Browse Items · Pediatric Palliative Care Library

The search for social safety and comfort in families raising children with complex chronic conditions

Social consequences of raising children who were medically fragile and developmentally delayed (MF/DD) were explored in an ethnographic study of 20 families with school-age children. The overarching theme was the families' search for safety and…

The parents' postoperative pain measure: replication and extension to 2-6-year-old children

Pain assessment is a difficult task for parents at home following children's surgery. The purpose of the present study was to confirm the psychometric properties of a behavioural measure of postoperative pain developed to assist parents with pain…

The Outcome of Critically Ill Pediatric Cancer Patients Admitted to the Pediatric Intensive Care Unit in a Tertiary University Oncology Center in a Developing Country: A 5-Year Experience

INTRODUCTION: Cancer remains a major cause of death in children, but recent advances in supportive care and progress in the use of chemotherapy have considerably improved the prognosis. The need for intensive care management in pediatric oncology…

The ocular features of the mucopolysaccharidoses

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…

The Need for a Shared Understanding: Domains of Care and Composition of Team in Pediatric Palliative Care Guidelines

Conceptual confusion is a primary barrier to providing quality palliative care. This study aimed to analyze pediatric palliative care (PPC) guidelines from a conceptual perspective to facilitate a shared understanding of palliative care in…

The natural history of type B Niemann-Pick disease: Results from a 10-year longitudinal study.

OBJECTIVES: Type B Niemann-Pick disease (NPD-B) caused by acid sphingomyelinase deficiency is a rare, autosomal recessive, lysosomal storage disorder with a broad range of disease severity. The objectives of this study were to document the natural…

The natural history of Niemann-Pick disease type C in the UK

Niemann-Pick disease type C (NPC) is an autosomal recessive, neurovisceral lipid storage disorder. Mutations in two genes (NPC1 and NPC2) produce indistinguishable clinical phenotypes by biochemical mechanisms that have not yet been entirely…

The natural history of medium-chain acyl CoA dehydrogenase deficiency in the Netherlands: clinical presentation and outcome

OBJECTIVES: To describe the clinical presentation and long-term follow-up of a large cohort of patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. STUDY DESIGN: A nationwide, retrospective analysis of clinical presentation and…

The natural history of juvenile or subacute GM2 gangliosidosis: 21 new cases and literature review of 134 previously reported.

OBJECTIVE: Juvenile GM2 gangliosidosis is a group of inherited neurodegenerative diseases caused by deficiency of lysosomal beta-hexosaminidase resulting in GM2 ganglioside accumulation in brain. The purpose of this study was to delineate the natural…

The natural history of degenerative ataxia: a retrospective study in 466 patients

The aim of the present study was (i) to compare disease progression and survival in different types of degenerative ataxia, and (ii) to identify variables that may modify the rate of disease progression. We included patients suffering from…

The journey through care: study protocol for a longitudinal qualitative interview study to investigate the healthcare experiences and preferences of children and young people with life-limiting and life-threatening conditions and their families in the West Midlands, UK

INTRODUCTION: The number of children and young people living with life-limiting and life-threatening conditions is rising. Providing high-quality, responsive healthcare for them and for their families presents a significant challenge. Their…

The impact on health-related quality of life from non-steroidal anti-inflammatory drugs, methotrexate, or steroids in treatment for juvenile idiopathic arthritis

OBJECTIVE: To assess and compare the impact of medication treatments on health-related quality of life (HRQOL), family function, and medical status in children with juvenile idiopathic arthritis (JIA). METHODS: Fifty-seven children diagnosed with JIA…

The impact of parental status on the risk of completed suicide.

BACKGROUND: Although some studies suggest that parenthood is associated with a reduced suicide risk, the impact of children on parental suicide has rarely been documented. METHODS: This study investigates the impact of parental status on the risk of…

The Functional Independence Measure for Children (WeeFIM). Conceptual basis and pilot use in children with developmental disabilities.

