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Keywords Catastrophic Antiphospholipid ...

Catastrophic Antiphospholipid Antibody syndrome

https://read.qxmd.com/read/37416028/penile-glans-necrosis-associated-with-antiphospholipid-syndrome-a-rare-complication
#21
Minh H Truong, Trung Q Ngo, Thang D Vu
Penile glans necrosis is a rare clinical condition caused by trauma, diabetes mellitus, adverse effect of vasoconstrictive solutions, and circumcision. Antiphospholipid syndrome (APS) is categorized as an autoimmune disease with the presence of antiphospholipid antibodies that results in an increased risk of vascular thrombosis and obstetrical complications. In this article, we report a rare case of a 20-year-old boy with penile glans necrosis due to penile vascular thrombosis following catastrophic antiphospholipid syndrome (CAPS) which we successfully treated at People's Hospital 115...
June 2023: Curēus
https://read.qxmd.com/read/37303325/catastrophic-antiphospholipid-syndrome-and-renal-failure-an-unexpected-recovery-after-three-years-on-dialysis
#22
Daniela Alferes, Susana Pereira, Vitória Paes de Faria, Ana Ventura, Maria Clara Almeida
Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening disorder characterised by arterial or venous thrombotic events, involving three or more organs in a short period of time, in the presence of persistent antiphospholipid antibodies. Long-term anticoagulation with warfarin is the standard of care to prevent recurrent vascular events. Besides supportive care, optimal management of CAPS is unclear and consensus among experts is lacking. We describe a patient with primary antiphospholipid syndrome who experienced probable CAPS after receiving rivaroxaban, resulting in extensive cutaneous ulceration, acute coronary syndrome and dialysis-dependent renal failure...
May 2023: Curēus
https://read.qxmd.com/read/37275894/an-update-on-the-biologics-for-the-treatment-of-antiphospholipid-syndrome
#23
REVIEW
Zelin Yun, Lizhi Duan, Xiangjun Liu, Qingmeng Cai, Chun Li
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis and pregnancy morbidity with the persistent presence of antiphospholipid antibodies (aPLs). Although anticoagulation is the primary treatment for APS, it fails in approximately 20-30% of obstetric APS cases and more than 30% of thrombotic APS cases. Therefore, there is a need for new, targeted treatments beyond anticoagulants. Biologics, such as rituximab and eculizumab, have been recommended for refractory catastrophic APS...
2023: Frontiers in Immunology
https://read.qxmd.com/read/37252601/early-hellp-syndrome-or-catastrophic-antiphospholipid-syndrome-a-diagnostic-dilemma
#24
Suhwoo Bae, Lizelle Comfort, Jason Ng, Kumar Sarkar, Sarah Pachtman
Hypertensive disorders of pregnancy typically occur in the third trimester, with earlier presentations associated with underlying disorders such as antiphospholipid syndrome (APLS). We describe a case of a young primigravida presenting at 15 weeks 6 days gestation with epigastric pain, vomiting, new-onset severe-range hypertension, and subsequent development of anemia, thrombocytopenia, and transaminitis. Antiphospholipid antibodies (aPL) were triple-positive and imaging was negative for thrombosis. She was treated with aspirin, therapeutic anticoagulation, and ultimately dilatation and evacuation with initial postoperative improvement...
April 2023: Curēus
https://read.qxmd.com/read/37123583/catastrophic-antiphospholipid-syndrome-a-case-series
#25
JOURNAL ARTICLE
Navya Christopher, Gopinath Periaswamy, Venkatesh Kasi Arunachalam, V Mangalakumar, Pankaj Mehta, Mathew Cherian
Catastrophic antiphospholipid syndrome (CAPS) is the rare but most severe form of antiphospholipid syndrome with multiple organ ischemia developing over a short period of time. CAPS should be considered when imaging suggests an acute and concurrent multiorgan ischemia, associated with positive antiphospholipid antibodies. As CAPS can have fulminant irreversible complications, its early recognition is important to initiate the treatment promptly. We present three patients of CAPS who were managed at our institution...
