Thrombotic thrombocytopenic purpura (TTP) is a spectrum of syndromes characterized by thrombocytopenia and microangiopathic hemolytic anemia, manifested by an elevated blood lactate dehydrogenase (LDH) concentration and red blood cell fragments. It classically occurs in patients with a hereditary or acquired lack of ADAMTS13, a metalloproteinase that cleaves large multimers of von Willebrand fa...

Phaeochromocytoma is a rare disease in childhood with a subtle and wide range of clinical presentations. We report two confirmed cases and one potential case of phaeochromocytoma, each belonging to a different disease spectrum or syndromal disorder, namely sporadic phaeochromocytoma, von Hippel-Lindau disease, and multiple endocrine neoplasia type 2a. Knowledge of the molecular basis of the con...

The compensated three-dimensional turbulent kinetic energy spectrum exhibits a peculiar bump at wave numbers in the vicinity of the crossover from inertial to viscous regimes due to pile up in turbulent kinetic energy, a phenomenon referred to as the bottleneck effect. The origin of this bump is linked to an inflection point in the second-order structure function in physical space caused by com...

Using the geometrical optics approximation, a theoretical prediction of the deflection angle correlation of a laser beam propagating in a hot turbulent jet is found as a functional form of the turbulent spectrum of the refractive index fluctuations. By applying the modified Von Karman model and Tatarskii model, the structure coefficient of the refractive index and the deflection angle correlati...

An analog of the Carnot engine reversibly operating within the framework of pure-state quantum mechanics is discussed. A general formula is derived for the efficiency of such an engine with an arbitrary confining potential. Its expression is given in terms of an energy spectrum and shows how the efficiency depends on a potential as the analog of a working material in thermodynamics, in general....

It has been generally assumed, since the work of von Karman and Howarth in 1938, that free decay of fully-developed turbulence is self-similar. We present here a simple phenomenological model of the decay of 3D incompressible turbulence, which predicts breakdown of self-similarity for low-wavenumber spectral exponents n in the range nc < n < 4, where nc is some threshold wavenumber. Calculation...

the aim of this study was to determine the effect of trenched zirconia bar on the von mises stress distribution of ips -empress ii core ceramics.the three-dimensional model including a three-unit bridge from the second premolar to the second molar was designed. the model was reinforced with zirconia bar (zb), zirconia bar with vertical trench (vzb) and zirconia bar with horizontal trench (hzb) ...

Botrocetin, originally called venom coagglutinin, is a Bothrops factor that causes aggregation of blood platelets in the presence of the von Willebrand component of the factor VIII macromolecular complex. The complex consists of a series of multimers with a molecular weight of about 1-20 x 10(6). Ristocetin, another agent that causes platelet aggregation dependent on von Willebrand factor, reac...

Severe cutaneous drug reactions include a wide spectrum of clinical manifestations ranging from mild morbilliform cutaneous rash, to severe forms of hypersensitivity. Special attention is given in this report to the acute generalized exanthematous pustulosis (AGEP), induced in 90% of cases by the use of systemic drugs, especially aminopenicillins and macrolides. The incidence of the disease is ...

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