The CAPS Registry: morbidity and mortality of the catastrophic antiphospholipid syndrome

Lupus ◽  
2009 ◽  
Vol 18 (10) ◽  
pp. 905-912 ◽  
Author(s):  
S Bucciarelli ◽  
G Espinosa ◽  
R Cervera
Keyword(s):  
Antiphospholipid Syndrome ◽  
Clinical Medicine ◽  
Clinical Laboratory ◽  
Case Reports ◽  
Lethal Outcome ◽  
Catastrophic Antiphospholipid Syndrome ◽  
European Forum ◽  
International Registry ◽  
The World ◽  
Medicine Today

Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant, its potentially lethal outcome emphasises its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. To put together all the published case reports as well as the new diagnosed cases from all over the world, an international registry of patients with catastrophic APS (CAPS Registry) was created in 2000 by the European Forum on Antiphospholipid Antibodies. Currently, it documents the entire clinical, laboratory and therapeutic data of more than 300 patients whose data has been fully registered. This registry can be freely consulted at the Internet (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM), and it is expected that the periodical analysis of these data will allow us to increase our knowledge of this condition.

Catastrophic antiphospholipid syndrome (CAPS): update from the ‘CAPS Registry’

Lupus ◽  
2010 ◽  
Vol 19 (4) ◽  
pp. 412-418 ◽  
Author(s):  
R. Cervera ◽  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Clinical Medicine ◽  
Clinical Laboratory ◽  
Case Reports ◽  
Lethal Outcome ◽  
Catastrophic Antiphospholipid Syndrome ◽  
European Forum ◽  
International Registry ◽  
The World ◽  
Medicine Today

Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant, its potentially lethal outcome emphasizes its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. In order to put together all of the published case reports as well as the new diagnosed cases from all over the world, an international registry of patients with catastrophic APS (CAPS Registry) was created in 2000 by the European Forum on Antiphospholipid Antibodies (see http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). Currently, it documents the entire clinical, laboratory and therapeutic data of more than 300 patients whose data has been fully registered.

The Ped-APS Registry: the antiphospholipid syndrome in childhood

Lupus ◽  
2009 ◽  
Vol 18 (10) ◽  
pp. 894-899 ◽  
Author(s):  
T Avčin ◽  
R Cimaz ◽  
B Rozman ◽  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Clinical Laboratory ◽  
Future Research ◽  
Juvenile Systemic Lupus Erythematosus ◽  
Collaborative Project ◽  
European Forum ◽  
International Registry ◽  
Low Prevalence

In recent years, antiphospholipid syndrome (APS) has been increasingly recognised in various paediatric autoimmune and nonautoimmune diseases, but the relatively low prevalence and heterogeneity of APS in childhood made it very difficult to study in a systematic way. The project of an international registry of paediatric patients with APS (the Ped-APS Registry) was initiated in 2004 to foster and conduct multicentre, controlled studies with large number of paediatric APS patients. The Ped-APS Registry is organised as a collaborative project of the European Forum on Antiphospholipid Antibodies and Juvenile Systemic Lupus Erythematosus Working Group of the Paediatric Rheumatology European Society. Currently, it documents a standardised clinical, laboratory and therapeutic data of 133 children with antiphospholipid antibodies (aPL)-related thrombosis from 14 countries. The priority projects for future research of the Ped-APS Registry include prospective enrolment of new patients with aPL-related thrombosis, assessment of differences between the paediatric and adult APS, evaluation of proinflammatory genotype as a risk factor for APS manifestations in childhood and evaluation of patients with isolated nonthrombotic aPL-related manifestations.

scholarly journals Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

2019 ◽  
Vol 6 (1) ◽  
pp. 13
Author(s):  
Carlos Anjo ◽  
Philip Fortuna ◽  
Luís Reis ◽  
Luís Bento
Keyword(s):  
Mortality Rate ◽  
Triple Therapy ◽  
Antiphospholipid Syndrome ◽  
Rare Complication ◽  
Multiorgan Failure ◽  
Lower Limbs ◽  
Rapid Progression ◽  
Muscular Tissue ◽  
Lethal Outcome ◽  
Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome (CAPS), an infrequent autoimmune disease, manifests as a widespread thrombotic disease with multiorgan failure with a 37% mortality rate. We report 39-year-old Caucasian women, medical history of Systemic Lupus Erythematous (SLE) complicated with APS and proliferative glomerulonephritis under dialysis. Patient was admitted at the ICU with septic shock with thoracotomy dehiscence origin. During the ICU stay, extensive lower limbs ulcers with polymorphonuclear infiltrate and steatonecrosis areas in adipose and muscular tissue. Lab studies: anti-β2-glycoprotein-Iantibody was detected. As probable CAPS diagnosis was made, triple therapy with methylprednisolone, immunoglobulin and anticoagulation immediately was started. Serious vasoplegic shock occurs with failure in vasopressor support increment and the patient died. The authors report an APS with rapid progression to catastrophic stage, rare complication that occurs only in 1% of catastrophic APS. In CAPS treatment, a statistical association between triple therapy and the decrease of mortality rate of patients with CAPS was observed, when compared with other combinations or with none of the treatments included in the triple therapy. Although the case had a lethal outcome, the authors emphasize the use of triple therapy in CAPS and the challenge of treating patients with CAPS and infection where the balance between the need for anticoagulation, aggressive immunosuppression and infection control is difficult to manage.