April 2023: Indian Journal of Radiology & Imaging
https://read.qxmd.com/read/37114699/catastrophic-antiphospholipid-syndrome-accompanied-by-complement-regulatory-gene-mutation
#26
Serim Pul, İbrahim Gökçe, Ece Demirci Bodur, Serçin Güven, Neslihan Çiçek, Mehtap Sak, Özde Nisa Türkkan, Deniz Filinte, Cemile Pehlivanoğlu, Betül Sözeri, Harika Alpay
BACKGROUND: Antiphospholipid syndrome (APS), particularly the catastrophic antiphospholipid syndrome (CAPS), is one of the rare causes of thrombotic microangiopathy (TMA). CAPS is the most severe form of APS, especially when accompanied by complement dysregulation, causes progressive microvascular thrombosis and failure in multiple organs. In this report, a case of CAPS with TMA accompanied by a genetic defect in the complement system is presented. CASE: A 13-year-old girl was admitted to the hospital with oliguric acute kidney injury, nephrotic range proteinuria, Coombs positive hemolysis, refractory thrombocytopenia, a low serum complement C3 level and anti-nuclear antibody (ANA) positivity...
2023: Turkish Journal of Pediatrics
https://read.qxmd.com/read/37065845/catastrophic-antiphospholipid-syndrome-in-an-immune-thrombocytopenia-patient-treated-with-avatrombopag
#27
Saartje Van de Vondel, Christophe Vandenbriele, Gerald Gheysens, Peter Verhamme, Ann Janssens
BACKGROUND: Avatrombopag is an orally administered second-generation thrombopoietin receptor agonist (TPO-RA) approved for the treatment of chronic immune thrombocytopenia (ITP). However, increased thrombogenicity in patients with ITP after initiation of TPO-RA treatment has been reported. KEY CLINICAL QUESTION: We report a case of a patient with ITP who developed a catastrophic antiphospholipid antibody syndrome (CAPS), following treatment with avatrombopag. CLINICAL APPROACH: A20-year-old known chronic patient with ITP presented at the emergency department with a 2-week history of headache, nausea, and abdominal pain, 3 weeks after initiating avatrombopag...
March 2023: Research and Practice in Thrombosis and Haemostasis
https://read.qxmd.com/read/37052044/catastrophic-antiphospholipid-syndrome-as-a-complication-of-covid-19-infection
#28
JOURNAL ARTICLE
Lauren Bitterman, Mahdis Solhjoo, Vivek Shah, Sue Min Kwon, Karina Torralba, Hana Kazbour
COVID-19 infection has been found to precipitate hypercoagulability and transiently increase antiphospholipid antibodies. However, it is yet to be determined how likely these transient changes contribute to thrombotic events and antiphospholipid syndrome. We present a case in which antiphospholipid antibodies were detected in the presence of significant thromboses. The patient was subsequently treated for suspected catastrophic antiphospholipid syndrome following COVID-19 infection.
2023: Journal of Investigative Medicine High Impact Case Reports
https://read.qxmd.com/read/37007308/a-probable-catastrophic-antiphospholipid-antibody-syndrome-thrombotic-storm-presenting-as-rapidly-evolving-multifocal-ischemic-and-hemorrhagic-strokes-a-case-report
#29
Mohammad Abu-Abaa, Ghassan Al-Qaysi, Sindhu Chadalawada, Adedeji Cole
Catastrophic antiphospholipid antibody syndrome (CAPS) is a life-threatening disorder. It is a rare and severe form of antiphospholipid antibody (APL) syndrome characterized by widespread multisystemic thrombosis. We present a 55-year-old male patient with acute cerebellar hemorrhagic stroke who developed widespread progressive microthrombosis and macrothrombosis manifesting as progressive bilateral ischemic strokes with lower extremities deep vein thrombosis (DVT) and acute renal failure within a week of presentation...
February 2023: Curēus
https://read.qxmd.com/read/36945288/demographic-clinical-and-serological-characteristics-of-antiphospholipid-syndrome-patients-from-the-anticoagulation-clinic-of-hospital-universitario-san-vicente-fundaci%C3%A3-n-medell%C3%A3-n-colombia
#30
JOURNAL ARTICLE
Santiago Álvarez-López, María Fernanda Ariza-Gómez, Vanessa López-Montoya, John Ubeimar Cataño-Bedoya, Diana Giraldo-Mendez, Fabian Jaimes
INTRODUCTION: Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia, characterized by vascular thrombosis or obstetric compromise, associated with the presence of antiphospholipid antibodies. Large international studies have analyzed the clinical/serological behavior of the disease and in Colombia, there are few cohorts that have been evaluated. OBJECTIVE: The main objective is to characterize the patients with APS followed in the anticoagulation clinic of a tertiary care hospital and to determine the clinical manifestations and serological findings at diagnosis...
