Biliary atresia (BA) is a rare fatal liver disease in children, and the aim of this study was to develop a method to diagnose BA early. We determined serum levels of matrix metalloproteinase-7 (MMP-7), the results of 13 liver tests, and the levels of 20 bile acids, and integrated computational models were constructed to diagnose BA. Our findings demonstrated that MMP-7 expression levels, as well as the results of four liver tests and levels of ten bile acids, were significantly different between 86 BA and 59 non-BA patients (P < 0.05). The computational prediction model revealed that MMP-7 levels alone had a higher predictive accuracy [area under the receiver operating characteristic curve (AUC) = 0.966, 95

Since the outbreak of coronavirus disease 2019(COVID-19),the entire world has been affected by the epidemic[1,2].On November 9,2021,a variant of the novel coronavirus B.1.1.529 was detected for the first time from case samples in South Africa.

目的 总结智能化随访系统的研发及在短肠综合征患儿中的应用.方法 设计研发智能化随访系统,采用便利抽样法,选取2021年1月—2022年12月入住浙江省某三级甲等儿童医院新生儿外科的34例短肠综合征患儿应用该随访系统,比较使用随访系统前后患儿并发症发生率、家属对医疗护理服务的满意度及健康教育知晓率的差异.结果 智能化随访系统应用后,患儿家属对医疗护理服务满意度逐年提高,显著高于对照组(P<0.05).家属在导管护理、饮食营养、并发症及应急情况处理方面的知识知晓率均高于对照组,患儿出院1年后并发症发生率低于对照组,差异有统计学意义(P<0.05).结论 智能化随访系统实现了以护士为主导的精准化、专业化、智能化及闭环化随访服务,有效降低了患儿并发症的发生率,提高了家属对医疗护理服务的满意度和健康教育知晓率.

Objective:To explore the correlation between age of Kasai portoenterostomy and early prognosis to provide references for timing of Kasai surgery in biliary atresia (BA) children.Methods:Retrospective review was conducted for clinical data of hospitalized BA children from January 2019 to December 2021.General profiles (gender & operative age) and parameters of liver function, coagulation function and blood ammonia were collected.According to the jaundice clearance at 3 months post-operation, they were assigned into two groups of non-jaundice clearance ( n=47) and jaundice clearance ( n=66). The cut-off value of operative age was calculated.Then two groups were divided according to operative age.The prognostic parameters of Kasai portoenterostomy at 3 months post-operation were compared, including alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST), direct bilirubin (DBIL), postoperative DBIL/ preoperative DBIL, gamma-glutamyltransferase (GGT), total bile acid (TBA), total bilirubin (TBIL), platelet count (PLT), aspartate aminotransferase-to-platelet ratio index (APRI) and international normalized ratio (INR). Results:A total of 113 cases were collected, including 49 males and 64 females. The operative age was 59.00 (40.50, 69.00) days. Univariate analysis of the preoperative indexes of the non-jaundice clearance group and the jaundice clearance group showed that the age of operation(64 (52, 79) d vs. 55 (33, 67) d), AST (202.00 (138.00, 332.00) U/L vs.147.50 (100.00, 242.00) U/L), indirect bilirubin (IBIL) ((70.60±20.31)μmol/L vs.(79.96±27.13)μmol/L), total protein(TP) ((55.88±6.59)g/L vs.(53.13±4.71)g/L) between the two groups were statistically significant ( P<0.05). The indexes with P<0.1 in univariate analysis [age of operation, albumin (ALB), ALT, AST, IBIL, TP, activated partial thromboplastin time(APTT)] were included in the multivariate Logistic regression analysis, and the results showed that the age of operation ( OR=0.974, 95% CI: 0.951-0.997, P=0.030) was an independent factor influencing the jnundice clearance at 3 months after Kasai protoentrostomy. According to the cut-off value (46.5d) of the operative age group, the results showed that the levels of ALP(365.00 (316.00, 514.75) U/L vs.492.00 (343.00, 684.00) U/L), ALT ((76.35±81.33) U/L vs.(134.09±97.45)U/L), AST (67.50 (41.50, 99.50) U/L vs.112.00 (79.00, 168.00) U/L), DBIL (2.55 (1.78, 6.95) μmol/L vs. 9.80 (3.60, 36.80) μmol/L), postoperative DBIL/ preoperative DBIL(0.05 (0.03, 0.09) vs.0.12 (0.05, 0.54)), TBA (45.50 (25.93, 92.93) μmol/L vs.106.20 (41.20, 187.50) μmol/L), TBIL (9.65 (6.98, 15.43) μmol/L vs.21.50 (10.40, 66.90) μmol/L) and APRI (0.32 (0.22, 0.48) vs.0.62 (0.41, 0.93)) in the operation age ≤46.5 d group were significantly lower than those in the operation age > 46.5 d group, the difference was statistically significant ( P<0.05). Conclusion:Early prognostic parameters are superior in group with operative age≤46.5 days to group with operative age >46.5 days.And early Kasai portoenterostomy may improve the prognosis of BA.

