Browse Items · Pediatric Palliative Care Library

A clinical rating scale for Batten disease Reliable and relevant for clinical trials

2005

Tags: 2005, Adams H, Adolescent, Adult, Backlog, Child, Children W/SNI, Clinical Trials/standards, de Blieck EA, DeYoung J, Disease Progression, Dure L, Female, Humans, Journal Article, Levy Erika, Longitudinal Studies, Male, Marshall FJ, McDonough T, Messing S, Mink JW, Neurologic Examination/methods/standards, Neurology, Neuronal Ceroid-Lipofuscinoses/diagnosis/physiopathology/psychology, Neuropsychological Tests/standards, others, Personality Tests/standards, Predictive Value of Tests, Preschool, Prognosis, Rothberg PG, Severity Of Illness Index, Treatment Outcome

A community survey of children with severe intellectual disability and their families: psychological adjustment, carer distress and the effect of respite care

The present paper describes a cross-sectional study of the psychosocial adjustment of 143 children with severe disability and their families identified from a regional case register for children with special needs. Thirty-eight per cent of the…

A modified Hammersmith functional motor scale for use in multi-center research on spinal muscular atrophy

The Hammersmith functional motor scale for children with spinal muscular atrophy was modified to establish a standard measure of functional ability in children with non-ambulant spinal muscular atrophy types 2 and 3 in a longitudinal multi-center…

A report of four cases of acute, severe pulmonary hemorrhage in infancy and support with extracorporeal membrane oxygenation

Introduction
Pulmonary hemorrhage is an uncommon event in infants. It has been described most commonly in the sick premature neonate, older child, or adolescent with chronic cardiopulmonary disease. Acute idiopathic pulmonary hemorrhage in…

Tags: 1994, Acute Disease, Artificial, Backlog, Extracorporeal Membrane Oxygenation, Female, Hemorrhage/complications/therapy, Humans, Infant, Journal Article, Lung Diseases/complications/therapy, Male, Moler FW, Pediatric Pulmonology, Respiration, Respiratory Insufficiency/etiology/therapy, Sanders GM, Severity Of Illness Index, Siden HB

Acute and chronic paediatric intensive care patients: current trends and perspectives on resource utilization

BACKGROUND: Advances in paediatric critical care have resulted in increased survival of critically ill patients, many of whom require long-term ventilation as a means of life support. AIM: To determine current trends in resource utilization, and…

Advance care planning in adolescents with cystic fibrosis: A quality improvement project

INTRODUCTION: Advance care planning (ACP), though recommended, has not been studied in adolescents with cystic fibrosis (CF). This quality improvement project engaged adolescents with advanced CF disease in ACP and assessed patient and CF provider…

Advanced illness index: Predictive modeling to stratify elders using self-report data

OBJECTIVES: Develop a prediction model to identify persons who have an increased risk of dying within the next 36 months, in order to focus additional resources and assessment in areas related to advanced care planning. DESIGN: Retrospective study…

Advice seeking and appropriate use of a pediatric emergency department

OBJECTIVES: To determine whether seeking advice prior to an unscheduled visit to a pediatric emergency department (PED) influences appropriate use of this setting for minor illnesses. DESIGN: Cross-sectional questionnaire survey. SETTING: The medical…

Anorexia/cachexia-related quality of life for children with cancer

BACKGROUND: Anorexia is a common symptom in patients with cancer, which can lead to poor tolerance of treatment and can contribute to cachexia in extreme cases. Children with advanced-stage cancer are especially vulnerable to malnutrition resulting…

Assessing the clinical importance of symptomatic improvements. An illustration in rheumatology

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…

Behavioral aspects of Lesch-Nyhan disease and its variants

Self-injury is a defining feature of lesch-nyhan disease (LND) but does not occur in the less severely affected Lesch-Nyhan variants (LNV). The aim of this study was to quantify behavioral and emotional abnormalities in LND and LNV. Thirty-nine…

Characteristics and Outcomes of Critical Illness in Children With Feeding and Respiratory Technology Dependence

OBJECTIVES: Children with dependence on respiratory or feeding technologies are frequently admitted to the PICU, but little is known about their characteristics or outcomes. We hypothesized that they are at increased risk of critical illness-related…

Chronic abdominal pain in children: an update

Despite the fact that chronic abdominal pain is a common complaint in children and adolescents, the complexity of this condition only recently has begun to be understood. This article summarizes new advances in the diagnosis, conceptualization, and…

Classification and definition of disorders causing hypertonia in childhood

OBJECTIVE: This report describes the consensus outcome of an interdisciplinary workshop that was held at the National Institutes of Health in April 2001. The purpose of the workshop and this article are to define the terms "spasticity," "dystonia,"…