Few tools are available to pediatricians for tracking and monitoring disability status in children. We describe the conceptual basis and pilot use of the Functional Independence Measure for Children (WeeFIM). Our pilot use of this instrument in…

The feasibility and validity of forced spirometry in ataxia telangiectasia

OBJECTIVES: To explore the feasibility and validity of forced spirometry in patients with ataxia telangiectasia (A-T). STUDY DESIGN: Twenty-eight patients (aged 3.7-19.3 years) performed spirometry on 47 occasions. Parameters studied were technical…

The Faces Pain Scale-Revised: toward a common metric in pediatric pain measurement

The Faces Pain Scale (FPS; Bieri et al., Pain 41 (1990) 139) is a self-report measure used to assess the intensity of children's pain. Three studies were carried out to revise the original scale and validate the adapted version. In the first phase,…

The emerging issue of euthanasia

2005

Tags: 2005, Adolescent, Archives Of Pediatrics & Adolescent Medicine, Backlog, Child, Decision Making, Euthanasia, Humans, Infant, Journal Article, Longitudinal Studies, Netherlands, Newborn, Pediatrics/statistics & numerical data, Preschool, Siden HB, Terminal Care

The Effects of Chronic Disease on Ambulatory Care–Sensitive Hospitalizations for Children or Youth

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 effect of continuity of care on emergency department use

OBJECTIVE: To examine whether continuity of care with an individual health care provider is associated with the number of hospital emergency department (ED) visits in a statewide Medicaid population. DESIGN: A cross-sectional study based on a 100%…

The decision to use topical anesthetic for intravenous insertion in the pediatric emergency department

OBJECTIVES: Topical anesthetic creams to reduce the pain of intravenous (IV) placement may be more effectively used in the emergency setting if they are applied by nurses in the triage area of the emergency department or soon after the patient is…

The death of a twin

Death or disability is much more common in multiple births than single children, especially in the perinatal period. Parents face particular problems in that their loss may be underestimated; their grieving may be impeded by the confusion between the…

Tags: 1995, Adolescent, Adult, Backlog, Bryan EM, Child, Child Psychology, Counseling, Death, Female, Fetal Death, Grief, Guilt, Humans, Infant, Journal Article, Male, Newborn, Palliative Medicine, Parent-child Relations, Preschool, sibling bereavement, Social Support, Twins

The clinical spectrum of mitochondrial disease in 75 pediatric patients

The clinical presentation of mitochondrial disorders in childhood is highly variable causing difficulties in diagnosis and management. We assessed records of 75 children (48 male, 27 female) with a biochemically and/or molecularly established…

The clinical and demographic characteristics of nonneuronopathic Gaucher disease in 887 children at diagnosis

OBJECTIVE: To describe the clinical and demographic characteristics of nonneuronopathic Gaucher disease (GD) in children at the time of diagnosis. DESIGN: Longitudinal observational database of the International Collaborative Gaucher Group Gaucher…

The Carter Neurocognitive Assessment for children with severely compromised expressive language and motor skills

In this paper, different means of assessing cognitive development in children with severe impairments in both their expressive language and their motor skills are reviewed. A range of techniques are considered, including traditional cognitive tests…

The association between environmental events and self-injurious behaviour in Cornelia de Lange syndrome

BACKGROUND: There has been limited empirical research into the environmental causes of self-injury in Cornelia de Lange syndrome. The present study examined the variability of self-injurious behaviour in Cornelia de Lange syndrome across…

Tay-Sachs disease: Progression of changes on neuroimaging in four cases.

The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted…

Task force 1: the changing profile of congenital heart disease in adult life

Talking with parents about end-of-life decisions for their children

BACKGROUND AND OBJECTIVE: Retrospective studies show that most parents prefer to share in decisions to forgo life-sustaining treatment (LST) from their children. We do not yet know how physicians and parents communicate about these decisions and to…

Talking about death with children who have severe malignant disease

BACKGROUND: One of the questions faced by the parents of a child who is terminally ill with a malignant disease is whether or not they should talk about death with their child. METHODS: In 2001, we attempted to contact all parents in Sweden who had…

Systemic carnitine deficiency--a treatable inherited lipid-storage disease presenting as Reye's syndrome

A 3 1/2-year-old boy presented at three months of age with an acute episode of lethargy, somnolence, hypoglycemia, hepatomegaly, and cardiomegaly, which responded poorly to restoration of the blood sugar level to normal. The absence of ketonuria…

Symptoms in the palliative phase of children with cancer

BACKGROUND: The aim of the study was to make a comprehensive inventory of the physical, psychological, and social symptoms of children with cancer and their parents during the palliative phase and the extent to which health professionals address…

Symptoms in children/young people with progressive malignant disease: United Kingdom Children's Cancer Study Group/Paediatric Oncology Nurses Forum survey.