Objective:To explore the correlation between the results of preoperative laboratory tests and the degree of liver fibrosis (LF) to formulate a better noninvasive prediction model for evaluating LF in children with biliary atresia (BA).Methods:From April 2021 to April 2023, the relevant clinical data were retrospectively reviewed for 100 BA children.Mann-Whitney U or Kruskal-Wallis H test was utilized for comparing the differences of preoperative laboratory parameters between children with varying Ohkuma's grades of LF and bile duct hyperplasia (BDH). According to multivariate Logistic regression analysis, an integrated model was established.And receiver operating characteristic (ROC) curve was plotted for individual laboratory parameters to compare its predictive efficiency for the degree of LF.Results:Age at Kasai operation, serum matrix metalloproteinase-7 (MMP-7), alanine aminotransferase (ALT), aspartate aminotransferase (AST), direct bilirubin (DBil), indirect bilirubin (IBil), prealbumin (PAB) and aspartate aminotransferase to platelet ratio index (APRI) varied markedly between children with varying grades of LF and BDH (all P<0.05). The higher the grade of LF, the higher the grade of BDH ( P<0.001). The area under the ROC curve of the integrated model was 0.876(95% CI: 0.806, 0.945) with a sensitivity of 68.3%, a specificity of 93.2%, an accuracy of 83.0%, a positive predictive value of 87.5% and a negative predictive value of 80.9%, which were higher than the prediction efficiency of each laboratory parameter alone. Conclusions:Age of Kasai operation and level of MMP-7/APRI are correlated with the grades of LF and BDH.The innovative integrated model of multiple laboratory parameters offers better diagnostic efficiency and accuracy for non-invasive prediction of the degree of LF in BA.

BackgroundInfants with Alagille syndrome (ALGS) need to be promptly differentiated from biliary atresia (BA) at an early stage. ALGS is an autosomal, dominant, multisystem disorder with variable phenotypic penetrance caused by heterozygous mutations in JAG1 or NOTCH2, which encode the Notch signaling pathway.Case presentationWe report two cases, both with cholestatic jaundice as the main manifestation, in which BA was excluded and finally diagnosed as ALGS based on characteristic facial features, serological tests, imaging, laparoscopic cholangiography, pathology and genetic findings. Both cases are novel mutant genes on chromosome 20 that have not been reported in the literature. The mutation in patient 1 was a novel heterozygous nonsense mutation (NM_000214 exon20, c.2419G > T, p.E807Ter), which was a spontaneous mutation. Followed up to 1 year and 6 months, the symptoms resolved with ursodeoxycholic acid and cholestyramine, and the jaundice has now subsided. Patient 2 was a novel heterozygous frameshift mutation (NM_000214 exon19, c.2367–2368dupTC, p.P790Lfs*31), which was inherited from his mother. This patient was followed up to 9 months and is currently awaiting liver transplantation.ConclusionBoth cholestatic infants reported combined to exclude BA, avoid Kasai portoenterostomy (KPE), and definitively diagnose ALGS. Broadening the spectrum of JAG1 gene mutations.