Thrombotic microangiopathy in the course of catastrophic antiphospholipid syndrome successfully treated with eculizumab: case report and systematic review of the literature

Lupus ◽  
2020 ◽  
Vol 29 (6) ◽  
pp. 631-639 ◽  
Author(s):  
Marta Skoczynska ◽  
Mark A Crowther ◽  
Malgorzata Chowaniec ◽  
Monika Ponikowska ◽  
Shruti Chaturvedi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Single Case ◽  
Case Reports ◽  
Review Of The Literature ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Total Remission

Objective The purpose of this study was to characterize the role of eculizumab, a monoclonal antibody against the terminal complement component C5, in patients with catastrophic antiphospholipid syndrome (CAPS). Methods We present a case report of a patient with systemic lupus erythematosus (SLE) and CAPS treated with eculizumab, as well as results of a systematic review of the literature. Results Including our patient, we identified 11 case reports of patients with CAPS treated with eculizumab. All of them had partial or total remission of symptoms. Conclusion Data on eculizumab efficacy in CAPS are promising but are limited to single case reports. More studies are needed to develop evidence-based recommendations for eculizumab use in CAPS.

Methods and clinical applications in nuclear cardiology: a position statement

2002 ◽  
Vol 41 (01) ◽  
pp. 3-13 ◽  
Author(s):  
M. Schäfers
Keyword(s):  
Nuclear Cardiology ◽  
Clinical Medicine ◽  
Imaging Techniques ◽  
Cost Savings ◽  
Cost Benefit ◽  
Position Statement ◽  
Electron Beam Tomography ◽  
Partial Functions ◽  
Cost Benefit Ratio ◽  
Medicine Today

SummaryNuclear cardiological procedures have paved the way for non-invasive diagnostics of various partial functions of the heart. Many of these functions cannot be visualised for diagnosis by any other method (e. g. innervation). These techniques supplement morphological diagnosis with regard to treatment planning and monitoring. Furthermore, they possess considerable prognostic relevance, an increasingly important issue in clinical medicine today, not least in view of the cost-benefit ratio.Our current understanding shows that effective, targeted nuclear cardiology diagnosis – in particular for high-risk patients – can contribute toward cost savings while improving the quality of diagnostic and therapeutic measures.In the future, nuclear cardiology will have to withstand mounting competition from other imaging techniques (magnetic resonance imaging, electron beam tomography, multislice computed tomography). The continuing development of these methods increasingly enables measurement of functional aspects of the heart. Nuclear radiology methods will probably develop in the direction of molecular imaging.

Catastrophic antiphospholipid syndrome with bilateral adrenal hemorrhage: a case report

2019 ◽  
Author(s):  
De Marchi Lucrezia ◽  
M K de Filette Jeroen ◽  
Sol Bastiaan ◽  
E Andreescu Corina ◽  
Kunda Rastislav ◽  
...  
Keyword(s):  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Adrenal Hemorrhage ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Bilateral Adrenal Hemorrhage

Histórias escritas com sangue: Uma análise de processos judiciários de assassinatos de mulheres em Goiânia (1970-1984)

2020 ◽  
Author(s):  
Neide Célia Ferreira Barros
Keyword(s):  
Power Relations ◽  
Case Reports ◽  
Border Region ◽  
Life And Death ◽  
Country Life ◽  
The World ◽  
Gender Power

This book analyzes the criminal processes of homicides or attempted homicides of women in Goiânia during the period of 1970-1984. We observed the gender power relations in the capital of Goiás, a border region, a mixture of country life elements and discourses of modernity. Hence, through case reports of women who suffered attacks on their lives in a period of intense changes, such as the organization of feminist groups in Brazil and the world, political and economic repercussions of the construction of Brasília in Goiás and mass immigration to Goiânia, we have pursued to understand what it meant socially to "be a man" and "to be a woman" in this capital and what consequences were brought into their bodies, concerning life and death, protection and punishment.

scholarly journals PCR-detectable Candida DNA exists a short period in the blood of systemic candidiasis murine model

2020 ◽  
Vol 15 (1) ◽  
pp. 677-682
Author(s):  
Zheng-Xin He ◽  
Hui-Hai Zhao ◽  
Fu-Kun Wang
Keyword(s):  
Heat Shock ◽  
Murine Model ◽  
Fungal Pathogens ◽  
Clinical Medicine ◽  
Diagnostic Techniques ◽  
Systemic Candidiasis ◽  
Blood Samples ◽  
Short Period ◽  
Polymerase Chain ◽  
Medicine Today

