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目的提高对遗传性毛细血管扩张症(hereditary hemorrhagic telangiectasia,HHT)造成的大咯血患儿的诊治水平。方法总结1例以大量咯血为临床表现的HHT患儿的临床诊治经过并复习相关文献。结果通过数字减影血管造影(digital subtraction angiography,DSA)及病史诊断为HHT,DSA结果提示右侧支气管动脉中下部发育异常、支气管动脉-肺动脉瘘、左侧支气管动脉发育异常,行经导管栓塞(transcatheter embolotherapy,TCE)治疗,随访2年,无咯血表现。在国内外报道儿童咯血原因中,血管因素属少见原因。40%的HHT患者存在肺动静脉畸形(pulmonary arteriovenous malformation,PAVM),70%的PAVM患者与HHT相关,2%~5%的PAVM患者为支气管动脉与肺动脉瘘。结论遗传性毛细血管扩张症为儿童大量咯血的少见原因。DSA可以明确病变部位。利用TCE治疗并应长期随访。
Objective To improve the diagnosis and treatment of hemoptysis in children with hereditary hemorrhagic telangiectasia (HHT). Methods One case of HHT with clinical manifestations of massive hemoptysis was reviewed and reviewed. Results The diagnosis of HHT by digital subtraction angiography (DSA) and medical history revealed that the right middle bronchial artery was abnormally developed, the bronchial artery - pulmonary artery fistula and left bronchial artery were abnormally developed, and the transcatheter embolotherapy, TCE) treatment, followed up for 2 years, no hemoptysis. In the domestic and international coverage of the causes of hemoptysis in children, vascular factors are rare causes. Forty percent of HHT patients have pulmonary arteriovenous malformation (PAVM), 70% of patients with PAVM are associated with HHT, and 2% to 5% of patients with PAVM are bronchial and pulmonary arterial fistulas. Conclusion Hereditary telangiectasia is a rare cause of massive hemoptysis in children. DSA can identify the lesion. The use of TCE treatment and should be long-term follow-up.