早期干预对高胆红素血症儿童智能发育的作用

目的 探讨早期干预对高胆红素血症儿童智力发育的作用。方法将88例高胆红素血症儿童随机分为早期干预组44例，对照组44例，早期干预组自新生儿期开始干预治疗至2岁，两组高胆红素血症儿童均于1岁、2岁时进行智能发育检测。结果早期干预组1岁、2岁时智力发育指数（MDI）和心理运动发育指数（PDI）均高于对照组，差别有统计学意义。结论早期干预可以促进高胆红素血症儿童的智力发育，有利于减少高胆红素血症对儿童智能发育的影响。     

两种蓝光治疗新生儿高胆红素血症对甲状腺功能影响的比较

目的比较两种蓝光疗法治疗新生儿高胆红素血症对甲状腺功能的影响.方法将患儿随机分为Bilibed婴儿蓝光床治疗（A组）18例、传统温箱加双面蓝光照射治疗（B组）18例.观察两组治疗前后高胆红素血症的含量变化并比较对甲状腺功能的影响.结果两组光疗前后血清胆红素较治疗前下降（P＜0.05）,A组治疗后血清胆红素下降较B组明显（P＜0.05）;A组副作用小于B组（P＜0.05～0.01）;两组光疗前后甲状腺功能指标变化无统计学意义（P＞0.05）.结论Bilibed婴儿蓝光床副作用小,疗效优于传统光疗法,对甲状腺功能无影响.     

Sieving coefficient inaccuracies during hemodiafiltration in patients with hyperbilirubinemia

Hemodiafiltration has assumed an important role in the supportive therapy of critically ill patients. The viability of the filter used for hemodiafiltration can be monitored by estimating the sieving coefficient of small molecules such as creatinine and/or urea. We report on three patients with severe hyperbilirubinemia whose creatinine sieving coefficient was spuriously elevated as a result of discordance in the accuracy of creatinine measurement in plasma and ultrafiltrate respectively. This discordance was a consequence of lack of bilirubin clearance during hemodiafiltration. As a result, while the plasma creatinine determination by the kinetic Jaffe method was negatively influenced by the hyperbilirubinemia, the ultrafiltrate creatinine was not. This report is the first to document the lack of bilirubin clearance during hemodiafiltration and its impact on the calculation of sieving coefficient based on creatinine. The use of urea as the solute for determining the sieving coefficient allows for an accurate estimate and provides a valid means of monitoring this parameter in the setting of hyperbilirubinemia. Received: 18 April 2000 / Revised: 16 June 2000 / Accepted: 20 June 2000     

6种母子ABO血型不合的新生儿血型血清学检验结果及新生儿溶血病发生率

目的:探讨6种母子ABO血型不合的新生儿脐血血型血清学检验结果及临床型HDN发生率。方法:按检验操作规程对1199例母子ABO血型不合的新生儿脐血做血型血清学检验,在出生7天内分组调查其高胆红素血症发生率。结果:①母/子血型O/A(B)组合的新生儿抗体释放试验、DAT、IAT阳性率、临床型HDN发生率均极显著高于母/子血型A/B、A/AB、B/A、B/AB组合的新生儿(P<0.01)。②新生儿脐血血型血清学检验结果与临床型HDN发生率呈显著正相关性(P<0.01)。③患儿血液中存在的IgG抗-A+B对临床型HDN发生率无显著影响(P>0.01)。结论:ABO血型系统发生的临床型HDN以母/子血型O/A(B)组合的患儿为主(89.19%),母/子血型A/B、A/AB、B/A、B/AB组合的患儿较少(10.81%)。抗体释放试验阳性患儿血液中存在的游离抗体对判断患儿疾病的发展趋势有一定的意义。     

Rotor综合征合并慢性肾衰1例家系调查并文献复习

目的：探讨Rotor综合征的发病机制及临床特点,提高临床医师对该病的认识.方法：报告1例合并有慢性肾衰的Rotor综合征的临床资料,复习相关文献,总结其临床表现,诊断方法及预后.结果：该患者通过其临床表现、家族史和肝活检诊断为Rotor综合征,并行肾移植,术后肝功并无明显受损.结论：该病罕见,临床自幼表现为波动性黄疸,肝活检及99mTc-HIDA胆道显像有助于明确诊断,预后较好.     

Impaired expression of hepatic multidrug resistance protein 2 is associated with posthepatectomy hyperbilirubinemia in patients with biliary cancer

Background and aims Hyperbilirubinemia is a critical complication following hepatectomy for biliary cancer. Hepatic multidrug resistance protein 2 (MRP2), a bilirubin transporter, is shown to be down-regulated by acute biliary obstruction in rats. However, little is known about the effect of chronic obstruction by malignancy on the MRP2 expression in patients or the association of MRP2 expression with posthepatectomy hyperbilirubinemia.Materials and Methods The MRP2 expression before hepatectomy was determined by immunostaining and Western blotting in patients with biliary cancer. To directly determine the effect of chronic bile duct obstruction on the MRP2 expression, the expression levels were compared between the cholestatic and noncholestatic lobes in each of seven patients. In another 39 patients, the correlation of the MRP2 expression of the anticipated remnant liver with the posthepatectomy severe hyperbilirubinemia, defined as a serum total bilirubin concentration200 mol/l, was evaluated.Results The MRP2 staining in the cholestatic lobes was weak and not restricted to the canalicular membrane, unlike the noncholestatic lobes. The expression levels in the cholestatic lobes were 45% of those in the noncholestatic lobes. Postoperative maximum bilirubin levels were significantly correlated with MRP2 expression of the anticipated remnant liver. The MRP2 expression had been already impaired before hepatectomy in all patients who eventually developed severe hyperbilirubinemia.Conclusions Decreased MRP2 expression, caused by biliary obstruction due to cancer, is a possible risk factor for posthepatectomy severe hyperbilirubinemia.     

