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目的探讨婴儿胆汁淤积症的临床特点和诊断方法。方法收集我科2009-2011年收治的88例胆汁淤积症患儿,对其病因、临床检查、病理及预后进行分析。结果婴儿胆汁淤积症男孩、足月儿多见;2~3月龄发病率高。本组患儿中胆道闭锁组19例;肝内淤胆组69例,其中仅10.1%明确病因。胆道闭锁组γ谷氨酰转肽酶(GGT)水平明显高于肝内淤胆组,其他生化指标差异无统计学意义。血筛瓜氨酸血症1例;尿筛40%患儿高度怀疑Citrin缺陷。肝内淤胆组中23例行LSC25A13基因12种突变位点的检测,结果均为阴性。胆道闭锁组B超确诊率为64.7%。49例肝组织病理提示两组胆汁性肝硬化发生率差异有统计学意义(P<0.01)。两组的病死率和好转痊愈率差异有统计学意义(P<0.01)。结论胆道造影术、内科动态观察是鉴别胆道闭锁的常用方法,前者更可靠。对于有肝内胆汁淤积病史或存在肝功能反复异常者,应积极找病因,注意代谢病检查,并长期随访。
Objective To investigate the clinical features and diagnosis of infant cholestasis. Methods Eighty-eight children with cholestasis admitted in our department from 2009 to 2011 were collected and their etiology, clinical examination, pathology and prognosis were analyzed. Results Infant cholestasis boy, full-term children more common; 2 to 3-month-old high incidence. The group of children with biliary atresia group of 19 cases; liver cholestasis in 69 cases, of which only 10.1% of the clear cause. The GGT level in biliary atresia group was significantly higher than that in intrahepatic cholestasis group, and the other biochemical indexes had no statistical significance. 1 case of serum citrullinemia; 40% of children with urinalysis were highly suspected of Citrin deficiency. Twenty-two cases of LSC25A13 gene mutations were detected in 23 cases of intrahepatic cholestasis group, and the results were negative. B ultrasound diagnosis of biliary atresia group was 64.7%. 49 cases of liver histology suggest that the incidence of biliary cirrhosis between the two groups was statistically significant (P <0.01). The difference between the two groups was statistically significant (P <0.01). Conclusion The cholangiography and dynamic observation of internal medicine is a common method to identify biliary atresia. The former is more reliable. For those who have a history of intrahepatic cholestasis or liver function abnormalities, should actively find the cause, pay attention to metabolic disease examination, and long-term follow-up.