD) cope with pain. We describe parental reported coping styles…

Pediatric surgeons can play an important role in offering procedures that may improve the quality of life for terminally ill children. As with all palliative interventions, surgical therapies should be evaluated in the context of explicitly defined…

BACKGROUND: Although the extent of hospital and intensive-care use at the end of life is well known, patterns of surgical care during this period are poorly understood. We examined national patterns of surgical care in the USA among elderly…

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