A Case of Antiphospholipid Syndrome Underwent Cardiac Surgery Performed Using Coagulation Management by Measuring Heparin Concentration during Extracorporeal Circulation / 日本心臓血管外科学会雑誌

A 72-year-old female was diagnosed with systemic lupus erythematosus and antiphospholipid syndrome (APS) in 2014 and was followed up. Severe mitral regurgitation coexisted with APS, but the case was nonsymptomatic, and surgery involved high risk. Therefore, the physicians continued their observation. In 2020, the patient experienced rheumatic severe mitral stenosis and shortness of breath on exertion. Paroxysmal atrial fibrillation and coronary stenosis were also detected. Therefore, we planned mitral valve replacement, tricuspid annuloplasty, coronary artery bypass, pulmonary vein isolation and left atrial appendage closure. During extracorporeal circulation (ECC), we performed coagulation management based on blood heparin concentration using HMS PLUS. Because the APS patient showed prolonged activated clotting time (ACT), and coagulation therapy based on ACT is unreliable. She was discharged from our hospital on postoperative day 23. No complications, including bleeding and thrombosis, were observed 2 years after the operation. We experienced a case of APS who underwent cardiac surgery and performed coagulation management by measuring heparin concentration during ECC. We targeted a 3.5 U/ml heparin concentration, and her clinical course was uneventful.

Connective tissue disorders in pregnancy: maternal and fetal perspective

Background: The objective of this study was to study maternal and fetal outcome in connective tissue disorders in pregnancy.Methods: This was a retrospective type of observational study done in department of obstetrics and gynecology and department of rheumatology at a King Edward Memorial hospital over a period of 1.5 years. 48 women were included in this study after informed consent. All these women presented with collagen disorders to ANC outpatient department or to rheumatology outpatient department or in emergency. All postpartum patients having connective tissue disorders not recruited during ANC were also included in this study after taking their written, informed and valid consent.Results: Connective tissue disorders are associated with multiple voluntary and involuntary abortions as well as intrauterine fetal deaths. Even in those women having live births, many undergo cesarean sections due to various indications like fetal distress, poor biophysical profile, non-reassuring non stress test etc. Neonates born to mothers with connective tissue disorders are growth restricted and many of them need intensive care admission. Also, these women were found to have multiple associated medical comorbidities in pregnancy.Conclusions: The data collected and the results arrived upon should help contribute significant literature regarding collagen disorders in pregnancy and help in better fetal and maternal management during pregnancy.

Incidence of thrombophilia in patients with recurrent pregnancy loss

Background: Approximately 1-3% of women of reproductive age suffer from recurrent pregnancy loss. Objective of this study was to evaluate the association between recurrent pregnancy loss and thrombophilia.Methods: This is a descriptive study, involving retrospective analysis of patients with recurrent pregnancy losses. Patients with recurrent pregnancy loss in whom associated morbidity factors were excluded underwent screening for both acquired and inherited thrombophilia.Results: A total of 20 patients were screened for acquired and inherited thrombophilia with recurrent pregnancy loss. Thrombophilia was diagnosed in 70% cases. Out of which, anticardiolipin antibodies was found positive in 57% of patients, protein C 7% and protein S deficiency was observed in 35% cases.Conclusions: Thrombophilias are associated with recurrent pregnancy loss. Patients in whom other associated morbid factors are excluded, should be offered screening for thrombophilia. Multidisciplinary management involving hematologist is vital for management.

Antiphospholipid antibody syndrome and infertility / Síndrome anticorpo antifosfolípide e infertilidade

Abstract Antiphospholipid antibody syndrome (APS) is a systemic, autoimmune, prothrombotic disease characterized by persistent antiphospholipid antibodies (aPLs), thrombosis, recurrent abortion, complications during pregnancy, and occasionally thrombocytopenia. The objective of the present study was to review the pathophysiology of APS and its association with female infertility. A bibliographic review of articles of the past 20 yearswas performed at the PubMed, Scielo, and Bireme databases. Antiphospholipid antibody syndrome may be associated with primary infertility, interfering with endometrial decidualization and with decreased ovarian reserve. Antiphospholipid antibodies also have direct negative effects on placentation, when they bind to the trophoblast, reducing their capacity for invasion, and proinflammatory effects, such as complement activation and neutrophil recruitment, contributing to placental insufficiency, restricted intrauterine growth, and fetal loss. In relation to thrombosis, APS results in a diffuse thrombotic diathesis, with global and diffuse dysregulation of the homeostatic balance. Knowing the pathophysiology of APS, which is closely linked to female infertility, is essential for new therapeutic approaches, specialized in immunomodulation andinflammatory signaling pathways, to provide important advances in its treatment.

