OBJECTIVE To develop a system to guide interpretation of scores generated from 2 new instruments measuring work-related physical and behavioral health functioning (Work Disability - Physical Function (WD-PF) and WD - Behavioral Function (WD-BH)). DESIGN Cross-sectional, secondary data from 3 independent samples to develop and validate the functional levels for physical and behavioral health f...

Background. Type 2 diabetes mellitus (T2DM) increases the risk of myocardial ischemia, followed by increased perioperative risk of cardiovascular morbidity. We investigated whether reducing caloric intake reduces ischemic injury and myocardial dysfunction and affects the protective effects of the volatile anesthetic sevoflurane in diet-induced T2DM rats. Methods. Rats received a western (WD) or...

Objective. Violent dream content and its acting out during rapid eye movement sleep are considered distinctive for rapid eye movement sleep behaviour disorder (RBD). This study reports first quantitative data on dreaming in a cohort of patients with treated Wilson's disease (WD) and in patients with WD with RBD. Methods. Retrospective questionnaires on different dimensions of dreaming and a pro...

Background Wilson disease (WD) is an inherited neurometabolic disorder that results in excessive copper deposition in the liver and the brain, affecting children and young adults. Without treatment the disease is invariably fatal. Though treatments for WD have been available since the 1950s, the disease continues to be associated with considerable morbidity and mortality because of missed diagn...

BACKGROUND Wolman disease (WD) is a rare lysosomal storage disorder that is caused by mutations in the LIPA gene encoding lysosomal acid lipase (LAL). Deficiency in LAL function causes accumulation of cholesteryl esters and triglycerides in lysosomes. Fatality usually occurs within the first year of life. While an enzyme replacement therapy has recently become available, there is currently no s...

wilson’s disease (wd) or hepato-lenticular degeneration is a rare autosomal-recessive disorder. a prevalence rate of 30 cases per million and a birth incidence rate of one per 30,000 to 40,000 are often quoted. in 40 to 50% of individuals with wd, hepatic dysfunction is the initial clinical manifestation. with the exception of liver transplantation, treatment of wd is only palliative and intend...

Wilson’s disease (WD), characterized by hepatic and neurological abnormity, is a rare autosomal recessive genetic disorder of copper metabolism. It was first described as a syndrome by Wilson in 1912 [1]. Its morbidity is 1/30 000 1/100 000 on human individuals. Nowadays, the early diagnosis of WD still puzzles clinical doctors and WD patients are often unable to obtain timely and effective tre...

Binary white dwarf (WD) coalescence driven by gravitational waves or collisions in triple systems are potential progenitors of Type Ia supernovae (SNe Ia). We combine the distribution of 56Ni inferred from observations of SNe Ia with the results of both sub-Chandrasekhar detonation models and direct collision calculations to estimate what mass WDs should be exploding in each scenario to reprodu...

BACKGROUND In patients with Wilson's disease (WD), an autosomal recessive disorder, toxic accumulation of copper results in fatal liver disease and irreversible neuronal degeneration. ATP7B, the gene mutated in WD, contains 21 exons and encodes a copper transporting ATPase. A novel disease causing mutation (4193delC) in exon 21 of the ATP7B gene has previously been detected by heteroduplex anal...

INTRODUCTION Both primary biliary cirrhosis (PBC) and Wilson's disease (WD) can cause copper retention in the liver, which is an important factor for liver cellular damage. Copper chelation may preserve liver cell function. It is challenging to distinguish WD from copper accumulation in patients with PBC. There have been few case reports of PBC co-occurrence with WD. CASE PRESENTATION Here we...