Posterior rectal advancement with fistula preservation in patients with anorectal malformation. A multicenter study.

Background: Anorectal malformations (ARM) with rectoperineal fistula are mainly repaired with a posterior sagittal anorectoplasty (PSARP), which can be challenging given the proximity of the fistula to the vagina and urethra. The posterior rectal advancement anoplasty (PRAA), preserves the anterior wall of the fistula without leaving an anterior or a posterior sagittal incision. It is indicated for selected cases of ARM with rectoperineal fistula in which the anterior aspect of the fistula is partially surrounded by sphincter complex.

New Insights Into the Spectrum of RASopathies: Clinical and Genetic Data in a Cohort of 121 Spanish Patients.

Noonan syndrome and related disorders are a group of well-known genetic conditions caused by dysregulation of the Ras/mitogen-activated protein kinase (RAS/MAPK) pathway. Because of the overlap of clinical and molecular features, they are now called RASopathies. In this study, we retrospectively analyzed the clinical data of 121 patients with a molecularly confirmed diagnosis of RASopathy, describing frequencies for clinical features in all organ systems as well as molecular data.

Clinical features of Chagas disease progression and severity.

Chagas disease, the most common form of nonischaemic cardiomyopathy globally, is one of the leading causes of morbidity and mortality in Latin America. Chagas cardiomyopathy has a wide clinical spectrum and prognosis, which is primarily determined by the severity of left ventricular dysfunction. Chagas disease also affects the brain, particularly manifesting as cardioembolic strokes and cognitive impairments.

Prostate-specific Antigen at 3 Months as a Predictor of Radiologic Progression-free Survival in Metastatic Hormone-sensitive Prostate Cancer Treated with Apalutamide: Analysis of 633 Patients in a Real-world Database.

Background And Objective: The depth of the prostate-specific antigen (PSA) decline after androgen receptor pathway inhibitor (ARPI) treatment combined with androgen deprivation therapy for patients with metastatic hormone-sensitive prostate cancer (mHSPC) may affect prognosis. The primary objective in our study was the correlation between the PSA response at 3 mo and radiologic progression-free survival (rPFS) at 24 mo. Three groups were defined according to the PSA decline: complete response (PSA ≤0.

Lung Ultrasound in the Acute Phase of ST-Segment-Elevation Acute Myocardial Infarction: 1-Year Prognosis and Improvement in Risk Prediction.

Background: Lung ultrasound (LUS) has emerged as a useful tool in the acute phase of patients admitted for ST-segment-elevation myocardial infarction. However, its long-term significance remains uncertain, and risk scores do not include LUS findings as a predictor. This study aims to assess the 1-year prognostic value of LUS and its ability to enhance existing risk scores.

Development and validation of the post-CAR prognostic index for large B-cell lymphoma patients after CAR-T progression in third or later line treatment.

Chimeric antigen receptor (CAR) T-cell therapy fails to achieve durable responses in over 60% of relapsed/refractory (R/R) large B-cell lymphoma (LBCL) patients in the third or later line setting. After CAR-T failure, survival outcomes are heterogeneous and a prognostic model in this patient population is lacking. A training cohort of 216 patients with progressive disease (PD) after CAR-T from 12 Spanish centers was used to develop the Post-CAR Prognostic Index (PC-PI); primary endpoint was overall survival (OS) from CAR-T progression.

International expert consensus statement on the diagnosis and management of congenital nephrogenic diabetes insipidus (arginine vasopressin resistance).

Congenital nephrogenic diabetes insipidus (NDI; also known as arginine vasopressin resistance) is a rare inherited disorder of water homeostasis, caused by insensitivity of the distal nephron to arginine vasopressin. Consequently, the kidney loses its ability to concentrate urine, which leads to polyuria, polydipsia and the risk of hypertonic dehydration. The diagnosis and management of NDI are very challenging and require an integrated, multidisciplinary approach.

Activities of the clinical pharmacist in the intensive care units.

The main objective of the activity carried out in an intensive care unit and in general in all hospitalization units, is to provide all the human and material resources to offer the best therapeutic care to admitted patients. Work in multidisciplinary teams, made up of specialists in Intensive Care Medicine as those responsible for the patients, doctors from other specialties, specialized nursing, physiotherapists, nutritionists and clinical pharmacists is an optimal approach to achieve the proposed objective. The activities of the clinical pharmacist can be developed at different levels (basic, intermediate and excellent) depending on the degree of involvement, the time dedicated, the training and the available resources.

Results from Arm A of Phase 1/2 DREAMM-6 trial: belantamab mafodotin with lenalidomide plus dexamethasone in patients with relapsed/refractory multiple myeloma.

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Mitral Annular Disjunction in Heritable Thoracic Aortic Disease: Insights From the Montalcino Aortic Consortium.

Background: Mitral annular disjunction (MAD), posterior displacement of the mitral valve leaflet hinge point, predisposes to arrhythmias or sudden cardiac death. We evaluated the burden of MAD, mitral valve prolapse (MVP), and mitral regurgitation (MR) by heritable thoracic aortic disease gene in a cross-sectional analysis of 2014-2023 data in the Montalcino Aortic Consortium registry.

Methods And Results: MAD was determined by direct measurement of echocardiographic images.

Hemophagocytic lymphohistiocytosis in a patient with Epstein-Barr virus-positive diffuse large B-cell lymphoma treated with chimeric antigen receptor T-cell therapy.

With the advent of chimeric antigen receptors T-cell therapy, understanding their role in the development of hemophagocytic lymphohistiocytosis has become increasingly complex. We describe a case of a young patient with Epstein-Barr virus-positive diffuse large B-cell lymphoma, who was treated with axicabtagene ciloleucel. The patient developed progressive cytopenia and, on Day 73 post-infusion, met criteria for hemophagocytic lymphohistiocytosis.

Signature Proteins in Small Extracellular Vesicles of Granulocytes and CD4 T-Cell Subpopulations Identified by Comparative Proteomic Analysis.

Several studies have described the proteomic profile of different immune cell types, but only a few have also analysed the content of their delivered small extracellular vesicles (sEVs). The aim of the present study was to compare the protein signature of sEVs delivered from granulocytes (i.e.

Bacterial Infection Features in Alcohol-Associated Hepatitis: Review of a 2016-2021 Cohort.

Bacterial infections (BI) are a major cause of mortality in patients with alcohol-associated hepatitis (AH); however, only a few studies have investigated BI in AH in the last decade. Therefore, we aimed to assess the features and outcomes of BI in patients with AH. This observational descriptive study included patients with AH admitted to a tertiary academic hospital between 2016 and 2021.