The results of total knee replacement in five patients aged between 22 and 37 with severe haemophilia A or B are described. All patients had been managed conservatively without success. Frequent bleeds, severe pain and limitation of movement were the indications for operation. Despite close haematological surveillance, bleeding problems occurred in three of the patients and large quantities of ...

Soon after the enzymatic defects in Gaucher disease and in Niemann-Pick disease were discovered, enzyme replacement or enzyme supplementation was proposed as specific treatment for patients with these and related metabolic storage disorders. While relatively straightforward in concept, successful implementation of this approach required many years of intensive effort to bring it to fruition. Pr...

Carbonation is the water-mediated replacement of silicate minerals, such as olivine, by carbonate, and is commonplace in the Earth's crust. This reaction can remove significant quantities of CO2 from the atmosphere and store it over geological timescales. Here we present the first direct evidence for CO2 sequestration and storage on Mars by mineral carbonation. Electron beam imaging and analysi...

In 1964, Christian de Duve first suggested that enzyme replacement might prove therapeutic for lysosomal storage diseases (LSDs). Early efforts identified the major obstacles, including the inability to produce large quantities of the normal enzymes, the lack of animal models for proof-of-concept studies, and the potentially harmful immune responses to the "foreign" normal enzymes. Subsequently...

The unique radiation exposure conditions that exist in computed tomography (CT), during which thin slices of the patient are irradiated by a narrow, fan-shaped beam of x rays emitted from the x-ray tube during its rotation around the patient, have required the use of special dosimetry techniques to characterize the radiation doses to patients and to monitor CT system performance. This section d...

It is essential to be able to incorporate representation of quantities into models of many domains. Many ontologies attempt to axiomatize a theory of quantities in OWL so that they may incorporate quantities in their ontologies. However, OWL based axiomatizations suffer from a number of problems including the impossibility of representing (and computing) conversions in OWL 1. While there are pr...