Browse Items · Pediatric Palliative Care Library

Peroxisome biogenesis disorders with prolonged survival: phenotypic expression in a cohort of 31 patients

The peroxisome biogenesis disorders (PBDs) with generalized peroxisomal dysfunction include Zellweger syndrome (ZS), neonatal adrenoleukodystrophy (NALD), and infantile Refsum disease (IRD). There is clinical, biochemical, and genetic overlap among…

Long-term follow-up, neurological outcome and survival rate in 28 Nordic patients with glutaric aciduria type 1

All 28 patients, 13 females and 15 males, with glutaric aciduria type 1 diagnosed between 1975 and 2001 in Denmark, Finland, Norway and Sweden were identified and studied retrospectively until 2001. Mass screening was not performed. Three were…

Short-term safety assessment in the use of intravenous zoledronic acid in children

The clinical side effects of the potent new bisphosphonate zoledronic acid in children are unknown. In this study of 34 children with various bone disorders, the frequency of postinfusion flu-like illness, hypocalcemia, and hypophosphatemia was 85%,…

Mortality in parents after death of a child in Denmark: A nationwide follow-up study.

BACKGROUND: Little is known about the effect of parental bereavement on physical health. We investigated whether the death of a child increased mortality in parents. METHODS: We undertook a follow-up study based on national registers. From 1980 to…

Bereaved parents' experience of research participation.

Despite the ethical codes guiding bereavement research, few studies have been conducted to evaluate the perceived stress experienced by the bereaved, and to explore which methodologies cause least distress. This article investigates how bereaved and…

Unilateral closed-lip schizencephaly and epilepsy: a comparison with cases of unilateral polymicrogyria

We compared the electroclinical features and evolution of patients with two different types of abnormal cortical organization: unilateral closed-lip schizencephaly (SCHZ) and unilateral polymicrogyria (PMG). Between February 1990 and June 2002, 51…

Assessing the impact of pediatric epilepsy and concomitant behavioral, cognitive, and physical/neurologic disability: Impact of Childhood Neurologic Disability Scale

Epilepsy has a significant impact on a child's life, the extent to which is based on four factors: epilepsy, cognition, behavioral, and physical/neurologic function. This study evaluates the ability of the 44-item Impact of Childhood Neurologic…

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…

A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenic purpura (ITP) from the Intercontinental Childhood ITP Study Group

OBJECTIVE: To analyze prospectively the impact of age at diagnosis in childhood idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: International registry from June 1997 to May 2001, with analysis of data from baseline and 6-month-follow-up…

Parental stress and burden following traumatic brain injury amongst children and adolescents.

PRIMARY OBJECTIVES: To assess parental stress following paediatric traumatic brain injury (TBI), and examine the relationship between self-reported problems, parental stress and general health. RESEARCH DESIGN: Parents of 97 children admitted with a…

Polyethylene glycol without electrolytes for children with constipation and encopresis

BACKGROUND: Children with functional constipation and encopresis benefit from behavior modification and from long-term laxative medication. Polyethylene glycol without electrolytes has become the first option for many pediatric gastroenterologists.…

Joubert syndrome: long-term follow-up

Emotionally focused interventions for couples with chronically ill children: a 2-year follow-up

Couples with chronically ill children are particularly at risk for experiencing marital distress. The study presented here is a 2-year follow-up of a randomized control trial that assessed the efficacy of Emotionally Focused Therapy (EFT) in…

Follow-up on seven adult patients with mild Sanfilippo B-disease.

This report describes 7 patients, aged 30 to 43 years, suffering from a mild variant of Sanfilippo B-disease. Somatic findings in these patients are unremarkable. Dementia and behavioral disturbances occurred late in the course of the disease. All 7…

Tags: 1987, Adult, Age Factors, American Journal Of Medical Genetics, Backlog, Dementia/complications/genetics, Female, Follow-up Studies, Humans, Journal Article, Longitudinal Studies, Male, Mental Retardation/complications/genetics, Mucopolysaccharidoses/genetics, Mucopolysaccharidosis III/genetics, Pedigree, van de Kamp JJ, van Schrojenstein-de Valk HM

Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease).

