转换西罗莫司治疗肾移植后高胆红素血症的临床观察

目的：对转换西罗莫司（SRL）并撤除钙调磷酸酶抑制剂（CNI）类药物对高胆红素血症影响及其安全性和可行性进行临床观察。方法：2006年2月～2007年11月对肾移植后伴有高胆红素血症患者37例转换SRL并撤除CNI类药物,观察转换后总胆红素（TB）水平变化、血清肌酐（Scr）变化和不良事件发生情况。结果：TB和直接胆红素（DB）由转换前的30．45μmol／L和10．10μmol／L下降至转换后的12．13μmol／L和3．7μmol／L（P〈0．01）。Scr和血尿酸（Ua）转换后较转换前均有较明显降低,肌酐清除率（Ccr）有所提高。移植肾和患者全部存活。不良事件主要为高脂血症。结论：转换SRI．治疗肾移植后高胆红素血症是有效和安全的。     

急性高胆红素血症豚鼠听功能研究

目的 探讨急性高胆红素血症对听功能的影响及病损部位。方法建立急性高胆红素血症豚鼠模型，检测其听性脑干反应（ABR）、听神经复合动作电位（CAP）阈值及潜伏期、耳蜗微音器电位（CM）、畸变产物耳声发射（DPOAE）。结果实验组动物出现ABR反应阈升高，伴波Ⅰ、Ⅲ、Ⅳ潜伏期及Ⅰ-Ⅲ、Ⅲ-Ⅳ波间期明显延长，与对照组比较有显著差异（P〈0．01）；CAPN1、N2波潜伏期延长，与对照组比较有显著差异（P〈0．01）；实验组有2只动物（3耳）ABR、CAP未引出；CM在8、4、1kHz处幅值下降，DPOAE各频率幅值下降；电镜示螺旋神经节细胞胞浆空泡状改变、包绕髓鞘板层松解。结论高胆红素血症不但引起中枢听觉传导通路的损害，亦可累及耳蜗，其耳蜗毒性机制尚需进一步探讨。     

高胆红素血症对新生儿神经行为影响的探讨

用NBAS_(20)项对新生儿高胆红素血症病婴的神经行为进行评估测定,结果显示高胆红素血症对新生儿行为能力、运动能力及神经反射均有影响,其中以视听定向力及肌张力方面的影响为著。血清胆红素≥205μmol/L时,胆红素水平与行为能力评分呈直线负相关关系。(r=-0.53,P<0.001)新生儿神经行为测定可以全面地估价新生儿中枢神经系统的发育状况,有助于早期发现新生儿中枢神统系统的轻微功能障碍。     

Guidelines for detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants (35 or more weeks�� gestation) �C Summary

Hyperbilirubinemia is very common and usually benign in the term newborn infant and the late preterm infant at 35 and 36 completed weeks’ gestation. Critical hyperbilirubinemia is uncommon but has the potential for causing long-term neurological impairment. Early discharge of the healthy newborn infant, particularly those in whom breastfeeding may not be fully established, may be associated with delayed diagnosis of significant hyperbilirubinemia. Guidelines for the prediction, prevention, identification, monitoring and treatment of severe hyperbilirubinemia are presented.     

改良振幅整合脑电图评分联合血清神经元特异性烯醇化酶水平对新生儿胆红素脑损伤的早期预测价值

目的探讨改良振幅整合脑电图(aEEG)评分联合血清神经元特异性烯醇化酶(NSE)水平,对新生儿胆红素脑损伤的早期预测价值。 方法选择2017年3月至2018年2月,于苏北人民医院新生儿病房住院治疗的80例高胆红素血症新生儿为研究对象。根据头颅MRI检查结果是否有苍白球高信号改变,将其分为脑损伤组(n＝37)及无脑损伤组(n＝43);并选择本院产科同期出生的40例健康足月新生儿为对照组。对3组受试儿进行aEEG监测,并进行改良aEEG评分,检测血清总胆红素(TBiL)浓度及NSE水平,以及脑干听觉诱发电位(BAEP)。对3组受试儿一般临床资料、改良aEEG评分、血清TBiL浓度及NSE水平,采用单因素方差分析及最小显著性差异法(LSD)-t检验,进行总体比较及两两比较;对改良aEEG评分、血清NSE水平及aEEG分度分别与BAEP分度的相关性,采用Spearman秩相关分析法;绘制受试者工作特征曲线(ROC),比较改良aEEG评分、血清NSE水平及二者联合,对高胆红素血症新生儿发生胆红素脑损伤的预测价值。本研究遵循本院伦理委员会所制定的伦理学标准,并得到批准(审批文号：2016KY-064),所有纳入对象监护人知情同意。 结果①3组新生儿性别构成,入院时日龄及胎龄,出生时体重、身长、头围及胸围,以及其母亲孕次、产次比较,差异均无统计学意义(P>0.05)。②脑损伤组新生儿的改良aEEG评分为(7.7±1.6)分,分别低于无脑损伤组及对照组的(10.4±1.5)分和(10.9±1.3)分;而血清TBiL浓度及NSE水平分别为(349.7±74.0) μmol/L、(47.6±13.2) μg/L,均分别高于无脑损伤组的(295.7±31.4) μmol/L、(32.3±7.7) μg/L及对照组的(185.1±35.9) μmol/L、(28.6±7.3) μg/L,并且差异均有统计学意义(均为P<0.001)。③本研究受试儿的改良aEEG评分与BAEP分度呈负相关关系(r_s＝-0.718,P<0.001),血清NSE水平及aEEG分度,均与BAEP分度呈正相关关系(r_s＝0.685,P<0.001;r_s＝0.647,P<0.001)。④改良aEEG评分、血清NSE水平及二者联合,预测高胆红素血症新生儿发生胆红素脑损伤的ROC曲线下面积(ROC-AUC)分别为0.875(95%CI：0.795～0.954,P<0.001),0.853(95%CI：0.769～0.937,P<0.001)及0.938(95%CI：0.861～0.980,P<0.001);3种方法预测脑损伤的敏感度分别为86.5%、62.2%、83.8%,特异度分别为74.4%、93.0%、90.7%。 结论改良aEEG评分联合血清NSE水平,对新生儿胆红素脑损伤的早期预测价值,较单一采用改良aEEG评分或血清NSE水平更高。     

