What we know and what we don't know about catastrophic antiphospholipid syndrome.

Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body. Analysis of the CAPS Registry has also shown that patients receiving anticoagulation, glucocorticoids and plasma exchange and/or IVIG have a better prognosis. However, there are still many unresolved questions. In this review we summarize what is known and what is still a matter of research in this condition.

Therapeutic Plasma Exchange in Catastrophic Antiphospholipid Syndrome (CAPS): A Rare Case Report and Literature Review.

BACKGROUND/AIM: Catastrophic antiphospholipid syndrome (CAPS) may be the first manifestation ("de novo") of antiphospholipid syndrome (APS) or a complication in the clinical course of patients known to have this syndrome. Approximately 40% of patients had an associated autoimmune disease, mainly, systemic lupus erythematosus (SLE). The trigger can be one of the following: infections, surgical interventions, neoplasms, pregnancy, discontinuation of anticoagulant treatment, and others. CAPS is a medical emergency in which early identification and prompt initiation of aggressive therapy is extremely important. According to the Guidelines for the use of Therapeutic Apheresis in Clinical Practice developed by the American Society for Apheresis (ASFA), last updated in April 2023, in CAPS, the indication for therapeutic plasma exchange (TPE) is category I, grade 2C. CASE REPORT: We present a case of probable CAPS secondary to systemic lupus erythematosus (SLE) in an elderly patient in whom clinical and biological improvement was achieved through a multidisciplinary approach and prompt initiation of TPE. Because TPE is considered first-line therapy in CAPS, we initiated the procedure as soon as the attending rheumatologist raised this suspicion. Four plasmapheresis sessions were performed in the Intensive Care Unit. We used TPE by membrane filtration. Following the therapeutic intervention with TPE, corticotherapy (Solumedrol in puls-therapy), cyclophosphamide and anticoagulant treatment, the evolution was favourable, with clinical and biological improvement. CONCLUSION: The prompt initiation of TPE, because of the suspicion of CAPS, increases the chances of a favourable evolution.

Severe parapharyngeal abscess that developed significant complications: management during the COVID-19 pandemic.

A 50-year-old Caucasian man presented to the emergency department during the early stages of the COVID-19 pandemic with a rapidly progressive facial swelling, fever, malaise and myalgia. The patient had recently travelled to a COVID-19-prevalent European country and was therefore treated as COVID-19 suspect. The day before, the patient sustained a burn to his left forearm after falling unconscious next to a radiator. A CT neck and thorax showed a parapharyngeal abscess, which was surgically drained, and the patient was discharged following an intensive care admission. He then developed mediastinitis 3 weeks post-discharge which required readmission and transfer to a cardiothoracic unit for surgical drainage. This report discusses the evolution of a deep neck space infection into a mediastinitis, a rare and life-threatening complication, despite early surgical drainage. This report also highlights the difficulties faced with managing patients during the COVID-19 pandemic.

Recomendaciones de consenso SEDAR-SEMICYUC sobre el manejo de las alteraciones de la hemostasia en los pacientes graves con infección por COVID-19 / SEDAR-SEMICYUC consensus recommendations on the management of haemostasis disorders in severely ill patients with COVID-19 infection

La infección por el coronavirus SARS-CoV-2, causante de la enfermedad denominada COVID-19, provoca alteraciones fundamentalmente en el sistema respiratorio. En los pacientes graves, con frecuencia la enfermedad evoluciona a un síndrome de distrés respiratorio agudo que puede predisponer a los pacientes a un estado de hipercoagulabilidad, con trombosis tanto a nivel venoso como arterial. Esta predisposición presenta una fisiopatología multifactorial, relacionada tanto con la hipoxia como con el grave proceso inflamatorio ligado a esta patología, además de los factores trombóticos adicionales presentes en muchos de los pacientes.Ante la necesidad de optimizar el manejo de la hipercoagulabilidad, los grupos de trabajo de las sociedades científicas de Anestesiología-Reanimación y Terapéutica del Dolor (SEDAR) y de Medicina Intensiva, Crítica y de Unidades Coronarias (SEMICYUC) han desarrollado un consenso para establecer unas pautas de actuación frente a las alteraciones de la hemostasia observadas en los pacientes graves COVID-19. Estas recomendaciones incluyen la profilaxis de la enfermedad tromboembólica venosa en pacientes graves y en el periparto, el manejo de los pacientes en tratamiento crónico con fármacos antiagregantes o anticoagulantes, de las complicaciones hemorrágicas en la evolución de la enfermedad y de la interpretación de las alteraciones generales de la hemostasia

