Gestational alloimmune liver disease treated with exchange transfusion and intravenous immunoglobulin: A case study

Gestational alloimmune liver disease (GALD) is a materno-fetal alloimmune disorder that targets the fetal liver and often causes neonatal liver failure. GALD most commonly presents as neonatal hemochromatosis (NH), which is a severe neonatal liver injury confirmed by extra-hepatic iron accumulation at various sites. With the discovery of the alloimmune mechanism of GALD, exchange transfusion and intravenous immunoglobulin (IVIG) administration are being used as novel treatments. Here, we present a rare case of an 11-day-old female infant who presented with marked hyperbilirubinemia. Laboratory findings showed significantly elevated direct and indirect bilirubin, high ferritin and alpha fetoprotein levels, high transferrin saturation, and severe coagulopathy. Abdominal magnetic resonance imaging revealed markedly reduced T2 signal intensity in the liver and pancreas compared to the spleen, suggesting iron deposition. The infant was diagnosed with NH and successfully treated with exchange transfusion and four doses of IVIG.     

稳定性冠心病血瘀证的血浆定量蛋白质组学探析＊

目的 基于数据非依赖性采集的定量蛋白质组学技术（data independent acquisition，DIA）对稳定性冠心病血瘀证患者的血浆蛋白质进行研究分析，初步探索冠心病血瘀证的关键生物学过程与核心靶点。方法 选择中国中医科学院广安门医院心血管科收治的5例稳定性冠心病患者作为疾病观察组，同时选取健康成人5人作为对照组，采用DIA蛋白质组学技术对受试者的血浆进行检测，确定蛋白质表达量后进行差异蛋白质表达分析、GO富集分析、KEGG信号通路图查询和蛋白质互作网络分析。结果 在差异倍数为1.5倍时，疾病观察组相比于健康对照组，共鉴定到22个蛋白质下调，9个蛋白质上调。进一步的生物信息学分析表明，冠心病血瘀证的生物学过程与补体参与的慢性炎症反应有关，关键信号通路是补体与凝血级联反应的信号通路，核心蛋白质为C反应蛋白、转甲状腺素蛋白、补体因子H、维生素D结合蛋白等。结论 冠心病血瘀证的生物学过程与补体参与的慢性炎症反应有关。     

胰腺切除患者围术期血糖管理的最佳证据总结

背景血糖紊乱是胰腺切除患者围术期常见问题。然而，目前国内开展的胰腺切除患者围术期血糖管理实践多以经验为指导，缺乏循证依据。目的总结胰腺切除患者围术期血糖管理的最佳证据。方法系统检索BMJ最佳临床实践、Up to Date、国际指南协作组、国际糖尿病联盟（IDF）、世界卫生组织（WHO）、美国国立指南数据库（NGC）、美国糖尿病协会、英国国家临床优化研究所（NICE）、新西兰指南研究组、加拿大糖尿病协会、澳大利亚糖尿病协会、苏格兰校际指南网络（SIGN）、PubMed、Web of Science、EMBase、CINHAL数据库、Cochrane图书馆、乔安娜布里格斯研究所（JBI）循证卫生保健中心、医脉通、万方数据知识服务平台、中国知网（CNKI）、生物医学数据库（CBM），筛选关于胰腺切除患者围术期血糖管理的文献。检索时间为建库至2020年12月。指南的质量评价采用国际AGREE协作组织2009年更新的AGREE Ⅱ量表。专家共识的质量评价采用澳大利亚JBI循证卫生保健中心专家共识评价标准2017版。随机对照试验的评价采用Jadad评分量表。结果通过初步检索筛选出文献6 637篇，最终纳入文献13篇，其中7篇为临床实践指南，4篇为专家共识，2篇为随机对照试验。评价结果显示，7篇临床实践指南中3篇总体质量评价为A级，剩下4篇为B级；4篇专家共识的质量评价在"观点与其他文献不一致的地方是否有合理解释？"条目的评价结果为"不清楚"，其余条目的评价结果均为"是"。2篇随机对照试验均为高质量文献。共汇总了62条最佳证据，主要涉及围术期组织管理、入院评估及处理、血糖控制目标、血糖监测、术前血糖管理策略、术中血糖管理策略、术后血糖管理策略、危急状况处理以及出院指导9个方面。结论临床医务人员应根据总结的9个方面最佳证据（包括围术期组织管理、入院评估及处理、血糖控制目标、血糖监测、术前血糖管理策略、术中血糖管理策略、术后血糖管理策略、危急状况处理以及出院指导）为胰腺切除患者制定个体化、全程化的围术期血糖管理方案。     

Effect of iron deficiency without anaemia on days alive and out of hospital in patients undergoing valvular heart surgery

Comprehensive evidence regarding the treatment of non-anaemic iron deficiency in patients undergoing valvular heart surgery is lacking. This study aimed to investigate the association between non-anaemic iron deficiency and postoperative outcomes in these patients. We retrospectively analysed 321 patients of which 180 (56%) had iron deficiency (defined as serum ferritin < 100 ng.ml^-1 or < 300 ng.ml^-1 with transferrin saturation < 20%). While the iron-deficient group had lower pre-operative haemoglobin levels than the non-iron deficient group (median (IQR [range]) 134 (127–141 [120–172]) g.l^-1, 143 (133–150 [120–179]) g.l^-1, p = 0.001), there was no between-group difference in allogeneic red blood cell transfusion. Median (IQR [range]) days alive and out of hospital at postoperative day 90 was 1 day shorter in the iron-deficient group (80 (77–82 [9–85]) days vs. 81 (79–83 [0–85]) days, p = 0.026). In multivariable analysis, only cardiopulmonary bypass duration (p = 0.032) and intra-operative allogeneic red blood cell transfusion (p = 0.011) were significantly associated with reduced days alive and out of hospital at postoperative day 90. Iron deficiency did not exert any adverse influence on secondary outcomes except length of hospital stay. Our findings indicate that non-anaemic iron deficiency alone is not associated with adverse effects in patients undergoing valvular heart surgery when it does not translate into an increased risk of allogeneic transfusion.     

Accurate detection of reemergent anti-donor ABO antibody is crucial for safe transfusion in ABO major incompatible HSCT patient after achieved the stages of full switch to donor type

Dynamic monitoring ABO chimera including erythroid ABO antigen and anti-A/B is crucial to not only assess the status of erythroid engraftment but also achieve personalized safety transfusion in patients post ABO incompatible hematopoietic stem cell transplantation. Transfusion support for ABO incompatible (ABOi) HSCT patients after achieved complete alteration to donor origin still remains cautious because the instant hematopoietic status on these transplant patients possibly returned to patient origin derived from early disease relapse and graft loss or failure. We reported that reemergent anti-B in a female patients (donor/patient: B/O) at the early phase after achievement complete donor type were not effectively found from partial automatic ABO blood grouping systems, which directly resulted in differential judgement of transplantation stage for about 15 days and disturbed the optimal recommendation on transfusion support. Meanwhile, the solely alteration of ABO chimera was found and earlier than changes of other markers such as MRD diagnosis, chimerism analysis by STR-PCR and sex chromosome assays, which can be an available predictors for bad transplant outcomes such as graft failure.