February 2023: Curēus
https://read.qxmd.com/read/36915843/an-unusual-case-of-catastrophic-antiphospholipid-syndrome-in-an-elderly-man
#31
Kayla Lam, Mohammad Selim
Antiphospholipid syndrome (APS) is a condition in which the body produces antiphospholipid antibodies, resulting in arterial and venous thrombosis. Catastrophic antiphospholipid syndrome (CAPS) is a rare APS subtype characterized by acute thrombotic microangiography. Antiphospholipid antibodies cause thrombosis through activating and inhibiting properties. CAPS is caused by conditions or factors that trigger the production of antiphospholipid antibodies: genetics that increases the risk of antiphospholipid antibody-associated thrombosis, infection, surgery, medications, and malignancy...
February 2023: Curēus
https://read.qxmd.com/read/36820549/catastrophic-antiphospholipid-syndrome-in-lupus-associated-immune-thrombocytopenia-treated-with-eltrombopag-a-case-series-and-literature-review
#32
REVIEW
Wakar Garra, Or Carmi, Shaye Kivity, Yair Levy
BACKGROUND: Eltrombopag, a thrombopoietin receptor (TPO-R) agonist, is considered a second-line treatment for patients with refractory immune thrombocytopenia (ITP). Systemic lupus erythematosus (SLE) is frequently associated with ITP. In some cases, thrombocytopenia in SLE patients is attributed to concurrent antiphospholipid antibodies (APLA). Currently, data regarding treatment with TPO-R agonists for ITP in SLE or APLA patients are limited. The incidence of SLE flare or antiphospholipid syndrome while on TPO-R agonists has not been well-studied...
February 10, 2023: Medicine (Baltimore)
https://read.qxmd.com/read/36800570/management-of-cardiovascular-surgery-in-patients-with-systemic-lupus-erythematosus-including-thromboembolism-and-multiple-organ-failure-prevention-a-retrospective-observational-study
#33
JOURNAL ARTICLE
Taira Yamamoto, Satoshi Matsushita, Daisuke Endo, Akie Shimada, Shizuyuki Dohi, Kan Kajimoto, Yasutaka Yokoyama, Yuichiro Sato, Yoichiro Machida, Tohru Asai, Atsushi Amano
Systemic lupus erythematosus is a chronic autoimmune disease that affects most tissues. Cardiovascular events are critical, life-threatening, long-term complications of systemic lupus erythematosus (SLE). We report our single-center experience of performing cardiovascular surgery in patients with SLE while avoiding postoperative complications. We also suggest a new approach for cardiopulmonary bypass and perioperative management. We applied the antiphospholipid antibody syndrome (APS) severity classification published by the Japan Intractable Disease Information Center to patients with SLE for perioperative management...
February 17, 2023: Medicine (Baltimore)
https://read.qxmd.com/read/36792010/precipitating-factors-of-catastrophic-antiphospholipid-syndrome-the-role-of-anticoagulant-treatment-in-a-series-of-112-patients
#34
MULTICENTER STUDY
Romain Stammler, Yann Nguyen, Cécile Yelnik, Véronique Le Guern, Marc Lambert, Romain Paule, Eric Hachulla, Luc Mouthon, Anastasia Dupré, Félix Ackermann, Virginie Dufrost, Denis Wahl, Bertrand Godeau, Gaëlle Leroux, Ygal Benhamou, Estibaliz Lazaro, Eric Daugas, Holy Bezanahary, Arsène Mekinian, Jean-Charles Piette, Nathalie Morel, Nathalie Costedoat-Chalumeau
BACKGROUND: The prevention of catastrophic antiphospholipid syndrome (CAPS), a rare complication of antiphospholipid syndrome (APS), is a major goal. OBJECTIVES: We analyzed its precipitating factors, focusing on anticoagulation immediately before CAPS episodes. METHODS: We retrospectively analyzed patients in the French multicenter APS/systemic lupus erythematosus database with at least 1 CAPS episode. Then we compared each patient with known APS before CAPS with 2 patients with non-CAPS APS matched for age, sex, center, and APS phenotype...
May 2023: Journal of Thrombosis and Haemostasis: JTH
https://read.qxmd.com/read/36575067/selected-severe-8222-haematological-8220-syndromes-in-adult-intensive-care-patients
#35
REVIEW
Jaromír Gumulec, Ivo Demel, Klára Lančová, Eva Drbohlavová, Alicia Piegzová, Zdeněk Kořístek, Milan Navrátil, Vladimír Černý
Haemophagocytic syndrome, diffuse alveolar haemorrhage, catastrophic antiphospholipid syndrome and various types of thrombotic microangiopathies are rare conditions with significant morbidity and mortality. A common feature is late diagnosis, which can affect the success of treatment. The aim of this review article is to summarize the basic diagnostic and therapeutic steps of the present subpopulation of critically ill patients.