The infection by the coronavirus SARS-CoV-2, which causes the disease called COVID-19, mainly causes alterations in the respiratory system. In severely ill patients, the disease often evolves into an acute respiratory distress syndrome that can predispose patients to a state of hypercoagulability, with thrombosis at both venous and arterial levels. This predisposition presents a multifactorial physiopathology, related to hypoxia as well as to the severe inflammatory process linked to this pathology, including the additional thrombotic factors present in many of the patients. In view of the need to optimise the management of hypercoagulability, the working groups of the Scientific Societies of Anaesthesiology-Resuscitation and Pain Therapy (SEDAR) and of Intensive, Critical Care Medicine and Coronary Units (SEMICYUC) have developed a consensus to establish guidelines for actions to be taken against alterations in haemostasis observed in severely ill patients with COVID-19. These recommendations include prophylaxis of venous thromboembolic disease in these patients, and in the peripartum, management of patients on long-term antiplatelet or anticoagulant treatment, bleeding complications in the course of the disease, and the interpretation of general alterations in haemostasis

Infarct Volume Before Hemicraniectomy in Large Middle Cerebral Artery Infarcts Poorly Predicts Catastrophic Outcome.

BACKGROUND AND PURPOSE: Infarct volumes predict malignant infarcts in patients undergoing decompressive hemicraniectomy (DH) for large middle cerebral artery territory infarcts. The aim of the study was to determine the optimal magnetic resonance imaging infarct volume threshold that predicts a catastrophic outcome at 1 year (modified Rankin Scale score of 5 or death). METHODS: We included consecutive patients who underwent DH for large middle cerebral artery infarcts. We analyzed infarct volumes before DH with semi-automated methods on b1000 diffusion-weighted imaging sequences and apparent diffusion coefficient maps. We studied infarct volume thresholds for prediction of catastrophic outcomes, and analyzed sensitivity, specificity, and the area under the curve, a value ≥0.70 indicating an acceptable prediction. RESULTS: Of 173 patients (109 men, 63%; median age 53 years), 42 (24.3%) had catastrophic outcomes. Magnetic resonance imaging b1000 diffusion-weighted imaging and apparent diffusion coefficient infarct volumes were associated to the occurrence of 1-year catastrophic outcome (adjusted odds ratio, 9.17 [95% CI, 2.00-42.04] and odds ratio, 4.18 [95% CI, 1.33-13.19], respectively, per 1 log increase). The optimal volume cutoff of were 211 mL on b1000 diffusion-weighted imaging and 181 mL on apparent diffusion coefficient maps. The 2 methods showed similar sensitivities and specificities and overlapping area under the curve of 0.64 (95% CI, 0.54-0.74). CONCLUSIONS: In patients with large middle cerebral artery infarcts, optimal magnetic resonance imaging infarct volume thresholds showed poor accuracy and low specificity to predict 1-year catastrophic outcome, with different b1000 diffusion-weighted imaging and apparent diffusion coefficient thresholds. In the setting of DH, optimal infarct volumes alone should not be used to deny DH, irrespectively of the method used.

Factors associated with catastrophic total costs due to tuberculosis under a designated hospital service model: a cross-sectional study in China.

