放射性核素肝胆显像联合动态十二指肠液检查对婴儿持续性黄疸鉴别诊断的意义

目的 探讨99mTc-EHIDA肝胆显像联合动态十二指肠液引流检查对婴儿期持续性黄疸鉴别诊断的意义.方法 应用SPECT仪对84例15～90 d持续性阻塞性黄疸息儿进行肝胆核素显像和动态十二指肠引流液检查.结果 核素肝胆显像对先天性胆道闭锁(EHBA)诊断灵敏度为100.0%(29/29),特异度为74.5%(41/55);核素肝胆显像联合动态十二指肠引流对EHBA诊断灵敏度为100.0%(29/29),特异度为100.0%(55/55).结论 ,99mTc-EHIDA肝胆显像是一种无创、安全、有效的检查方法,与动态十二指肠引流法结合对婴儿持续性黄疸鉴别诊断具有较高的临床价值.     

Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature

Hepatobiliary scintigraphy is an important diagnostic modality for work-up of neonatal cholestasis. Therefore, our objective was to evaluate the literature regarding the accuracy of hepatobiliary scintigraphy in differentiating biliary atresia from non-biliary atresia causes of cholestasis (collectively called neonatal hepatitis). Our search included Medline, SCOPUS and Google Scholar. Only studies using Tc-99 m-labeled immunodiacetic acid (IDA) derivatives were included. Overall, 81 studies were included in the meta-analysis. Pooled sensitivity and specificity were 98.7% (range 98.1–99.2%) and 70.4% (range 68.5–72.2%), respectively. Factors that increased specificity included the use of radiotracers with high hepatic extraction, administration of hepatic-inducing drugs (such as phenobarbital), use of a calculated dose/kg and administration of a booster dose in cases of non-excretion of the tracer in the bowel. SPECT imaging and duodenal fluid sampling also had high specificity; however, they need further validation because of the low number of studies. Semiquantitative imaging methods do not seem to have any incremental value. We conclude that hepatobiliary scintigraphy using IDA derivatives can be very useful for diagnostic work-up of neonatal cholestasis. To improve the specificity, several measures can be followed regarding type and dose of the radiotracer and imaging protocols. Non-imaging methods seem to be promising and warrant further validation.     

Living donor liver transplantation in children: a single North American center experience over two decades

Little data concerning hospital charges and long-term outcomes of LDLT in North American children according to transplant indications have been published. To compare outcomes of patient and graft survival and healthcare charges for LDLT for those with BA vs. other diagnoses (non-BA). A retrospective review of 52 children receiving 53 LDLT (38 BA and 14 non-BA) from 1992 to 2010 at our institution was performed. One-, five-, and 10-yr patient and graft survival data were comparable to national figures reported to UNOS. Average one-yr charges for recipients and donors were $242 849 for BA patients and $183 614 for non-BA (p = 0.074). BA patients were 1.23 ± 1.20 yr of age vs. 4.25 ± 5.02 for non-BA, p = 0.045. Examination of the total population of patients who were alive in 2010 in five chronological groupings showed that the crude five-yr survival rates were 1992-1995: 9/11 (82%); 1995-1997: 6/10 (60%); 1997-1999: 8/10 (80%); 1999-2001: 9/10 (90%); and 2001-2003: 7/7 (100%). Thus, examination of the clinical and financial data together over the entire period of the transplant program suggests that the dramatic improvement in patient survival was accomplished without a dramatic increase in indexed charges. All 53 donors survived, and only 10% had complications requiring hospitalization. LDLT in children results in excellent outcomes for patients and donors. Ways to lower costs and maximize graft outcome should be investigated.     

肝胆同位素动态显像和超声检查对婴儿肝外胆道闭锁的诊断价值

目的探讨99Tcm-二乙基乙酰苯胺亚氨二醋酸(99Tcm-EHIDA)肝胆平面+断层显像和肝胆超声检查对婴儿肝外胆道闭锁(EHBA)的诊断价值。方法临床表现高度怀疑EHBA的持续性黄疸患儿98例,予肝胆平面+断层显像和超声检查。参照临床最终诊断,评价两种检查方法对不同日龄段EHBA的诊断效能和联合应用价值。结果99Tcm-EHIDA肝胆平面显像诊断EHBA的灵敏度、特异度和准确性分别为92.6%、60.6%和68.4%;联合6～8h肝胆断层显像,分别提高到100.0%、77.5%和83.7%。对日龄<60d组患儿,肝胆平面+断层显像对EHBA灵敏度、特异度和准确性分别为100.0%、86.2%、89.7%,高于以胆囊大小、收缩率为诊断标准的超声检查(80.0%、69.0%、71.8%)和以肝门纤维块为诊断标准的超声检查(40.0%、93.1%、79.5%);而对日龄>60d者,三者对EHBA的效能分别为100%、71.4%、79.7%和88.2%、57.1%、66.1%和76.5%、92.9%、88.1%。其中,肝胆平面+断层显像持续未见肠道放射性出现的29例患儿,联合超声检查以三角形索带征为诊断标准,诊断E...     