2022: Vnitr̆ní Lékar̆ství
https://read.qxmd.com/read/36289603/antiphospholipid-antibody-syndrome-associated-increased-surface-expression-of-vla4-integrin-on-human-monocytes
#36
JOURNAL ARTICLE
Ula Štok, Neža Štucin, Elizabeta Blokar, Aleš Ambrožič, Snežna Sodin-Šemrl, Saša Čučnik, Polona Žigon
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombosis and/or obstetric complications in the presence of antiphospholipid antibodies (aPL). Catastrophic APS (CAPS) is the most severe form of the disease, in which microvascular thromboses develop rapidly, leading to multiorgan failure. Monocytes, along with endothelial cells, are critical players in the pathogenesis of APS. Recruitment of these cells to the site of injury/inflammation involves a series of events, including capture, rolling, adhesion enhancement, and transmigration, which are controlled by surface adhesion molecules...
September 20, 2022: Biomedicines
https://read.qxmd.com/read/36274788/rapidly-evolving-necrotic-skin-lesions-of-the-face
#37
JOURNAL ARTICLE
Laura Mengeot, Liliane Marot, Marie Baeck
No abstract text is available yet for this article.
October 2022: JAAD Case Reports
https://read.qxmd.com/read/36272841/emerging-therapies-in-antiphospholipid-syndrome
#38
REVIEW
Anne Hubben, Keith R McCrae
The antiphospholipid syndrome (APS) is the most common cause of acquired immune-mediated thrombophilia. This syndrome is broadly defined by the presence of arterial or venous thrombosis, or pregnancy morbidity, in the presence of high levels of antiphospholipid antibodies. Despite recognition of this disorder more than 50 years ago, a fundamental unifying pathogenesis has not been determined. Due to this, mechanism-based therapies for APS are not available, and current management following thrombotic events suggests anticoagulation of indeterminate duration, or for obstetric complications, heparin/low molecular weight heparin and aspirin...
October 2022: Transfusion Medicine Reviews
https://read.qxmd.com/read/36195247/covid-19-and-the-antiphospholipid-syndrome
#39
REVIEW
Manuel Serrano, Gerard Espinosa, Antonio Serrano, Ricard Cervera
Coronavirus disease 2019 (COVID-19) has resulted in a global pandemic. Most COVID-19 patients are asymptomatic or have flu-like symptoms. However, around 15% of the patients may have severe disease, including unilateral or bilateral pneumonia with acute respiratory distress syndrome and progressive hypoxemia that may require mechanical ventilation assistance. A systemic inflammatory response syndrome occurs in the most severe forms of COVID-19, with multiorgan involvement which can be life threatening caused by a cytokine storm...
December 2022: Autoimmunity Reviews
https://read.qxmd.com/read/36126365/prevalence-characteristics-and-outcome-of-cardiac-manifestations-in-critically-ill-antiphospholipid-syndrome-patients
#40
JOURNAL ARTICLE
Lévi-Dan Azoulay, Marc Pineton de Chambrun, Romaric Larcher, Frédéric Pène, Laurent Argaud, Julien Mayaux, Matthieu Jamme, Remi Coudroy, Alexis Mathian, Aude Gibelin, Elie Azoulay, Yacine Tandjaoui-Lambiotte, Auguste Dargent, François Beloncle, Jean-Herlé Raphalen, Antoine Troger, Nicolas de Prost, Jérôme Devaquet, Damien Contou, Samuel Gaugain, Pierre Trouiller, Steven Grangé, Stanislas Ledochowski, Jérémie Lemarie, Stanislas Faguer, Vincent Degos, Quentin Moyon, Charles-Edouard Luyt, Mathieu Kerneis, Alain Combes, Zahir Amoura
AIMS: Antiphospholipid syndrome (APS) is a rare autoimmune disease defined by thrombotic events occurring in patients with persistent antiphospholipid antibodies. Cardiac manifestations in critically-ill APS patients are poorly investigated. We conducted a study to assess the prevalence, the characteristics and the prognosis of cardiac manifestations in thrombotic APS patients admitted to intensive care unit (ICU). METHODS AND RESULTS: A French, national, multicentre, retrospective study, conducted, from January 2000 to September 2018, including all APS patients admitted to 24 participating centres' ICUs with any new thrombotic (arterial, venous or microvascular) manifestation...
December 2022: Journal of Autoimmunity
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