The tailor-made treatment in a particular case of pulmonary hypertension in thalassaemia intermedia: A case report

Background: Pulmonary hypertension (PH) is a haemodynamic condition, secondary to different causes. Thalassaemia may lead to PH of different origin and needs a comprehensive analysis to be correctly characterized and possibly treated. Case summary: We present a case study of a patient with a non-transfusion-dependent thalassaemia and a previous diagnosis of group 5 PH. A complete diagnostic assessment led to a specific diagnosis of chronic thromboembolic PH. Thus, we were able to start a specific therapy with riociguat that provided an improvement in terms of haemodynamic, imaging, and functional status. Discussion: A correct characterization and treatment of PH are essential in order to change the patient's prognosis. Chronic thromboembolic PH is a treatable cause of PH in thalassemic patients and should be investigated

Artroplastia total no cementada en la coxartrosis secundaria a displasia y luxación congenita de cadera

Se revisan 13 prótesis no cementadas implantadas en 12 paciente s que presentaban una coxartrosis secundaria a una displasia congénita de cadera. Los paciente s han sido seguidos más de 2 años de evolución. En todos los casos se utilizó la via de abordaje postero-lateral. Se implantó siempre la prótesis tipo PCA procediéndose en 7 casos a la realización, a nivel acetabular, de un alo o autoinjerto complementario. A nivel femoral se utilizaron 7 vástagos estándar, 3 medianos y 3 largos para facilitar la restauración del centro rotatorio original de la cadera. En los pacientes que presentaban una luxación importante se llevaron a cabo osteotomías de acortamiento a nivel subtrocantérico. Los pacientes han sido evaluados pre y post-operatoriamente tanto desde el punto de vista radiográfico como clínico. Desde el punto de vista clínico, se obtuvieron 8 resultados excelentes, 3 buenos, 1 discreto y 1 malo. Todos los pacientes estaban plenamente satisfechos con el resultado de la operación habiendo aumentado drásticamente su nivel de actividad. Solamente en un caso fue necesario practicar una intervención de revisión a los 4 años de la artroplastia primitiva, a causa de una grave osificación periprotésica. Solamente se tuvo una complicación intraoperatoria consistente en una fractura de la diálisis femoral.Thirteen non-cemented hips prostheses implanted in 12 patients with an osteoartritis secundar y to congenita l dislocation and dysplasi a of the hip wer e reviewed . Patients had a more than 2 years follow-up. The postero-lateral surgical approach was employed in all cases. On the acetabular side, we always used the PCA socket type. On the femoral side we implanted a PCA standard stem in 7 cases, mid stem in 3 cases and a long stem in 3. Acetabular bone autograft was added in 7 patients. In order to restore the original center of rotacion of the hip, in patients with a severe dislocation we perfomed also a shortening sub-trochanteric osteotomy; all the patients wer e evaluated pre and post-operatively with two separate forms. The first regarding the clinical evaluation, the second for radiographic assessment. All the patients were fully satisfied with the operation increasing dramatically their activity level. We obtained 8 excellent results, 3 good, 1 fair and 1 poor. One case required a revision for a severe heterotopic bone formation about 4 years after the first implant. We had a diaphyseal femoral fracture as the sole inly an intraoperative complication

Investigation of Squaramide Catalysts in the Aldol Reaction En Route to Funapide

Funapide is a 3,3’-spirocyclic oxindole with promising analgesic activity. A reported pilot-plant scale synthesis of this chiral compound involves an asymmetric aldol reaction, catalyzed by a common bifunctional thiourea structure. In this work, we show that the swapping of the thiourea unit of the catalyst for a tailored squaramide group provides an equally active, but rewardingly more selective, catalyst for this aldol reaction (from 70.5 to 85 % ee). The reaction was studied first on a model oxindole compound. Then, the set of optimal conditions was applied to the target funapide intermediate. The applicability of these conditions seems limited to oxindoles bearing the 3-substituent of funapide. Exemplifying the characteristics of target-focused methodological development, this study highlights how a wide-range screening of catalysts and reaction conditions can provide non-negligible improvements in an industrially viable asymmetric transformation

Necrodes littoralis (Coleoptera: Silphidae) visiting and breeding on a carcass in Italy