Meconium peritonitis (MP) combined with intestinal atresia (IA) is a rare neonatal condition, and it is even rarer in combination with biliary atresia (BA). We describe a case of an infant who developed short bowel syndrome after partial intestinal resection due to MP and IA, along with a Santullienterostomy. During continuous enteral and parenteral nutrition, the stool color became paler. BA was identified by elevated direct bilirubin (DBIL), gamma-glutamyltransferase (GGT), serum matrix metalloproteinase-7 (MMP-7), and hepatobiliary ultrasound; then, Kasai portoenterostomy (KPE) was performed promptly. The Roux-en-Y limb was adjusted intraoperatively to preserve the maximum length of the small intestine while closing the enterostomy. After the operation, the infant gradually adapted to enteral nutrition, his bilirubin level returned to normal, and his weight gradually caught up to the normal range. Although rare, BA should be suspected when MP is combined with IA and when the stool becomes paler in color in the enterostomy state.

Abstract Purpose Blood transfusion is a common and life-saving procedure in congenital heart surgery (CHS), and it is critical for patients to identify risk factors prior to surgery. Our objective is to conduct an analysis of the preoperative factors that influence blood use during CHS and to offer guidance on preoperative blood preparation. Methods A total of 1550 cases were retrospectively analyzed in our institution between May 2019 and June 2020. We determined whether to employ red blood cells (RBCs), platelets, and plasma as dependent variables; we treated the data from characteristics and laboratory tests as binary data, except for the Risk Adjustment for Congenital Heart Surgery (RACHS) methods as multinomial data, and finally taken into binary logistic regression analysis. Results The total amounts of transfused RBCs, platelets, and plasma were 850.5 U (N = 713, 46%), 159 U (N = 21, 1.4%), and 1374.2 U (N = 953, 61.5%), respectively. Multivariate analysis found age (OR 0.142, 95% CI 0.099–0.203, P < 0.001), weight (0.170, 0.111–0.262, P < 0.001) RACHS method (RACHS2 vs. RACHS1, 3.444, 2.521–4.704, P < 0.001; RACHS3 vs. RACHS1, 9.333, 4.731–18.412, P < 0.001; RACHS4 vs. RACHS1, 31.327, 2.916–336.546, P = 0.004), and hemoglobin (0.524, 0.315–0.871, P = 0.013) to be independent risk predictors of RBC transfused volume; age (9.911, 1.008–97.417, P = 0.049), weight (0.029, 0.003–0.300, P = 0.029), RACHS method (RACHS3 vs. RACHS1, 13.001, 2.482–68.112, P = 0.002; RACHS4 vs. RACHS1, 59.748, 6.351–562.115, P < 0.001) to be platelets; and age (0.488, 0.352–0.676, P < 0.001), weight (0.252, 0.164–0.386, P < 0.001), RACHS method (RACHS2 vs. RACHS1, 2.931, 2.283–3.764, P < 0.001; RACHS3 vs. RACHS1, 10.754, 4.751–24.342, P < 0.001), APTT (1.628, 1.058–2.503, P = 0.027), and PT (2.174, 1.065–4.435, P = 0.033) to be plasma. Conclusion Although patients' age, weight, routine blood test, coagulation function, and protein levels should all be considered for preparing blood before CHS, the RACHS method is the most important factor influencing intraoperative blood transfused volume and should be considered first in clinical blood preparation.

