Karimzadeh Parvaneh, Fallahi Minoo, Kazemian Mohammad, Taslimi Taleghani Naeeme, Nouripour Shamsollah, Radfar Mitra
Pediatric Neurology Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Pediatric Neurology Department, Mofid Children's Hospital, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Iran J Child Neurol. 2020 Winter;14(1):7-19.
Hyperbilirubinemia is one of the most common neonatal disorders. Delayed diagnosis and treatment of the pathologic and progressive indirect hyperbilirubinemia lead to neurological deficits, defined as bilirubin induced encephalopathy (BIE) (2). The incidence of this disorder in underdeveloped countries is much more than developed areas. All neonates with the risk factors for increased the blood level of indirect bilirubin are at risk for BIE, especially preterm neonates which are prone to low bilirubin kernicterus . BIE can be transient and acute (with early, intermediate and advanced phases)or be permanent, chronic and lifelong ( with tetrad of symptoms including visual (upward gaze palsy), auditory (sensory neural hearing loss), dental dysplasia abnormalities, and extrapyramidal disturbances (choreoathetosis cerebral palsy).Beside the abnormal neurologic manifestations of the jaundiced neonates ,brain MRI is the best imaging modality for the confirmation of the diagnosis. Although early treatment of extreme hyperbilirubinemia by phototherapy and exchange transfusion can prevent the BIE, unfortunately the chronic bilirubin encephalopathy does not have definitive treatment.
高胆红素血症是最常见的新生儿疾病之一。病理性和进行性间接胆红素血症的延迟诊断和治疗会导致神经功能缺损,即胆红素诱导的脑病(BIE)(2)。这种疾病在欠发达国家的发病率远高于发达地区。所有具有间接胆红素血水平升高风险因素的新生儿都有患BIE的风险,尤其是容易发生低胆红素核黄疸的早产儿。BIE可以是短暂和急性的(有早期、中期和晚期阶段),也可以是永久性、慢性和终身性的(有包括视觉(向上凝视麻痹)、听觉(感觉神经性听力损失)、牙齿发育异常和锥体外系障碍(舞蹈手足徐动症型脑瘫)的四联征症状)。除了黄疸新生儿的异常神经表现外,脑部MRI是确诊的最佳影像学检查方法。虽然通过光疗和换血疗法对极重度高胆红素血症进行早期治疗可以预防BIE,但不幸的是,慢性胆红素脑病没有确切的治疗方法。