AbstractInvasive candidiasis is a major challenge to clinical medicine today. However, traditional fungal diagnostic techniques and empirical treatments have shown great limitations. Although efforts are necessarily needed in methodology standardization and multicenter validation, polymerase chain reaction (PCR) is a very promising assay in detecting fungal pathogens. Using a “heat-shock” DNA preparation method, a rapid and simple PCR protocol for quantification of the Candida albicans (C. albicans) ribosomal DNA was established. The PCR assay could detect Candida DNA as low as 10 CFU/mL in samples prepared by the heat-shock protocol, without any cross-reaction with DNA prepared from other Candida spp. and bacterial pathogens. For simulated blood samples, the PCR test sensitivity of whole blood samples was better than that of plasma and blood cells. In the systemic candidiasis murine model, detectable DNA was only observed within 24 h after C. albicans SC5314 injection, which is much shorter than that observed in the kidney.

scholarly journals Cardio-Pulmonary Sequelae in Recovered COVID-19 Patients: Considerations for Primary Care

2021 ◽  
Vol 12 ◽  
pp. 215013272110237
Author(s):  
Zouina Sarfraz ◽  
Azza Sarfraz ◽  
Alanna Barrios ◽  
Radhika Garimella ◽  
Asimina Dominari ◽  
...  
Keyword(s):  
Primary Care ◽  
Primary Infection ◽  
Clinical Laboratory ◽  
Point Of Care ◽  
Case Reports ◽  
Case Series ◽  
Infectious Complications ◽  
Care Physician ◽  
Cross Sectional ◽  
Infection Period

Background: Current literature lacks characterization of the post-recovery sequelae among COVID-19 patients. This review characterizes the course of clinical, laboratory, radiological findings during the primary infection period, and the complications post-recovery. Primary care findings are presented for long-COVID care. Methods: Adhering to PRISMA guidelines, 4 databases were searched (PubMed, Embase, CINAHL Plus, Scopus) through December 5, 2020, using the keywords “COVID-19 and/or recovered and/or cardiovascular and/or long-term and/or sequelae and/or sub-acute and/or complication.” We included published peer-reviewed case reports, case series, and cross-sectional studies providing the clinical course of COVID-19 infection, and cardiopulmonary complications of patients who recovered from COVID-19, while making healthcare considerations for primary care workers. Results: We identified 29 studies across 9 countries including 37.9% Chinese and 24.1% U.S. studies, comprising 655 patients (Mean Age = 45) with various ethnical backgrounds including Asian and European. Based on the WHO COVID-19 severity classification scale, initial disease severity was mild for 377 patients and severe for 52 patients. Treatments during primary infection included corticosteroids, oxygen support, and antivirals. The mean value (in days) for complication onset after acute recovery was 28 days. Complete blood counts and RT-PCR tests were the most common laboratory results described. In 22 of the studies, patients showed signs of clinical improvement and were prescribed medications such as anticoagulants or corticosteroids. Conclusion: Post-recovery infectious complications are common in long-COVID-19 patients ranging from mild infections to life-threatening conditions. International thoracic and cardiovascular societies need to develop guidelines for patients recovering from COVID-19 pneumonia, while focused patient care by the primary care physician is crucial to curb preventable adverse events. Recommendations for real-time and lab-quality diagnostic tests are warranted to establish point-of-care testing, detect early complications, and provide timely treatment.

scholarly journals 2019 novel coronavirus disease with secondary ischemic stroke: two case reports

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Bin Fu ◽  
Yun Chen ◽  
Ping Li
Keyword(s):  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Clinical Laboratory ◽  
Case Reports ◽  
Small Vessel ◽  
Vessel Occlusion ◽  
Aged Patients ◽  
Health Crisis ◽  
Physical Examinations ◽  
Novel Coronavirus

Abstract Background The COVID-19 pandemic, which broke out in Wuhan in 2019, has become the global health crisis of our time. Elderly patients with certain fundamental diseases are more likely to develop severe cases. The secondary lesion following viral infection have only rarely been reported. Case presentation We here report two cases of coronavirus-infected pneumonia with acute ischemic stroke in middle-aged patients. In both COVID-19 cases, neurological physical examinations showed normal results before infection. Lymphocytopenia, accompanied by elevated cytokines and D-dimers, were found from serum clinical laboratory examination at admission. Dysarthria and limb muscle weakness are initial manifestations, occurring one week after infect-causative pathogen, SARS-CoV-2. The head CT and head/neck arterial CTA showed small-vessel occlusion. The patients were diagnosed with coronavirus diseases with secondary acute ischemic stroke. They were treated with tirofiban and followed up with daily aspirin and atorvastatin. Conclusions These cases suggested that secondary ischemic stroke, mainly manifested as small-vessel occlusion, should be considered for COVID-19 patients and diagnosed and treated promptly.

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