Resumo A Síndrome do anticorpo antifosfolípide (SAF) é uma doença sistêmica, autoimune e prótrombótica caracterizada por anticorpos antifosfolípides, trombose, aborto recorrente, complicações durante a gestação, e, ocasionalmente, trombocitopenia. O objetivo do presente estudo foi revisar a fisiopatologia da SAF e sua associação com a infertilidade feminina. Foi feita uma revisão bibliográfica dos últimos 20 anos nas bases de dados PubMed, Scielo e Bireme. A SAF pode estar associada à infertilidade primária, interferindo na decidualização endometrial e combaixas reservas ovarianas. Os anticorpos antifosfolípides também apresentam efeito negativo direto na placentação, se ligando ao trofoblasto e diminuindo sua capacidade de invasão, além de efeitos pró-inflamatórios, tais como ativação do sistema de complemento e recrutamento de neutrófilos, contribuindo para a insuficiência placentária, crescimento intrauterino restrito e perda fetal.Quanto a trombose, a SAF resulta em distúrbios trombóticos difusos, com uma desregulação do balanço homeostático. Conhecer a fisiopatologia da SAF, que apresenta associação importante com a infertilidade feminina, é essencial para novas abordagens terapêuticas, principalmente no que tange imunomodulação e os caminhos de ativação inflamatórios.

Antiphospholipid Antibody Syndrome Complicating Pregnancy: Anaesthetic Management for Caesarean Delivery.

Antiphospholipid antibody (APLA) syndrome is an acquired autoimmune disorder characterized by venous or arterial thrombosis. It causes recurrent fetal losses in females of reproductive age group. However, with appropriate anticoagulant therapy in antepartum and the postpartum period, favourable pregnancy outcomes are possible. Elective caesarean sections are quite common in view of bad obstetric history. Here we discuss the anaesthetic management of a 25 year old female patient with APLA syndrome scheduled for elective caesarean section.

The role of corticosteroids in primary antiphospholipid antibody syndrome presenting as cerebral venous thrombosis in young females at peripartum

The clinical study of cerebral venous thrombosis in antiphospholipid antibody syndrome in young females at peripartum was done to study the incidence of antiphospholipid antibodies in highly susceptible population groups most commonly at peripartum women. The presence of these antibodies points towards increased susceptibility to thrombosis and ischemic stroke apart from other manifestations in peripartum period. The age group most affected was between 20-25 years. Most of them were primipara. Many of the patients underwent Cesarean section before the presentation with the specific neurological complaint. None of the patients gave positive history for use of oral contraceptive pills. This study showed a 69% incidence of antiphospholipid antibodies out of the total patients studied. It was also found that 66% of the APL positive patients had radiological evidence of cerebral venous thrombosis. To help clarify the significance of aCL in CVT, this study was systematically analyzed and the clinical, radiological, treatment, and outcome information of patients with CVT tested for aCL immunereactivity at our institution and the pertinent literature was systematically reviewed. It was also studied that the most of the patients improved with corticosteroids.

Correlation between antiphospholipid antibody syndrome and the early onset of preeclampsia / 实用医学杂志

Objective To investigate the correlation between antiphospholipid antibody syndrome and the early onset of preeclampsia. Methods From May 2010 to July 2013, one hundred and threecases in-patient treatment of the early onset preeclampsia were enrolled in this study. The maternal serum anticardiolipin antibodies(ACA)and anti-β2-glycoproteinⅠantibody (Aβ2-GPⅠ) were detected by ELISA method. 58 cases of pregnant women were randomly divided into the routine treatment group (30 cases) and the anticoagulant therapy group (28 cases). Results ACA positive predictive value of the early onset preeclampsia value was 3.9%. No significant difference was found in the prolonged anticoagulation of early onset preeclampsia time between the control group and the treatment group. Conclusion ACA may not be used to predict the early onset preeclampsia. Anticoagulation therapy can′t extend the early onset preeclampsia time and improve the outcome of pregnancy.

A Case of Refractory Headache with Antiphospholipid Antibody Syndrome Improved by High-Intensity Warfarin Medication

Antiphospholipid antibody syndrome (APS) is a coagulation disorder associated with antiphospholipid antibodies. Headache is common in APS patients and often unresponsive to analgesics. We report a case of refractory headache in a patient with APS, who was improved by high-intensity warfarin treatment. The mechanisms of the headache in patients with APS were presumed to be hypercoagulability of microcirculation and thrombotic occlusion of the capillaries, which were associated with antiphospholipid antibodies. Therefore, high-intensity warfarin could be considered as one of the treatments for refractory headache in patients with APS.

