Epidemiological, Demographic, and Clinical Characteristics of Von Willebrand Disease Patients in Zahedan City, Iran: A Descriptive Study

Majid Naderi, Benyamin Rhmati, Hoora Ganjali, Saeedeh Yaghoubi, Mohammad Sadra Harifi-Mood, Seyed Ghader Azizi
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Abstract

Background: Von Willebrand disease (VWD) is one of the most common coagulative diseases, so identifying the effective factors in preventing this complication is essential. The study aimed to evaluate the frequency of demographic and epidemiological findings in VWD patients referred to a hospital in Zahedan, Iran. Materials and Methods: This study was performed on 76 patients with VWD referred to Hazrat Ali-Asghar Hospital in Zahedan City, Sistan, and Baluchestan province. After obtaining consent from the patients, the demographic information and clinical symptoms of the disease were recorded. All statistical analyses were performed using SPSS 22.0 software. All descriptive data were expressed as mean ±SD and percent (%) depending on the continuous and dichotomous variables. A P-value ≤0.05 was considered significant statistically. Results: The present study results showed that the highest age group of VWD patients at the time of disease diagnosis was in the age group 1-5 years (47.3%), and most patients had type III VWD (80.3%). It was also found that 67.1% of patients had a positive family history and their parents' consanguineous marriage (77.6%). The most common complaints were epistaxis (88.15%), cutaneous bleeding (78.94%), and oral cavity bleeding (61.84%), respectively. Conclusion: Due to the high prevalence of VWD in consanguineous marriages and an increase in adverse complications and symptoms in VWD patients, proper diagnosis and screening at an early age, especially in people with family history, is essential. Efforts are needed to develop national registries and widely provide the required and available basic services for diagnosis and treatment.  
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伊朗扎黑丹市冯-威廉布兰德病患者的流行病学、人口统计学和临床特征:描述性研究
背景:冯-威廉氏病(VWD)是最常见的凝血疾病之一,因此确定预防这种并发症的有效因素至关重要。本研究旨在评估转诊至伊朗扎黑丹一家医院的 VWD 患者的人口统计学和流行病学发现的频率。材料和方法:研究对象为转诊至锡斯坦和俾路支斯坦省扎黑丹市 Hazrat Ali-Asghar 医院的 76 名 VWD 患者。在征得患者同意后,对其人口统计学信息和临床症状进行了记录。所有统计分析均使用 SPSS 22.0 软件进行。根据连续变量和二分变量,所有描述性数据均以均数±SD和百分比(%)表示。P值≤0.05被认为具有统计学意义。结果本研究结果显示,1-5 岁是 VWD 患者确诊时的最高年龄组(47.3%),大多数患者为 III 型 VWD(80.3%)。研究还发现,67.1%的患者有阳性家族史,其父母为近亲结婚(77.6%)。最常见的主诉分别是鼻衄(88.15%)、皮肤出血(78.94%)和口腔出血(61.84%)。结论由于 VWD 在近亲结婚中的高发病率以及 VWD 患者不良并发症和症状的增加,因此在早期进行适当的诊断和筛查至关重要,尤其是对有家族史的人群。需要努力建立国家登记册,广泛提供诊断和治疗所需的基本服务。
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