Browse Items · Pediatric Palliative Care Library

Warfarin for atrial fibrillation. The patient's perspective

OBJECTIVE: To determine the minimal clinically important difference (MCID) of warfarin therapy for the treatment of nonvalvular atrial fibrillation from the perspective of patients using 2 different elicitation methods. DESIGN: All patients completed…

Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

Purpose: Preliminary validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) for motor skill assessment in spinal muscular atrophy type I. Methods: A total of 27 subjects 3 to 260 months old (mean =…

Tags: (health care), 2011, atrophies of childhood/diagnosis, Child, Child Development/physiology, childhood/physiopathology, CHOP INTEND, Chung W K, Darras B T, De Vivo D C, Deng L Y, Disability Evaluation, Disease Severity, Dunaway S, Female, Finkel R S, Flickinger J, Glanzman A M, Humans, Infant, Kaufmann P, Male, Martens W B, McDermott M P, Montes J, Motor Skills/physiology, Muscle Study Group, natural-history, O'Hagen J, outcome assessment, Pediatric Neuromuscular Clinical Research, Pediatric Physical Therapy, Pediatrics, physical therapy/methods, Preschool, Psychometrics/methods, Quigley J, Rehabilitation, Riley S, scale development, Severity Of Illness Index, Sma, SMA1, smn2 copy number, spinal muscular, spinal muscular atrophies of, spinal muscular-atrophy, Sproule D, Tawil R, tone and motor problems, tool development, Validity, Yang M

Validation of an Electronic Pediatric Index of Mortality 2 Score in a Mixed Quaternary PICU

OBJECTIVE: To assess the validity of an electronic version of the Pediatric Index of Mortality 2 score. DESIGN: Retrospective observational study. SETTING: Pediatric and cardiac ICUs at a quaternary medical center. PATIENTS: Patients more than 60…

Tags: 2020, Child, Cohort Studies, Crana CM, Electronics, Hospital Mortality, Humans, Infant, Intensive Care Units Pediatric, Joyce EL, Kellum JA, March 2021 List, Pediatric Critical Care Medicine, Retrospective Studies, Severity Of Illness Index, Yabes J

Usefulness of consecutive biomarkers measurement in the management of community-acquired pneumonia.

The aim of this study was to investigate whether procalcitonin (PCT), neopterin,

Use of bisphosphonate therapy for osteoporosis in childhood and adolescence

Congenital and acquired forms of osteoporosis in childhood and adolescence can result in morbidity from fracture and pain in childhood, and place an individual at significant risk for problems in adult life. A range of therapies exist for the…

Treatment of children with unexplained chronic pain

2006

Tags: 2006, Adolescent, Backlog, Child, Chronic Disease/therapy, Cognitive Therapy/methods, Humans, Journal Article, Kashikar-Zuck S, Lancet, Pain/classification/psychology/therapy, PedPal Lit, Severity Of Illness Index

Transdermal fentanyl in the management of children with chronic severe pain: results from an international study

BACKGROUND: The current study was conducted to assess the safety and tolerability of a transdermal fentanyl delivery system for the relief of chronic pain in a pediatric population, and also to validate titration recommendations and conversion to…

The use of clonidine for severe and intractable sleep problems in children with neurodevelopmental disorders--a case series

This paper reports on the use of clonidine for the treatment of severe sleep problems associated with behavioural difficulties in children with neurodevelopmental disabilities. Data were obtained from reviewing the case notes of a series of six…

The Spanish version of the dementia quality of life questionnaire: a validation study

BACKGROUND: The aim of the study was to adapt and validate culturally the dementia-specific health-related quality of life instrument (HRQoL) into Spanish for patients with mild to moderate dementia. METHODS: Two forward translations, a reconciled…

The natural history of Unverricht-Lundborg disease: a report of eight genetically proven cases

We report eight cases of genetically proven ULD, with the aim of reassessing the clinical characteristics and natural history of ULD in genetically characterized patients. The eight patients had their first symptoms at mean age of 10.6 years (range:…

Tags: 2008, Adolescent, Adult, Age Factors, Atrophy/complications/pathology, Auditory, Backlog, Bhatia KP, Brain Stem/physiology, Brain/pathology/physiopathology, Cerebellar Ataxia/complications/diagnosis, Cerebellum/pathology/physiopathology, Chew NK, Child, Chromosomes, Cordivari C, Dementia/complications/diagnosis, Diagnosis, Differential, Disease Progression, Dystonia/complications/diagnosis, Edwards MJ, Electroencephalography, Electromyography, Evoked Potentials, Evoked Potentials/physiology, Female, Human, Humans, Journal Article, Kim HT, Magnetic Resonance Imaging, Male, Martino D, Mir P, Movement Disorders: Official Journal Of The Movement Disorder Society, Myoclonus/complications/diagnosis, Neuropsychological Tests, Pair 21/genetics, Q3 Scoping Review Results, Quinn NP, Schneider SA, Seizures/complications/diagnosis, Severity Of Illness Index, Unverricht-Lundborg Syndrome/diagnosis/genetics/physiopathology

