Buchanan G R, Glader B E
J Pediatr. 1977 Jul;91(1):21-4. doi: 10.1016/s0022-3476(77)80436-8.
Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20.4 to 57.6 mg/dl with approximately one half conjugated). All of the children improved within days to weeks and currently are well, without recurrence of hyperbilirubinemia or evidence of chronic liver disease. The patients described here suggest that sickling within the liver, previously reported to be a serious and even fatal syndrome, usually is a benign and self-limited process.
由于镰状细胞贫血患者极重度高胆红素血症的管理方法及预后尚不明确，我们回顾了6例镰状细胞病患儿出现显著高胆红素血症的治疗经验。肝内镰变（镰状细胞性肝病）而非肝炎或胆结石似乎是至少4例患儿极重度黄疸的主要原因，可能也是所有6例患儿黄疸的主要原因。症状和体征较少，除显著高胆红素血症外（血清总胆红素浓度为20.4至57.6mg/dl，约一半为结合胆红素），实验室检查异常并不明显。所有患儿在数天至数周内病情改善，目前情况良好，未再出现高胆红素血症，也无慢性肝病迹象。本文所述病例提示，此前报道的肝脏镰变这一严重甚至致命的综合征，通常是一个良性的自限性过程。