目的 探讨谷氨酰转肽酶(GGT)联合直接胆红素(DBIL)、天冬氨酸转氨酶/血小板指数(APRI)诊断胆道闭锁(BA)的价值.方法 回顾性分析283例婴儿胆汁淤积性肝病患者的临床资料,经胆道探查、胆道造影及肝活检确诊为BA者138例为BA组,术中排除或内科治疗后黄疸消退者145例为Non-BA组.收集患者首次入院的一般资料、首次同期血液学检查结果,包括日龄、性别、体质量、GGT、DBIL、间接胆红素(IBIL)、总胆汁酸(TBA)、天冬氨酸转氨酶(AST)、丙氨酸转氨酶(ALT)、血小板计数(PLT);并计算APRI.采用ROC曲线评价各指标对BA的诊断价值.结果 BA组患者体质量、GGT、TBA、DBIL、IBIL、AST、ALT、APRI水平均显著高于Non-BA组(P<0.05).ROC曲线显示,GGT诊断BA的曲线下面积(AUC)最大,为0.845(95％CI:0.801～0.890),当GGT为临界值264 U/L时,其敏感度和特异度分别为0.732和0.814;GGT+DBIL、GGT+APRI联合诊断BA的AUC分别为0.880(95％CI:0.840～0.919)和0.871(95％CI:0.830～0.912).结论 GGT对BA具有较好的诊断价值,GGT联合DBIL、APRI对BA的诊断价值优于GGT单独诊断.

目的 建立医用耗材采购信息变动通知系统,优化医用耗材采购信息变动通知流程管理.方法 改进医用耗材采购信息变动通知的管理流程,在医院采购软件系统表中增加相应触发器实时抓取数据,设计开发软件系统,结合自动短信通知,替代原先的纸质通知单.结果 实现医用耗材采购信息变动通知的信息化管理,优化通知管理流程,实现跨院区、多部门信息化联动,通知时效性得到有效提高.运行8个月的数据显示,通知时间与计算时间、计算时间与审核时间间隔均在1b以内,使用部门总体满意度由54.8％提高至98.5％.结论 医用耗材采购信息变动通知系统的开发应用,提高了采购变动信息通知的时效性、解决了跨院区信息传递障碍、降低了人工流程管理成本,提升了医用耗材精细化管理水平.

目的 研究杭州地区门诊儿童哮喘流行病学特征及其与PM2.5和气温的关系,为哮喘的预防控制提供依据.方法 收集2015-2019年浙江大学医学院附属儿童医院门诊哮喘患儿就诊信息,采集2015-2019年每日的天气信息记录并和哮喘门诊量进行相关性分析.结果 2015-2019年,本院门诊有784 137例患儿被诊断为哮喘,3岁以下患儿是哮喘的主要人群,占总人数的49.66％,7岁以下儿童占总人数的84.38％;秋冬季节的哮喘发病率明显高于其他季节.哮喘就诊患儿的门诊数量和PM2.5浓度和气温存在相关性,与PM2.5浓度呈正相关(r=0.237,P＜0.001),而与日最高气温(r=-0.436)、日最低气温(r=-0.418)、日温差(r=-0.433)、日平均气温(r=-0.433)均呈负相关(P＜0.01).结论 学龄前儿童哮喘发生率更高,患儿家长应该在气温较低和PM2.5较高的秋冬季节密切关注患儿的身体情况,防止重型哮喘疾病的发生.

随着临床输血管理方面法律法规的完善,人们的法制意识也在逐渐得到加强,目前医院的输血管理基本上实现了合理、安全和有效输血.为了更好地对临床输血实施全面系统的管理,有必要进行输血的全面信息化管理,从输血的申请、输血信息的登记以及血液入库到血液发放等整个规范的输血过程,都应该实行信息化电子管理.本文从医院输血全面信息化改进这一角度切入,探讨每一个输血管理环节的信息化管理改进.

This article mainly elaborates ISO15189 laboratory quality system, which based on the reality of transmitting specimens between two hospitals,and focus on the improvement of the Laboratory Information System. It indicates the importance of the quality improvement when applying this standard.