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。     

高胆血红素血症新生儿T细胞亚群、NK细胞表达水平的研究

目的 探讨高胆血红素血症新生儿T细胞亚群、NK细胞表达水平,为高胆血红素血症新生儿的早期诊疗、干预保健提供科学依据.方法 无菌采集健康足月自然分娩1分钟Apger评分≥8分,5分钟评分10分,体重在2.8～4Kg新生儿脐静脉肝素抗凝血2ml及不同高胆血红素血症新生儿股静脉肝素抗凝血2ml,采用美国RD有限公司提供的试剂,采用间接免疫荧光技术和S.P法,测定其T细胞亚群及NK细胞的水平.结果 （1）三个组CD3、CD4、CD8、NKCD16、NKCD57与对照组比较,均P＜0.01差异显著,有统计学意义.（2）一组与二组、三组比较,除了二组CD8 P＞0.05差异无统计学意义外,其他均P＜0.01差异显著,有统计学意义.结论 证实了高胆血红素血症新生儿胆红素毒性对其T细胞亚群及NK细胞有免疫抑制作用,可致患儿细胞免疫功能低下.这也是高胆血红素血症新生儿易并发感染的理论基础.为高胆血红素血症新生儿早期诊疗、干预及保健提供了基础免疫学依据.     

IgG亚型与新生儿ABO溶血病的关系

目的 探讨IgG亚型与新生儿ABO溶血病高胆红素血症的发生及危重程度的关系。方法 对实验室诊断为新生儿ABO溶血病的患儿，用德国欧盟公司的亚型试剂盒测定其血清IgG亚型，并检测胆红素水平。结果 153例实验室诊断为新生儿ABO溶血病的患儿中检测出IgG1和IgG3亚型的27例，其中24例发生高胆红素血症，检出IgG3亚型的全部发生高胆红素血症且胆红素增高明显。结论 测定新生儿IgG亚型抗体，可有助于诊断新生儿ABO溶血病高胆红素血症的发生并预测其严重程度。     

新生儿高胆红素血症30例病因及治疗探讨

目的分析新生儿高胆红素血症的病因及治疗方法。方法选择30例新生儿高胆红素血症的临床资料,进行回顾性分析。结果病人均采用蓝光和白蛋白治疗,其中27例治愈;3例自动放弃治疗。结论ABO溶血、感染和母乳黄胆是新生儿高胆红素血症的病因。早期诊断和正确治疗可以减少并发症的发生率,提高治愈率。     

Clinical,pathogenetic and neuropathological correlates in dystonic cerebral palsy

Studies were made of three autopsied cases of dystonic (changing tone) cerebral palsy with the aim of correlating the clinical characteristics and pathogenetic factors with the neuropathological lesions.Clinically all three patients were small-for-dates and born at gestational ages of 31–34 weeks. They sustained either severe birth asphyxia or repeated hypoxic episodes with respiratory arrest, and all three had moderate hyperbilirubinemia during the perinatal period. All of them developed an identical non-progressive extrapyramidal cerebral palsy syndrome. Mental capacity was less affected than motor performance, which remained at a neonatal level.From the pathogenetic point of view, prenatal malnutrition, preterm birth, pre- and perinatal hypoxia, acidosis and icterus were considered to be cumulative brain-damaging factors in each of the three patients. The pattern of combined partial and total asphyxia could be discerned in all the cases.The outstanding neuropathological correlate was selective bilateral sclerosis of the globus pallidus.It is proposed that the time of operation of the above factors in relation to the developmental stage of the globus pallidus determined the site of the cerebral damage. This hypothesis finds support in recent experiments with graded asphyxia in Rhesus monkeys.Supported by¹ the Swedish Medical Research Council, and² the Norrbacka-Eugenia Foundation and the newspaper Expressen's Prenatal Research Foundation     

β-Thalassemia trait and hyperbilirubinemia in G-6-PD deficient newborn infants

Hb A₂ was determined in 50 subjects with erythrocyte G-6-PD deficiency who presented with hyperbilirubinemia in the neonatal period and in 100 non-hyperbilirubinemic G-6-PD deficient newborn infants, at the age of 12 months or more. Six subjects in the first group and 13 in the second were found to be carriers of the -thalassemia trait. Statistical analysis of the data did not show any significant difference between the two groups. It seems that the -thalassemia trait does not provide any protection against neonatal hyperbilirubinemia associated with G-6-PD deficiency.     

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。     

新生儿高胆红素血症484例临床分析

目的了解住院新生儿高胆红素血症的发病情况及探讨采用间歇性蓝光照射治疗新生儿高胆红素血症的疗效。方法总结分析我院2002年1月至2007年8月收治的484例高胆红素血症患儿临床资料。结果高胆红素血症发病率占同期新生儿的40．5％，引起本组高胆红素血症的病因中，围产期因素占37．4％，感染占21．6％，母乳性黄疸占18．5％，新生儿溶血占6．2％。其中发病日龄在7天以内占69％。435例治愈，49例好转。结论加强围产期保健，对所有新生儿特别是7天内新生儿应进行每天至少1次的临床评估。对所有24小时内出现黄疸的新生儿应进行经皮测胆红素及血清总胆红素的监测。对耶些在血总胆红素达到高峰水平前离院的新生儿应随访。对于出生24小时内出现黄疸，早产儿低于正常值但有高危因素，健康足月儿总胆红素〉256μmol／L，无高危因素，都应积极治疗以降低高胆红素的发病率、死亡率和伤残率。