Aspiration of biological viscoelastic drops

Spherical cellular aggregates are in vitro systems to study the physical and biophysical properties of tissues. We present a novel approach to characterize the mechanical properties of cellular aggregates using micropipette aspiration technique. We observe an aspiration in two distinct regimes, a fast elastic deformation followed by a viscous flow. We develop a model based on this viscoelastic behavior to deduce the surface tension, viscosity, and elastic modulus. A major result is the increase of the surface tension with the applied force, interpreted as an effect of cellular mechanosensing.Comment: 4 pages, 4 figures

JDM treatment with rituximab Personal non-commercial use only

ABSTRACT. Objective. To evaluate the safety and efficacy of rituximab (RTX) in juvenile dermatomyositis (JDM) in off-trial patients. Methods. We conducted a multicenter prospective study of patients with JDM included in the French Autoimmunity and Rituximab (AIR) registry. Results. Nine patients with severe JDM were studied. The main indication for RTX treatment was severe and/or refractory muscle involvement (7 patients), severe calcinosis (1 patient), or severe chronic abdominal pain associated with abdominal lipomatosis (1 patient). RTX was associated with corticosteroids, immunosuppressive drugs, and plasma exchange therapy in 9/9, 5/9, and 2/9 patients, respectively. Mild infections of the calcinosis sites occurred in 2 patients and an infusion-related event in 1. Complete clinical response was achieved in 3/6 patients treated with RTX for muscle involvement. In these responders steroid therapy was stopped or tapered to < 15% of the baseline dosage, with no relapse, with a followup ranging from 1.3 to 3 years. Calcinosis did not improve in the 6 affected patients. Conclusion. This small series suggests that rituximab may be effective for treating muscle and skin involvement in a small subset of children with severe JDM, and that its safety profile was satisfactory. Further studies are needed to identify predictive factors of response to RTX in patients with sever

Connectivites mixtes, connectivites indifférenciées et syndromes de chevauchement associés aux connectivites à début pédiatrique (présentation et évolution)

TOULOUSE3-BU Santé-Centrale (315552105) / SudocSudocFranceF

Syndrome de Bartter néonatal, corrélation phénotype génotype (à partir d'une cohorte de 34 patients)

Le syndrome de Bartter néonatal (BS) est une tubulopathie héréditaire due à des mutations de gènes codant pour des molécules participant à la réabsorption du sel dans le néphron distal : SLC12A1 (BS type I), KCNJ1 (BS type II), CLCNKB (BS type III) ou BSND (BS type IV). 34 enfants présentant un BS génétiquement confirmé ont été suivis avec un recul moyen de 7,4 ans. Le BS de type II est le plus fréquent avec une hyperkaliémie initiale notée chez 61% des enfants. A l inverse, l'hypokaliémie est constante et profonde dans les BS de type III. Un hydramnios, une prématurité et une polyurie caractérisent les BS de type I, II et IV alors que l'expression clinique des patients porteurs de mutations du gène CLCNKB est variable, soit une forme anténatale soit une forme proche d'un BS classique. Sous supplémentation hydroélectrolytique et indométacine, la majorité des enfants ont un rattrapage staturo-pondéral. L'évolution vers l'insuffisance rénale est rare et probablement multifactorielle.TOULOUSE3-BU Santé-Centrale (315552105) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Soft Matter Models of Developing Tissues and Tumors

International audienceAnalogies with inert soft condensed matter-such as viscoelastic liquids, pastes, foams, emulsions, colloids, and polymers-can be used to investigate the mechanical response of soft biological tissues to forces. A variety of experimental techniques and biophysical models have exploited these analogies allowing the quantitative characterization of the mechanical properties of model tissues, such as surface tension, elasticity, and viscosity. The framework of soft matter has been successful in explaining a number of dynamical tissue behaviors observed in physiology and development, such as cell sorting, tissue spreading, or the escape of individual cells from a tumor. However, living tissues also exhibit active responses, such as rigidity sensing or cell pulsation, that are absent in inert soft materials. The soft matter models reviewed here have provided valuable insight in understanding morphogenesis and cancer invasion and have set bases for using tissue engineering within medicine

