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Fetal cardiac function in pregnancy affected by congenital heart disease: protocol for a multicentre prospective cohort study.
BMC Pregnancy Childbirth
January 2025
Royal Hospital for Women and UNSW, School of Clinical Medicine, Level 0, Royal Hospital for Women, Barker Street (Locked Bag 2000), Sydney, NSW, 2031, Australia.
Background: Congenital heart disease (CHD) is the most common fetal malformation, and it can result first in cardiac remodeling and dysfunction and later in cardiac failure and hydrops. A limited number of studies have evaluated cardiac function in fetuses affected by CHD. Functional parameters could potentially identify fetuses at risk of cardiac failure before its development.
View Article and Find Full Text PDFSacubitril/valsartan is associated with improvements in quality of life in adult congenital heart disease patients with systemic right ventricular failure.
Open Heart
January 2025
Center for Congenital Heart Disease Amsterdam Leiden (CAHAL), Leiden University Medical Center, Leiden, Zuid-Holland, Netherlands
Background: Short-term improvements in quality of life (QOL) have been reported in adult congenital heart disease patients with systemic right ventricle (sRV) failure after treatment with sacubitril/valsartan. This study aimed to evaluate the medium-term QOL changes in sRV failure patients treated with sacubitril/valsartan.
Methods: In this single-centre, prospective cohort study, patients with symptomatic sRV failure completed the Netherlands Organisation for Applied Scientific Research/Academic Hospital Leiden Questionnaire for Adult's Health-Related Quality of Life (TAAQOL) at baseline and after starting treatment with sacubitril/valsartan.
Alemtuzumab and thrombotic thrombocytopenic purpura: Analysis of an international surveillance database and systematic literature review.
Transfus Apher Sci
January 2025
Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Objectives: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy associated with severe deficiency in ADAMTS13. ADAMTS13 deficiency may be secondary to absent or dysfunctional protein production due to mutations in the ADAMTS13 gene (congenital TTP) or autoantibody-mediated clearance and/or inhibition (immune-mediated TTP). This autoimmunity may, albeit rarely, occur secondary to certain medications (eg, ticlopidine).
View Article and Find Full Text PDFImpaired spatial coding of the hippocampus in a dentate gyrus hypoplasia mouse model.
Proc Natl Acad Sci U S A
February 2025
Zanvyl Krieger Mind/Brain Institute, Johns Hopkins University, Baltimore, MD 21218.
The hippocampal dentate gyrus (DG) is thought to orthogonalize inputs from the entorhinal cortex (pattern separation) and relay this information to the CA3 region. In turn, attractor dynamics in CA3 perform a pattern completion or error correction operation before sending its output to CA1. In a mouse model of congenital hypoplasia of the DG, a deficiency in the (Wls) gene, specifically in cells expressing , which targets neuronal progenitors, led to an almost total absence of dentate granule cells and modestly impaired performance in spatial tasks.
View Article and Find Full Text PDFAcquired hypothyroidism, iodine status and hearing impairment in adults: A pilot study.
PLoS One
January 2025
Institute of Endocrinology, Prague, Czech Republic.
Objectives: Hearing impairment can have major impacts on behavior, educational attainment, social status, and quality of life. In congenital hypothyroidism, the incidence of hearing impairment reaches 35-50%, while in acquired hypothyroidism there is a reported incidence of 25%. Despite this, knowledge of the pathogenesis, incidence and severity of hearing impairment remains greatly lacking.
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