Browse Items (332 total)

We obtained information about the behavioral, psychiatric, and functional status of 26 children (13 males, 13 females) with juvenile neuronal ceroid lipofuscinosis (JNCL; mean age 12y 3mo [SD 3y 4mo]; range 6y 9mo to 18y 8mo). Twenty-five children…

BACKGROUND: Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disorder that is associated with significant esophageal strictures, resulting in dysphagia and nutritional failure. Although endoscopically guided balloon…

Cornelia de Lange syndrome is reported to be associated with self-injurious behavior (SIB) and social avoidance. We used analog methodology to examine the effect of manipulating adult social contact on social communicative behaviors and SIB in 16…

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…

It is estimated that between 14.8% and 18% of all youths in North America have a chronic health condition or a special health care condition (eg, impairments, such as musculoskeletal impairments, speech defects, deafness and hearing loss, blindness…

The objectives of the study were to evaluate the psychometric properties and appropriateness of instruments for the study of resilience in adolescents. A search was completed using the terms resilience and instruments or scales using the EBSCO…

Family management styles (FMSs) explain some of the complexities embedded in a family with a child who has chronic illness. The FMS typologies provide descriptions of family adjustment and management of care. These 5 distinct patterns may be valuable…

This article reviews the unique challenges of pediatric palliative medicine. These challenges originate from the specific epidemiology of pediatric diseases for which palliative care is indicated and the necessity to provide child-focused,…

AIMS: The mucopolysaccharidoses (MPS) are a heterogeneous group of rare disorders characterised by accumulation of glycosaminoglycans within multiple organ systems. This study aimed to determine the prevalence and severity of ocular complications in…

Is it necessary - and possible - to discuss death with a child in palliative phase? How should one approach the subject? A recent Swedish study demonstrated the benefits for parents who discussed with their child his or her imminent death, and raised…

Telomeres have emerged as crucial cellular elements in aging and various diseases including cancer. To measure the average length of telomere repeats in cells, we describe our protocols that use fluorescent in situ hybridization (FISH) with labeled…

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…

Cancer metastases (spread to distant organs from the primary tumor site) signify systemic, progressive, and essentially incurable malignant disease. Anorexia and wasting develop continuously throughout the course of incurable cancer. Overall, in…

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…

CONTEXT: Magnetic resonance imaging (MRI) findings have been reported for specific clinical cerebral palsy (CP) subgroups or lesion types but not in a large population of children with all CP subtypes. Further information about the causes of CP could…

Objective To review empirical studies of the efficacy of psychological interventions as adjuvanttherapies for children with pediatric diabetes, cancer, cystic fibrosis, and sickle celldisease. Methods A search was conducted for qualifying studies…

This qualitative systematic review of the clinical methodology used in randomised, controlled trials of oral opioids (morphine, hydromorphone, oxycodone) for cancer pain underlines the difficulties of good pain research in palliative care. The…

Risk behaviors, including tobacco, alcohol, and drug use, are common in adolescents and young adults. Those who engage in one risk behavior are likely to engage in additional health risk behaviors, and as the number of risk behaviors increase,…

Critically ill children are treated with opioid medication in an attempt to decrease stress and alleviate pain during prolonged pediatric intensive care. This treatment plan places children at risk for opioid dependency. Once dependent, children need…

OBJECTIVES: Teaching hospitals are perceived to provide a higher quality of care for the treatment of rare disease and complex patients. A substantial proportion of stage I palliation for hypoplastic left heart syndrome (HLHS) may be performed in…

The changing environment in neonatology and perinatology has led to the examination of issues surrounding palliative care. Newborn palliative care should be considered in three general areas: (1) Neonates at the limits of viability. As advances in…

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,…

Individuals with Duchenne muscular dystrophy may benefit from gastrostomy tube feeding due to progressive dysphagia and malnutrition. However, due to their severely impaired pulmonary function, these individuals are at risk of severe complications…

Results are described for a survey assessing prevalence of missing data and reporting practices in studies with missing data in a random sample of empirical research journal articles from the PsychINFO database for the year 1999, two years prior to…

A retrospective, multicenter study of 180 children with cytochrome c oxidase (COX) deficiency analyzed the clinical features, prognosis, and molecular bases of the COX deficiency. Clinical symptoms including failure to thrive, encephalopathy,…

OBJECTIVES: To assess parents' and health care providers' perceptions of the name and description of a pediatric palliative care (PPC) program. METHODS: Survey conducted at three pediatric health care sites; asked respondents (parents and staff)…

This article reviews mood disorders in children and adolescents, the diagnostic criteria, and important differential diagnoses to be considered. Critical points in the treatment of mood disorders and comorbid conditions and the important role the…

Because predicting and outlining guidance for all possible scenarios is difficult, advance directives are rarely sufficiently precise to dictate patient preferences in specific situations as a disease progresses. Nonetheless, advance care planning is…

The objective of this article is to review all published normative ethical and clinical guidelines concerning the genetic carrier testing of minors. The databases Medline, Philosopher's Index, Biological Abstracts, Web of Science, and Google Scholar…

Pain and cancer cannot be dissociated. A particular feature observed in children is that the child exhibits a certain psychomotor atony with increasing pain. The child complains less and less, making it difficult to recognize the degree of suffering…

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…

Children and young people with advanced cancer frequently have a central venous access device (central line) in place which can be used for intravenous medication for symptom management. Those that do not may need a subcutaneous device for medication…

The promise of theory and model development makes grounded theory an attractive methodology to follow. However, it has been argued that many researchers fall short and provide a detailed description of only the research area or simply a quantitative…

OBJECTIVES: Develop a prediction model to identify persons who have an increased risk of dying within the next 36 months, in order to focus additional resources and assessment in areas related to advanced care planning. DESIGN: Retrospective study…
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