Abdelrazik Nabil, Rashad Hayam, Selim Tarek, Tharwat Layla
Department of Pediatrics, Mansoura University Children Hospital, Mansoura, Egypt.
Hematology. 2007 Aug;12(4):309-14. doi: 10.1080/10245330701255205.
Disorders of coagulation in children often prove challenging to the medical care team. The aims of this study were to assess the spectrum and prevalence of coagulation disorders among children attending Mansoura University Children Hospital (MUCH), Mansoura, Egypt. A total of 105 pediatric patients were referred to MUCH. They were divided into two groups: congenital coagulation disorders (75 cases, age 45.36 +/- 48.59 months), and acquired coagulation disorders (30 cases, age 56.13 +/- 61.61 months). All patients were subjected to thorough history taking including the nature of bleeding, family, past history, mode of inheritance, and detailed physical findings. Hemostatic tests included: platelet count, bleeding time (BT), prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time (TT). Specific tests in the congenital group include assay of coagulation factors according to each disorder, Von Willebrand factor assay, ristocetin aggregation test, APTT mixing study for detection of inhibitors in complicated hemophilia cases, F VIII C to VWAg ratio with cut off 0.7 for detection of carriers in some hemophilia A families. Congenital disorders constituted 71.4% of the studied cases vs. 28.6% for acquired disorders. Hemophilia A (42.85%), hemophilia B (14.28%) and liver diseases (14.28%) represented the majority of the studied cases. Mild and moderate cases of hemophilia A and B are more frequent than severe cases in both types. Male sex is more frequent than female in the congenital group (94.7 vs. 5.3%, P < 0.001). Direct correlation existed between factor level assay and severity of hemophilia (r = 0.73, P = 0.006). Three mothers and one sister were identified as carrier out of four families. Anti-clotting factors inhibitor was detected in 18.2% of patients with hemophilia A and in 9.1% with hemophilia B. In conclusion, our study found that hemophilias are the most prevalent congenital coagulation disorders among children. Attention must be given for detection of hemophilia carriers and inhibitors of clotting factors.
儿童凝血障碍常常给医疗团队带来挑战。本研究的目的是评估埃及曼苏拉曼苏拉大学儿童医院（MUCH）就诊儿童中凝血障碍的范围和患病率。共有105名儿科患者被转诊至MUCH。他们被分为两组：先天性凝血障碍（75例，年龄45.36 +/- 48.59个月）和获得性凝血障碍（30例，年龄56.13 +/- 61.61个月）。所有患者均接受了全面的病史采集，包括出血的性质、家族史、既往史、遗传方式以及详细的体格检查结果。止血检查包括：血小板计数、出血时间（BT）、凝血酶原时间（PT）、活化部分凝血活酶时间（APTT）、凝血酶时间（TT）。先天性组的特异性检查包括根据每种疾病检测凝血因子、血管性血友病因子检测、瑞斯托霉素聚集试验、用于检测复杂血友病病例中抑制剂的APTT混合试验、F VIII C与VWAg比值（截断值为0.7）以检测某些甲型血友病家族中的携带者。先天性疾病占研究病例的71.4%，获得性疾病占28.6%。甲型血友病（42.85%）、乙型血友病（14.28%）和肝病（14.28%）占研究病例的大多数。甲型和乙型血友病的轻度和中度病例比重度病例更常见。先天性组中男性比女性更常见（94.7%对5.3%，P < 0.001）。因子水平检测与血友病严重程度之间存在直接相关性（r = 0.73，P = 0.006）。在四个家族中，有三名母亲和一名姐妹被确定为携带者。在18.2%的甲型血友病患者和9.1%的乙型血友病患者中检测到抗凝血因子抑制剂。总之，我们的研究发现血友病是儿童中最常见的先天性凝血障碍。必须重视血友病携带者和凝血因子抑制剂的检测。
