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Essential Thrombocythemia: A Review.
JAMA
January 2025
CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, University of Florence, AOU Careggi, Florence, Italy.
Importance: Essential thrombocythemia, a clonal myeloproliferative neoplasm with excessive platelet production, is associated with an increased risk of thrombosis and bleeding. The annual incidence rate of essential thrombocythemia in the US is 1.5/100 000 persons.
View Article and Find Full Text PDFNew and Emerging Biological Therapies for Myasthenia Gravis: A Focussed Review for Clinical Decision-Making.
BioDrugs
January 2025
Department of Neurology, Neuroscience Clinical Research Center (NCRC) and Integrated Myasthenia Gravis Center, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, 10117, Charitéplatz 1, Germany.
Myasthenia gravis (MG) is a rare autoimmune disease characterised by exertion-induced muscle weakness that can lead to potentially life-threatening myasthenic crises. Detectable antibodies are directed against specific postsynaptic structures of the neuromuscular junction. MG is a chronic condition that can be improved through therapies, but to date, not cured.
View Article and Find Full Text PDFAcute decompensated right heart failure potentially triggered by multiple factors including pulmonary vasodilator removal during plasma exchange: a case report.
JA Clin Rep
January 2025
Department of Anesthesiology and Critical Care Medicine, Hokkaido University Hospital, N14W5, Kita-ku, Sapporo, 060-8648, Japan.
Background: Plasma exchange (PE) removes high-molecular-weight substances and is sometimes used for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) with alveolar hemorrhage. Hypotension during PE is rare, except in allergic cases. We report a case of shock likely caused by increased pulmonary vascular resistance (PVR) during PE.
View Article and Find Full Text PDFVariable treatment response to lumasiran in pediatric patients with primary hyperoxaluria type 1.
Pediatr Nephrol
January 2025
University Medical Center Hamburg-Eppendorf, University Children's Hospital, Martinistrasse 52, Hamburg, 20246, Germany.
Background: Primary hyperoxaluria type 1 (PH 1) is a rare genetic condition due to mutations in the AGXT gene. This leads to an overproduction of oxalate in the liver. Hyperoxaluria often causes kidney stones, nephrocalcinosis, and chronic kidney disease.
View Article and Find Full Text PDFTestosterone-induced Seminal Vesicle-like Differentiation in Cervical Mesonephric Duct Remnants in a Female to Male Transgender Patient.
Int J Gynecol Pathol
January 2025
Department of Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK.
Pure ductal-type mesonephric remnants in the uterine cervix are rare. We report an unusual case in a 31-yr-old of cervical mesonephric remnants of predominantly ductal type exhibiting seminal vesicle-like differentiation in a female-to-male transgender patient receiving long-term testosterone therapy. To the best of our knowledge, this phenomenon has not been previously reported.
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