Browse Items (147 total)

The records of 52 children with Niemann-Pick disease type C were reviewed to establish whether the disease process and outcome varied with the initial clinical pattern; 34 children (65%) had cholestatic liver disease and hepatosplenomegaly in…

The goal of this study was to examine sources of variation in the utilities assigned to health states. The authors selected a common clinical problem, carcinoma of the rectum, and examined the utilities assigned to colostomy, a common outcome of…

Although most clinicians agree that patients should be informed about treatment alternatives, little is known about the way patients perceive probabilistic information about treatment outcomes and how it influences the choices they make. The purpose…

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…

A retrospective study of 54 patients who underwent feeding jejunostomy because of dysphagia on a neurologic basis was performed to determine risk factors affecting a postoperative mortality of 33% and six-month mortality of 67%. Advanced patient age,…

Predicting survival and disclosing the prediction to patients with advanced disease, particularly cancer, is among the most difficult tasks that physicians face. With the de-emphasis of prognosis in favor of diagnosis and therapeutics in the medical…

Cachexia is a syndrome and therefore does not have a specific definition. Patients are characterized by the presence of anorexia, early satiety, weight loss, weakness, anaemia and oedema. These features occur to a variable extent in different…

OBJECTIVES: The objective of this study is to investigate the relationship between a physician's subjective mortality prediction and the level of confidence with which that mortality prediction is made. DESIGN AND PARTICIPANTS: The study is a…

The aim of this study was to reach consensus about the prognostic factors when deciding the discharge destination from a hospital stroke unit, and to construct a prognostic conceptual framework. To realise an optimal integration of knowledge from…

OBJECTIVE: To identify priorities for quality end-of-life care from the parents' perspective. DESIGN: Anonymous, self-administered questionnaire. SETTING: Three pediatric intensive care units in Boston. PARTICIPANTS: Parents of children who had died…

The presence in blood of proteins normally confined to the cytoplasm of brain cells is considered peripheral evidence of brain damage. Only recently have these proteins been measured in the blood of children at risk of brain damage. To show the value…

We studied 42 individuals, including 8 patients with either complete or partial syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS), 8 patients with either complete or partial syndrome of myoclonic…

Cachexia, i.e. body wasting, has long been recognised as a serious complication of chronic illness. The occurrence of wasting in chronic heart failure (CHF) has been known for many centuries, but it has not been investigated extensively until…

The overall cure rate for cancer in childhood now exceeds 70% and is projected to reach 85% by the year 2010 in industrialized countries. Therefore, major attention is being placed on reducing the side effects of therapy. However, 85% of the world's…

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…

OBJECTIVE: This study examined the relationships between pain characteristics, psychosocial factors, and quality of life among adolescents with chronic pain that existed for at least 3 months, either recurrently (ie, pain with pain-free intervals) or…

OBJECTIVES: We sought to determine the long-term clinical and biochemical outcome of newborns with mitochondrial cytopathies (MCs) and to identify possible prognostic factors that may modify the course of these diseases. MATERIAL AND METHODS:…

A retrospective, multicenter study of 180 children with cytochrome c oxidase (COX) deficiency analyzed the clinical features, prognosis, and molecular bases of the COX deficiency. Clinical symptoms including failure to thrive, encephalopathy,…

AIM: To describe the epidemiology of idiopathic thrombocytopenic purpura (ITP) in the Nordic countries, to define clinical subgroups and to investigate factors predicting chronic disease. METHODS: A prospective registration was done from 1998 to…

PURPOSE OF REVIEW: Pediatric palliative care, with its emphasis on symptom management and quality of life, is an important aspect of care of children with life-threatening illnesses. We review recent publications with implications for care of these…

Late-onset Pompe's disease (acid maltase deficiency, glycogen storage disease type II) is a slowly progressive myopathy caused by deficiency of acid alpha-glucosidase. Current developments in enzyme replacement therapy require detailed knowledge of…

BACKGROUND: Accurate prognostication of the trajectory of an illness provides multiple benefits in end-of-life care. Prognostic information facilitates more realistic decision making regarding ongoing treatment, fosters risk-benefit considerations of…

Mitochondrial diseases affect all age groups, but those with childhood onset often seem to experience the greatest burden of disability. In some paediatric patients this can be explained by a cumulative disability acquired over many years. In others,…

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…

Output Formats

atom, dcmes-xml, json, omeka-xml, rss2