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This work is a review of the scientific evidence on the oral treatment of adult patients with Gaucher disease type 1 (GD1) with a clinical guideline format according to the Agree II regulations. It describes the main differences between the two oral treatments currently available for treating this disease (miglustat and eliglustat). This review reminds us that the criteria for starting oral treatment in patients with GD1 must be assessed individually. Although miglustat and eliglustat are both glucosylceramide synthase (GCS) enzyme inhibitors, they have different mechanisms of action and pharmacological properties and should never be considered equivalent. Miglustat is indicated in patients with non-severe GD1 who cannot receive other first-line treatments, while eliglustat is indicated as first-line treatment for patients with GD1 of any severity without the need for prior stabilization with enzyme replacement therapy (ERT). It is important to emphasize that in order to start treatment with eliglustat, we must know the CYP2D6 metabolic phenotype and its association with drugs metabolized through the CYP2D6 and CYP3A4 cytochromes-or alternatively those that use P-Glycoprotein must be evaluated on an individual basis. During pregnancy, the use of eliglustat should be avoided; only ERT can be used. Unlike miglustat, whose adverse effects have limited its use, eliglustat has not only demonstrated similar efficacy to ERT but has also been shown to improve the quality of life of patients with GD1.This updated version of the Spanish Society for Research in Osteoporosis and Mineral Metabolism (SEIOMM) osteoporosis guides incorporate the most relevant information published in the last 7 years, since the 2015 guides, with imaging studies, such as vertebral fracture assessment and bone trabecular score analysis. In addition, therapeutic advances include new anabolic agents, comparative studies of drug efficacy, and sequential and combined therapy. Therefore, therapeutic algorithms are also updated.

Guidelines regarding congenital penile curvature (CPC) are lacking, and this pathology has not been the subject of French recommendations to date. The Andrology and Sexual Medicine Committee (CAMS) of the French Association of Urology (AFU) propose a series of clinical practice recommendations (CPR) by answering five clinical questions concerning the diagnosis and treatment of this pathology.

After a bibliographic research between 2000 and 2021, followed by a critical reading according to the CRP method. These recommendations were written to answer five questions (1) What are the different types of CPC? (2) What are the reasons for consultation? (3) What are the assessment methods for CPCs and their consequences? (4) What are the indications for CPCs treatment? (5) What are the corrective modalities for the treatment of CPC?

There are two main phenotypes CPC type 4 (the most common) and chordee without hypospadias. The diagnosis of CPC is clinical and established through enquiry and clinical examination associated with photos of the erect penis. Support can be offered if the curvature is responsible for a disability and/or sexual dissatisfaction linked to a deformation making penetration difficult and/or in the event of significant psychological impact. Only surgical treatments have demonstrated their effectiveness. For type 4 CPCs, corporoplasty (excisional, incisional, or incisionless techniques) is the gold standard.

These recommendations provide support for the management of patients consulting with CPC.

These recommendations provide support for the management of patients consulting with CPC.

Radical prostatectomy (RP) can generate multidimensional physiological changes, like decrease in physical and emotional functioning, as well as Health Related Quality of Life (HRQoL). However, only pelvic floor muscle training (PFMT) is commonly recommended as conservative treatment after RP. More comprehensive interventions than only PFMT, such as physiotherapy promoting general coordination, flexibility, strength, endurance, fitness and functional capacity may seem more relevant and patient-centered.

Our aim was to evaluate whether a more Comprehensive Functional Physical Therapy (CFPT) than PFMT alone, focused on lower limb and lumbo-pelvic exercises, would improve physical capacities and functions (including urinary continence (UI)), emotional functions and HRQoL in patients after RP.

A systematic review was performed in accordance with the PRISMA reporting guidelines. A literature search was conducted in PubMed, PEDro, Web of Science and Cochrane Library databases from inception to January 2022. Thult in more positive outcomes, including physical capacities, physical and emotional functioning and HRQoL, than PFMT alone. Further standardized, physiotherapist-guided and well-designed clinical trials conducted by experienced multidisciplinary clinicians are still called for.

The aim of this study was to assess the methodological quality and accuracy of reporting within systematic reviews (SRs) that provide evidence to form clinical practice guidelines (CPGs) in the management and treatment of breast cancer.

The 5 included CPGs for breast cancer management among National Comprehensive Cancer Network and European Society for Medical Oncology were searched for all SRs and meta-analyses. selleck chemicals llc The characteristics of each study along with their methodological reporting were extracted from each SR using the PRISMA (Preferred Reporting Instrument for Systematic Reviews and Meta-Analyses) and AMSTAR-2 (A Measurement Tool to Assess Systematic Reviews 2) tools. Our second objective was to compare SRs produced by Cochrane groups vs non-Cochrane.

