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1.
Haemophilia ; 29(Supplement 1):51, 2023.
Article in English | EMBASE | ID: covidwho-2252805

ABSTRACT

Introduction: Acquired hemophilia A (AHA) is a rare autoimmune disease due to anti-factor VIII antibodies. It may be associated with infections and malignancies. The association with Covid vaccine is extremely rare. Immunosuppressive therapy with steroids, cytotoxic agents, is the traditionalmainstay for antibodies eradication. Rituximab standard doses have been used with success. There are few reports on low-dose Rituximab for AHA.We present a case of AHA post Covid-19 vaccination successfully treated with low dose of Rituximab. Method(s): case report Results: A non hemophilic 69-year-old male with no medical history consulted for multiple ecchymosis that spontaneously occurred with no context of trauma. Two months previously he received a second dose of CoronaVac-Sinovac vaccine. Coagulation tests revealed an isolated and prolonged aPTT (100 sec/30s;ratio=3.33) not corrected with normal plasma. The coagulation factors assay revealed an isolated decrease of factor VIII to 1% with a titer of 121 Bethesda units/ml confirming the diagnosis of AHA. Hepatitis B and C and HIV tests were negative. A full body-computed tomography scan was normal. Treatment with Prednisolone 1 mg/kg/d was started with tranexamic acid. Bypassing therapy was not considered because of the absence of life-threatening bleeding. Seventeen days after corticosteroid initiation, a worsening of the ecchymosis was noted with the non-improvement of the aPTT. A low-dose rituximab (100 mg/week) was added for 4 weeks. After 3 doses of Rituximab a complete clinical response was achieved. Factor VIII inhibitor was completely eradicated. Corticosteroid was discontinued. At 3-month follow-up the patient remains in remission without further treatment Discussion/Conclusion:More than 50 cases of AHA following COVID-19 vaccine have been reported. To our knowledge only 2 cases of AHA were successfully treated with low dose of rituximab. Low-dose Rituximab appears to be effective for Factor VIII inhibitor eradication in AHA with a lower cost.

2.
Haemophilia ; 29(Supplement 1):159, 2023.
Article in English | EMBASE | ID: covidwho-2252804

ABSTRACT

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia ascribable to platelet-rich microthrombi. TTP is related to a severe deficiency in ADAMTS13, the specific von Willebrand factor-cleaving metalloprotease. The aim of our study is to analyze the clinical characteristics, treatment and outcome of patients with TTP. Method(s): We retrospectively analyzed 15 patients with TTP treated Farhat Hached hospital, Sousse, Tunisia, from2004 to 2021. Result(s): Among the 15 patients, there were 7 males and 8 females, with a median age of 45,5 (30~72) years. Two of them had evolutive cancer and 1 had covid-19 vaccination 3 months earlier. Twelve patients had neurological presentations (80%), 4 had mucocutaneous bleeding and 2 had fever. Biology showed thrombopenia, mechanical hemolytic anemia in all patients and renal failure in 2 patients. No abnormalities in coagulation tests were detected. The ADAMTS13 activity was performed in only 3 patients (Due to test unavailability) showing a level <=10% in all 3 patients. All patients were treated with plasma exchange/ plasma infusion + glucocorticoid, combined with rituximab in 2 cases. Ten patients showed complete remission, 1 relapsed within the first year, 2 died and 2 others were lost to follow-up. Discussion/Conclusion: Most TTP patients presented with the triad of microangiopathic anemia, thrombocytopenia, and neurologic abnormalities and improved with plasma therapy.

3.
Journal of Applied Accounting Research ; 24(1):47-69, 2023.
Article in English | Scopus | ID: covidwho-2243467

ABSTRACT

Purpose: This study expands the performance management literature by developing a strategy map and balanced scorecard (BSC) for a large performing arts theater (PAT). Design/methodology/approach: First, interviews with significant stakeholders identify key success factors (KSFs). Next, a survey is administered, and a structural model is employed to determine the importance of each KSF and their interdependent causal relationships within the PAT. Each KSF's controllability and room for improvement are also measured to facilitate implementation strategies. Findings: The results reveal that the Financial Perspective plays a critical role in the PAT's success, while significant changes can be enacted by focusing on the Internal Processes Perspective. Regarding the individual KSF, the following emerge as the most critical: excellent reputation, attendance growth, increasing sponsorship and donation, and supporting the local arts community;however, PAT managers will have to be creative to enact change through these KSF as some are difficult to control or have little perceived room for improvement. Research limitations/implications: The data were collected prior to, or at the beginning of the coronavirus disease 2019 (COVID-19) pandemic. Post-pandemic priorities for the organization may have changed. Practical implications: By highlighting the relationships between different KSFs, this study provides PAT managers with a frame of reference for developing their BSC and performance metrics. It also offers PAT's managers a structured and adaptable approach for prioritizing their strategic choices and developing implementation plans for improved outcomes. Originality/value: This study exemplifies the need for applied BSC studies in various sectors, including nonprofit organizations. Specifically, this study extends the performance management literature by providing an example of a large PAT's performance measures, the inter-relationships among KSF and the resulting strategy map. The results are significant because arts management is a unique discipline based upon a specific body of knowledge (Weinstein and Bukovinsky, 2009). © 2022, Emerald Publishing Limited.

4.
Journal of Applied Accounting Research ; 2022.
Article in English | Scopus | ID: covidwho-1784458

ABSTRACT

Purpose: This study expands the performance management literature by developing a strategy map and balanced scorecard (BSC) for a large performing arts theater (PAT). Design/methodology/approach: First, interviews with significant stakeholders identify key success factors (KSFs). Next, a survey is administered, and a structural model is employed to determine the importance of each KSF and their interdependent causal relationships within the PAT. Each KSF's controllability and room for improvement are also measured to facilitate implementation strategies. Findings: The results reveal that the Financial Perspective plays a critical role in the PAT's success, while significant changes can be enacted by focusing on the Internal Processes Perspective. Regarding the individual KSF, the following emerge as the most critical: excellent reputation, attendance growth, increasing sponsorship and donation, and supporting the local arts community;however, PAT managers will have to be creative to enact change through these KSF as some are difficult to control or have little perceived room for improvement. Research limitations/implications: The data were collected prior to, or at the beginning of the coronavirus disease 2019 (COVID-19) pandemic. Post-pandemic priorities for the organization may have changed. Practical implications: By highlighting the relationships between different KSFs, this study provides PAT managers with a frame of reference for developing their BSC and performance metrics. It also offers PAT's managers a structured and adaptable approach for prioritizing their strategic choices and developing implementation plans for improved outcomes. Originality/value: This study exemplifies the need for applied BSC studies in various sectors, including nonprofit organizations. Specifically, this study extends the performance management literature by providing an example of a large PAT's performance measures, the inter-relationships among KSF and the resulting strategy map. The results are significant because arts management is a unique discipline based upon a specific body of knowledge (Weinstein and Bukovinsky, 2009). © 2022, Emerald Publishing Limited.

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