中国循证儿科杂志 ›› 2016, Vol. 11 ›› Issue (2): 157-160.

• 病案报告 • 上一篇    

以血小板减少为首发症状的婴儿自身免疫性肝炎2例并分析

杨梅雄1 唐清1 吕自力2 王琳琳1 欧佳琏1 单庆文1   

  1. 广西医科大学第一附属医院 1 儿科,2 病理科 南宁,530021
  • 收稿日期:2016-01-24 修回日期:2016-04-21 出版日期:2016-04-25 发布日期:2016-04-21
  • 通讯作者: 单庆文

Analyzed 2 infant patients with autoimmune hepatitis whose original symptom was thrombocytopenia

YANG Mei-xiong1, TANG Qing1, LV Zi-li2, WANG Lin-lin1, OU Jia-lian1, SHAN Qing-wen1   

  1. 1 Department of Pediatrics, 2 Department of Pathology,The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China
  • Received:2016-01-24 Revised:2016-04-21 Online:2016-04-25 Published:2016-04-21
  • Contact: SHAN Qing-wen

摘要:

摘要:目的 总结自身免疫性肝炎婴儿患者临床特点及诊治情况,为该病的诊断、治疗提供依据。方法 分析我院收治的2例婴儿期起病的自身免疫性肝炎患儿的症状、体征、辅助检查,结合文献资料进行分析并提出相应的治疗方案。结果 2例患儿均以血小板减少为首发症状,以肝功能异常、肝脾肿大为主要临床表现,结合肝功能、免疫球蛋白、血清学抗体及肝穿检查结果确诊为自身免疫性肝炎,对激素或联合硫唑嘌呤应答良好。结论 自身免疫性肝炎临床症状可不典型,在婴儿中较少见,临床医师面对合并血小板减少的肝功能异常的婴儿时,需注意考虑是否存在自身免疫性肝炎,应尽早行肝穿以明确诊断,期为治疗、停药、预后提供依据,一旦诊断明确,需积极予激素或联合硫唑嘌呤治疗,以防病情恶化。

Abstract:

【Abstract】 Objective:The purpose of this paper is to investigate,summarize the clinical characteristics,and provide evidence for the diagnosis and treatment of infants with autoimmune hepatitis (AIH).Methods:Based on 2 cases of AIH in our hospital we analyzed patient's symptoms,physical signs,results of their auxiliary examination,the corresponding theurapeutic schemes results,and the literature data.Results:Thrombocytopenia and abnormal liver function were the original symptoms.Hepatosplenomegaly were the main clinical manifestations in those 2 patients.Patient's liver function test,immunoglobulin,serum antibodies,liver biopsy results,and good response to corticosteroid alone or in combination with azathiprine (AZA) all supported the diagnosis of AIH.Conclusions:The clinical symptoms of AIH disease may not specific and AIH is considered rare in the infant population.Clinicians should always consider AIH in children presenting with chronic or acute liver disease.Liver biopsy is required as soon as possible to establish the diagnosis,it can provide evidence for the treatment,drug withdrawal,and prognosis.Once the diagnosis is clear, treatment should be instituted as soon as possible to avoid progression of the disease and it consist of corticosteroids alone or in combination with azathioprine (AZA).