BACKGROUND: Certain districts and counties in China designated local general hospital as the designated hospital for tuberculosis (TB) management after the promulgation of the Law of Practicing Physicians in 2009. To our knowledge, there is limited research on catastrophic payments of TB patients under this service model, often with inconsistent conclusions. In addition, there has been no published studies from China using the updated 2018 World Health Organization (WHO) definition of catastrophic total costs due to TB. This study used the latest criterion recommended by the WHO to analyze the incidence of catastrophic total costs for households affected by TB under the designated hospital model and explore its influencing factors. METHODS: A cross-sectional analysis was carried out in all ten designated hospitals in Ningbo, China. Eligible pulmonary TB cases confirmed by sputum culture of Mycobacterium tuberculosis were recruited and surveyed from September 2018 to October 2018. We evaluated catastrophic total costs using total costs for TB treatment exceeding 20% of the household's annual pre-TB income. A sensitivity analysis was performed while varying the thresholds. The least absolute shrinkage and selection operator (LASSO) regression were applied to select variables, and multiple logistic regression analysis were used to identify the determinants of catastrophic total costs. RESULTS: A total of 672 patients were included, with a median age of 41 years old. The rate of catastrophic total costs of surveyed households was 37.1%, and that of households affected by MDR was 69.6%. Medical cost accounted for more than 60% of the total cost. 57.7% cases were hospitalized. The hospitalization rates of patients with no comorbidities, no severe adverse drug reactions, and rifampin-sensitive TB were 53.9, 54.9, and 55.3%, respectively. Patients in the poorest households had the highest hospitalization rates (Q1:54.8%, Q2:61.4%, Q3:52.2%, Q4:49.5%, Q5:69.7%, P = 0.011) and the highest incidence of severe adverse drug reactions (Q1:29.6%, Q2:19.6%, Q3:28.0%, Q4:33.7%, Q5:35.3%, P = 0.034). Factors such as elderly, minimum living security, unemployed before or after illness, poor economic status, seeking medical care outside the city, hospitalization, absence of local basic medical insurance coverage and MDR were positively associated with catastrophic costs. CONCLUSION: Substantial proportions of patients and households affected by pulmonary TB faced catastrophic economic risks in Ningbo, China. The existing policies that focus on expanding the coverage of basic medical insurance and economic protection measures (such as cash transfers to compensate low-income households for direct non-medical costs and income loss) might be insufficient. Tailored program that mitigate inappropriate healthcare and address equity of care delivery are worthy of attention.

Study of the mental health status of medical personnel dealing with new coronavirus pneumonia.

This paper studied the relationship between personality traits and mental health conditions of medical personnel to provide a basis and reference for the implementation of targeted education on mental health. A self-report inventory, the Symptom Checklist-90 (SCL-90), was used to investigate the mental health status of 548 medical personnel dealing with the new coronavirus pneumonia in eight provinces and cities of China. The overall mean SCL-90 score and mean values of factors (somatization, obsessive-compulsive, anxiety, phobic anxiety, and psychoticism) of the medical personnel were significantly higher than in the norm group (p < 0.05), while their average interpersonal sensitivity score was significantly lower (p < 0.01). In addition, personal factors affecting the mental health status of medical personnel were identified (all p < 0.05). The overall mental health status of medical personnel responding to new coronavirus pneumonia is generally higher than that of the norm group in China. The results of this study should contribute to measures to alleviate the psychological pressures on medical personnel dealing with the new coronavirus epidemic in China.

Utilization of Predictive Modeling to Determine Episode of Care Costs and to Accurately Identify Catastrophic Cost Nonwarranty Outlier Patients in Adult Spinal Deformity Surgery: A Step Toward Bundled Payments and Risk Sharing.

STUDY DESIGN: Retrospective review of prospectively-collected, multicenter adult spinal deformity (ASD) database. OBJECTIVE: The aim of this study was to evaluate the rate of patients who accrue catastrophic cost (CC) with ASD surgery utilizing direct, actual costs, and determine the feasibility of predicting these outliers. SUMMARY OF BACKGROUND DATA: Cost outliers or surgeries resulting in CC are a major concern for ASD surgery as some question the sustainability of these surgical treatments. METHODS: Generalized linear regression models were used to explain the determinants of direct costs. Regression tree and random forest models were used to predict which patients would have CC (>$100,000). RESULTS: A total of 210 ASD patients were included (mean age of 59.3 years, 83% women). The mean index episode of care direct cost was $70,766 (SD = $24,422). By 90 days and 2 years following surgery, mean direct costs increased to $74,073 and $77,765, respectively. Within 90 days of the index surgery, 11 (5.2%) patients underwent 13 revisions procedures, and by 2 years, 26 (12.4%) patients had undergone 36 revision procedures. The CC threshold at the index surgery and 90-day and 2-year follow-up time points was exceeded by 11.9%, 14.8%, and 19.1% of patients, respectively. Top predictors of cost included number of levels fused, surgeon, surgical approach, interbody fusion (IBF), and length of hospital stay (LOS). At 90 days and 2 years, a total of 80.6% and 64.0% of variance in direct cost, respectively, was explained in the generalized linear regression models. Predictors of CC were number of fused levels, surgical approach, surgeon, IBF, and LOS. CONCLUSION: The present study demonstrates that direct cost in ASD surgery can be accurately predicted. Collectively, these findings may not only prove useful for bundled care initiatives, but also may provide insight into means to reduce and better predict cost of ASD surgery outside of bundled payment plans. LEVEL OF EVIDENCE: 3.

