Browse Items (193 total)

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.…

Sanfilippo syndrome is a rare degenerative disorder which has severe intellectual and behavioural sequelae, commonly including sleep problems. A parental questionnaire was used to gather information on the sleep patterns of 80 children with…

AIM: To assess reliability and predictive validity of the neurological scale of the Standardized Infant NeuroDevelopmental Assessment (SINDA), a recently developed assessment for infants aged 6 weeks to 12 months. METHOD: To assess reliability, three…

BACKGROUND: There is a dearth of literature that investigates life transitions of young adults (YAs) with life-limiting conditions, families and professionals. The scant literature that is available has methodological limitations, including not…

Cancer is the leading cause of disease-related death for adolescents and young adults (AYAs) in the United States. Parents of AYAs with life-threatening illnesses have expressed the desire to talk to their children about end of life (EOL) care, yet,…

INTRODUCTION: The number of children and young people living with life-limiting and life-threatening conditions is rising. Providing high-quality, responsive healthcare for them and for their families presents a significant challenge. Their…

Background: One goal of pediatric palliative care is to maintain quality of life for children and their families. Quality-of-life investigations may be enhanced by considering clinically important metrics in addition to statistical significance.…

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…

OBJECTIVES: To determine whether new-onset clinical depression emerges over time, and whether positive and negative mood levels change among patients with terminal cancer. METHODS: In this two-site study, 58 cancer patients seen at least twice were…

OBJECTIVE: Firstly, this paper aims to systematically review the mitochondrial disease literature to identify studies assessing the needs and problems in the daily life of children with a mitochondrial disease and of their parents and family. The…

OBJECTIVES: To determine parental participation in medical decision-making (MDM) during hospitalization and its association with parental self-efficacy and to explore other factors associated with participation. STUDY DESIGN: We surveyed parents of…

To describe the clinical and neurophysiological spectrum and prognosis in a large cohort of biochemically and genetically proven late onset Pompe patients. Thirty-eight diagnosed with late onset Pompe disease at our neuromuscular department during…

Paediatric palliative care is an emerging subspecialty that focuses on achieving the best possible quality of life for children with life-threatening conditions and their families. To achieve this goal, the individuals working in this field need to:…

In this Review, we look at the relation between bereavement and physical and mental health. Although grief is not a disease and most people adjust without professional psychological intervention, bereavement is associated with excess risk of…

OBJECTIVE: A longitudinal pediatric palliative care curriculum was introduced into the pediatric residency program at the University of California, Los Angeles. The present study explores the possible effects of this curriculum on the interns'…

The death of a child is a traumatic event that can have long-term effects on the lives of parents. This study examined bereaved parents of deceased children (infancy to age 34) and comparison parents with similar backgrounds (n = 428 per group)…

OBJECTIVE: To review selected measures of stress and coping in pediatric populations. Stress and coping are presented within a risk and resiliency framework. METHODS: The Society of Pediatric Psychology (SPP) surveyed the membership to identify the…

PURPOSE OF REVIEW: Over the past 15 years, the lysosomal storage diseases have become paradigms for the specific treatment of monogenic disorders, particularly those affecting children. This review summarizes the phenotypes and recent literature…

BACKGROUND: Interactive voice response (IVR) systems that collect survey data using automated, push-button telephone responses may be useful to monitor patients' pain and function at home; however, its equivalency to other data collection methods has…

BACKGROUND: There is an increasing trend towards lower participation in questionnaire surveys. This reduces representativeness, increases costs and introduces particular challenges to longitudinal surveys, as researchers have to use complex…

BACKGROUND: Children with progressive metabolic, neurological, or chromosomal conditions and their families anticipate an unknown lifespan, endure unstable and often painful symptoms, and cope with erratic emotional and spiritual crises as the…

BACKGROUND: Advances in biomedical science and technology have resulted in dramatic improvements in the healthcare of pediatric chronic conditions. With enhanced survival, health-related quality of life (HRQOL) issues have become more salient. The…

ABSTRACT: BACKGROUND: Bone pain in malignancy can be challenging to treat. Bisphosphonates have been found to be useful in adults with bone pain, but there are no reports of their use in children for this indication. In pediatric palliative medicine…

Fifty-two Australian couples who had experienced the death of at least one member of a multiple birth (twin or higher order), with at least one survivor of that birth, were interviewed about their experiences at the time of the death, and since. This…

OBJECTIVE. The goal was to explore barriers to palliative care experienced by pediatric health care providers caring for seriously ill children. METHODS. This study explored pediatric provider perceptions of end-of-life care in an academic children's…

OBJECTIVE: Mucopolysaccharidosis types IIIA through IIID (Sanfilippo syndrome) are caused by deficiencies of enzymes involved in the degradation of heparan sulfate. The onset and severity of the disease are highly variable. The purpose of this study…

OBJECTIVE: The goal was to determine whether the type and severity of the child's impairments and the family's psychosocial, social, and economic characteristics influence parent-reported child quality of life across the spectrum of severity of…

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