Browse Items (332 total)

The alleviation of symptoms, with the ultimate intention of improvement of quality of life, is a fundamental component of pediatric palliative medi-cine. Psychological factors can exacerbate physical symptoms or influence the perception of symptoms…

The purpose of this work was to retrospectively evaluate the medical needs of children with chronic and/or life-threatening illness attending summer camp at Barretstown Gang Camp (Ireland). Data on medical facility visits collected over 28 sessions…

In this prospective longitudinal study with a follow-up of 6 months, the course of serum concentration of C-reactive protein was measured by an ultrasensitive immunoassay in 31 patients with acute lumbosciatic pain and 41 patients with chronic low…

OBJECTIVES: This study sought to determine what adolescents and young adults with moderate to complex congenital heart disease (CHD) believe their life span to be and to examine correlates of their beliefs. BACKGROUND: Patients with moderate to…

The neuropeptide nociceptin/orphanin FQ (N/OFQ) is the endogenous ligand for the opioid-like receptor ORL-1 and is thought to be involved in pain transmission and modulation. Human studies have not yet defined its role in pain patients. The aims of…

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…

BACKGROUND: While developing and distributing clinical practice guidelines are important in optimising clinical healthcare, insight into actual care is necessary to achieve successful implementation. Developing quality indicators may be the first…

We compare the clinical course of 74 boys 10-18 years of age with Duchenne muscular dystrophy (DMD) treated (40) and not treated (34) with deflazacort. Treated boys were able to rise from supine to standing, climb stairs and walk 10 m without aids,…

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,…

Duchenne muscular dystrophy (DMD) is a fatal disorder affecting approximately 1 in 3,500 live born males, characterized by progressive muscle weakness. Several different strategies are being investigated in developing a cure for this disorder. Until…

Mammals survive starvation by activating proteolysis and lipolysis in many different tissues. These responses are triggered, at least in part, by changing hormonal and neural statuses during starvation. Pathways of proteolysis that are activated…

Evaluation of children who have hip pain can be a diagnostic challenge. This article reviews pertinent history taking, physical examination, laboratory testing, and imaging studies that assist in reaching a correct diagnosis. It also reviews the…

Males with Duchenne muscular dystrophy have sub-average cognitive capacities and may manifest more specifically language-related deficits. In the current study, the information-processing capacity, reading performance, and behavioral functioning of…

The purpose of this article is to present a model of factors that may influence a child's response to a painful procedure when parents are distraction coaches during the procedure. Nonpharmacological interventions, in particular, distraction, and…

This qualitative study investigated the caregiving experiences of mothers of children with thalassemia. Using a semistructured questionnaire, the researchers interviewed a convenient sample of 19 mothers who have children with thalassemia. A…

A diagnosis of a long-term illness in a child will bring on a major upheaval in the lives of the whole family involved and leads to a long-standing relationship with health care personnel. The purpose of this study was to describe parents'…

Transition has been described by many disciplines. However, the concept of transition has yet to be applied to the sibling experience of childhood cancer. Understanding the transitions that siblings encounter is important because it will offer nurses…

Progressive respiratory failure is a common modality of death in children with a terminal illness. The management of respiratory failure, and in particular symptoms of dyspnoea and musculoskeletal chest pain in children receiving palliative care,…

Chronically painful conditions are frequently associated with sleep disturbances, i.e. changes in sleep continuity and sleep architecture as well as increased sleepiness during daytime. A new hypothesis, which has attracted more and more attention,…

This paper uses panel data from the Statistics Canada National Longitudinal Survey of Children and Youth (1994 through 2000) to study the implications of parenting a child with a disability or chronic condition for subjective assessments of parental…

This study describes and conceptualizes the experiences of stigma in a group of children living with HIV in Sao Paulo, Brazil, and evaluates the impact of access to highly active antiretroviral therapy (HAART) over the social course of AIDS and over…

In addition to its importance in clinical assessment, N-terminal pro-brain natriuretic peptide (NT pro-BNP) is a valuable marker for evaluation of treatment and prognosis of heart failure. However, there are situations where NT pro-BNP is not related…

The aim of this study was to investigate long-term survival and examine causes of death in adult patients with cerebral palsy (CP). A 1940-1950 birth cohort based on paediatric case referral allows for long-term survival follow-up. Survival is…

A prospective controlled study with repeated measures before and after surgery examined the medical, surgical, and health outcomes of gastrostomy for children with disabilities at a tertiary paediatric referral centre in the North Thames area, UK.…

Scoliosis affects 75 to 90% of patients with non-ambulant Duchenne muscular dystrophy (DMD). Spinal surgery is the treatment of choice but the indication varies among centres. Some offer surgery to all non-ambulant patients, irrespective of scoliosis…

A review of the measures relating to the monitoring of disease progression in Duchenne muscular dystrophy was undertaken as part of the work of the North Star Clinical Network for Paediatric Neuromuscular Disease Management developing a standardized…

Pyruvate dehydrogenase (PDH) deficiency is a major cause of neurological dysfunction and lactic acidosis in infancy and early childhood. The great majority of cases (>80%) result from mutations in the X-linked gene for the E1alpha subunit of the…
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