Efectividad del consejo médico para dejar de fumar en pacientes con orbitopatía de Graves / Effectiveness of oral counselling for smoke cessation in Graves orbitopathy patients

Objetivo: El tabaco es un factor de riesgo en la orbitopatía de Graves (GO) y es modificable. El consejo médico para dejar de fumar se ha incluido en todas las guías de práctica clínica de esta enfermedad, aunque su efectividad no ha sido evaluada. El objetivo de este estudio es conocer el cambio producido en el hábito tabáquico en pacientes con GO tras una recomendación verbal para dejar de fumar. Material y métodos: Se estudió una cohorte retrospectiva de pacientes con GO. Todos los pacientes recibieron consejo médico sobre la importancia de dejar de fumar en la primera consulta del oftalmólogo. Entre 2013 y 2014 se atendió a 33 pacientes en la consulta, que fueron preguntados sobre el cambio en su hábito tabáquico en 2015. La variable principal de estudio fue el número de cigarros fumados al día antes y después de las consultas en endocrinología y oftalmología. Aparte, se recogieron otros datos personales médicos y socioeconómicos. Resultados: El número medio de cigarros fumados fue de 13,6 (DE 9,66) y 6,3 (DE 7,73) antes y después de la primera consulta en oftalmología, respectivamente (test t pareado, p < 0,05). El 42,42% de los pacientes dejó de fumar y el 30,3% disminuyó el consumo de cigarrillos. Los pacientes que consiguieron dejar de fumar presentaban con mayor frecuencia formas de enfermedad de Graves activas y graves, tenían trabajos estables y estaban apoyados por sus familiares y amigos. Conclusión: Un consejo fuerte y firme recomendando dejar de fumar es efectivo en pacientes con GO. Esta enfermedad afecta seriamente la calidad de vida de los pacientes, lo que hace que sean más susceptibles de cambiar a la hora de modificar sus hábitos

Objective: Smoking is an important risk factor for Graves orbitopathy (GO) and it is modifiable. The advice to stop smoking has been included in all the clinical practice guidelines of GO. However, the effectiveness of this practice remains unknown. The purpose of this study is to assess the change in the smoking habit in patients affected with GO after an oral counselling for smoking cessation. Material and methods: A retrospective cohort of GO patients was studied. The patients received a significant oral counsel during the first consultation with the ophthalmologist. 33 GO patients were explored in the ophthalmology clinic during 2013 and 2014 and the study was done throughout a telephone questionnaire in 2015. The main outcome was the number of cigarettes smoked daily before and after consultation with the endocrinologist and the ophthalmologist. Other medical and socioeconomic factors were recorded. Results: The mean number of cigarettes that were smoked was 13.6 (SD 9.66) and 6.3 (SD 7.73) before and after the consultation done at the ophthalmology office (T-test paired, P = 0.0006). 42.42% achieved smoking cessation and 30.3% decreased their smoking habit. Patients who stopped smoking suffered usually from active and severe GO, had more stable jobs and received greater support from their relatives and friends. Conclusion: A firm and strong oral counsel held for smoke cessation was effective in GO patients. This disease deeply affects patients’ quality of life, making them more prone to change their habits

Measuring Burden of Care After Catastrophic Illness or Injury.

Burden of care (BoC) is the amount of time a patient requires direct, daily assistance from another person to meet basic needs in the home; it is based on a patient's functional level, obtained using the Functional Independence Measure. Inpatient BoC is a patient's projected resource utilization during a stay at an inpatient facility, assessed using the Northwick Park Dependency Scale. At the outpatient level, function and BoC can be assessed using the LIFEware System. Measuring and monitoring outcomes of all care result in reduced health care expenditures, more streamlined patient care, and improved quality of life for patients and families.

Value-Based Pricing for Emerging Gene Therapies: The Economic Case for a Higher Cost-Effectiveness Threshold.

