OBJECTIVE: Infantile Pompe's disease is a lethal cardiac and muscular disorder. Current developments toward enzyme replacement therapy are promising. The aim of our study is to delineate the natural course of the disease to verify endpoints of…
The aim of the present study was (i) to compare disease progression and survival in different types of degenerative ataxia, and (ii) to identify variables that may modify the rate of disease progression. We included patients suffering from…
AIMS: To describe clinical and demographic characteristics at baseline of a European cohort of adults with congenital heart disease (CHD) and to assess mortality and morbidity in a 5 year follow-up period. METHODS AND RESULTS: Data collected as part…
The aim of our study was to describe trends in opioid use and perceptions of having hastened the end of life of a patient. In 2005, a questionnaire was sent to 6860 physicians in The Netherlands who had attended a death. The response rate was 78%. In…
The study was designed to evaluate a treatment-tradeoff method for its potential in helping lung cancer patients make treatment decisions. A treatment-tradeoff interview was conducted to determine how patients weighed potential survival benefits…
BACKGROUND: Physicians are often asked to prognosticate patient survival. However, prediction of survival is difficult, particularly with critically ill and dying patients within the hospitals. The Palliative Performance Scale (PPS) was designed to…
OBJECTIVE: Little is known about factors that influence whether children with chronic conditions die at home. We sought to test whether deaths attributable to underlying complex chronic conditions (CCCs) were increasingly occurring at home and to…