Browse Items (88 total)

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…

Considerable research has focused on hospitalizations for ambulatory care–sensitive conditions (ACSHs), but little of that research has focused on the role played by chronic disease in ACSHs involving children or youth (C/Y). This research…

The sleep patterns of a cohort of 83 Rett syndrome females were characterized using a sleep diary for 7 consecutive days and nights and compared with normative sleep data. The mean total sleep time of the cohort was 10.75 h, daytime sleep 0.77 h,…

In juvenile neuronal ceroid-lipofuscinosis (JNCL), sleep disorders are common. The purpose of this study was to investigate the sleep structure of 28 patients with JNCL compared with healthy controls subjects and to clarify the pathophysiology…

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.…

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…

BACKGROUND: Advance care planning and goals of care discussions involve the exploration of what is most important to a person, including their values and beliefs in preparation for health-care decision-making. Advance care planning conversations…

We report the second family with AIMP1 deficiency, due to a homozygous truncating AIMP1 (g.107248613 C > T) mutation. This female showed early-onset developmental arrest, intractable epileptic spasms, microcephaly, and a rapid clinical course leading…

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…

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…

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…

BACKGROUND: Niemann-Pick type C disease (NPC) is an inherited neurodegenerative disorder characterised by an intracellular lipid-trafficking defect with secondary accumulation of glycosphingolipids. Miglustat, a small iminosugar, reversibly inhibits…

Previously overlooked factors in elders' depressive symptomatology were examined, including death fear, sibling death, and sibling closeness. Participants were 150 elders (61 men, 89 women) aged 65-97 years with at least one sibling. Measures were…

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)…

OBJECTIVE: To describe the practice reported by pediatric emergency department (PED) medical directors regarding age limits and transition of health care in their emergency departments and institutions. METHODS: A 28-question survey was sent by…

PURPOSE OF REVIEW: The transition of healthcare from pediatric to adult settings has become more significant over the past 20 years as the survival of young people with chronic illness and disability has increased and healthcare delivery has become…

As increasing numbers of young people with chronic illness reach adulthood, their ongoing medical care must evolve to be delivered in an adult rather than paediatric setting, a process known as transition. Towards this goal, increasing numbers of…

AIM: To determine the prevalence and associations of self-reported and parent-reported pain in children with cerebral palsy (CP) of all severities., METHOD: Cross-sectional design using a questionnaire; analysis using ordinal regression. Children…

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…

Young people with a chronic illness or disability originating in childhood ultimately need transition to adult care. The process of leaving a familiar paediatric service and effectively engaging in appropriate adult health care can be challenging and…

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…

This study examined posttraumatic growth in 30 male veterans captured and held as prisoners of war during the Vietnam War. Participants were assessed with structured diagnostic interviews administered by trained clinicians as well as with the…

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…

The main objective of this review was to provide a quantitative and methodologically sound evaluation of existing treatments for bereavement and grief reactions in children and adolescents. Two meta-analyses were conducted: 1 on controlled studies…

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 aim was to compare results of studies performed in different settings worldwide and identify common therapeutic areas to allow for focused interventions, because off-label drug use can be a measure of the lack of knowledge concerning paediatric…

STUDY OBJECTIVES: More than two thirds of family members visiting intensive care unit (ICU) patients have symptoms of anxiety or depression during the first days of hospitalization. Identifying determinants of these symptoms would help caregivers…
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