Chen Wen-Xiong, Wong Virginia C N, Wong Kar-Yin
Division of Neurodevelopmental Pediatrics, Department of Pediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong ROC.
J Child Neurol. 2006 Jun;21(6):474-9. doi: 10.1177/08830738060210061301.
We recruited 128 neonates with hyperbilirubinemia over a 5-year period (1995-2000) to study the short- and long-term effects of hemolytic hyperbilirubinemia on the auditory brainstem pathway and neurodevelopmental status. These children were divided into two groups: (1) a hemolytic group (n = 29; ABO incompatibility [n = 19], Rh incompatibility [n = 1], glucose-6-phosphate dehydrogenase deficiency [n = 8] and both ABO incompatibility and glucose-6-phosphate dehydrogenase deficiency [n = 1]) and (2) a nonhemolytic group (n = 99). All received phototherapy. Exchange transfusions were performed for four (13.8%) in the hemolytic group and three (3%) in the nonhemolytic group. The brainstem auditory evoked potential was recorded at a mean age of 3.2 months in the hemolytic group and 3.1 months in the nonhemolytic group. Serial brainstem auditory evoked potential assessments were performed until 2 years of age (3 in the hemolytic group and 18 in the nonhemolytic group). All had regular physical, neurologic, visual, and auditory evaluation until 3 years of age. The rate of exchange transfusion was significantly higher in the hemolytic group than in the nonhemolytic group (P < .05). Brainstem auditory evoked potential abnormalities at the initial assessment occurred in three (10.4%) in the hemolytic group (all related to ABO incompatibility) and nine (9.1%) in the nonhemolytic group. At 2 years, the brainstem auditory evoked potential returned to normal except in three cases with a slightly increased hearing threshold (one [3.5%] in the hemolytic group at 60 dB nHL and two [2%] in the nonhemolytic group at 50 dB nHL]). There were no significant differences in the rate of brainstem auditory evoked potential abnormalities at the initial or subsequent assessments between both groups. All except five cases had a normal neurodevelopmental outcome at 3 years (three [two with ABO incompatibility and one with glucose-6-phosphate dehydrogenase deficiency] in the hemolytic group [10.4%] and two [2%] in the nonhemolytic group). All had mild motor delay and hypotonia, which returned to normal at 3 years. The rate of abnormal neurodevelopmental outcome was higher in the hemolytic group than in the nonhemolytic group, although with no significant difference between both groups (P = .08). All five cases in both groups with abnormal neurodevelopment had a normal brainstem auditory evoked potential at the initial assessment. There was no relationship between the abnormal initial brainstem auditory evoked potential and the final neurodevelopmental outcome. The toxic effect of hyperbilirubinemia on the auditory brainstem pathway and neurodevelopmental status in our cohort was transient. The prognosis of neonatal hemolytic hyperbilirubinemia in our Chinese cohort is excellent, possibly owing to an aggressive early-intervention approach.
我们在5年期间（1995 - 2000年）招募了128例高胆红素血症新生儿，以研究溶血性高胆红素血症对听觉脑干通路和神经发育状况的短期和长期影响。这些儿童被分为两组：（1）溶血组（n = 29；ABO血型不合[n = 19]，Rh血型不合[n = 1]，葡萄糖-6-磷酸脱氢酶缺乏症[n = 8]以及ABO血型不合合并葡萄糖-6-磷酸脱氢酶缺乏症[n = 1]）和（2）非溶血组（n = 99）。所有患儿均接受了光疗。溶血组有4例（13.8%）进行了换血治疗，非溶血组有3例（3%）进行了换血治疗。溶血组在平均年龄3.2个月时记录脑干听觉诱发电位，非溶血组在平均年龄3.1个月时记录。对两组患儿进行连续脑干听觉诱发电位评估直至2岁（溶血组3次，非溶血组18次）。所有患儿在3岁前均进行了定期的体格、神经、视觉和听觉评估。溶血组的换血治疗率显著高于非溶血组（P <.05）。初始评估时，溶血组有3例（10.4%）出现脑干听觉诱发电位异常（均与ABO血型不合有关），非溶血组有9例（9.1%）出现异常。到2岁时，除3例听力阈值略有升高外，脑干听觉诱发电位恢复正常（溶血组1例[3.5%]在60 dB nHL，非溶血组2例[2%]在50 dB nHL）。两组在初始或后续评估中脑干听觉诱发电位异常率无显著差异。除5例患儿外，所有患儿在3岁时神经发育结局均正常（溶血组3例[2例ABO血型不合和1例葡萄糖-6-磷酸脱氢酶缺乏症][10.4%]，非溶血组2例[2%]）。所有患儿均有轻度运动发育迟缓及肌张力低下，在3岁时恢复正常。溶血组神经发育异常结局的发生率高于非溶血组，尽管两组之间无显著差异（P =.08）。两组中神经发育异常的5例患儿在初始评估时脑干听觉诱发电位均正常。初始脑干听觉诱发电位异常与最终神经发育结局之间无关联。在我们的队列中，高胆红素血症对听觉脑干通路和神经发育状况的毒性作用是短暂的。我们中国队列中新生儿溶血性高胆红素血症的预后良好，可能归因于积极的早期干预措施。