Transient liver injury and severe neonatal cholestasis in infant with glucose-6-phosphate dehydrogenase deficiency due to a new mutation

We report the case of a neonate with a new, previously undescribed, glucose-6-phosphate dehydrogenase (G6PD) gene mutation, which was revealed by severe cholestasis, hyperbilirubinemia, and transient liver dysfunction. The severity of the clinical phenotype with ongoing chronic hemolytic anemia suggests that this mutation belongs to class 1 G6PD deficiency. The hemizygous mutation «c.675G>c; p.Trp225Cys» was detected by genomic sequencing. Since severe G6PD deficiency can be revealed by cholestasis, it is important to check G6PD enzyme activity when faced with a case of liver dysfunction in the neonatal period.     

红细胞储存时间对高胆红素血症新生儿换血治疗的疗效及内环境的影响

目的 探讨不同储存时间的红细胞对高胆红素血症新生儿换血治疗的疗效及内环境的影响。方法 回顾性分析2015年1月至2018年8月经换血治疗的135例高胆红素血症新生儿的临床资料,按输入红细胞的储存时间分为短期储存组（储存时间≤ 7 d,n=56）和长期储存组（储存时间 > 7 d,n=79）。比较两组患儿换血后0 h、12 h的血清总胆红素（TBIL）水平与TBIL下降率,以及换血后继续光疗时间及再次换血率。比较分析两组患儿换血前及换血后0 h的血常规、电解质、血糖及血气分析指标的变化。结果 换血后0 h,两组患儿TBIL水平及TBIL下降率比较差异无统计学意义（P > 0.05）;换血后12 h,长期储存组TBIL水平高于短期储存组,TBIL下降率低于短期储存组（P < 0.01）;长期储存组换血后的继续光疗时间更长（P < 0.05）;长期储存组低血钠、高血钾及代谢性酸中毒等换血并发症的发生率较短期储存组增加（P < 0.05）。结论 使用储存时间 > 7 d的红细胞输入不影响新生儿换血治疗的即时效果,但换血后继续光疗效果较差,亦增加了低血钠、高血钾及代谢性酸中毒的发生风险。     

Multiple portions enteral nutrition and chyme reinfusion of a blunt bowel injury patient with hyperbilirubinemia undergoing open abdomen: A case report

Blunt bowel injury (BBI) is relatively rare but life-threatening when delayed in surgical repair or anastomosis. Providing enteral nutrition (EN) in BBI patients with open abdomen after damage control surgery is challenging, especially for those with discontinuity of the bowel. Here, we report a 47-year-old male driver who was involved in a motor vehicle collision and developed ascites on post-trauma day 3. Emergency exploratory laparotomy at a local hospital revealed a complete rupture of the jejunum and then primary anastomosis was performed. Postoperatively, the patient was transferred to our trauma center for septic shock and hyperbilirubinemia. Following salvage resuscitation, damage control laparotomy with open abdomen was performed for abdominal sepsis, and a temporary double enterostomy (TDE) was created where the anastomosis was ruptured. Given the TDE and high risk of malnutrition, multiple portions EN were performed, including a proximal portion EN support through a nasogastric tube and a distal portion EN via a jejunal feeding tube. Besides, chyme delivered from the proximal portion of TDE was injected into the distal portion of TDE via a jejunal feeding tube. Hyperbilirubinemia was alleviated with the increase in chyme reinfusion. After 6 months of home EN and chyme reinfusion, the patient finally underwent TDE reversal and abdominal wall reconstruction and was discharged with a regular diet. For BBI patients with postoperative hyperbilirubinemia who underwent open abdomen, the combination of multiple portions EN and chyme reinfusion may be a feasible and safe option.     

思密达和胆维他佐治新生儿高胆红素血症

本文观察24例高胆患儿在常规治疗的基础上加用思密达，胆维他口服治疗。结果24例观察组高胆患儿治疗前血清总胆红素（μmol／L，X±S）为292±47，治疗后17例高胆患儿复查血清总胆红素为87±38。说明能更有效降低血清总胆红素，对高胆具有良好的近期疗效。     

不同方案治疗感染所致新生儿高胆红素血症成本-效果分析

目的观察阿莫西林克拉维酸钾干混悬剂对感染引起的新生儿高胆红素血症的治疗效果并进行成本分析。方法将2006年1月-2008年3月皖南医学院附属池州医院因感染所致新生儿高胆红素血症新生儿104例患儿随机分为观察组和对照组两组。观察组予口服阿莫西林克拉维酸钾干混悬剂抗感染治疗,而对照组予阿洛西林静脉点滴治疗,两组患儿均予蓝光光疗等综合退黄治疗措施。观察两组患儿治疗效果,采用最小成本分析法对两组进行评价。结果两组患儿在痊愈率、有效率、药物不良反应发生率、疗程及住院费用方面差异无统计学意义（P〉0.05）;观察组在药品费用、总成本及成本-效果比方面较对照组差异有统计学意义（P〈0.01）。结论口服阿莫西林克拉维酸钾干混悬剂对感染引起的新生儿高胆红素血症相对于静脉应用阿莫西林具有明显的药物经济学优势。