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 Memorial Symptom Assessment Scale: an instrument for the evaluation of symptom prevalence, characteristics and distress

The Memorial Symptom Assessment Scale (MSAS) is a new patient-rated instrument that was developed to provide multidimensional information about a diverse group of common symptoms. This study evaluated the reliability and validity of the MSAS in the…

The management of pain in children with life-limiting illnesses

The management of pain in children with life-limiting illnesses is complex and unfortunately not often done effectively. Pain is a multidimensional symptom that can overshadow all other experiences of both the child and family. This article focuses…

The Impact of Mortality on Total Costs Within the ICU

OBJECTIVES: The high cost of critical care has engendered research into identifying influential factors. However, existing studies have not considered patient vital status at ICU discharge. This study sought to determine the effect of mortality upon…

The health and well-being of caregivers of children with cerebral palsy.

OBJECTIVE: Most children enjoy healthy childhoods with little need for specialized health care services. However, some children experience difficulties in early childhood and require access to and utilization of considerable health care resources…

The gross motor function measure is a valid and sensitive outcome measure for spinal muscular atrophy

Spinal muscular atrophy is a genetic disease of the anterior horn cell with high morbidity rate in childhood. Certain drugs may be of benefit and are in or under consideration for Phase II trials. Outcome measures that are age appropriate and…

The development and evaluation of the pain indicator for communicatively impaired children (PICIC)

A previous study found that parents of communicatively impaired children with severe cognitive impairments identified six core cues as indicating definite or severe pain in their child (J. Pediatr. Psychol. 27 (2002) 209). The frequency of each cue…

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…

Symptoms of anxiety and depression in family members of intensive care unit patients before discharge or death. A prospective multicenter study

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…

Sleep in individuals with Cri du Chat syndrome: a comparative study

BACKGROUND: Sleep problems are common in individuals with intellectual disability. Little is known about sleep in children and adults with Cri du Chat syndrome (CDC).;METHOD: Sleep was investigated in 30 individuals with CDC using a sleep…

Sleep disturbance in Mowat-Wilson syndrome

Mowat-Wilson syndrome (MWS) is a multiple congenital anomaly syndrome caused by a heterozygous mutation or deletion of the ZEB2 gene. It is characterized by a distinctive facial appearance in association with intellectual disability (ID) and variable…

Tags: 0 (Homeodomain Proteins), 0 (Repressor Proteins), 0 (ZEB2 protein human), 2016, Adolescent, Adult, American Journal of Medical Genetics Part A, Australia, Behavior Rating Scale, characteristics, Child, Child Preschool, Einfeld S, Evans E, Facies, Female, Gene Expression, Hirschsprung Disease/co [Complications], Hirschsprung Disease/di [Diagnosis], Hirschsprung Disease/ge [Genetics], Hirschsprung Disease/pp [Physiopathology], Homeodomain Proteins/ge [Genetics], Humans, Intellectual Disability/co [Complications], Intellectual Disability/di [Diagnosis], Intellectual Disability/ge [Genetics], Intellectual Disability/pp [Physiopathology], Male, Microcephaly/co [Complications], Microcephaly/di [Diagnosis], Microcephaly/ge [Genetics], Microcephaly/pp [Physiopathology], Middle Aged, Mowat D, Mowat-Wilson syndrome, Mutation, Repressor Proteins/ge [Genetics], Severity Of Illness Index, sleep disturbance/disorders, Sleep Initiation and Maintenance Disorders/co [Complications], Sleep Initiation and Maintenance Disorders/di [Diagnosis], Sleep Initiation and Maintenance Disorders/ge [Genetics], Sleep Initiation and Maintenance Disorders/pp [Physiopathology], Surveys And Questionnaires, Trajectory, Wilson M

Sleep alterations in juvenile neuronal ceroid-lipofuscinosis

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…

Self-injurious behaviour in young children with Lesch-Nyhan syndrome

The early development of self-injurious behaviour in three young boys (aged 17, 25, and 30 months at start of study) with Lesch-Nyhan syndrome was examined by means of parental interviews and by direct observations completed at 3 to 4 monthly…

Tags: 2001, behavioral problems, characteristics, Child Preschool, Developmental Medicine and Child Neurology, Environment, Extinction Psychological, Hall S, Humans, Lesch-Nyhan syndrome, Lesch-Nyhan Syndrome/diagnosis/prevention & control, Male, Murphy G, Oliver C, Self-Injurious Behavior/prevention & control, self-injury, Severity Of Illness Index, Sign Language, Trajectory

