Browse Items (90 total)

The records of 52 children with Niemann-Pick disease type C were reviewed to establish whether the disease process and outcome varied with the initial clinical pattern; 34 children (65%) had cholestatic liver disease and hepatosplenomegaly in…

BACKGROUND AND OBJECTIVE: Although there are general guidelines for adolescents with chronic illnesses making the transition from paediatric to adult health care, there are few studies which evaluate transition programs. This cross-sectional study…

PURPOSE: To examine bereavement mental health service use, barriers to use, and factors associated with use in parents bereaved by cancer. PATIENTS AND METHODS: A multicenter, cross-sectional study of 120 parents bereaved by cancer between 6 months…

Children grieve differently than adults. Although the unit of care in hospice is the patient and family, emphasis is often on the grown members of the family and the anticipatory grief and bereavement needs of children and adolescents are sometimes…

AIM: We investigated relationships between hand function and genotype and aspects of phenotype in Rett syndrome. METHOD: Video assessment in naturalistic settings was supplemented by parent-reported data in a cross-sectional study of 144 females with…

Transition is a process that attends to the medical, psychosocial and educational needs of young people as they transfer to adult-orientated care. With a growing population of adolescents surviving with chronic illness well into adulthood, it is…

BACKGROUND: Advances in biomedical science and technology have resulted in dramatic improvements in the healthcare of pediatric chronic conditions. With enhanced survival, health-related quality of life (HRQOL) issues have become more salient. The…

BACKGROUND: To help design population-based pediatric palliative care services, we sought to describe the hospital care received in the last year of life by children and young adults who died. We also determined the proportion with complex chronic…

Objectives: This study examined short- and long-term psychological adjustment to parental bereavement in later life for mothers and fathers. Methods: Using 9 waves of data from the United States (1998-2014 Health and Retirement Study), I estimated…

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…

This article investigates the relationships of child- and family-related variables with family function in families with children who have Duchenne muscular dystrophy. Child-related variables included level of disability (indicator: Barthel Index)…

Hypotheses generated by the precaution adoption process model, a stage model of health behavior, were tested in the context of home radon testing. The specific idea tested was that the barriers impeding progress toward protective action change from…

BACKGROUND: Research found that low levels of professional confidence and personal comfort among neonatal clinicians regarding palliative care may indicate a lack of competence and hesitancy to offer neonatal palliative care services. PURPOSE: This…

We report on an 8 years and 3 months old boy with severe idiopathic juvenile osteoporosis (IJO). Clinical features included multiple fractures, especially of the vertebrae, and neurological symptoms. Biological studies showed non-parathyroid…

The cyclical nature of chronic illness requires that children with inflammatory bowel disease be transitioned to the adult medical system with as much continuity of care as possible. Transition from pediatric to adult medical care continues to…

OBJECTIVE: Comparing demographic and clinical characteristics associated with receipt of palliative care (PC) among children who died in children's hospitals to those who did not receive PC and understanding the trends in PC use. METHODS: This…

Transition of young adults with cystic fibrosis (CF) from pediatric to adult medical care is an important priority, because many patients are living well into their fourth decade, and by 2010 more than half of all people living with CF will be older…

We present an evidence-based clinical practice guideline for the administration of the dissociative agent ketamine for emergency department pediatric procedural sedation and analgesia. Substantial research in recent years has necessitated updates and…

OBJECTIVES: This study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD). BACKGROUND: Historically, most deaths in patients with CHD occurred in early childhood. Notable advances have since…

Rett syndrome is a rare but severe neurological disorder typically associated with a mutation in the MECP2 gene. We describe change in gross motor function over 3 to 4 years for 70 subjects participating in the Australian Rett Syndrome Database.…

Bisphosphonates are synthetic analogues of pyrophosphate that inhibit bone resorption by their action on osteoclasts. Bisphosphonates have been extensively used in the elderly with primary and secondary osteoporosis, Paget's disease, and…

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:…

OBJECTIVE: To estimate when a difference in disability symptoms is sufficiently large to be important to individual patients. DESIGN: Cross-sectional analysis of two groups: derivation set (n = 46) and validation set (n = 57). SETTING: The Arthritis…

BACKGROUND: Some consider the loss of a child as the most stressful life event. When the death is caused by a malignancy, the parents are commonly exposed not only to their own loss, but also to the protracted physical and emotional suffering of the…
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