Introduction: Healthcare systems face rising demand and unsustainable cost pressures. In response, health policymakers are adopting Value-Based Health Care (VBHC), targeting available resources to achieve the best possible patient outcomes at the lowest possible cost and actively disinvesting in care of low-value. This requires the evaluation of longitudinal clinical and patient reported outcome measures (PROMs) at an individual-level and population-scale, which can create significant data challenges.
View Article and Find Full Text PDFThis chapter focuses on the historic aspects of the development of much of our current knowledge of the diagnosis and treatment of Wilson disease. Included are descriptions of the clinical signs of neurologic and hepatic disease, the natural history of disease progression, studies of disease pathogenesis and a unique perspective on the development of diagnostic testing and pharmacological therapy.
View Article and Find Full Text PDFBackground: Renal disease is being increasingly diagnosed in the elderly. However, reports on biopsy-confirmed renal disease in this population are limited. The aim of this study was to give an overview of the most important indications, diagnoses and outcomes of renal biopsies in the elderly in our center.
View Article and Find Full Text PDFHerein we present one of the largest single-center reports of the response of hemodialysis patients to a two-vaccine hepatitis B virus vaccination protocol in a European dialysis population. A hepatitis B recombinant DNA vaccine, HBvaxPRO, was given at a dose of 40 µg intramuscularly using a four-dose schedule at 0, 1, 2, and 12 months. Responses were (1) a titer >100 mIU/mL = patient immune, (2) a titer level 10-99 mIU/mL = give a booster dose and recheck level 2 months later, and (3) 0 ≤ 10 mIU/mL = repeat vaccination course using a different vaccine, Engerix-B.
View Article and Find Full Text PDFBefore 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die.
View Article and Find Full Text PDFThis study covers patients seen at a Wilson disease clinic between 1960 and 2002. The diagnosis of Wilson disease was confirmed in 316. Of 40 patients with a neurological presentation who were found not to have the disease, caeruloplasmin concentration was found to be reduced in 19.
View Article and Find Full Text PDFThe 2.89- to 2.76-gigayear-old conglomerates of the Central Rand Group of South Africa host an immense concentration of gold.
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