Browse Items (924 total)

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…

BACKGROUND: Research ethics committees are commonly perceived as a 'barrier' to research involving seriously ill children. Researchers studying seriously ill children often feel that committees view their applications more harshly compared to…

Illness narratives have become very popular. The stories of children, however, are rarely ever studied. This paper aims to provide insight into how children, parents and physicians make sense of progressive childhood cancer. It also explores how this…

Tuberous sclerosis complex (TSC) (OMIM191100) is a genetic disorder with multi-system involvement including neurodevelopmental manifestations. There is great interest in understanding the pathogenetic mechanisms underlying these neurobehavioral and…

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…

Aim: To quantify, in individuals with Rett syndrome with the capacity to walk, walking-based activity and sedentary time, and to analyse the influences of age, walking ability, scoliosis, and the severity of epilepsy. Method: Sixty-four participants…

Background Autism spectrum disorder (ASD) is a neurodevelopmental condition estimated to affect 1 in 66 children in Canada and 1 in 270 individuals worldwide. As effective therapies for the management of ASD core and associated symptoms are limited,…

This paper describes the development of a video-based evaluation tool for use in Rett syndrome (RTT). Components include a parent-report checklist, and video filming and coding protocols that contain items on eating, drinking, communication, hand…

ImportanceThe associations of spiritual and religious factors with patient-reported outcomes among adolescents with cancer are unknown.ObjectiveTo model the association of spiritual and religious constructs with patient-reported outcomes of anxiety,…

Melatonin was tested as a sleeping pill in five patients with neuronal ceroid lipofuscinoses. The single-blind, placebo-controlled study consisted of motor activity recordings, sleep logs, and administration of placebo or melatonin (2.5 or 5 mg).…

BACKGROUND: Managing transition of adolescents/young adults with life-limiting conditions from children's to adult services has become a global health and social care issue. Suboptimal transitions from children's to adult services can lead to…

BACKGROUND: Better tools are needed to diagnose and identify children at risk of clinical malnutrition. OBJECTIVES: We aimed to compare body composition (BC) and malnutrition screening tools (MSTs) for detecting malnutrition on admission; and examine…

OBJECTIVES: Children with medical complexity (CMC) have high readmission rates, but relatively little is known from the parent perspective regarding care experiences surrounding and factors contributing to readmissions. We aimed to elicit parent…

BACKGROUND: Despite the increasing number of studies using photo-elicitation for data collection in qualitative research, there is a need to further explore its use among families of children and adolescents living with chronic illness. OBJECTIVE(S):…

Background: In 2018, >75,000 children were newly affected by the diagnosis of advanced cancer in a parent. Unfortunately, few programs exist to help parents and their children manage the impact of advanced disease together as a family. The Enhancing…

OBJECTIVE: To determine the impact of palliative care (PC) on end-of-life (EoL) care and the place of death (PoD) in children, adolescents, and young adults with life-limiting conditions. METHOD(S): Eight online databases (PubMed, Medline, EMBASE,…

BACKGROUND: Although perinatal deaths are still a common pregnancy outcome in developing countries, little is known about the effect perinatal death has on fathers. OBJECTIVE: The aim of the study was to understand and describe the meaning of…

Sleep disturbances are prevalent in mucopolysaccharidosis Type III (MPS III), yet there is a lack of objective, ecologically valid evidence detailing sleep quantity, quality or circadian system. Eight children with MPS III and eight age-matched…

BACKGROUND: Communication in the pediatric intensive care unit (PICU) between families and the health care team affects the family experience, caregiver psychological morbidity, and patient outcomes. OBJECTIVE: To test the feasibility of studying and…

BACKGROUND: Cornelia de Lange syndrome is associated with abnormalities on chromosomes 5, 10 and X. AIMS: To delineate the behavioural phenotype of Cornelia de Lange syndrome with specific reference to autistic-spectrum disorder. METHOD: A total of…

Children with medical complexity (CMC) are patients with one or more complex chronic conditions dependent on medical technologies. In our unit (Pediatric Pulmonology and Respiratory Intermediate Care Unit, Department of Pediatrics, "Bambino Gesù"…

OBJECTIVES: Hospital discharge offers an opportunity to initiate coordination of follow-up care, preventing readmissions or emergency department (ED) recidivism. We evaluated how revisits and costs of care varied in a 12-month period between children…

INTRODUCTION: A growing number of children and young people are surviving severe acquired brain injuries due to advances in healthcare. However, many fail to emerge from coma and continue to live with disorders of consciousness (DOC). Diagnostic,…

BACKGROUND: Families and professionals caring for children with life-limiting conditions face difficult healthcare decisions. Shared decision-making is promoted in many countries, however little is known about factors influencing these processes.…

BACKGROUND: On average, people will experience 2.28 hospital admissions in the last year of life with the likelihood of a hospital admission increasing in the last 2 weeks of life. Reducing hospital admissions has become a focus for high-income…

OBJECTIVES: To assess the ability of two illness severity scores, Pediatric Logistic Organ Dysfunction Score 2 and Pediatric Index of Mortality 3, in predicting PICU-acquired morbidity. DESIGN: Retrospective chart review conducted from April 2015 to…

BACKGROUND: A substantial proportion of young people with Complex Chronic Conditions (CCCs) experience some degree of discontinuation of follow-up care, which is an umbrella term to describe a broken chain of follow-up. Discontinuation of follow-up…

BACKGROUND: Very little research has been reported examining nonpharmacologic symptom management strategies for very young, hospitalized children receiving palliative care, and none has involved Reiki-a light touch therapy. OBJECTIVES: To determine…

OBJECTIVES: To characterize patterns of surgery among pediatric patients during terminal hospitalizations in children's hospitals. METHODS: We reviewed patients ≤20 years of age who died among 4 424 886 hospitalizations from January 2013-December…

Friedreich's ataxia is the most common autosomal recessive form of neurodegenerative ataxia. We present a longitudinal study on the gait pattern of children and adolescents affected by Friedreich's ataxia using Gait Analysis and the Scale for the…

OBJECTIVE: To assess the rate and type of urinary incontinence in a large sample of children and adolescents with spinal muscular atrophy (SMA), a genetic disorder characterized by loss of motor function caused by anterior horn degeneration.…

Data show that children are less severely affected with SARS-Covid-19 than adults; however, there have been a small proportion of children who have been critically unwell. In this systematic review, we aimed to identify and describe which underlying…

Children who require palliative care are being 'short changed or ignored', according to a survey of services in England by the charity Together for Short Lives.
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