While one-time gene replacement therapies may offer transformative innovation for the management of ultrarare, health-catastrophic diseases, they also pose challenges to the current U.S. health care system. Historically, the United States and other countries have demonstrated a willingness to support higher prices for health gains in rare diseases. However, payers may be ill-prepared to address reimbursement based on single administrations associated with gene therapies. As yet, there is no consensus on how to appropriately reward gene therapy innovation. The purpose of this article is to characterize challenges for traditional approaches to assessing the value of one-time gene replacement therapies and to provide a health economic rationale for a higher value-based cost-effectiveness threshold (CET). There is a general recognition that ultrarare, health-catastrophic conditions should be judged against a higher CET. The Institute for Clinical and Economic Review in the United States has discussed a range of up to $500K per quality-adjusted life-year (QALY) gained for ultrarare diseases, and the National Institute for Health and Care Excellence in the United Kingdom has described a variable threshold up to £300,000 per QALY depending on the magnitude of the health gains. In practice, health technology assessment decision makers often make comparisons to "benchmarks" to justify both standard and extraordinary CETs. We briefly review and present a list of relevant benchmarks. We also sketch out how a broader concept of value could provide the basis for higher CETs for some ultrarare diseases. This approach is outlined by the recent International Society for Pharmacoeconomics and Outcomes Research Special Task Force on Value Assessment Frameworks. In addition to the QALY gains, other elements of value related to uncertainty may also be important. They include insurance value, severity of disease, real option value, value of hope, and equity. A gene therapy currently in development for the treatment of spinal muscular atrophy (SMA) provides an exemplar for discussing the issues that accompany one-time gene replacement therapies. It is imperative that we find a consensus on how to appropriately reward value created by these gene therapies to incentivize appropriate risk taking and investments by their developers-a higher CET would, by economic logic, support a higher value-based price. If consensus on appropriate rewards cannot be found for safe and effective gene therapies for diseases such as SMA with clear criticality and unmet need, it will be even more difficult to do so for diseases where the value provided is less apparent. DISCLOSURES: Funding for the writing of this article was provided by AveXis Pharmaceuticals, which reviewed the manuscript and contributed feedback during manuscript development. The authors had final editorial control. Jackson and Paul are employees of MME, a biopharmaceutical consulting firm that received funding from AveXis for work on this project. Jackson and Paul also report consulting fees from numerous other biopharmaceutical companies outside of this project. Garrison reports consulting fees from AveXis for work on this project and advisory/consultancy fees from BioMarin, Roche, Novartis, and Pfizer unrelated to this project. Kenston is a former employee of AveXis and reports consulting fees from AveXis for this project and for other projects outside of this work.

Emergency Action Planning in Secondary School Athletics: A Comprehensive Evaluation of Current Adoption of Best Practice Standards.

CONTEXT: Emergency action plans (EAPs) are policies that improve response times and ensure access to emergency equipment for the management of patients with acute injuries and medical conditions, yet the extent to which EAP standards are adopted and implemented is unknown. OBJECTIVE: To describe the extent of EAP adoption and implementation in secondary school (SS) athletics with athletic trainer (AT) services in the United States. DESIGN: Cross-sectional study. SETTING: Web-based questionnaire. PATIENTS OR OTHER PARTICIPANTS: A national sample of ATs (n = 9642) was invited to participate in a Web-based questionnaire. MAIN OUTCOME MEASURE(S): Twelve components of EAP minimum best practices were derived from the "National Athletic Trainers' Association (NATA) Position Statement: Emergency Planning in Athletics." Emergency action plan components were analyzed using descriptive statistics with 95% confidence intervals (CIs) around proportions. Contingency tables (2 × 2) were used to calculate odds ratios (with 95% CIs) to assess adoption of the components (dichotomized as yes or no), employment factors (eg, full time versus part time, employed by clinic/district), and access to emergency equipment. RESULTS: The response rate for the questionnaire was 13.2% (n = 1273). A majority of ATs (89.1%) reported having an EAP; however, only 9.9% described implementing all 12 components cited in the NATA position statement. Athletic trainers stated that they created the EAP in 62.8% (95% CI = 60.1%, 65.4%) of schools with an EAP. Athletic trainers employed full time were at greater odds of adopting 9 or more components of the EAP compared with ATs employed part time (odds ratio = 2.42 [95% CI = 1.66, 3.53]). A total of 85.7% of ATs noted access to an automated external defibrillator. CONCLUSIONS: Although a majority of SSs had EAPs, the EAPs were often incomplete and lacked the necessary components for full compliance with the NATA position statement. These findings demonstrate the need for efforts to promote the adoption and implementation of comprehensive EAPs in SS athletics.

Catastrophic health expenditure among type 2 diabetes mellitus patients: A province-wide study in Shandong, China.

