Unravelling Acquired Hemophilia A in an Ambiguous Clinical Picture.

Acquired hemophilia A (AHA) is characterized by the development of neutralizing autoantibodies, called "inhibitors," against intrinsic factor VIII. Its presentation differs profoundly from congenital hemophilia. Here, we present the case of a 69-year-old patient presenting with right-sided flank pain and hematuria, initially diagnosed with acute pyelonephritis, who was found to have bilateral renal hemorrhage during the course of his hospitalization. Later, after a thorough diagnostic evaluation, he was deemed to have AHA.

TACTUM: Trends in Access to Cellular Therapies in Multiple Myeloma, Perspectives of Treating Versus Referring Physicians.

Chimeric antigen receptor T cell therapy (CAR-T) and bispecific T cell engagers (TCE) for multiple myeloma (MM) are readily available at many large US medical centers. However, many potentially eligible patients may not be referred to the specialized centers administering these therapies. Perspectives regarding potential barriers for MM cellular therapy from referring-center oncologists (ROs) versus treating-center oncologists (TOs) have not been reported previously. We conducted TACTUM-23, a survey of US oncologists who treat MM, to identify perceived barriers to these cellular therapies. This 24-question survey, which focused on demographics and perceived barriers to CAR-T and TCE, was conducted between June and August 2023. Of 247 oncologists, 37 (15%) completed the survey including 26 (70%) TOs who prescribed both CAR-T and TCEs, 4 (11%) TOs who only prescribed TCEs, and 7 (19%) ROs who referred patients. The top RO-stated barrier to CAR-T was financial toxicity, while the top TO-stated barrier to CAR-T was leukapheresis/ manufacturing slot availability. The top RO-stated barrier to TCE was financial toxicity, while the top TO-stated barrier to TCE was the hospitalization requirement. In conclusion, financial concerns are perceived by ROs to be the top barrier to both CAR-T and TCEs in myeloma. In contrast, TOs perceive logistical concerns to be the top barrier. Interventions to lower financial toxicity during these therapies, and outreach to raise awareness of such interventions among ROs, are needed alongside strategies to streamline manufacturing (for CAR-T) and monitoring.

COVID-19 in patients with Down syndrome: A systematic review.

INTRODUCTION: Down syndrome (DS) is associated with multiple comorbid conditions and chronic immune dysfunction. Persons with DS who contract COVID-19 are at high risk for complications and have a poor prognosis. We aimed to study the clinical symptoms, laboratory and biochemical profiles, radiologic findings, treatment, and outcomes of patients with DS and COVID-19. METHOD: We systematically searched PubMed, MEDLINE, Web of Science, Scopus, and the Cochrane Library using the keywords COVID-19 or coronavirus or SARS-CoV-2 and DS or trisomy 21. Seventeen articles were identified: eight case reports and nine case series published from December 2019 through March 2022, with a total of 55 cases. RESULTS: Patients averaged 24.8 years (26 days to 60 years); 29 of the patients were male. The most common symptoms were fever, dyspnea, and cough. Gastrointestinal and upper respiratory tract symptoms were commonly reported for pediatric patients. The most common comorbidities present in patients with DS were obesity (49.0%), hypothyroidism (21.6%) and obstructive sleep apnea (15.6%). The patients were hospitalized for a mean of 14.8 days. When the patients were compared with the general COVID-19 population, the mean number of hospitalized days was higher. Most patients had leukopenia, lymphopenia, and elevated inflammatory markers (d-dimer and C-reactive protein). Bilateral infiltrations and bilateral ground-glass opacifications were frequently seen in chest radiographs and chest computed tomographic imaging. Most of the patients were treated with methylprednisolone, macrolides, and hydroxychloroquine. Of the 55 patients, 22 died. The mean age of the patients who died was 42.8 years. Mortality rate was higher in individuals with DS over 40 years of age. CONCLUSION: More studies are needed to better understand COVID-19 infections among persons with DS. In addition, the study was limited by a lack of statistical analyses and a specific comparison group.

The critical pharmaceutical situation in Sudan 2023: A humanitarian catastrophe of civil war.

The situation in Sudan have deteriorated since the ongoing war outbreak in April 2023. This article sheds light on the pharmacological status in Sudan in terms of shortage of supply, rising demands, and regulatory issues. The ongoing civil war has acutely impacted the dilapidated pharmaceutical status of Sudan, patients have suffered from the paucity of medical services forcing an out-of-control rise in underreported morbidity and mortality. To mitigate this uprising issue, an increase in stakeholder communication is crucial to deal with this national threat and establish a system for reporting the shortage.

Amplatzer amulet versus watchman device for percutaneous left atrial appendage closure: A systematic review and meta-analysis.

BACKGROUND: The Amplatzer Amulet (AA) and Watchman devices (WD) are the 2 most frequently used devices for percutaneous LAA closure globally. OBJECTIVE: To evaluate the safety and clinical outcomes associated with these 2 devices in patients undergoing percutaneous LAA closure. METHOD: We systematically searched all electronic databases from inception until February 21, 2023. The primary endpoint was procedure related complications. Secondary endpoints were device related thrombus, stroke, cardiovascular mortality, peri device leak, systemic embolism, and all-cause mortality. RESULTS: A total of 3 randomized clinical trials with 2150 patients were included in this meta-analysis. The mean age was 75 and 76 years in the Amplatzer group and in the Watchman group, respectively. The odds of procedure-related complications (OR, 1.80 [95% CI: 1.21-2.67], P < .001) were significantly higher among patients with AA compared to the WD. However, the odds of all-cause mortality (OR, 0.75 (95% CI: 0.49-1.16), P = .20), stroke (OR, 0.79 [95% CI: 0.47-1.34], P = .39), systemic/pulmonary embolism (OR, 1.34 [95% CI: 0.30-6.04], P = .70), and major bleeding (OR, 1.10 [95% CI: 0.83-1.48], P = .50) were comparable between the two devices. The odds of device related thrombus (OR, 0.72 [95% CI: 0.46-1.14], P = .17) was comparable between both the group of patients, however the incidence of peri device leak was significantly lower in AA group (OR, 0.41 [95% CI: 0.26-0.66], P < .001) compared with WD group of patients. CONCLUSION: The AA was not superior to the Watchman device in terms of safety and efficacy. However, the Amulet occluder was associated with a higher incidence of procedure-related complications, and lower peri device leak.