活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

Gallbladder contraction in biliary atresia: a pitfall of ultrasound diagnosis

In 3 (9 %) of 34 children with biliary atresia, US revealed gallbladder contraction following an oral feed, given on admission, but not with subsequent feeds. Surgery revealed a Kasai type IIIa biliary atresia with a patent communication between the gallbladder and duodenum. We propose that the bile ducts may initially have been patent, but then gradually became obliterated secondary to inflammation. These cases may explain the development of one type of biliary atresia. Received: 28 July 1997 Accepted: 1 December 1997     

活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

Pediatric tracheotomy: 3-year experience at a tertiary care center with 54 children

The aim of this study was to evaluate the indications, complications and outcomes of pediatric tracheotomies at a tertiary care center. Data were obtained retrospectively from 54 patients who underwent tracheotomy from July 2007 to May 2010. Over the three-year period, 54 tracheotomies were performed. Thirty-two patients (59.3%) were male and 22 (40.7%) were female. The mean and median ages of the patients were 54 and 14 months (6 days-17 years), respectively. Twenty-six patients (48.1%) were under 1 year of age. The most common indication for tracheotomy was prolonged intubation (87%), followed by upper airway obstruction (13%). Five patients (9.2%) underwent tracheotomy under semiurgent settings. The overall complication rate was 29.6% (16/54). Early complications occurred in 7 patients (13%), including accidental decannulation in 2 patients, subcutaneous emphysema in 4 patients and hemorrhage in 1 patient. Late complications occurred in 9 patients (16.7%) (stomal granulation in 7 patients and stomal infection in 2 patients). Eight patients (14.8%) were decannulated successfully. No tracheotomy-related deaths occurred, with an overall mortality rate of 27.7% (15/54). Pediatric tracheotomy is a relatively safe procedure with a low incidence of procedure-related morbidities. The indication for the majority of the procedures was prolonged intubation (87%). The lower decannulation rate is related to the higher percentage of patients needing assisted ventilation and the relatively short follow-up period.     

Early differential diagnosis methods of biliary atresia: a meta-analysis

Purpose

To evaluate the accuracy of early differential diagnosis methods of biliary atresia in patients with infantile cholestasis.

Methods

We searched PubMed, EMBASE and the Web of Science databases for articles evaluated the early differential diagnosis methods of biliary atresia. The methodological quality of each study was assessed with version 2 of the Quality Assessment of Diagnostic Accuracy Studies tool. Two reviewers extracted data independently. Pooled sensitivity, specificity, positive likelihood ratio (LR?+), negative likelihood ratio (LR??), diagnostic odds ratio (DOR) with 95% CIs were calculated to assess each diagnosis method.

Results

A total of 38 articles were included. Summary sensitivity and specificity were 77% (95% CI 74–80%) and 93% (95% CI 91–94%), respectively, for B-US in 23 studies; 96% (95% CI 92–98%) and 58% (95% CI 51–65%), respectively, for MRCP in five studies; 87% (95% CI 82–91%) and 78% (95% CI 74–82%), respectively, for acholic stool in seven studies; 84% (95% CI 78–89%) and 97% (95% CI 97–98%), respectively, for serum liver function test in seven studies; 96% (95% CI 94–97%) and 73% (95% CI 70–76%), respectively, for hepatobiliary scintigraphy in 18 studies; 98% (95% CI 96–99%) and 93% (95% CI 89–95%), respectively, for percutaneous liver biopsy in 11 studies.

Conclusion

The accuracy rate of percutaneous liver biopsy is better than all of the noninvasive methods. Take into consideration the advantages and disadvantages of the six methods, combination of multidisciplinary noninvasive diagnosis methods is the first choice for differential diagnosis of BA from other causes of neonatal cholestasis.

     

活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

Prolonged neonatal jaundice and the diagnosis of biliary atresia: a single-center analysis of trends in age at diagnosis and outcomes

Age at diagnosis is a modifiable risk factor in outcomes after hepatoportoenterostomy in biliary atresia; however, distinguishing biliary atresia from other more common causes of prolonged neonatal jaundice can be difficult. To focus attention on diagnosis of biliary atresia, we analyzed secular trends in the age at diagnosis, and other factors that might influence outcome. We performed a retrospective analysis of 55 consecutive infants with biliary atresia presenting to a single academic pediatric center over 15-year period from 1990 to 2004. The median age at diagnosis was 60 days (range: 21-152). In recent era (2000-2004), the median age was 69.0 days, compared with 48.5 days (1990-1994) and 59.5 days (1995-1999), respectively. Consistent with previous studies, the median age at diagnosis of those with poor outcomes (death or liver transplant) exceeded those with good outcomes after the hepatoportoenterostomy (72 vs 52 days, P < .001). The lack of improvement, or a concerning trends toward an increase in the age at diagnosis of biliary atresia, is perhaps attributable to neonatal follow-up practices. Efforts to make an earlier diagnose of this important condition deserve wider application and study.