Clinical rating scale for pantothenate kinase-associated neurodegeneration: A pilot study

BACKGROUND: Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the…

Tags: 2017, Adolescent, Adult, Aged, Aguilera-Albesa S, Behavior, Castro C A, Child, Coelho M, Cognitive Dysfunction/di [Diagnosis], Cognitive Dysfunction/et [Etiology], Correia G L, Costa C, Cross-sectional Studies, Cuadras P D, Darling A, Disabled Persons, dystonia/di [Diagnosis], Dystonia/et [Etiology], Espinos C, Ferreira J, Garcia P J, Garrido C, Gaston I, Gavilan I T, Gonzalez G L, Gutierrez F, Hernandez L C, Humans, IND, Lin J P, Lorenzo S G, Lupo V, Madruga M, Magalhaes M, Marti M J, Marti S L, Martinez-Martin P, Martorell L, Mental Disorders/di [Diagnosis], Mental Disorders/et [Etiology], Middle Aged, Moreira F, Movement Disorders, Nardocci N, Ocular Motility Disorders/di [Diagnosis], Ocular Motility Disorders/et [Etiology], Ortigoza E J D, Pantothenate Kinase-Associated Neurodegeneration/co [Complications], Pantothenate Kinase-Associated Neurodegeneration/di [Diagnosis], Pantothenate Kinase-Associated Neurodegeneration/ge [Genetics], Parkinsonian Disorders/di [Diagnosis], Parkinsonian Disorders/et [Etiology], Perez D B, Pilot Projects, Pires P, PKANDRS, Pons R, Pujol M, Ramos L J, Rego P, Reproducibility of Results, Rodriguez-Blazquez C, scale development, Severity Of Illness Index, Stamelou M, Stefanis L, Tello C, Temudo T, Tomas V M, tone and motor problems, tool development, Tustin K, Vela L, Young Adult, Zorzi G

Clinician predictions of intensive care unit mortality

OBJECTIVE: Predicting outcomes for critically ill patients is an important aspect of discussions with families in the intensive care unit. Our objective was to evaluate clinical intensive care unit survival predictions and their consequences for…

Comparison of continuous subcutaneous and intravenous hydromorphone infusions for management of cancer pain

To compare the safety and efficacy of subcutaneous and intravenous infusion of opioid analgesics, a randomised, double-blind, crossover trial was carried out in inpatients. 15 patients with severe cancer pain received two 48 h infusions of…

Coping with Dravet syndrome: parental experiences with a catastrophic epilepsy

The aim of this study was to investigate how parents cope with and care for a child with Dravet syndrome, a severe myoclonic epilepsy with three distinct stages. Twenty-four parents of children with Dravet syndrome participated in a semi-structured…

Coping, self-efficacy and psychiatric history in patients with both chronic widespread pain and chronic fatigue

OBJECTIVE: To investigate the relationship of coping style and self-efficacy to functional impairment in a group of patients with both chronic widespread pain (CWP) and chronic fatigue, as well as the possible mediating role of psychiatric diagnosis.…

COVID-19 Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children and adolescents: a systematic review of critically unwell children and the association with underlying comorbidities

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…

Decisions to forgo life-sustaining therapy in ICU patients independently predict hospital death

OBJECTIVE: More than one-half the deaths of patients admitted to intensive care units (ICUs) occur after a decision to forgo life-sustaining therapy (DFLST). Although DFLSTs typically occur in patients with severe comorbidities and intractable acute…

Determinants of sleep disturbances in Rett syndrome: Novel findings in relation to genotype

Rett syndrome is a rare but severe neurological disorder associated with a mutation in the methyl CpG binding protein 2 (MECP2) gene. Sleep problems and epilepsy are two of many comorbidities associated with this disorder. This study investigated the…

Development of global rating instruments for pediatric patients with ataxia telangiectasia

INTRODUCTION: Ataxia telangiectasia (AT) is a neurodegenerative disorder with cerebellar and extrapyramidal features. Interventional and epidemiological studies in AT should rely on specific scales which encompass the specific neurological features,…

Diagnostic evaluation using whole-body technetium bone scan in children with cerebral palsy and pain.

