Bleeding tendency as a first symptom in children with congenital biliary dilatation
Bleeding tendency as a first symptom in children with congenital biliary dilatation
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DOI：
10.1055/s-2007-964928
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发表时间：
2007-02-01
影响因子：
1.8
通讯作者：
Ono, S.
中科院分区：
文献类型：
--
作者：
Fumino, S.;Iwai, N.;Ono, S.
研究目的：尽管以出血倾向为首发症状的先天性胆管扩张症(CBD)是一种危急状态，但这种症状的临床细节仍不清楚。材料和方法：1983-2004年间，我所收治了65例CBD患儿。以颅内出血、便血等出血表现为首发症状的儿童为出血组，其余有腹痛、呕吐等消化症状的儿童为消化组。结果：65例中有6例有出血表现(9.2%)。6例均为囊性胆总管扩张。出血组的平均年龄明显低于消化组，出血更频繁，特别是在12个月以下的婴儿。在一项实验室研究中，出血组比消化组表现出更长的凝血时间。出血组血清淀粉酶和脂肪酶水平基本正常，而消化组则明显升高。出血组的直接胆红素水平明显高于消化系统疾病组。结论：胆汁淤积与维生素K缺乏相关的凝血功能障碍导致CBD患儿有出血倾向。因此，儿科医生应该意识到这种罕见但危急的情况，可以通过补充维生素K进行快速和准确的治疗来预防。
Aim of the Study: Although a bleeding tendency as a first symptom is a critical condition in congenital biliary dilatation (CBD), the clinical details of this symptom remain unclear. We assessed this condition in children with CBD in this paper.Materials and Methods: Sixty-five children with CBD were treated at our institute between 1983 and 2004. The children, initially presenting with bleeding manifestations such as intracranial hemorrhage and bloody stools, were defined as the bleeding group, and the remaining children with digestive symptoms such as abdominal pain and vomiting were defined as the digestive group. The clinical features were compared between these two groups.Results: In 6 of the 65 cases, bleeding manifestations were noted (9.2%). All six had cystic-type choledochal dilatation. The mean age of the bleeding group was significantly younger than that of the digestive group, and bleeding was more frequent, especially in infants less than 12 months of age. In a laboratory study, the bleeding group showed a more prolonged blood coagulation time than the digestive group did. Serum amylase and lipase levels in the bleeding group were almost normal, while those in the digestive group were significantly higher. The direct bilirubin level in the bleeding group was significantly higher than that in the digestive group.Conclusions: Disturbed blood coagulation due to vitamin K deficiency related to cholestasis results in a bleeding tendency in children with CBD. Therefore, pediatric surgeons should be aware of this rare but critical condition which can be prevented by rapid and precise treatment with vitamin K supplementation.