Transcutaneous bilirubinometry in very low birthweight infants

AIM: To evaluate whether transcutaneous bilirubinometry (TcB) would be a reliable and efficient screening technique for hyperbilirubinaemia in very low birthweight (VLBW, < or =1500 g) infants in an intensive care unit setting. METHODS: TcB measurements (Minolta Airshield Jaundice Meter JM-102, Osaka, Japan) were obtained immediately before or within 10 min following routine blood sampling for plasma bilirubin concentration measurements in 124 VLBW infants not receiving phototherapy. The relationship between the two techniques was analysed by linear regression analysis. A plasma bilirubin > or =150 micromol/l was defined as hyperbilirubinaemia. The sensitivity and specificity of possible TcB cut-off readings to detect hyperbilirubinaemia was evaluated. RESULTS: There was a significant correlation between the measurements of both techniques (p < 0.0001, r = 0.68). In the present study, a TcB cut-off reading of 14 would have reduced the need for plasma bilirubin measurements by 26% without missing true hyperbilirubinaemia. CONCLUSION: The data suggest that TcB will improve VLBW infant care in an intensive care unit setting by reducing the need for invasive bilirubin concentration measurements.     

Newborn jaundice and kernicterus—health and societal perspectives

Kernicterus, a preventable injury to the brain from severe neonatal jaundice, has re-emerged in the United States as a public and societal health concern. Kernicterus, in its usually recognized form, causes devastating disabilities, including athetoid cerebral palsy and speech and hearing impairment. This condition not only ranks amongst the highest cost per new case (per CDC’s Financial Burden of Disability study, 1992), but also results in profound and uncompromising grief for the family and loss to siblings of healthy, talkative playmates. And for the child with kernicterus (usually remarkably intelligent, but trapped in an uncontrollable body), grief and frustration are enormous. In 2001 national healthcare organizations, including Centers for Disease Control (CDC), the Joint Commission for the Accreditation of Healthcare Organizations (JACHO) and the American Academy of Pediatrics (AAP) issued alerts to all accredited hospitals and public health professionals in the United States that all healthy infants are at potential risk of kernicterus if their newborn jaundice is unmonitored and inadequately treated. The re-emergence of kernicterus in the United States is the result of interacting phenomena including (a) Early hospital discharge (before extent of jaundice is known and signs of impending brain damage have appeared); (b) Lack of adequate concern for the risks of severe jaundice in healthy term and near newborns; (c) An increase in breast feeding; (d) Medical care cost constraints; (e) Paucity of educational materials to enable parents to participate in safeguarding their newborns; and (f) Limitations within in healthcare systems to monitor the outpatient progression of jaundice. A multidisciplinary approach that encompasses both healthcare and societal needs should be evaluated at a national level for practical and easy to implement strategies. An approach that is based on principles of evidence-based medicine, patient-safety and family centeredness is presented in this article. These strategies should also be based on public awareness campaign such that the healthcare providers can attempt to achieve a “Zero Tolerance of Kernicterus” and thereby decrease both childhood disabilities and infant mortality within the community.     

NICU高胆红素血症新生儿听力筛查

目的通过对NICU高胆红素血症新生儿进行听力筛查,发现NICU中高胆红素血症新生儿听力障碍发病情况。方法对我院2005年10月至2006年12月期间NICU收住的高胆红素血症新生儿用DPOAE模式进行初筛,对初筛未通过者用DPOAE+ABR进行复筛,复筛未通过者进行听力学评估及确诊。结果共筛查高胆红素血症178人,确诊(6月龄)5例,听力障碍发病率为2.96%。结论高胆红素血症是NICU新生儿听力障碍发病的一个高危因素。     

双重病因对新生儿高胆红素血症发生率影响探讨

目的:探讨存在葡萄糖6磷酸脱氢酶(G6PD)缺乏和新生儿溶血病(HDN)双重病因对新生儿高胆红素血症发生率的影响。方法:按检验操作规程对足月健康新生儿脐血进行G6PD和HDN血型血清学检验,对存在G6PD缺乏和HDN病因的新生儿调查其高胆红素血症发生率。结果:①G6PD缺乏组、HDN病因组、G6PD缺乏与HDN双重病因组的患儿高胆红素血症发生率均极显著高于正常对照组的新生儿(P<0.01);②存在G6PD缺乏和HDN双重病因的患儿高胆红素血症发生率极显著高于G6PD缺乏组和HDN病因组的患儿(P<0.01)。结论:存在G6PD缺乏和HDN双重病因的患儿红细胞溶血破坏的几率增高,其发生高胆红素血症的可能性更大。