Fake Superpotential for Large and Small Extremal Black Holes

We consider the fist order, gradient-flow, description of the scalar fields coupled to spherically symmetric, asymptotically flat black holes in extended supergravities. Using the identification of the fake superpotential with Hamilton's characteristic function we clarify some of its general properties, showing in particular (besides reviewing the issue of its duality invariance) that W has the properties of a Liapunov's function, which implies that its extrema (associated with the horizon of extremal black holes) are asymptotically stable equilibrium points of the corresponding first order dynamical system (in the sense of Liapunov). Moreover, we show that the fake superpotential W has, along the entire radial flow, the same flat directions which exist at the attractor point. This allows to study properties of the ADM mass also for small black holes where in fact W has no critical points at finite distance in moduli space. In particular the W function for small non-BPS black holes can always be computed analytically, unlike for the large black-hole case.Comment: 30 pages, LaTeX source. Discussion on the radial evolution of the scalar fields, in relation to the symmetries of the W-function, extended. Table 1 added. Typos correcte

Histopathological differences of myotonic dystrophy type 1 (DM1) and PROMM/DM2

Muscle biopsy findings in DM2 have been reported to be similar to those in DM1. The authors used myosin heavy chain immunohistochemistry and enzyme histochemistry for fiber type differentiation on muscle biopsies. Their results show that DM2 patients display a subpopulation of type 2 nuclear clump and other very small fibers and, hence, preferential type 2 fiber atrophy in contrast to type 1 fiber atrophy in DM1 patients

European survey on criteria of aesthetics for periodontal evaluation: The ESCAPE study

Objective: The ESCAPE multicentre survey was designed to (a) compare the agreement of three relevant aesthetic scoring systems among different centres, and (b) evaluate the reproducibility of each question of the questionnaires. / Materials and Methods: EFP centres (n = 14) were involved in an e‐survey. Forty‐two participants (28 teachers, 14 postgraduate students) were asked to score the one‐year aesthetic outcomes of photographs using the Before–After Scoring System (BASS), the Pink Esthetic Score (PES) and the Root coverage Esthetic Score (RES). Mean values of kappa statistics performed on each question were provided to resume global agreement of each method. / Results: Between teachers, a difference of kappa ≥ 0.41 (p = .01) was found for BASS (75%) and PES (57%). Similarly, RES (84%) and PES (57%) were different (p < .001). No difference was found between BASS (75%) and RES (84%). No difference was found between students, whatever the scoring system. Questions of each scoring system showed differences in their reproducibility. / Conclusions: The outcomes of this study indicate that BASS and RES scoring systems are reproducible tools to evaluate aesthetic after root coverage therapies between different centres. Among the various variables, lack of scar, degree of root coverage, colour match and gingival margin that follows the CEJ show the best reliability

Clinical and histologic findings in autosomal centronuclear myopathy

Centronuclear myopathy (CNM) is a congenital myopathy characterized by chains of centrally located nuclei in a large number of muscle fibers. Clinically, an early-onset form was reported in several autosomal-recessive (AR) families and many sporadic patients, whereas a late-onset form was found in most autosomal-dominant (AD) families. The boundary between these two forms remains unclear, and the molecular basis of autosomal CNM is still unresolved. To better define the clinical and morphologic characteristics of autosomal CNM, the authors analyzed a series of 29 patients from 12 families. Two subgroups were identified in three AD families: two families had a relatively late onset of disease and a slow progression of diffuse weakness, whereas the third family, who had a similar clinical course, also presented a unique diffuse muscle hypertrophy. Two presumed AR families and seven sporadic patients were analyzed together, and three subgroups were identified: 1) an early-onset form with ophthalmoparesis; 2) an early-onset form without ophthalmoparesis; and 3) a late-onset form without ophthalmoparesis. Overall, 23 muscle biopsies were reviewed; a majority of patients had &gt;20% central nuclei, fiber type 1 predominance, and a radial distribution of sarcoplasmic strands on oxidative stains. A marked endomysial fibrosis was observed in three sporadic patients with a relatively severe clinical course. The classification reported in this study will be useful for the diagnosis and the follow-up evaluation of patients with autosomal CNM and for the research into the molecular defects underlying the condition

A propos d'un essai de Phase I de thérapie génique effectué avec un plasmide contenant l'intégralité du gène dystrophine dans la Myopathie de Duchenne/Becker

Un premier essai de thérapie génique a été réalisé chez des patients atteints de myopathie de Duchenne/ Becker, à l'aide d'un plasmide contenant l'intégralité de la séquence codante du gène dystrophine. Cet essai de Phase I était destiné à apprécier le degré de transfection obtenue et à évaluer la tolérance au produit injecté ainsi qu'à l'expression du segment de dystrophine nouvellement synthétisé. Neuf patients ont été inclus dans l'essai, répartis en trois cohortes de trois. La première cohorte a reçu une injection intramusculaire dans les muscles radicaux de 200 $\mu$g, la seconde une injection de 600 $\mu$g, et la troisième deux injections à deux semaines d'intervalle de 600 $\mu$g. L'inclusion des patients s'est faite de façon séquentielle, après examen des résultats du patient précédent par un comité de pilotage comprenant des experts indépendants. Le plasmide a été retrouvé dans les neuf prélèvements effectués trois semaines après la première (ou unique) injection. Une expression de la dystrophine exogène a été retrouvée chez 6 patients sur 9. Cette expression a toujours été faible, jusqu'à 6% de fibres présentant un marquage périphérique complet, et jusqu'à 26 % de fibres présentant un marquage partiel. Les messagers de la dystrophine ont été détectés par une RT-PCR “nichée” dans cinq des six prélèvements présentant des fibres marquées. Il est remarquable de noter qu'aucune réponse immunitaire humorale ou cellulaire vis-à-vis de la dystrophine exogène n'a été détectée. Aucune réaction secondaire locale ou générale n'a été observée. Ces résultats, modestes mais significatifs, ouvrent la voie à de nouveaux développements en terme de thérapie génique des maladies musculaires humaines, et en particulier dans les myopathies de Duchenne et de Becker