Browse Items (195 total)

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…

Improving our ability to prevent or diminish suffering in dying children and adolescents and their families is dependent on the completion of high-quality pediatric end-of-life studies. The purpose of this article is to provide useful evidence-based…

Palliative care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and…

OBJECTIVE: To describe the clinical and demographic characteristics of nonneuronopathic Gaucher disease (GD) in children at the time of diagnosis. DESIGN: Longitudinal observational database of the International Collaborative Gaucher Group Gaucher…

Management of neonates with trisomy 18 is controversial, supposedly due to the prognosis and the lack of precise clinical information concerning efficacy of treatment. To delineate the natural history of trisomy 18 managed under intensive treatment,…

The study aimed to analyse the clinical courses of aggressively treated neonates with cytogenetically confirmed trisomy 18, with special attention focused on the efficiency of prenatal diagnostics, associated malformations, therapeutic dilemmas and…

Sleep disturbances are common in many progressive metabolic encephalopathies. The possible presence of disturbed sleep-wake behaviour in the lysosomal storage disorder aspartylglucosaminuria, has not been previously studied, however. The sleep-wake…

A longitudinal feasibility study regarding quality of life and interventions for patients with advanced cancer or AIDS and their family caregivers was conducted to determine issues related to their recruitment and retention and to obtain pilot data…

OBJECTIVE: To characterize the natural progression of infantile-onset Pompe disease. STUDY DESIGN: Retrospective chart reviews of 168 patients with documented acid alpha-glucosidase deficiency and symptom onset by 12 months of age; Kaplan-Meier…

OBJECTIVES: To describe the clinical presentation and long-term follow-up of a large cohort of patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. STUDY DESIGN: A nationwide, retrospective analysis of clinical presentation and…

Mitochondrial diseases affect all age groups, but those with childhood onset often seem to experience the greatest burden of disability. In some paediatric patients this can be explained by a cumulative disability acquired over many years. In others,…

This paper reports the development and validation of a disease-specific measure of health status and well-being of children with severe cerebral palsy (CP). The Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) was…

The purpose of this study was to determine the correlation of perceived stress with selected physiological and psychological factors in an HIV-infected, predominantly African American population and to assess the multivariable effects on perceived…

Stress, burden, and sorrow are not surprising responses for mothers of children who acquire life-altering disabilities. What is largely unforeseen is how maternal caregivers transform in positive ways through trauma and diversity. This article offers…

Metabolic storage disorders are caused by mutations in genes that result in insufficient activity of enzymes required for the catabolism of substances that arise from the turnover of senescent cells in the body. Among the most prevalent of these…

OBJECTIVE: To examine how individual- and family-level predictors in late childhood and preadolescence relate to psychosocial adaptation (i.e., scholastic success, social acceptance, and positive self-worth) in early adolescence. METHOD: This…

PURPOSE OF REVIEW: Pediatric palliative care, with its emphasis on symptom management and quality of life, is an important aspect of care of children with life-threatening illnesses. We review recent publications with implications for care of these…

BACKGROUND: Chronic disease of childhood may have implications for the psychosocial well-being of children and their families. The purpose of this paper is to provide an overview of the current literature regarding the psychosocial well-being of…

BACKGROUND: Children with severe neurological impairment may have significant oral motor dysfunction and are at increased risk of nutritional deficiencies, poor growth, and aspiration pneumonia. Gastrostomy tube feeding is increasingly being used for…

OBJECTIVE: The aim of family focused grief therapy is to reduce the morbid effects of grief among families at risk of poor psychosocial outcome. It commences during palliative care of terminally ill patients and continues into bereavement. The…

BACKGROUND: There is tension around the notion of research with bereaved parents. While it is recognised that the care of children with palliative care needs will only improve with better understanding of parent perspectives, the vulnerability of…

There is growing interest in researching posttraumatic growth and understanding how to enhance positive outcomes in trauma survivors. This study undertook a systematic exploration of the posttraumatic growth in bereaved caregivers of people with…

PURPOSE: The main objective was to compare parent-reported health-related quality of life (HRQL) of child and adolescent survivors of childhood cancer to that of controls who had no history of cancer. METHODS: We assessed HRQL of 800 child and…

Children with neurodegenerative, life-threatening illnesses (NLTIs) account for a significant proportion of children requiring palliative care, yet there is a lack of of research that examines families' experiences. This grounded theory study…
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