Reliability and predictive validity of the Standardized Infant NeuroDevelopmental Assessment neurological scale

AIM: To assess reliability and predictive validity of the neurological scale of the Standardized Infant NeuroDevelopmental Assessment (SINDA), a recently developed assessment for infants aged 6 weeks to 12 months. METHOD: To assess reliability, three…

Tags: 2019, Developmental Medicine and Child Neurology, Diagnostic Techniques, Female, Hadders-Algra M, Humans, Infant, Longitudinal Studies, Male, Neurodevelopmental Disorders/diagnosis, Neurological/standards, November 2019 List, Philippi H, Pietz J, Reproducibility of Results, Rupp A, Severity Of Illness Index, Tacke U

Relationship of illness severity and length of stay to functional outcomes in the pediatric intensive care unit: a multi-institutional study

OBJECTIVE: The purpose of this study was to establish relationships between illness severity, length of stay, and functional outcomes in the pediatric intensive care unit (PICU) by using multi-institutional data. We hypothesized that a positive…

Pyruvate dehydrogenase E3 binding protein (protein X) deficiency

Pyruvate dehydrogenase (PDH) deficiency is a major cause of neurological dysfunction and lactic acidosis in infancy and early childhood. The great majority of cases (>80%) result from mutations in the X-linked gene for the E1alpha subunit of the…

Prognosis for survival and improvement in function in children with severe developmental disabilities

OBJECTIVE: To derive prognostic data for survival and clinical improvement in children with severe developmental disabilities. STUDY DESIGN: A 13-year follow-up study of several cohorts of children initially evaluated before their first birthday. The…

Prevalence and severity of feeding and nutritional problems in children with neurological impairment: Oxford Feeding Study

The aim of this study was to estimate the prevalence and severity of feeding and nutritional problems in children with neurological impairment within a defined geographical area. In a cross-sectional study, a validated questionnaire was sent to 377…

Prediction of Acquired Morbidity Using Illness Severity Indices in Pediatric Intensive Care Patients

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…

Pediatric risk of mortality (PRISM) score

The Pediatric Risk of Mortality (PRISM) score was developed from the Physiologic Stability Index (PSI) to reduce the number of physiologic variables required for pediatric ICU (PICU) mortality risk assessment and to obtain an objective weighting of…

Tags: 1988, Backlog, Child, Critical Care Medicine, Getson PR, Humans, Infant, Infant Mortality, Intensive Care Units, Journal Article, Mortality, Multicenter Studies as Topic, Pediatric, Pollack MM, Preschool, Risk Factors, ROC Curve, Ruttimann UE, Severity Of Illness Index

Pediatric Ethics and Communication Excellence (PEACE) Rounds: Decreasing Moral Distress and Patient Length of Stay in the PICU

This paper describes a practice innovation: the addition of formal weekly discussions of patients with prolonged PICU stay to reduce healthcare providers' moral distress and decrease length of stay for patients with life-threatening illnesses. We…

Palliative medicine consultation for preparedness planning in patients receiving left ventricular assist devices as destination therapy.

OBJECTIVE: To assess the benefit of proactive palliative medicine consultation for delineation of goals of care and quality-of-life preferences before implantation of left ventricular assist devices as destination therapy (DT)., PATIENTS AND METHODS:…

Pain, anxiety, distress, and suffering: interrelated, but not interchangeable

Pain in children with cerebral palsy: a review.

Children with cerebral palsy (CP) face many challenges including impaired motor control and coordination, functional impairment, sensory disturbances, and, sometimes, communication difficulties and cognitive deficits. Pain also may be a problem for…

Pain in children with cerebral palsy: a cross-sectional multicentre European study.

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…

Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a cross-sectional study with long-term follow-up

PURPOSE: To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency - a life-threatening metabolic disease - and the relation to age at diagnosis,…

Objective assessment of changing mortality risks in pediatric intensive care unit patients.

OBJECTIVE: To develop and validate a mortality risk predictor based on physiologic data that estimates daily the probability of a patient dying within the next 24 hrs as that probability changes with disease and recovery. SETTING: Nine pediatric ICUs…

Nurses' attitudes to pain management during routine venepuncture in young children

Venepuncture is one of the most commonly performed clinical procedures carried out on young children. Evidence indicates inconsistency in the use of pain management strategies during these procedures. A survey method was used to explore nurses' views…

Neurological presentation in pediatric patients with congenital disorders of glycosylation type Ia

OBJECTIVE: Congenital disorders of glycosylation (CDG), formerly called carbohydrate-deficient glycoprotein syndromes, constitute a newly identified group of multisystem disorders characterized by defective glycosylation of N-glycosylated proteins.…

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