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.
Cardiac tamponade is a rare presentation in breast cancer and may be associated with poor prognosis. In this article, we reviewed the characteristics and survival outcomes of patients with breast cancer who developed cardiac tamponade. Three databases (PubMed, EMBASE and SCOPUS) were searched for relevant articles published from 1978 to 2022 and 16 articles were identified comprising 64 cases. The median age of the cases was 52 years. Cardiac tamponade was diagnosed with echocardiogram or computerized tomography of the chest or both in 91.9%, 1.6% and 6.5% of the cases, respectively. Cytology of the pericardial fluid was done in 90.5% of the cases while biopsy in addition to cytology was done in 9.5% of cases. Tamponade was proven to be malignant in 97.4% of the cases. The initial treatment for tamponade was pericardiocentesis. Adjunct therapies ranged from the insertion of a pericardial window, pericardiectomy, radiotherapy and chemotherapy. The median time from the first treatment of breast cancer to the onset of tamponade was 24 months while the median survival following diagnosis of tamponade was 13 months. There was no significant correlation (spearman rank-sum correlation coefficient = 0.35, p = 0.165) between time to tamponade (interval time from the first diagnosis of breast cancer and the onset of cardiac tamponade) and survival. Cardiac tamponade may adversely affect survival in patients with breast cancer. Early diagnosis with echocardiogram and cytology may guide management and expectations. Further observational studies are needed to determine the predictors of cardiac tamponade and optimal treatment in patients with breast cancer.
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.
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.
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