Mokhtar Galila M, Tantawy Azza A G, Adly Amira A M, Telbany Manal A S, El Arab Sahar Ezz, Ismail Mona
Pediatric Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Blood Coagul Fibrinolysis. 2012 Jul;23(5):411-8. doi: 10.1097/MBC.0b013e3283540bf8.
Keeping an updated registry of bleeding disorders is crucial for planning care and documenting prevalence. We aimed to assess the prevalence of various bleeding disorders including rare inherited coagulation and platelet disorders concerning their clinico-epidemiological, diagnostic data and bleeding manifestations severity. Patients suffering from manifestations of bleeding or coagulation disorders presented to Hematology Clinic during 16 years were included and prospectively followed up. Demographics, clinical characteristics, complete blood count, bleeding, prothrombin and activated partial thromboplastin times, platelet aggregation tests and bone marrow aspiration were recorded. Overall 687 patients with bleeding disorders from total 2949 patients were identified. Inherited coagulation defects were found in 27.2%; hemophilia A (70.6%), hemophilia B (13.9%), factor I deficiency (2.3%), factor V deficiency (1.6%), factor X deficiency (4.2%), factor VII deficiency (2.6%), factor XIII deficiency (1.1%), combined factor deficiency (2.1%) and unclassified coagulation disorders in 1.6% of studied patients. Overall 72.7% had diagnosed with platelet disorders; immune thrombocytopenia was the commonest (74.8%), and inherited conditions represent (25.2%) in the following order: Glanzman's thrombasthenia (11.2%), von Willebrand disease (6.6%), Bernard-Soulier syndrome (1%) and Chediak Higashi in 0.4% and unclassified in 6%. Median age of diagnosis of coagulation and platelet disorders were 33 and 72 months. Presenting symptoms of coagulation disorders were: 25.1% post circumcision bleeding, 22.5% ecchymosis, 20.9% hemoarthrosis and 15% epistaxis. Symptoms of rare coagulation disorders were postcircumcision bleeding (20%), bleeding umbilical stump (20%), epistaxis (12%), hemoarthrosis (8%) and hematomas (4%). Presenting symptoms in rare inherited platelet disorders were purpura, ecchymosis, epistaxis and bleeding gums, respectively. Analysis of the clinico-epidemiological data of patients with bleeding disorders is a useful tool for monitoring and improving their quality of care.
建立最新的出血性疾病登记册对于规划治疗和记录患病率至关重要。我们旨在评估各种出血性疾病的患病率,包括罕见的遗传性凝血和血小板疾病,涉及它们的临床流行病学、诊断数据和出血表现的严重程度。纳入16年间到血液科门诊就诊的有出血或凝血障碍表现的患者,并进行前瞻性随访。记录人口统计学、临床特征、全血细胞计数、出血情况、凝血酶原时间和活化部分凝血活酶时间、血小板聚集试验及骨髓穿刺结果。在总共2949例患者中,共识别出687例出血性疾病患者。遗传性凝血缺陷患者占27.2%;其中血友病A(70.6%)、血友病B(13.9%)、因子I缺乏症(2.3%)、因子V缺乏症(1.6%)、因子X缺乏症(4.2%)、因子VII缺乏症(2.6%)、因子XIII缺乏症(1.1%)、联合因子缺乏症(2.1%),1.6%的研究患者存在未分类的凝血障碍。总体而言,72.7%的患者被诊断为血小板疾病;免疫性血小板减少症最为常见(74.8%),遗传性疾病占(25.2%),依次为:血小板无力症(11.2%)、血管性血友病(6.6%)、伯纳德 - 索利尔综合征(1%)、切 - 东综合征(0.4%),6%未分类。凝血和血小板疾病的诊断中位年龄分别为33个月和72个月。凝血障碍的表现症状为:包皮环切术后出血占25.1%、瘀斑占22.5%、关节积血占20.9%、鼻出血占15%。罕见凝血障碍的症状为包皮环切术后出血(20%)、脐带残端出血(20%)、鼻出血(12%)、关节积血(8%)和血肿(4%)。罕见遗传性血小板疾病的表现症状分别为紫癜、瘀斑、鼻出血和牙龈出血。分析出血性疾病患者的临床流行病学数据是监测和改善其治疗质量的有用工具。