AIMS/INTRODUCTION: Diabetes mellitus often causes high economic burden on the patients and their households. The present study aimed to assess the incidence and intensity of catastrophic health expenditure (CHE) relating to type 2 diabetes mellitus care, and to explore its determinants in China. MATERIALS AND METHODS: A total of 1,948 type 2 diabetes patients were included in the analysis. CHE for type 2 diabetes mellitus was defined as out-of-pocket payments for diabetes care that were ≥40% of the non-food expenditure of a household. The Chi-square-test was used to identify the factors associated with CHE. Multivariate logistic regression was used to assess the effects of explanatory analysis variables. RESULTS: The incidence of CHE for type 2 diabetes mellitus care was 13.8%. An association was observed between CHE incidence and household income level, and the poorest group was more likely to experience CHE as a result of diabetes mellitus care. The type 2 diabetes mellitus patients with complications were found to be more likely to experience CHE. Diabetes patients who experienced outpatient or inpatient services increased the likelihood of CHE, and those who experienced inpatient services were more likely to incur CHE. CONCLUSIONS: Type 2 diabetes mellitus has a significantly catastrophic effect on patients and their households in China. Early screening for type 2 diabetes mellitus patients among the high-risk groups and effective management of the detected cases should be priorities to reduce the overall healthcare expenditure for type 2 diabetes mellitus.

A comparative study of catastrophic health expenditure in Zhejiang and Qinghai province, China.

BACKGROUND: China has made great achievements in health insurance coverage and healthcare financing; however, the rate of catastrophic health expenditure (CHE) was 13.0% in China in 2008, which is higher than that in some other countries. There remain some differences in life-style, national customs, medical conditions, and health consciousness in different provinces in China. This study aimed to compare the rates of households with CHE, further to explore the different performance of factors influencing CHE between Zhejiang and Qinghai province, China. METHODS: Data were derived from the household surveys conducted in Zhejiang and Qinghai. Sampling on multi-stage stratified cluster random method was adopted. Household with CHE occurs when the out-of-pocket payment for health care equals to or exceeds 40% of a household's income. Univariate and multivariate logistic regression analyses were used to identify the performance of factors of CHE. RESULTS: A total of 1598 households were included in this study, including 995 in Zhejiang and 603 in Qinghai. The average rates of CHE in Zhejiang and Qinghai were 9.6 and 30.5%, respectively. We found that economic status of households and households headed by an employed person are the protective factors for CHE; and number of members with chronic diseases and number of inpatients in household are the risk factors for CHE in the two provinces. Besides, poor/low-insured households in Zhejiang; and households having outpatients and households headed by a minority person in Qinghai are more likely to experience the risk of CHE. CONCLUSIONS: This study highlights the importance of improving economic development, expanding employment, and adjusting policies to make greater efforts to protect chronic diseases patients, outpatients, and inpatients, further to reduce the risk of CHE. The Chinese government should pay more attention to the actual conditions in different provinces, further to make policy decisions according to the local knowledge.

The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement.

BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome (APS) involving many organs and leading to their insufficiency. The pathogenesis of CAPS is associated with the presence of antiphospholipid antibodies (aPL). Typical therapy includes anticoagulation, glucocorticoids, therapeutic plasma exchanges and/or intravenous immunoglobulin. Despite this aggressive treatment, the mortality rate of 37% is still high. Novel therapeutic agents are required. Rituximab (RTX) is the most studied drug in APS also used in CAPS. Because of the rarity of CAPS occurrence it is impossible to plan a controlled, randomized study exploring its efficacy in CAPS. Therefore, case reports of its usage can be a source of our knowledge in this matter. CASE PRESENTATION: A 35-year-old woman who displayed dyspnoea and peripheral edema was admitted to the Nephrology Clinic because of rapidly progressive renal insufficiency. Her history included autoimmune hemolytic anemia anemia, two miscarriages and the diagnosis of APS with the treatment of heparin and acetylosalicylic acid during her next pregnancy. In spite of this treatment, she gave birth to a dead fetus in 22 Hbd. She then developed CAPS with involvement of the kidneys, brain, skin, peripheral veins and central retinal artery. Lupus anticoagulant and ß2-glicoprotein-I antibodies were positive. Immediately upon admission to the nephrology clinic, she received anticoagulation and corticosteroids along with therapeutic plasma exchanges. As a supportive treatment hemodialysis sessions were necessary. Under this treatment the amelioration of the general state was observed but renal failure persisted, therefore intravenous immunoglobulin was added. Afterwards, the kidney function recovered and the renal replacement therapy could be stopped. After this therapy, aPL became negative. Four weeks later lupus anticoagulant began to increase. Taking into account the risk factors of the relapse and the life-threatening course of the disease, rituximab was introduced. After administration of 2 g of RTX in three separate doses, we observed no new thrombotic events, the further amelioration of renal function and the negative profile of aPL. CONCLUSIONS: CAPS is a life-threatening condition and a prompt, complex treatment is necessary. Rituximab together with conventional therapy can be an additional option in case of the risk of relapse.

