Background: Hand stereotypies are considered a hallmark of Rett syndrome (RTT) and are usually described as symmetric movements at the midline. However, related pathologies may show the same type of involuntary movement. Furthermore, patients with…
We present a baby with spinal muscular atrophy type 1, an inherited disorder causing progressive weakness, leading to complete paralysis of respiratory, facial and limb muscles. Without intervention, death occurs in infancy due to respiratory…
Archival data from a survey of parent observations was used to determine the prevalence of social and behavioral problems in children with agenesis of the corpus callosum (ACC). Parent observations were surveyed using the Child Behavior Checklist…
CONTEXT: The place where children with complex chronic conditions are dying may be shifting toward residential homes due to the evolving epidemiology of life-threatening childhood conditions, advances in home-based medical technology, and changes in…
BACKGROUND: Metachromatic leukodystrophy (MLD) is a lysosomal storage disease with infantile and juvenile onset with a poor prognosis and magnetic resonance imaging (MRI) plays a fundamental role in its diagnosis. Procedural sedation is needed to…
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…
In the history of science, technical advances often precede periods of rapid accumulation of knowledge. Within the past three decades, discoveries that enabled the noninvasive measurement of the psychobiology of stress (in saliva) have added new…
The characteristic clinical presentation, especially the appearance of muscle symptoms, is quite unique in children carrying the mtA8344G mutation. The diagnosis of MERRF syndrome is seldom made in the pediatric age. Fatigue is a common finding in…
BACKGROUND: Respiratory depression is the most feared opioid-related side-effect yet research on the topic is sparse. We evaluated changes in respiratory parameters during parenteral opioid titration for cancer pain to determine if opioid titration…
Pediatric palliative care is increasingly recognized to be a specialized type of care requiring specific skills and knowledge, yet, as found in several countries, there is little available research evidence on which to base care. Objectives: The goal…
This study applied three family measures (ratings of service adequacy or implementation, satisfaction with the family–professional partnership, and family quality of life) to a sample of families of young children in one Midwestern U.S. state. The…
PURPOSE: To determine the levels of beta-endorphin and cortisol in children with multiple injuries and to determine whether there is any difference between and compare the severity of trauma and beta-endorphin and cortisol release as calculated using…
Over 8,000 children, young people and adults in the UK are affected by cystic fibrosis and although no cure exists, comprehensive therapy started early and administered consistently delays disease progression. This article explores three aspects of…
Quality end-of-life care includes the management of distressing symptoms; provisions of care, including the assessment and management of psychosocial and spiritual needs; and respite from diagnosis through death and bereavement. Meeting the…
Caring for children who have a chronic life-limiting illness can be emotionally and physically challenging. Just as families may struggle with whether they are making the right decisions, care providers struggle with whether they are giving the right…
This article describes the use and effectiveness of adjuvant propofol for pain control for pediatric oncology patients at the end of life. All patients experienced severe pain and agitation, not well controlled by continuous infusion opioids and…
OBJECTIVES: The purpose of this study was to identify the factors that predict good adherence to health regimens by adolescents with diabetes (insulin-dependent diabetes mellitus). METHODS: Altogether, 300 individuals aged 13-17 years were randomly…
The importance of measuring growth outcomes following a traumatic event has been highlighted in recent literature (e.g., Linley, Joseph: Journal of Traumatic Stress 17:11-21, 2004). Although reports of growth are abundant, the relationship between…
OBJECTIVE: To determine the effectiveness of a psycho-educational group intervention for chronically ill children. METHODS: Based on principles from cognitive behavior therapy and information from previous research about children's experiences with…
PURPOSE: The purpose of this study was to describe and show effectiveness of the outreach team model of palliative care (PC) in allowing home death for children with incurable cancer. PATIENTS AND METHODS: Over 7 months, 185 children from 22 United…
OBJECTIVE: Caregiving has been described in the literature as a risk factor for ill health in the carer. This controlled, prospective study examines the course of physical and mental health problems in parents of adolescent survivors of a mass burn…
Transition to adulthood for youth with developmental disabilities has become an important concern internationally of service providers working with these young people. Reflecting on the useful review by Binks and colleagues in this issue of the…
OBJECTIVES: To describe the patients who received care from the 8 dedicated pediatric palliative care programs in Canada in 2002 and to estimate the number of children who may have benefited but did not receive services from these programs. DESIGN:…
Optimizing the quality of medical care at the end of life has achieved national status as an important health care goal. Palliative care, a comprehensive approach to treating the physical, psychosocial and spiritual needs of patients and their…
OBJECTIVE: The purpose of this review of published literature was to identify the number and focus of empirically based papers that included research methods used to directly solicit patient-reported outcomes (PRO) from pediatric oncology patients at…
OBJECTIVE: To investigate parents' perspectives on the desirability, content, and conditions of a physician-parent conference after their child's death in the pediatric intensive care unit (PICU). STUDY DESIGN: Audio-recorded telephone interviews…
OBJECTIVE: To characterize parental perception of clinical research, particularly in emergency settings. To identify specific aspects of clinical research that concern parents and to discuss how these concerns can be addressed. STUDY DESIGN:…
OBJECTIVE: To examine whether physicians or parents assume responsibility for treatment decisions for critically ill children and how this relates to subsequent parental experience. A significant controversy has emerged regarding the role of parents,…
PURPOSE: Palliative sedation therapy (PST) is a controversial issue. There is a need for internationally accepted definitions and standards. METHODS: A systematic review of the literature was performed by an international panel of 29 palliative care…
The cure rate of cancer in adolescents is high but between 10% and 40% of them will develop incurable disease depending on tumor type and prognostic factors. These patients will need palliative care defined as supportive care to optimize comfort,…
