Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.
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http://dx.doi.org/10.1016/j.reuma.2020.07.013 | DOI Listing |
Front Pediatr
December 2024
Pediatric Rheumatology Department, Hospital Para El Niño Poblano, Puebla, Mexico.
A female patient in middle childhood was diagnosed with coarctation of the aorta at one month of age and underwent a successful cortectomy. At 11 years old, she developed re-coarctation, which was managed through interventional cardiology. Shortly after the procedure, she experienced a sudden and severe clinical decline, presenting with hypoperfusion of the lower extremities, gastrointestinal bleeding, acute kidney injury, and pancreatitis.
View Article and Find Full Text PDFCardiol Young
January 2025
The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
A variety of autoimmune disorders are associated with an increased risk of thrombosis. Previous studies have suggested combined therapy of heparin and therapeutic plasma exchange (TPE) with fresh frozen plasma (FFP) as the replacement fluid is beneficial in some cases of acute flare-up of autoimmune diseases complicated by thrombotic events. Nevertheless, it remains unknown whether clinicians do more harm than good by exposing patients to a "thrombotic storm" through simultaneous administration of heparin and the clotting factors in the FFP during TPE.
View Article and Find Full Text PDFBr J Haematol
January 2025
Division of Nephrology, Department of Medicine, University of Illinois at Chicago, Chicago, Illinois, USA.
We describe a patient with sickle cell disease (SCD) and elevated antiphospholipid antibodies (aPL) who developed multi-organ failure resembling catastrophic antiphospholipid syndrome. Autoimmune screening revealed several autoantibodies characteristic of systemic lupus erythematosus (SLE). Notably, routinely housed and unmanipulated transgenic sickle mice displayed significantly elevated titres of aPL- and SLE-associated autoantibodies.
View Article and Find Full Text PDFFront Pediatr
December 2024
Pediatric Intensive Care Unit, The Second Hospital of Lanzhou University, Lanzhou, China.
Background: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by recurrent vascular thrombotic events. Catastrophic APS (CAPS), which can result in multiple organ failure and even death, is the most severe manifestation of APS. Herein, we report the case of a pediatric patient with CAPS, including the clinical course, diagnosis, and treatment, with the goal of expanding the literature on this condition, as reports of CAPS in pediatric patients are rare.
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