Disease-specific out-of-pocket and catastrophic health expenditure on hospitalization in India: Do Indian households face distress health financing?

BACKGROUND: Rising non-communicable diseases (NCDs) coupled with increasing injuries have resulted in a significant increase in health spending in India. While out-of-pocket expenditure remains the major source of health care financing in India (two-thirds of the total health spending), the financial burden varies enormously across diseases and by the economic well-being of the households. Though prior studies have examined the variation in disease pattern, little is known about the financial risk to the families by type of diseases in India. In this context, the present study examines disease-specific out-of-pocket expenditure (OOPE), catastrophic health expenditure (CHE) and distress health financing. METHODS AND MATERIALS: Unit data from the 71st round of the National Sample Survey Organization (2014) was used for this study. OOPE is defined as health spending on hospitalization net of reimbursement, and CHE is defined as household health spending exceeding 10% of household consumption expenditure. Distress health financing is defined as a situation when a household has to borrow money or sell their property/assets or when it gets contributions from friends/relatives to meet its health care expenses. OOPE was estimated for 16 selected diseases and across three broad categories- communicable diseases, NCDs and injuries. Multivariate logistic regression was used to understand the determinants of distress financing and CHE. RESULTS: Mean OOPE on hospitalization was INR 19,210 and was the highest for cancer (INR 57,232) followed by heart diseases (INR 40,947). About 28% of the households incurred CHE and faced distress financing. Among all the diseases, cancer caused the highest CHE (79%) and distress financing (43%). More than one-third of the inpatients reported distressed financing for heart diseases, neurological disorders, genito urinary problems, musculoskeletal diseases, gastro-intestinal problems and injuries. The likelihood of incurring distress financing was 3.2 times higher for those hospitalized for cancer (OR 3.23; 95% CI: 2.62-3.99) and 2.6 times for tuberculosis patients (OR 2.61; 95% CI: 2.06-3.31). A large proportion of households who had reported distress financing also incurred CHE. RECOMMENDATIONS: Free treatment for cancer and heart diseases is recommended for the vulnerable sections of the society. Risk-pooling and social security mechanisms based on contributions from both households as well as the central and state governments can reduce the financial burden of diseases and avert households from distress health financing.

Porous safety net: catastrophic health expenditure and its determinants among insured households in Togo.

BACKGROUND: In Togo, about half of health care costs are paid at the point of service, which reduces access to health care and exposes households to catastrophic health expenditure (CHE). To address this situation, the Togolese government introduced a National Health Insurance Scheme (NHIS) in 2011. This insurance currently covers only employees and retirees of the State as well as their dependents, although plans for extension exist. This study is the first attempt to examine the extent to which Togo's NHIS protects its members financially against the consequences of ill-health. METHODS: Data was obtained from a cross-sectional representative households' survey involving 1180 insured households that had reported illness in the household in the 4 weeks preceding the survey or hospitalization in the 12 months preceding the survey. The incidence and intensity of CHE were measured by the catastrophic health payment method. A logistic regression was used to analyse determinants of CHE. RESULTS: The results indicate that the proportion of insured households with CHE varies widely between 3.94% and 75.60%, depending on the method and the threshold used. At the 40% threshold, health care cost represents 60.95% of insured households' total monthly non-food expenditure. This study showed that the socioeconomic status, the type of health facility used, hospitalization and household size were the highest predictors of CHE. Whatever the chosen threshold, care in referral and district hospitals significantly increases the likelihood of CHE. In addition, the proportion of households facing CHE is higher in the lowest income groups. The behaviour of health care providers, poor quality of care and long waiting time were the main factors leading to CHE. CONCLUSION: A sizable proportion of insured households face CHE, suggesting gaps in the coverage. To limit the impoverishment of insured households with low income, policies for free or heavily subsidized hospital services should be considered. The results call for an equitable health insurance scheme, which is affordable for all insured households.