Our study included 5 CPGs for the management of breast cancer, containing 1341 total references with 69 being unique SRs we analyzed. PRISMA completeness percent had a mean 76.3% (n=69), while AMSTAR-2 completeness score mean was 66.5% (n=59). Cochrreased utilization of CPGs in clinical decision making.

Hormone receptor (HR) and human epidermal growth factor receptor-2 (HER2) status is critical for determining management of breast cancer. Previous reports of small cohorts with weak HR-positive (HR+)/HER2-negative (HER2-) disease showed similar rates of pathologic complete response (pCR) following neoadjuvant chemotherapy (NAC) as triple negative breast cancer (TNBC). This study aims to further characterize this group, focusing on pCR rates following NAC.

Patients with stage I-III, HR+/HER2- breast cancer were identified using the University of Wisconsin Hospital Cancer Registry. Medical records were reviewed for demographics, tumor characteristics with quantification level of estrogen and progesterone receptor (≤33%), treatment, and follow-up data.

Data was reviewed from 2,900 patients and a total of 64 patients met inclusion criteria. Eighty percent received chemotherapy, about half with NAC (n=30, 48%). Of 28 patients who received NAC followed by breast and axillary surgery, 12 (43%; 95% CI 25%-63%) had pCR (ypT0/Tis/ypN0). Of the 11 patients who had biopsyproven nodal disease at diagnosis and NAC followed by axillary surgery, 7 (64%, 95% CI 31%-89%) patients had pCR at the axilla. Only one patient with pCR developed recurrent disease. For those that recurred, median time to recurrence was 13.6 (5.6-48.7) months.

Breast cancers that are HER2- and weakly HR+ treated with NAC demonstrated pCR rate more similar to TNBC than breast cancers that are strong HR+. Neoadjuvant approaches may improve pCR rates, which provides important prognostic information. Clinical trials should be developed to focus on this unique patient cohort.

Breast cancers that are HER2- and weakly HR+ treated with NAC demonstrated pCR rate more similar to TNBC than breast cancers that are strong HR+. Neoadjuvant approaches may improve pCR rates, which provides important prognostic information. Clinical trials should be developed to focus on this unique patient cohort.

Despite remarkable advances in surgical techniques and perioperative management, left hepatic trisectionectomy (LHT) remains a challenging procedure with a somewhat high postoperative morbidity rate compared with less-extensive resections. This study aimed to analyze the short- and long-term outcomes of LHT and identify factors associated with the postoperative morbidity of this technically demanding surgical procedure.

The medical records of 53 patients who underwent LHT between June 2005 and October 2019 at a single institution were retrospectively reviewed. The independent prognostic factor of postoperative morbidity was analyzed using the logistic regression model.

Hepatocellular carcinoma was the most common indication for surgery (n=21), followed by hilar cholangiocarcinoma (n=14), intrahepatic cholangiocarcinoma (n=10), and other pathologies (including colorectal liver metastasis, hepatolithiasis, gallbladder cancer, living donor, hemangioma, and multilocular biliary cyst; n=8). The rates of posttive and appropriate preoperative management for obstructive cholangitis and compensatory hypertrophy of the future remnant posterior sector, and the experience of the surgeon.Shiga Toxin-producing Escherichia coli (STEC) is recognized as being responsible for a large number of foodborne illnesses around the world. The pathogenicity of STEC has been related to Stx toxins. However, the ability of STEC to colonize the host and other surfaces can be essential for developing its pathogenicity. Different virulence profiles detected in STEC could cause the emergence of strains carrying new genes codified in new pathogenicity islands linked to metabolism and adherence. Biofilm formation is a spontaneous mechanism whereby STEC strains resist in a hostile environment being able to survive and consequently infect the host through contaminated food and food contact surfaces. Biofilm formation shows intra-and inter-serotype variability, and its formation does not depend only on the microorganisms involved. Other factors related to the environment (such as pH, temperature) and the surface (stainless steel and polystyrene) influence biofilm expression. The «One Health» concept implies the interrelation between public, animal, and environmental health actors to ensure food safety, prevent cross-contamination and resistance to sanitizers, highlighting the need to identify emerging pathogens through new molecular markers of rapid detection that involve STEC strains carrying the Locus of Enterocyte Effacement or Locus of Adhesion and Autoaggregation.This paper investigates the distributed event-triggered control design problem for networked linear time-invariant (LTI) systems with partially measurable states, in the presence of bounded external disturbances. A distributed interval observer-based event-triggered control scheme has been proposed, the main features of this scheme lie in a) designing a novel distributed interval observer to deal with the unknown states and bound disturbances problem; b) proposing a distributed feedback control method using interval estimation information, the partially measurable limitation has been solved; c) introducing a decreased time-varying function with dead-zone modification to avoid the Zeno behavior. Moreover, sufficient conditions for the stability of closed-loop systems are given in the Lyapunov sense by using matrix inequalities and transmission strategy. Finally, the uniformly ultimately bounded stability and Zeno behavior avoidance of the proposed approach have been numerically demonstrated.