CASE PRESENTATION: We present a successful aortic valve replacement with cardiopulmonary bypass in a 48 years old lady with antiphospholipid syndrome, who has severe aortic regurgitation as a result of Libman-sacks endocarditis. Antiphospholipid antibodies were positive and the clinical data showed both negative cultures and infective parameters. Surgically resected vegetations revealed sterile fibrinous and verrucous vegetations on aortic valve. Valve replacement and the course of cardiopulmonary bypass was uneventful, and the patient was discharged well.
CONCLUSIONS: Classically Libman-Sacks endocarditis is often and more commonly associated with autoimmune diseases such as systemic lupus erythematosus, although it can occur in both primary and secondary antiphospholipid syndrome. It is not a common entity, and it is a frequent underestimated disease as most clinicians do not routinely screen for valvular lesion in patients with antiphospholipid syndrome unless they are symptomatic. However, due to its high prevalence of cardiac involvement, clinicians should have a high index of suspicion in the attempt to minimize cardiovascular and haemodynamic complications. Valve surgery in patients with antiphospholipid syndrome carries considerable early and late morbidity and mortality, usually caused by thromboembolic and bleeding events. The perioperative anticoagulation management and haemostatic aspect of antiphospholipid syndrome present an exceptional challenges to clinicians, surgeons, anaesthetists and laboratory personnel.
METHODS: Different electronic databases were searched in a non-systematic way to find out the literature of interest.
RESULTS: The level of ferritin rises in many inflammatory conditions including autoimmune disorders. However, in four inflammatory diseases (i.e., adult-onset Still's diseases, macrophage activation syndrome, catastrophic antiphospholipid syndrome, and sepsis), high levels of ferritin are observed suggesting it as a remarkable biomarker and pathological involvement in these diseases. Acting as an acute phase reactant, ferritin is also involved in the cytokine-associated modulator of the immune response as well as a regulator of cytokine synthesis and release which are responsible for the inflammatory storm.
CONCLUSION: This review article presents updated information on the role of ferritin in inflammatory and autoimmune diseases with an emphasis on hyperferritinaemic syndrome.
Materials and Methods: The coagulometer for factor VIII assay is Sysmex CS-5100. All data were expressed as mean ± standard deviation (SD).
Results: A total of 135 cases were studied. Of these, 100 cases were of mild hemophilia A diagnosed by molecular genetics and, 15 cases were positive for LAC, which were confirmed by dilute Russell Viper venom test. Clot-based one-stage APTT assay showed 65% sensitivity and 80% specificity in diagnosing mild hemophilia A cases and out of 15 LAC cases, it showed false positivity in five cases. Chromogenic assay showed 85% sensitivity and 90% specificity in diagnosing mild hemophilia cases and was 100% specific in excluding LAC cases.
Conclusions: One-stage APTT assay is the most commonly used test for determining factor VIII levels but chromogenic assay are considered as the gold standard and recommended as the reference method by European Pharmacopoeia and ISTH subcommittee. Mild hemophilia A patients with missense mutations show discrepancy between the one-stage clot-based APTT assay and chromogenic assays for determination of factor VIII level and this can lead to misdiagnosis or misclassification of mild hemophilia A. Therefore, it is recommended that both the assays should be used in the evaluation of mild hemophilia cases.
OBJECTIVES: We evaluated the leading causes of all-cause hospitalizations and their predictive factors in the Malaysian multi-ethnic SLE patients.
METHODS: This is a retrospective study involving 300 SLE patients. Demographic data and details of hospitalizations from the year 1988 until 2019 were reviewed. Baseline and latest disease activity (SLEDAI-2 K) and SLICC/ACR damage index (SDI) scores were evaluated. To be eligible for this study, their SLE diagnosis and disease duration was at least one year; this is to ensure that the baseline disease damages were measured at least after 6 months of diagnosis and subsequent disease damage indexes were captured.
RESULTS: Majority were of Chinese ethnicity 150 (50%). The cohort's median age was 48 (18-82) years and median disease duration was 13 (1-52) years. 133 (44.3%) had SDI score of ≥1 at baseline (early damage). 69 (23%) had developed new organ damage during this study period.There were 222 (74%) patients ever hospitalized from this cohort. The main cause of hospitalization was lupus flare which included concurrent infection (n = 415 admissions, 46%), followed by elective admissions for procedures and others (n = 284 admissions, 31.5%). Admissions for treatment and disease related complications were 13.8%. 8.7% of admissions were due to infections alone. Median length of stay for SLE-related cause admissions was longer compared to non-SLE related causes. Jointly predictive factors for hospitalization were anti-phospholipid syndrome (OR 5.82), anti-Smith (OR 6.30), anti-SSA (OR 3.37), serositis (OR 14.56), neurological (OR 5.52) and high baseline SDI (OR 1.74), all p