The community that progressively colonizes a decaying corpse can be considered a small ecosystem mostly composed of sarcosaprophagous arthropods belonging to the orders Diptera and Coleoptera. Studies on these species are often performed through animal models to obtain data on their succession, behaviour and life cycle, together with information on habitat, corpse conditions, season and association with other species. These data may be relevant for forensic investigations, especially concerning the estimation of Post Mortem Interval (PMI). An investigation on the sarcosaprophagous insect community in a rural area was set in Calabria (Southern Italy), using a pig, Sus scrofa Linnaeus, 1758 (Artiodactyla: Suidae) as experimental model. Analyses of the community of Diptera and Coleoptera revealed the massive presence of Necrodes littoralis (Linnaeus, 1758) (Coleoptera: Silphidae). Adults of this species reached the carcass during the bloated stage and a large amount of larvae was detected from the decay stage onwards, simultaneous to the sharp decrease in dipteran larvae and pupae. The occurrence and the activity of N. littoralis should be considered to avoid misinterpretation and errors in estimating PMI in forensic investigation

Cardiopulmonary exercise testing in a combined screening approach to individuate pulmonary arterial hypertension in systemic sclerosis

Objectives The DETECT algorithm has been developed to identify SSc patients at risk for pulmonary arterial hypertension (PAH) yielding high sensitivity but low specificity, and positive predictive value. We tested whether cardiopulmonary exercise testing (CPET) could improve the performance of the DETECT screening strategy. Methods Consecutive SSc patients over a 30-month period were screened with the DETECT algorithm and positive subjects were referred for CPET before the execution of right-heart catheterization. The predictive performance of CPET on top of DETECT was evaluated and internally validated via bootstrap replicates. Results Out of 314 patients, 96 satisfied the DETECT application criteria and 54 were positive. PAH was ascertained in 17 (31.5%) and pre-capillary pulmonary hypertension in 23 (42.6%) patients. Within CPET variables, the slope of the minute ventilation to carbon dioxide production relationship (VE/VCO2 slope) had the best performance to predict PAH at right-heart catheterization [median (interquartile range) of specificity 0.778 (0.714\u20130.846), positive predictive value 0.636 (0.556\u20130.750)]; exploratory analysis on pre-capillary yielded a specificity of 0.714 (0.636\u20130.8) and positive predictive value of 0.714 (0.636\u20130.8). Conclusion In association with the DETECT algorithm, CPET may be considered as a useful tool in the workup of SSc-related pulmonary hypertension. The sequential determination of the VE/VCO2 slope in DETECT-positive subjects may reduce the number of unnecessary invasive procedures without any loss in the capability to capture PAH. This strategy had also a remarkable performance in highlighting the presence of pre-capillary pulmonary hypertension

Analysis of Cometary Dust Impact Residues in the Aluminum Foil Craters of Stardust

In January 2006, the sample return capsule from NASA s Stardust spacecraft successfully returned to Earth after its seven year mission to comet Wild-2. While the principal capture medium for comet dust was low-density graded silica aerogel, the 1100 series aluminum foil (approximately 100 m thick) which wrapped around the T6064 aluminum frame of the sample tray assembly (STA) contains micro-craters that constitute an additional repository for Wild-2 dust. Previous studies of similar craters on spacecraft surfaces, e.g. the Long Duration Exposure Facility (LDEF), have shown that impactor material can be preserved for elemental and mineralogical characterization, although the quantity of impact residue in Stardust craters far exceeds previous missions. The degree of shock-induced alteration experienced by the Wild-2 particles impacting on foil will generally be greater than for those captured in the low-density aerogel. However, even some of the residues found in LDEF craters showed not only survival of crystalline silicates but even their solar flare tracks, which are extremely fragile structures and anneal at around 600 C. Laboratory hypervelocity experiments, using analogues of Wild-2 particles accelerated into flight-grade foils under conditions close to those of the actual encounter, showed retention of abundant projectile residues at the Stardust encounter velocity of 6.1 km/s. During the preliminary examination (PE) of the returned foils, using optical and electron microscopy studies, a diverse range in size and morphologies of micro-craters was identified. In this abstract we consider the state of residue preservation in a diverse range of craters with respect to their elemental composition and inferred mineralogy of the original projectiles

HIV-1 mediated insertional activation of STAT5B promotes the formation of a viral reservoir in T regulatory cells

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