Mechanics of Biomimetic Liposomes Encapsulating an Actin Shell

International audienceCell-shape changes are insured by a thin, dynamic, cortical layer of cytoskeleton underneath the plasma membrane. How this thin cortical structure impacts the mechanical properties of the whole cell is not fully understood. Here, we study the mechanics of liposomes or giant unilamellar vesicles, when a biomimetic actin cortex is grown at the inner layer of the lipid membrane via actin-nucleation-promoting factors. Using a hydrodynamic tube-pulling technique, we show that tube dynamics is clearly affected by the presence of an actin shell anchored to the lipid bilayer. The same force pulls much shorter tubes in the presence of the actin shell compared to bare membranes. However, in both cases, we observe that the dynamics of tube extrusion has two distinct features characteristic of viscoelastic materials: rapid elastic elongation, followed by a slower elongation phase at a constant rate. We interpret the initial elastic regime by an increase of membrane tension due to the loss of lipids into the tube. Tube length is considerably shorter for cortex liposomes at comparable pulling forces, resulting in a higher spring constant. The presence of the actin shell seems to restrict lipid mobility, as is observed in the corral effect in cells. The viscous regime for bare liposomes corresponds to a leakout of the internal liquid at constant membrane tension. The presence of the actin shell leads to a larger friction coefficient. As the tube is pulled from a patchy surface, membrane tension increases locally, leading to a Marangoni flow of lipids. As a conclusion, the presence of an actin shell is revealed by its action that alters membrane mechanics

Severe Global Inflammatory Involvement of Ocular Segments and Optic Disc Swelling in a 12-Year-Old Girl with Kawasaki Disease

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Clonal cytophagic histiocytic panniculitis in children may be cured by cyclosporine a

Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis in childhood, associated either with nonmalignant conditions or with subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and often also associated with macrophage activation syndrome (MAS). Discriminating between these 2 conditions is therapeutically important because nonmalignant CHP often improves under cyclosporine and prednisone, whereas most cases of SPLT may be best treated with more aggressive therapy. We report the cases of a 6-month-old boy and a 16-month-old girl who, after viral infection, developed multiple infiltrating skin nodules on the limbs and face, associated with MAS. Histopathologic findings for skin biopsy specimens revealed CHP associated with heavily cellular lobular panniculitis. Hemophagocytosis and immunohistochemical staining features were consistent with typical characteristics of in situ MAS in adipose tissue: the lymphocytes were mostly TCD8+ cells with an activated phenotype (human leukocyte antigen (HLA) -DR+) and expressed interferon-γ; CD68+ macrophages expressed tumor necrosis factor-α and interleukin-6. A monoclonal rearrangement of the T-cell receptor γ gene was present in skin tissue but not in peripheral blood or bone marrow lymphocytes. Cyclosporine A treatment resulted in the complete remission of cutaneous and systemic manifestations in both patients for 66 and 29 months, respectively. This report suggests that the diagnosis of a reactive T-cell lymphoproliferation should be the treatment of choice in young children with severe CHP, even if there is a SPTCL-like aspect with an in situ T-cell clonality. It also suggests that CSA is the optimal treatment of this condition and postulates the possible pathologic process underlying this efficacy.status: publishe

Tuning functions for automatic detection of brief changes of facial expression in the human brain

International audienceEfficient decoding of even brief and slight intensity facial expression changes is important for social interactions. However, robust evidence for the human brain ability to automatically detect brief and subtle changes of facial expression remains limited. Here we built on a recently developed paradigm in human electrophysiology with full-blown expressions (Dzhelyova et al., 2017), to isolate and quantify a neural marker for the detection of brief and subtle changes of facial expression. Scalp electroencephalogram (EEG) was recorded from 18 participants during stimulation of a neutral face changing randomly in size at a rapid rate of 6 Hz. Brief changes of expression appeared every five stimulation cycle (i.e., at 1.2 Hz) and expression intensity increased parametrically every 20 s in 20% steps during sweep sequences of 100 s. A significant 1.2 Hz response emerged in the EEG spectrum already at 40% of facial expression-change intensity for most of the 5 emotions tested (anger, disgust, fear, happiness, or sadness in different sequences), and increased with intensity steps, predominantly over right occipito-temporal regions. Given the high signal-to-noise ratio of the approach, thresholds for automatic detection of brief changes of facial expression could be determined for every single individual brain. A time-domain analysis revealed three components, the two first increasing linearly with increasing intensity as early as 100 ms after a change of expression, suggesting gradual low-level image-change detection prior to visual coding of facial movements. In contrast, the third component showed abrupt sensitivity to increasing expression intensity beyond 300 ms post expression-change, suggesting categorical emotion perception. Overall, this characterization of the detection of subtle changes of facial expression and its temporal dynamics open promising tracks for precise assessment of social perception ability during development and in clinical populations

Interferential therapy for chronic constipation in adults: The CON‐COUR randomizedcontrolled trial

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