Introduction: Von Willebrand Disease (VWD) is the second most common inherited bleeding disorder. There is paucity of the literature describing the prevalence and clinical characteristics of VWD in this part of the world. The aim of the current study is to detect the prevalence, describe the spectrum of the different types of VWD, their mode of presentation, bleeding phenotype and outcome in Oman. Methods: A retrospective cross-sectional study was carried out in the 2 available referral tertiary care facilities in Oman namely; Sultan Qaboos University and the Royal Hospitals. The study included all children and adults diagnosed with VWD in Oman until June 2019. The patients were subtyped as per the International Society of Thrombosis and Haemostasis (ISTH) criteria. Data was collected from the electronic hospital systems in both hospitals. Out of 700 entries of VWD in both hospitals, only 140 were true cases and 560 were tested negative but wrongly labelled. Patients or their next of ken were called and interviewed to obtain the necessary information that was not documented in the electronic system. Results: A total of 140 patients are confirmed to have VWD giving a prevalence of 1:20000. Fifty eight patients are males (41.5%), 82 patients are females (58.5%). Sixty six patients have type I (47%), 38 patients have type II (27%) and 36 patients have type III (26%). The majority of patients 90 (64%) were diagnosed before the age of 20 years and 62 of them (68%) had positive family history of the disease. The most common presentation was recurrent unexplained bruising. As expected, patients with type III tend to have a significant bleeding phenotype with a bleeding score more than 5 in adults and 3 for paediatric patients. All of them were admitted to hospital at some point electively (for surgery) or for bleeding control, however, they were not put on prophylaxis. None of the patients had serious or intra-cranial bleeding. Conclusion: Von Willebrand Disease is not uncommon in Oman with an overall prevalence of 1:20000, however, it is much less than what was originally reported in previous studies in developing countries and the WFH website. The majority of patients are type 1 and have a positive family history of the disease. The disease is more common in females. All patients with type III have abnormal bleeding score and required VW factor replacement at one point. None of the patients had a serious bleed and they are not on prophylaxis. Keywords: Von Willebrand disease, Prevalence, Oman. Disclosures Al-Khabori: AstraZeneca: Honoraria; Amgen: Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees; SOBI: Honoraria; NovoNardisk: Membership on an entity's Board of Directors or advisory committees; Shire (Takeda): Membership on an entity's Board of Directors or advisory committees; Servier: Membership on an entity's Board of Directors or advisory committees; Roche: Membership on an entity's Board of Directors or advisory committees; Abbvie: Membership on an entity's Board of Directors or advisory committees.
Introduction There is a paucity of data on the outcomes of coronavirus disease 2019 (COVID‐19) in patients with sickle cell disease (SCD) in the United States. We examined the outcomes of patients with COVID‐19 and SCD. Methods We utilized the National Inpatient Sample (NIS) to identify the data of patients diagnosed with COVID‐19 and SCD in 2020 using the International Classification of Disease, Tenth Revision codes. In‐hospital outcomes (invasive mechanical ventilation and mortality) were compared between SCD and non‐SCD groups. Results Of the 1 057 550 COVID‐19 hospitalizations, 2870 (0.3%) had SCD. The median age of the SCD group was 42 (IQR: 31) vs. 66 (IQR: 23) in the non‐SCD group (p < .0001). Patients with SCD were likely to be females (62.02% vs. 37.98%, p < .0001), Blacks (87.81% vs. 12.19%, p < .0001), and in the lowest income quartile (50.62% vs. 11.15%, p < .0001). There was no difference in the outcomes between the two groups. There were increased odds of invasive mechanical ventilation and in‐hospital mortality in COVID‐19 in Asians, Hispanics, Native Americans, and Blacks (except for in‐hospital mortality) compared to Whites. Conclusion In‐hospital mortality and invasive mechanical ventilation outcomes in SCD are comparable to that in non‐SCD patients hospitalized with COVID‐19.
Renal cell carcinoma is the most common renal neoplasm. Its presentation is often very occult, and it may be discovered incidentally. It may present with the classic symptoms of back pain, flank pain, hematuria, or hypertension. Renal cell carcinoma may also present with malignant pleural effusion at diagnosis; however, it is very rare. In this case report and literature review, we describe a 77-year-old male who was diagnosed with renal cell carcinoma after presenting with a malignant pleural effusion -an extremely rare phenomenon. An analysis of the literature yielded 13 case reports, including ours, where the diagnostic presentation of renal cell carcinoma was a malignant pleural effusion.Our patient presented with left-sided chest pain. Imaging was suggestive of pleural effusion. CT and MRI imaging demonstrated masses in the upper and lower poles of the right kidney suggestive of renal cell carcinoma. CT imaging also showed lung nodules that were suggestive of pulmonary metastases. Biopsy and immunostaining of pleural tissue were positive for clear cell renal cell carcinoma. Therapeutic thoracentesis was performed. Despite this, the patient developed recurrent large-volume pleural effusions requiring drainage and placement of a pleural catheter. Our patient's extremely rare presentation of malignant pleural effusion as the diagnostic presentation of renal cell carcinoma along with recurrent, large-volume effusions requiring drainage has only been reported in the form of case reports in the literature.