A Case of Secondary Antiphospholipid Antibody Syndrome with Thyroid Cancer

Antiphospholipid antibody syndrome (APS) is defined as the presence of lupus anticoagulant antibody or anticardiolipin antibody with vascular thrombosis or pregnancy complications. APS can be associated with autoimmune disease or infectious disease. APS has also been reported in conjunction with variety of solid and hematologic malignancies. There were some reports on APS which were accompanied by hematologic malignancy, but there was no report with solid malignancy in Korea. We experienced one case of secondary APS, which was diagnosed during pre-operative evaluation of thyroid cancer. This patient had prolonged aPTT (activate partial thromboplastin time) and decreased coagulation factors which were regarded as hemophilia at first. Although the precise mechanism of the relationship between APS and cancer has not been proven thoroughly, APS can be accompanied by various malignancies. So proper screening and early detection of malignancies in APS patients are recommended.

Endocardite de Libman-Sacks, anticorpos antifosfolípides e trombose arterial no lúpus ertitematoso sistêmico: relato de caso / Libman-Sacks endocarditis, antiphospholipid antibodies and arterial thrombosis in systemic lupus erythematosus: case report

Relato de caso de paciente de 38 anos, feminina, com lúpus eritematoso sistêmico (LES) que apresentou evento tromboembólico arterial agudo em membro inferior direito. A investigação evidenciou a presença de anticorpos antifosfolípides e vegetação asséptica em válvula mitral, endocardite de Libman-Sacks (eLS). São discutidas as possíveis causas de eventos tromboembólicos arteriais no LES, com ênfase nas recomendações atuais para diagnóstico e tratamento da eLS.

Case report of a 38-year-old female patient with systemic lupus erythematosus (SLE) who presented an acute arterial thromboembolic event in the right lower limb. Investigation showed the presence of antiphospholipid antibodies and sterile vegetation in the mitral valve, Libman-Sacks endocarditis (LSE). Possible causes of thromboembolic events in SLE are discussed, with emphasis on current recommendations for diagnosis and treatment of LSE.

Anesthetic management of right atrial mass removal and pulmonary artery thrombectomy in a patient with primary antiphospholipid antibody syndrome.

Antiphospholipid antibody syndrome (APLAS) characterises a clinical condition of arterial and venous thrombosis associated with phospholipids directed antibodies. APLAS occurs in 2% of the general population. However, one study demonstrated that 7.1% of hospitalised patients were tested positive for at least one of the three anticardiolipin antibody idiotype. Antiphospholipid antibodies often inhibit phospholipids dependent coagulation in vitro and interfere with laboratory testing of hemostasis. Therefore, the management of anticoagulation during cardiopulmonary bypass can be quite challenging in these patients. Here, we present a case of right atrial mass removal and pulmonary thrombectomy in a patient of APLAS.

A importância da intervenção nutricional na redução do peso corpóreo em pacientes com síndrome do anticorpo antifosfolípide / The importance of nutritional intervention in the reduction of body weight in patients with the antiphospholipid antibody

OBJETIVO: Avaliar a eficácia da intervenção nutricional na redução do excesso de peso (EP), em pacientes com síndrome do anticorpo antifosfolípide (SAF). MÉTODO: Incluídos 40 pacientes, acima de 18 anos, com diagnóstico de SAF primária ou secundária, acompanhados no Serviço de Reumatologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP) e recrutados no período de outubro de 2005 a fevereiro de 2006. Foram coletados dados demográficos e realizados a revisão de prontuários, a mensuração de peso e da altura, o cálculo do índice de massa corpórea (IMC) atual e a adequação da dieta pelo IMC ideal. Foi realizada avaliação na primeira consulta e após intervalo mínimo de dois meses. RESULTADOS: A média de idade dos pacientes foi de 41 anos, sendo 93 por cento de mulheres. Os pacientes com SAF primária perfaziam 25 por cento, com média de dois anos da doença. A trombose venosa ocorreu em 63 por cento, arterial em 48 por cento e manifestações obstétricas em 27 por cento. Na primeira consulta, 68 por cento apresentavam EP, 27 por cento eram eutróficos e 5 por cento estavam com baixo peso (BP). Após três meses de intervenção, os eutróficos mantiveram o peso e os de BP tornaram-se eutróficos, segundo o IMC. Interessantemente, entre os pacientes com EP (n = 27), 82 por cento emagreceram, 14 por cento engordaram e 4 por cento se mantiveram. Especificamente, 11 pacientes apresentaram 1 por cento a 3 por cento de perda ponderal de peso, oito perderam de 4 por cento a 7 por cento, dois reduziram 8 por cento a 9 por cento e um reduziu 13,6 por cento com o acompanhamento nutricional. CONCLUSÃO: Foi demonstrado no presente estudo que a intervenção nutricional conseguiu atingir metas para redução de peso, possibilitando diminuição no risco trombótico num curto período, sendo, portanto, uma modalidade terapêutica inicial e de eleição para corrigir o EP em pacientes com SAF.

