Browse Items (94 total)

BACKGROUND: Some pregnant patients with complex fetal anomalies meet with paediatric palliative care subspecialists prior to delivery, but referral to antenatal palliative care consultation (APCC) is not standard. Little is known about its role in…

Importance: The death of a close relative is associated with an increased mortality risk among the bereaved, but much less is known about the potential association of the death of a sibling in childhood with mortality in this population. Objective:…

BACKGROUND: We investigated the clinical characteristics that represent risk factors for death in pediatric patients with mitochondrial diseases. METHODS: The medical records of mitochondrial disease pediatric patients attended between 2006 and 2015…

Previous findings have suggested that the loss of a family member is associated with mortality among bereaved family members. The least-studied familial relationship in the bereavement literature is that of siblings, although loss of a sibling may…

BACKGROUND: When curative treatments are no longer options for patients dying of cancer, the focus of care often turns from prolonging life to promoting quality of life (QOL). Few data exist on what predicts better QOL at the end of life (EOL) for…

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…

The purpose of this study was to assess the receptiveness of parents to information given about their child's life threatening illness. Three months after the child's diagnosis, an independent interviewer, using a structured questionnaire,…

We documented seizures in 33 of 68 (48.5%) children with congenital hydrocephalus not associated with myelomeningocele. Mental retardation (MR) and CNS malformations correlated with seizure occurrence; age at shunt insertion and number of shunt…

OBJECTIVE: To investigate cardiac function in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and clarify the clinical features of cardiomyopathy in MELAS. PATIENTS: 11 consecutive patients with MELAS…

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…

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…

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:…

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…

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 (…

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)…

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…

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…

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…

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…

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…

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…

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…

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)…

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…

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,…

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…

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…

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…
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