AIM: The purpose of this study was to survey symptoms in children/young people with progressive cancer and identify which are the most important and which are the most difficult to treat effectively. PATIENTS AND METHODS: This was a questionnaire…

Symptom profile of common colds in school-aged children

BACKGROUND: Signs and symptoms of a common cold reported in young children are those perceived by caretakers. Objective signs include cough, fever, and sneezing. Subjective symptoms include nasal congestion, feverishness, headache, and sore throat.…

Tags: 2008, Backlog, Child, Common Cold/physiopathology, Haemophilus influenzae/isolation & purification, Hayden FG, Hendley JO, Humans, Journal Article, Medical Records/statistics & numerical data, Moraxella (Branhamella) catarrhalis/isolation & purification, Nasopharynx/microbiology/virology, Pappas DE, Pediatric Infectious Diseases Journal, Preschool, Rhinovirus/isolation & purification, Streptococcus pneumoniae/isolation & purification, Time Factors, Winther B

Swedish parents' experiences of parenthood and the need for support to siblings when a baby is stillborn

BACKGROUND: It has been argued that having a stillborn baby in the family affects older siblings more than parents realize. The aim of this study was to describe parenthood and the needs of siblings after stillbirth from the parents' perspective.…

Tags: 2011, Adolescent, Adult, Avelin P, Backlog, Birth (berkeley, Calif.), Child, Erlandsson K, Female, Hildingsson I, Humans, Infant, Journal Article, Male, Parenting, Preschool, Rådestad I, sibling bereavement, Siblings, Stillbirth, Sweden

Survival without disability to age 5 years after neonatal caffeine therapy for apnea of prematurity

CONTEXT: Very preterm infants are prone to apnea and have an increased risk of death or disability. Caffeine therapy for apnea of prematurity reduces the rates of cerebral palsy and cognitive delay at 18 months of age. OBJECTIVE: To determine whether…

Tags: 2012, Anderson PJ, Apnea/drug therapy, Asztalos EV, Backlog, Barrington KJ, Blindness/epidemiology/etiology/prevention & control, Caffeine/adverse effects/therapeutic use, Central Nervous System Stimulants/adverse effects/therapeutic use, Cerebral Palsy/epidemiology, Child, Child Development, Cognition Disorders/epidemiology/etiology/prevention & control, Davis PG, Deafness/epidemiology/etiology/prevention & control, Developmental Disabilities/epidemiology/etiology/prevention & control, Dewey D, Doyle LW, Female, Follow-up Studies, Grunau RE, Humans, Incidence, Infant, JAMA, Journal Article, Male, Moddemann D, N2N, Newborn, Odds Ratio, Ohlsson A, Premature, Preschool, Roberts RS, Schmidt B, Solimano A, Survival Analysis, Tin W, Treatment Outcome, Trial Investigators Caffeine for Apnea of Prematurity (CAP), Very Low Birth Weight

Survival rates among children with severe neurologic disabilities

BACKGROUND: This study was done to determine survival rates in subpopulations of severely neurologically disabled children who reside in pediatric skilled nursing facilities and to compare these survival rates with those in previously published…

Survival estimates of severely disabled children

Supporting the family after the death of a child.

The death of a child can have a devastating effect on the family. The pediatrician has an important role to play in supporting the parents and any siblings still in his or her practice after such a death. Pediatricians may be poorly prepared to…

Sudden, unexpected infant death due to pulmonary arterial hypertension

A 3-year-old girl with no particular medical history complained of a stomachache and died on the way to the hospital. The autopsy revealed marked right ventricular hypertrophy and dilation with no other cardiac abnormalities. Microscopically, the…

Tags: 2014, Autopsy, Backlog, Cause Of Death, Child, Death, Female, Funayama M, Hashiyada M, Hayashizaki Y, Hosoya T, Humans, Hypertension, Igari Y, Journal Article, Kawasumi Y, Legal Medicine (tokyo, Japan), Ohuchi T, Preschool, Pulmonary, Sudden, Usui A

Studies on nutrition in severely neurologically disabled children in an institution

Severe neurological handicaps in children are frequently accompanied by growth retardation. We have studied 13 severely neurologically impaired children in an institution to see if their poor growth was related to a low intake of energy and…

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