OBJECTIVE: To evaluate the efficacy of the nutricional intervention in the loss of the weight excess (WE), in patients with the antiphospholipid antibody syndrome (APS). METHODS: Forty patients older than 18 years-old were included, with a diagnosis of either primary or secondary APS, followed at the the Rheumatology Outpatient Clinic from HCFM-USP and were recruited between october/2005 and february/06. Demographic data and patient records were reviewed. Body weight, height and current Body Mass Index (BMI) were recorded as well as diet adjustment according to ideal BMI. Patients were subjected to a first medical and nutritional evaluation and subsequently at least 2 months after baseline. RESULTS: The mean age was 41 years old and 93 percent of the patients were female. The patients with primary APS were 25 percent, the mean disease duration was 2 years. Venous thrombosis occurred in 63 percent, arterial in 48 percent and obstetric events in 27 percent. In the first consultation 68 percent presented WE, 27 percent were euthrophic and 5 percent were of low weight (LW). After three months of intervention, the eutrophics maintained body weight while those with LW became eutrophic. Among the patients with WE (n=27), 82 percent lost weight, 14 percent had gained weight and 4 percent kept their weight. Specifically, 11 patients had-3 percent of weight loss, 8 lost 4-7 percent, 2 lost 8-9 percent, and 1 patient lost 13.6 percent while under nutritional counseling. CONCLUSION: The data demonstrate that nutritional intervention help achieving reduction in body weight. This may lead to reduction of the thrombotic risk in a short period of time. Nutritional intervention may thus be a valuable initial therapeutic approach to adjust body weight in patients with APS.

Síndrome de anticuerpos antifosfolipídico en obstetricia: presentación de dos casos / Antiphospholipid antibody syndrome in obstetrics: report of two cases

Se presentan dos casos de gestantes con diagnóstico previo de síndrome de anticuerpos antifosfolipídicos (SAF) con historias de pérdidas de embarazos anteriores por diversos motivos, que tuvieron un seguimiento durante todo el embarazo y se aplicaron tratamientos distintos de acuerdo a la situación clínica. Se obtuvieron resultados perinatales diferentes. Se hacen comentarios con relación a la terapéutica utilizada y la repercusión de esta enfermedad en el embarazo, con comentarios finales basados en nuestra experiencia.

Two cases of pregnant women with previous diagnosis of antiphospholipid antibody syndrome that had lost previous pregnancies for different reasons are presented. They were followed-up during the whole gestation and diverse treatments were applied according to the clinical situation. Various perinatal results were obtained. Comments are made on the therapeutics used and the impact of this disease on pregnancy. Finally, observations based on our experience are exposed.

A Case of Primary Adrenal Insufficiency Presenting as the Initial Clinical Manifestation of Primary Antiphospholipid Antibody Syndrome / 대한류마티스학회지

Primary adrenal insufficiency is one of the rare clinical manifestations of antiphospholipid antibody syndrome. The pathogenesis is mainly ascribed to the hemorrhagic infarction secondary to thrombosis. We describe a 19-year-old man who developed symptoms of adrenal insufficiency and then was diagnosed of primary antiphospholipid antibody syndrome, which is the first reported case in adult patients in Korea. The possibility of adrenal failure should be considered in the management of antiphospholipid antibody syndrome and also, when primary adrenal insufficiency is accompanied by no definite etiology, the antiphospholipid antibody syndrome should be considered as a possible etiology.

Antiphospholipid Antibody Syndrome: A Case Report

Antiphospholipid antibody (APLA) syndrome, which is presented by seizure, is uncommon. Most seizures in APLA syndrome are associated with systemic lupus erythematosus (SLE) or other autoimmune diseases. Its pathophysiology is not clear; however, many authorities suggest that autoantibodies related to APLA syndrome are related to an ischemic change in the brain. We experienced a man with APLA syndrome, who presented with a seizure, but he had no evidence of an ischemic change in the brain. He underwent conservative management for 2 weeks and was discharged. He was admitted twice due to seizure. One year later, he had a cerebrovascular attack and was hospitalized.