BACKGROUND: Pain in noncommunicative children can be difficult to localize and diagnose. The purpose of this study is to report our experience using a 3-phase whole-body technetium bone scan as a screening tool in identifying the source of persistent…

Differences in pediatric ICU mortality risk over time

OBJECTIVES: To compare pediatric intensive care unit (ICU) mortality risk using models from two distinct time periods; and to discuss the implications of changing mortality risk for severity systems and quality-of-care assessment. DATA SOURCES AND…

Direct measurement of intracranial pressure at high altitude and correlation of ventricular size with acute mountain sickness: Brian Cummins' results from the 1985 Kishtwar expedition

OBJECTIVE AND IMPORTANCE: The "tight-fit" hypothesis and subsequent current understanding of acute mountain sickness (AMS) is that individuals with less compliant cerebrospinal fluid systems (smaller ventricles and cerebrospinal fluid spaces) have a…

Disease severity in children and adults with Pompe disease related to age and disease duration

Information about 255 children and adults with Pompe disease was gathered through a questionnaire. Disease severity was associated with disease duration and not with age; an early manifestation of the disease implied earlier wheelchair or ventilator…

Disorders of Upper Limb Movements in Ataxia-Telangiectasia

Ataxia-telangiectasia is known for cerebellar degeneration, but clinical descriptions of abnormal tone, posture, and movements suggest involvement of the network between cerebellum and basal ganglia. We quantitatively assessed the nature of…

Driscoll Children's Hospital respiratory syncytial virus database: risk factors, treatment and hospital course in 3308 infants and young children, 1991 to 2002

BACKGROUND: Treatment of respiratory syncytial virus (RSV) lower respiratory tract infection has historically been one of the most frequent reasons for admission to Driscoll Children's Hospital. OBJECTIVE: The objective of this study was to examine…

Duration and morbidity of newly diagnosed idiopathic thrombocytopenic purpura in children: A prospective Nordic study of an unselected cohort

OBJECTIVE: To determine the duration of the risk period with platelet counts 1 month and 25 had 30 events. Among 93 patients with chronic ITP, 73 were at risk >1 month and 44 had 111 events. Events occurred with an average frequency of 0.39 per month…

End-of-life Decision-making For Children With Severe Developmental Disabilities: The Parental Perspective.

BACKGROUND AND AIMS:
The objectives of this integrative review were to understand how parents of children with severe developmental disorders experience their involvement in end-of-life decision-making, how they prefer to be involved and what factors…

Ethical Dilemmas In Postnatal Treatment Of Severe Congenital Hydrocephalus.

Severe congenital hydrocephalus manifests as accumulation of a large amount of excess fluid in the brain. It is a paradigmatic example of a condition in which diagnosis is relatively straightforward and long-term survival is usually associated with…

Factors influencing family participation in a longitudinal study: comparison of pediatric and healthy samples.

Compared participation levels in a longitudinal study of parent-child relationships from infancy to 4 years in families of children with cystic fibrosis, congenital heart disease, and with no chronic illness. Demographic (parent's age, education) and…

Functional electrical stimulation therapy for recovery of reaching and grasping in severe chronic pediatric stroke patients

Stroke affects 2.7 children per 100,000 annually, leaving many of them with lifelong residual impairments despite intensive rehabilitation. In the present study the authors evaluated the effectiveness of 48 hours of transcutaneous functional…

Gabapentin Successfully Manages Chronic Unexplained Irritability in Children With Severe Neurologic Impairment

Neurologically impaired children have an increased frequency of recurrent pain and irritability that persist in some despite comprehensive evaluation and management of possible pain sources. We hypothesized that visceral hyperalgesia was a source of…

Health and sleep problems in Cornelia de Lange Syndrome: a case control study

BACKGROUND: Self-injury, sleep problems and health problems are commonly reported in Cornelia de Lange Syndrome (CdLS) but there are no comparisons with appropriately matched participants. The relationship between these areas and comparison to a…

Health care resource utilization in adults with congenital heart disease

The number of adults with congenital heart disease (CHD) is increasing. However, rates of health care resource utilization in this population are unknown. The objectives of this study were to describe the use of general health care resources in…

How Parents And Physicians Experience End-of-life Decision-making For Children With Profound Intellectual And Multiple Disabilities.

BACKGROUND:
End-of-life decisions (EoLD) often concern children with profound intellectual and multiple disabilities (PIMD). Yet, little is known about how parents and physicians discuss and make these decisions.
AIMS:
The objective of this research…

Identifying futility in a paediatric critical care setting: a prospective observational study

AIMS: To determine the extent of futile care provided to critically ill children admitted to a paediatric intensive care setting. METHODS: Prospective evaluation of consecutive admissions to a 20 bedded multidisciplinary paediatric intensive care…

Impact of research participation on parents of seriously ill children.

BACKGROUND: There is a paucity of research evidence to guide health care providers' practice in pediatric palliative care. At the same time, some clinicians and Institutional Review Boards are reluctant to approve such studies because of concerns…

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