Browse Items · Pediatric Palliative Care Library

Follow-up in children with Joubert syndrome

Although Joubert syndrome (JS) was first reported in 1969 by Joubert et al (21), the long-term outcome is not yet documented. We report 19 children (4 pairs of siblings) from a single institution diagnosed with JS. Nine children were last seen…

Prognosis for survival and improvement in function in children with severe developmental disabilities

OBJECTIVE: To derive prognostic data for survival and clinical improvement in children with severe developmental disabilities. STUDY DESIGN: A 13-year follow-up study of several cohorts of children initially evaluated before their first birthday. The…

Magnetic resonance spectroscopy in Niemann-Pick disease type C: Correlation with diagnosis and clinical response to cholestyramine and lovastatin.

Niemann-Pick type C is an autosomal-recessive, neurovisceral storage disorder that results from defective cholesterol esterification. Cholesterol-lowering agents have been demonstrated to decrease hepatic lipids in Niemann-Pick type C patients. The…

End-of-life practices in the Netherlands under the Euthanasia Act

BACKGROUND: In 2002, an act regulating the ending of life by a physician at the request of a patient with unbearable suffering came into effect in the Netherlands. In 2005, we performed a follow-up study of euthanasia, physician-assisted suicide, and…

Follow-up on seven adult patients with mild Sanfilippo B-disease.

This report describes 7 patients, aged 30 to 43 years, suffering from a mild variant of Sanfilippo B-disease. Somatic findings in these patients are unremarkable. Dementia and behavioral disturbances occurred late in the course of the disease. All 7…

Tags: 1987, Adult, Age Factors, American Journal Of Medical Genetics, Backlog, Dementia/complications/genetics, Female, Follow-up Studies, Humans, Journal Article, Longitudinal Studies, Male, Mental Retardation/complications/genetics, Mucopolysaccharidoses/genetics, Mucopolysaccharidosis III/genetics, Pedigree, van de Kamp JJ, van Schrojenstein-de Valk HM

Information exchange among physicians caring for the same patient in the community

BACKGROUND: The exchange of information is an integral component of continuity of health care and may limit or prevent costly duplication of tests and treatments. This study determined the probability that patient information from previous visits…

The use of conscious sedation for pain control during forearm fracture reduction in children: does race matter?

Recent reports in the medical literature indicate that certain racial disparities have been identified in healthcare. The authors sought to identify the potential relationship between the use of pain medications in African-American and Caucasian…

Predictors of burden in spouse caregivers of individuals with Alzheimer's disease

This study used the following model of distress: Distress = [Exposure to Stress + Vulnerability]/[Psychological and Social Resources]. The constructs in the model were operationalized as (a) distress in response to caregiver experiences (burden); (b)…

Congenital malformations of the central nervous system in a 1-year birth cohort followed to the age of 14 years

A 1-year birth cohort from northern Finland comprised 12,058 children, 96% of all live-born infants born in the region in 1966. The development and morbidity of these children were followed up to the age of 14 years. Altogether, 40 children (25 boys…

A two-step multidisciplinary approach to treat recurrent esophageal strictures in children with epidermolysis bullosa dystrophica

In children with severe generalized recessive dystrophic epidermolysis bullosa (RDEB), esophageal scarring leads to esophageal strictures with dysphagia, followed by malnutrition and delayed development. We describe a two-step multidisciplinary…

Psychological Distress and the Impact of Social Support on Fathers and Mothers of Pediatric Cancer Patients: Long-Term Prospective Results

Parents of children diagnosed with cancer: work situation and sick leave, a five-year post end-of-treatment or a child's death follow-up study

BACKGROUND: Cancer in a child is associated with a significant impact on parental employment. We assessed the proportions of parents of survivors and bereaved parents working and reporting sick leave five years after end of successful treatment…

Association of Mortality With the Death of a Sibling in Childhood.

Importance: The death of a close relative is associated with an increased mortality risk among the bereaved, but much less is known about the potential association of the death of a sibling in childhood with mortality in this population. Objective:…

Factors important to patients' quality of life at the end of life

BACKGROUND: When curative treatments are no longer options for patients dying of cancer, the focus of care often turns from prolonging life to promoting quality of life (QOL). Few data exist on what predicts better QOL at the end of life (EOL) for…

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