نتایج جستجو برای: lefèvre disease
تعداد نتایج: 1490300 فیلتر نتایج به سال:
Optical flow is a powerful tool for the study and analysis of motion in a sequence of images. In this paper we study a Horn–Schunck-type spatio-temporal regularization functional for image sequences that have a non-Euclidean, time varying image domain. To that end we construct a Riemannian metric that describes the deformation and structure of this evolving surface. The resulting functional can...
Activation of granzyme B, a key cytolytic effector molecule of natural killer (NK) cells, requires removal of an N-terminal pro-domain. In mice, cathepsin C is required for granzyme processing and normal NK cell cytolytic function, whereas in patients with Papillon-Lefèvre syndrome (PLS), loss-of-function mutations in cathepsin C do not affect lymphokine activated killer (LAK) cell function. He...
C O N TE N TS Urals, which are enjoying a return to economic growth and are once again attracting manual workers [1]. The population of Moscow, where much of the country’s wealth is concentrated, is also growing fast. However, the decrease in Siberia and the Far East is especially acute. The region of Magadan has lost more than 200,000 of the 386,000 inhabitants that it counted in 1989. In the ...
In this study, a possible new syndrome affecting 18 members of a family spanning 4 generations is described. The main features include palmoplantar hyperkeratosis, proportionate short stature, facial dysmorphism, clinodactyly, epilepsy, deafness, and hypodontia. This syndrome is inherited in an autosomal dominant manner with a high degree of penetrance but variable expressivity. This syndrome d...
A rare case of recurrent pyogenic liver abscess since childhood: A case of Papillon-Lefèvre syndrome
Zusammenfassung Hintergrund Lehrer gehören zu den Berufen mit hohen psychoemotionalen Belastungen, denen Persönlichkeitsmerkmale und der individuelle Umgang Stress entgegenwirken, um die negativen Beanspruchungsfolgen vermeiden. Das Ziel dieser Arbeit bestand in altersabhängigen Analyse des individuellen Umgangs Stresssituationen ihrer situationsbedingten Persönlichkeitsprofile. Methodik Die St...
INTRODUCTION Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder characterised by palmoplantar keratoderma and severe, early onset periodontitis, which results from deficiency of cathepsin C activity secondary to mutations in the cathepsin C gene. To date, 13 different cathepsin C mutations have been reported in PLS patients, all of which are homozygous for a given mutation, refl...
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