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Isolated loss of vaccine immunity in the protein losings syndrome in a patient with a reverse one and a half ventricle palliation ("failing Fontan-like physiology").
Cardiol Young
January 2025
Department of Pediatric Cardiology, Intensive Care Medicine and Congenital Heart Disease, Justus Liebig University, Giessen, Germany.
Background: A subgroup of CHDs can only be treated palliatively through a Fontan circulation. In case of a failing Fontan situation, serum proteins are lost unspecifically and can also lead to a loss of vaccine antibodies. In a failing Fontan situation, heart transplantation may be the only feasible option.
View Article and Find Full Text PDFAn evaluation of pozelimab for the treatment of CHAPLE disease.
Expert Opin Biol Ther
January 2025
Division of Allergy and Immunology, Department of Pediatrics, School of Medicine, Marmara University, Istanbul, Turkey.
Introduction: CHAPLE disease is a severe, ultra-rare disorder caused by CD55 gene mutations, leading to uncontrolled complement hyperactivation, protein-losing enteropathy, and systemic thrombosis. Recent advances in targeted therapies, particularly the C5 inhibitor pozelimab (Veopoz), offer new treatment options by addressing complement dysregulation, marking a shift from symptomatic to precision therapy.
Areas Covered: This review explores the pathophysiology, clinical manifestations, and current treatments for CHAPLE disease.
Overview of secondary immunodeficiency.
Allergy Asthma Proc
September 2024
From the Division of Pediatric Allergy and Immunology, Department of Pediatrics, University of Rochester Medical Center, Rochester, New York; and.
In contrast to inborn errors of immunity (IEI), which are inherited disorders of the immune system that predispose to infections, malignancy, atopy, and immune dysregulation, secondary immunodeficiencies and immune dysregulation states (SID) are acquired impairments in immune cell function and/or regulation, and may be transient, reversible, or permanent. SIDs can derive from a variety of medical comorbidities, including protein-losing conditions, malnutrition, malignancy, certain genetic syndromes, prematurity, and chronic infections. Medications, including immunosuppressive and chemotherapeutic drugs, can have profound effects on immunity and biologic agents used in rheumatology, neurology, and hematology/oncology practice are increasingly common causes of SID.
View Article and Find Full Text PDFConstrictive Pericarditis and Protein-Losing Enteropathies: Exploring the Heart-Gut Axis.
J Clin Med
August 2024
Gastroenterology Unit, National Institute of Gastroenterology-IRCCS "Saverio de Bellis", 70013 Castellana Grotte, Italy.
Article Synopsis
- Constrictive pericarditis can lead to protein-losing enteropathy (PLE) through a mechanism involving intestinal lymphangiectasia, although this is a rare occurrence.
- A comprehensive review of the literature was conducted using specific medical terms to explore the clinical management of PLE related to constrictive pericarditis.
- Diagnosing constrictive pericarditis typically involves cardiac catheterization, with imaging techniques like MRI, CT scans, and endoscopic methods aiding in the identification of lymphangiectasias; treatment such as pericardiectomy may help reverse PLE symptoms.
Empiric anti-Giardia therapy in non-diarrheal protein-losing enteropathy: A focus on children with monogenic humoral immunodeficiency.
Qatar Med J
November 2023
Pediatric allergy and clinical immunology, The Royal Hospital. Muscat-Oman Email:
Chronic giardia infection can lead to non-erosive gastrointestinal disorders, including protein-losing enteropathy (PLE). This report describes non-diarrheal PLE in chronic giardiasis in children with defective humoral immunity. Methods: The retrospective report is related to 2 children known to have a monogenic inborn error of immunity.
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