Five patients with juvenile neuronal ceroid-lipofuscinosis, who were shown to have absent or reduced serum prebetalipoprotein and a deficiency of polyunsaturated fatty acids in erythrocyte membrane lipids, were treated for a period of one year with…

Congenital malformations of the central nervous system in a 1-year birth cohort followed to the age of 14 years

A 1-year birth cohort from northern Finland comprised 12,058 children, 96% of all live-born infants born in the region in 1966. The development and morbidity of these children were followed up to the age of 14 years. Altogether, 40 children (25 boys…

Radiological manifestations of bisphosphonate treatment with APD in a child suffering from osteogenesis imperfecta

A 12-year-old female suffering from osteogenesis imperfecta (OI) was treated with 3-amino-1-hydroxypropylidene-1,1-bisphosphonate (APD) orally, 250 mg daily, for periods of 2 months, alternating with periods of 2 months of abstinence. Total duration…

Tags: 1987, Backlog, Child, Clodronate, Devogelaer JP, Diphosphonates/therapeutic use, Female, Follow-up Studies, Humans, Journal Article, Maldague B, Malghem J, Nagant de Deuxchaisnes C, Osteogenesis Imperfecta/drug therapy/radiography, Skeletal Radiology

Wilson's disease: a longitudinal study of psychiatric symptoms

One hundred and twenty-nine cases of Wilson's disease (WD) were assessed at index admission and two follow-ups (F1 and F2) on a range of clinical and biochemical variables. The commonest psychiatric symptoms throughout were incongruous behavior,…

Beta-endorphin levels of children in acute stress

In this study aiming to clarify the relationships between beta-endorphin and glucose levels, beta-endorphin levels were determined in children in acute stress. The study was carried out on 32 critically ill children between 5 days and 12 years…

Juvenile neuronal ceroid lipofuscinosis (JNCL): quantitative description of its clinical variability

The clinical courses of 17 JNCL patients were analyzed retrospectively with the use of a simple, disease-specific scoring system. The mean observation period was 14 years (range 8-18 years). Scores of 0 (maximal dysfunction) to 3 (normal function)…

Tags: 1988, Acta Paediatrica, Adolescent, Adult, Albani M, Backlog, Child, Female, Follow-up Studies, Humans, Journal Article, Kohlschutter A, Laabs R, Longitudinal Studies, Male, Neuronal Ceroid-Lipofuscinoses/diagnosis, Retrospective Studies

Continuity of care: an approach to measurement

Buffers for the bereaved: the impact of social factors on the emotional health of bereaving parents

Emotional health of bereaving parents (N = 62; control N = 56) were explored 5 years after the death of a child (age 0-12) in Israel. The Symptom Check List-90 was utilized as the primary measurement instrument. Comparisons to controls according to…

Neuronal ceroid-lipofuscinosis: A clinical and morphological study of 19 patients.

We report on clinical, electrophysiological, neuroradiological, and morphological data from 19 patients with different types (late infantile, juvenile, and adult) of neuronal ceroid-lipofuscinosis (NCL), observed in the last 10 years at the…

Causal links between stressful events, coping style, and adolescent symptomatology

In a longitudinal study, the causal links between different types of stressors, coping styles and adolescent symptomatology were investigated. A total of 94 adolescents and their mothers participated in three annual assessments of critical life…

Tags: 2000, Adaptation, Adolescent, Adolescent Behavior/psychology, Backlog, Female, Follow-up Studies, Humans, Journal Article, Journal Of Adolescence, Life Change Events, Male, Prospective Studies, Psychological, Psychological/psychology, Seiffge-Krenke I, Stress

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…

Magnetic resonance spectroscopy in Niemann-Pick disease type C: Correlation with diagnosis and clinical response to cholestyramine and lovastatin.

Niemann-Pick type C is an autosomal-recessive, neurovisceral storage disorder that results from defective cholesterol esterification. Cholesterol-lowering agents have been demonstrated to decrease hepatic lipids in Niemann-Pick type C patients. The…

Living with cerebral palsy and tube feeding: A population-based follow-up study

OBJECTIVES: To assess the impact of surgically placed feeding tubes on children with severe cerebral palsy (CP) and their families and to determine the survival of these children after initiation of tube feeding (TF). METHODS: Virtually all children…

Structural equation modeling with longitudinal data: strategies for examining group differences and reciprocal relationships

This article describes the use of structural equation modeling with latent variables to examine group differences and test competing models about cause-effect relationships in passive longitudinal designs. This approach is compared with several other…

Postbereavement depressive mood and its prebereavement predictors in HIV+ and HIV- gay men

Prebereavement predictors of the course of postbereavement depressive mood were examined in 110 gay men who were their partner's caregiver until the partner's death of AIDS. In all, 37 HIV+ and 73 HIV- bereaved caregiving partners were assessed…

Vulnerability and resilience to combat exposure: can stress have lifelong effects?