Anesthetic Management of a Patient with Antiphospholipid Antibody Syndrome: A case report / 대한마취과학회지

The antiphospholipid antibody syndrome (APS) is characterized by the presence of auto antibodies to phospholipids in association with in vitro prolongation of phospholipid-dependent coagulation tests, recurrent pregnancy loss, multiple thrombotic events, etc. Due to strong predilection for thrombosis in both the arterial and venous system, perioperative thromboprophylaxis for APS patients is very important to avoid catastrophic major cardiovascular complications. We experienced general anesthesia for a total abdominal hysterectomy in a 47-year-old patient diagnosed as having APS by a past medical history of deep vein thrombosis, fetal wastages, and laboratory confirmation. Pre and postoperative management for hypercoagulability was done with oral warfarin and low dose heparin. To prevent intraoperative thrombosis, we maintained an activated clotting time (ACT) over 200 seconds by heparin 3000 U injection, applied antithrombotic stockings, and tried to avoid dehydration, hypothermia and infections.

Cerebral Infarct Caused by Ovarian Hyperstimulation Syndrome after Using an Ovulation Induction Agent: A case report

Ovarian hyperstimulation syndrome (OHSS) is a serious complication of ovulation induction agents used for patients with ovulatory dysfunction. It's pathogenesis is uncertain, although increased capillary permeability with extravasation offluid into the abdominal cavity is most likely suggested. Early features of the syndrome include nausea, vomiting,diarrhea, ascites, pleural effusion, and ovarian enlargement. In severe cases, increased viscosity with hemoconcentration hypovolemia, oliguria, coagulopathy, and thromboembolism may appear, and eventually develop to cerebral infarct. We experienced a case of cerebral infarct caused by OHSS with antiphospholipid antibody syndrome after using an ovulation induction agent, and we present this rare case with the review of literature.

A Case of Primary Antiphospholipid Antibody Syndrome with Multiple Thromboses Including Thrombotic Occlusion of Abdominal Aorta

In antiphospholipid antibody syndrome (APS), multiple thromboses affecting large arteries and veins through the whole body is rare. In this report, a case of a 65 year old women suffering from APS with multiple thromboses of the renal and iliac artery, the infrarenal abdominal aorta, the deep vein, and a pulmonary thromboembolism is presented. The patient was treated successfully with intensive anticoagulation and steroid pulse therapy. There was no evidence of recurrent thrombotic complications during a three year follow-up period with oral warfarin and prednisolone

A Case of Leukocytoclastic Vasculitis Associated with Antiphospholipid Antibody Syndorme / 대한피부과학회지

The antiphospholipid antibody syndrome is an acquired multisystemic disorder characterized by persistent elevated antiphospholipid antibodies and/or hypercoagulation in veins or arteries, or both. The clinical manifestations of the antiphospholipid antibody syodrome are recurrent thrombosis, fetal loss, thrcenbocytopenia, and various cutaneous lesions. Skin lesions are the first sign of this syndrome in 41% of patients and systemic thrombosis develops in 40% of them. Livedo reticularis is the most common cutaneous finding of the antiphosphotipid antibody syndrome. Although vasculitis has not been frequently noted in antiphospholipid antibody syndrome, some vasculitis such as polyarteritis nodosa, giant cell arteritis, and other nonspecific vasculitides have been found in association with antiphospholipid antibody syndrome. We present a male patient with typical manifestations of leukocytoclastic vasculitis with deep vein thrombosis and positive antiphospholipid antibodies. It suggests that a case of antiphospholipid antibody syndorme was accompanied with cutaneous leukocytoclastic vasculitis.

A case of primary antiphospholipid antibody syndrome showing vegetation on the mitral valve through echocardiography / 영남의대학술지

Antiphospholipid antibody syndrome(APS) is a well-known clinical syndrome characterized by recurrent arterial or venous thromboses, recurrent fetal loss, thrombocytopenia, together with high titers of sustained anticardiolipin antibody(aCL) or lupus anticoagulant(LA). Although systemic lupus erythematosus(SLE) and APS may coexist, a high proportion of patients manifesting the APS do not suffer from classical lupus or other connective tissue disease. The patient has been defined as having a primary antiphospholipid antibody syndrome. We experienced one case of primary APS with recurrent fetal loss, recurrent cerebral infarctions, positive anticardiolipin antibody IgG and fluttering vegetation on the mitral valve, without other connective tissue diseases including SLE. Forty-three old female had 2 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia and positive antinuclear antibody, but did not meet whole criteria. The patient was treated with ticlopidine, and anticoagulant therapy was recommended.