Disseminated intravascular coagulopathy (DIC) is infrequently associated with COVID-19 infection. COVID-19 infection can predispose to thrombotic events through inflammation and microvascular injury. DIC is rarely associated with coronary artery disease, especially myocardial infarction (MI). In this case report, we present an uncommon case of a patient with concurrent DIC and MI in the setting of COVID-19 infection. A 73-year-old male patient with no known cardiovascular risk factor presented with syncope. Assessment in the field by emergency medical service (EMS) showed the patient had a third-degree atrioventricular block and a heart rate of 40 beats per minute. He was given atropine and transcutaneously paced. Upon admission, he was found to have an inferior wall ST-elevation myocardial infarction (STEMI) and tested positive for COVID-19. Cardiac catheterization was performed urgently and revealed triple vessel disease. Attempts to revascularize the vessels were unsuccessful. He subsequently developed cardiogenic shock. He was started on multiple pressor support. Laboratory workup was suggestive of DIC, and he later developed multiorgan failure. Continuous renal replacement therapy was initiated but failed due to persistent thrombosis of the dialysis access. Despite all measures, the patient developed cardiac arrest and passed away on the third day of hospitalization. Our understanding of COVID-19 and its complications has grown exponentially since the beginning of the pandemic. The pro-inflammatory state induced by the disease creates a hypercoagulable state that may result in thrombotic complications, including MI. In severe cases, a consumptive coagulopathy may develop, leading to DIC. This unique case report seeks to highlight the importance of staying vigilant about the potential complications of MI and DIC induced by COVID-19 so that prompt diagnosis can be made to reduce morbidity and mortality in these patients.
Introduction The outcomes of pulmonary embolism (PE) in sickle cell disease (SCD) are poorly established in the literature. This study examined the prevalence and outcomes of patients with PE and SCD. Methods The National Inpatient Sample was used to identify patients' data with a diagnosis of PE and SCD in the United States from 2016 to 2020 using the International Classification of Disease, 10th Revision codes. Logistic regression was used to compare outcomes between those with and without SCD. Results Of the 405 020 patients with PE, 1504 (0.4%) had SCD, and 403 516 (99.6%) did not have SCD. The prevalence of PE with SCD was stable. Patients in the SCD group were more likely to be female (59.5% vs. 50.6%; p < .0001), Black (91.7% vs. 54.4%; p < .0001), with a lower rate of comorbidities. The SCD group had higher in‐hospital mortality (odds ratio [OR] = 1.41, 95% confidence interval [CI]:1.08–1.84; p = .012) but lower catheter‐directed thrombolysis (OR = 0.23, 95% CI: 0.08–0.64; p = .005), mechanical thrombectomy (OR = 0.59, 95% CI: 0.41–0.64; p < .0029), and inferior vena cava filter placement (OR = 0.47, 95% CI: 0.33–0.66; p < .001). Conclusion In‐hospital mortality remains high in PE with SCD. A proactive approach, including maintaining a high index of suspicion for PE, is needed to reduce in‐hospital mortality.
Bradycardia, renal failure, atrioventricular (AV) nodal disease, shock, and hyperkalemia (BRASH) syndrome is a well-recognized constellation of distinct clinicopathologic entities comprising bradycardia, renal failure, AV nodal disease, shock, and hyperkalemia. Our patient is an 89-year-old female with a past medical history significant for hypertension and diabetes, who was newly started on labetalol and had recent gastroenteritis; she presented to our Emergency Department with bradycardia and shock. Upon presentation, she showed physical signs of volume depletion, and her blood pressure was 50 mmHg systolic and heart rate was 25 beats per minute. The initial electrocardiogram showed an idioventricular rhythm. The laboratory workup revealed hyperkalemia. The patient was given repeated doses of atropine with no significant response. She was resuscitated with isotonic fluids. The patient improved clinically, her blood pressure stabilized, her potassium level, renal function, and heart rate were normalized, and normal sinus rhythm was restored with a narrow QRS complex. A diagnosis of BRASH syndrome was made retrospectively. Overall, the treatment of this syndrome is largely symptomatic. Hemodynamic support with fluid and treatment of hyperkalemia remains the goal of care. The overall prognosis is good if identified early and managed appropriately.
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