The purpose of this study was to examine whether appraisals of desirable and undesirable effects of military service mediated the effect of combat stress on posttraumatic stress disorder (PTSD) symptoms in later life in 1,287 male veterans, aged…

Predictors of burden in spouse caregivers of individuals with Alzheimer's disease

This study used the following model of distress: Distress = [Exposure to Stress + Vulnerability]/[Psychological and Social Resources]. The constructs in the model were operationalized as (a) distress in response to caregiver experiences (burden); (b)…

Efficacy and complications of morphine infusions in postoperative paediatric patients

The aim of the study was to evaluate the efficacy and the incidence of clinically significant adverse drug reactions (ADRs) in paediatric patients receiving continuous intravenous morphine infusions for acute postoperative pain. Definitions were…

Follow-up in children with Joubert syndrome

Although Joubert syndrome (JS) was first reported in 1969 by Joubert et al (21), the long-term outcome is not yet documented. We report 19 children (4 pairs of siblings) from a single institution diagnosed with JS. Nine children were last seen…

Predictors of husbands' and wives' grief reactions following infant death: the role of marital intimacy

This follow-up study examined how bereaved couples' grief reactions change over time and how the quality of the marriage can predict these reactions for men and women. A group of 31 bereaved couples who 2 to 4 years earlier had lost an infant (…

Tags: 1996, Amsel R, Backlog, Death studies, Fetal Death, Follow-up Studies, Gottlieb LN, Grief, Humans, Infant, Infant Mortality, Journal Article, Lang A, Marriage, Newborn, Parent caregivers, Questionnaires, Spouses/psychology

Spiritual aspects of loss among partners of men with AIDS: postbereavement follow-up

This article is a follow-up study of bereaved caregiving male partners of men with AIDS (T.A. Richards & S. Folkman, 1997). The earlier study examined spiritual beliefs, experiences, and practices reported in interviews with 125 caregivers conducted…

Serious adverse events after measles-mumps-rubella vaccination during a fourteen-year prospective follow-up

BACKGROUND: Several disorders have been attributed to measles-mumps-rubella (MMR) vaccination during the past decade. The aim of this prospective follow-up study was to identify serious adverse events causally related to MMR vaccination. METHODS:…

Status epilepticus in children: aetiology, treatment, and outcome

This retrospective study includes 65 children treated for status epilepticus at Tampere University Hospital in Finland. Aetiology of the condition, effectiveness of the treatment protocol, including short barbiturate anaesthesia to prevent prolonged…

Tags: 1997, Adolescence, Age Distribution, Anesthetics, Anticonvulsants, Backlog, Barbiturates/ad [Administration & Dosage], Chi-Square Distribution, Child, Developmental Medicine & Child Neurology, Diazepam/ad [Administration & Dosage], Drug Administration Schedule, Electroencephalography, Encephalitis/co [Complications], Eriksson KJ, Female, Follow-up Studies, Human, Infant, Intravenous/ad [Administration & Dosage], Journal Article, Koivikko MJ, Male, Meningitis, Nervous System Diseases/ep [Epidemiology], Neuropsychological Tests, Non-U.S. Gov't, Preschool, Prevalence, Retrospective Studies, Risk Factors, Septic/co [Complications], Sex Distribution, Shock, Status Epilepticus/di [Diagnosis], Status Epilepticus/dt [Drug Therapy], Status Epilepticus/ep [Epidemiology], Status Epilepticus/et [Etiology], Streptococcal Infections/co [Complications], Support, Time Factors, Treatment Outcome, Viral/co [Complications]

Influence of epilepsy on mortality in mental retardation: an epidemiologic study

PURPOSE: A cohort consisting of all persons with known mental retardation (MR) and living in a Swedish province on December 31, 1985, was followed for 7 years (1987-1992) to study the mortality pattern. METHODS: A file of the cohort was linked to the…

Reduced life expectancy in 40 cases of early onset cerebellar ataxia with retained tendon reflexes: a population-based study

A survival analysis of 40 cases of early onset cerebellar ataxia (EOCA) with retained tendon reflexes was performed. They represent all cases of EOCA diagnosed between 1945 and 1990 among residents of a defined area of Northwestern Italy, followed up…

Late onset globoid cell leukodystrophy (Krabbe's disease)--Swedish case with 15 years of follow-up.

We describe a male patient with late onset globoid cell leukodystrophy (GLD) (Krabbe's disease) still alive at 24 years of age, with a well preserved intellectual and communicative capacity, in contrast to visual failure and severe central pyramidal…

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