Al Atrash Eman, Azaz Amer, Said Samar, Miqdady Mohammad
Department of Pediatrics, Division of Pediatric Gastroenterology, Sheikh Khalifa Medical City, Abu Dhabi 971, United Arab Emirates.
Department of Pathology, Mayo Clinic, Rochester, MN 55905, United States.
World J Clin Pediatr. 2024 Jun 9;13(2):92263. doi: 10.5409/wjcp.v13.i2.92263.
Acute fulminant liver failure rarely occurs in the neonatal period. The etiologies include viral infection (15%), metabolic/genetic disease (10%), hematologic disorders (15%), and ischemic injury (5%). Gestational alloimmune liver disease usually manifests as severe neonatal liver failure, with extensive hepatic and extrahepatic iron overload, sparing the reticuloendothelial system. Empty liver failure is a rare cause of liver failure where a patient presents with liver failure in the neonatal period with no hepatocytes in liver biopsy.
A 5-week-old male presented with jaundice. Physical examination revealed an alert but deeply icteric infant. Laboratory data demonstrated direct hyperbilirubinemia, a severely deranged coagulation profile, normal transaminase, and normal ammonia. Magnetic resonance imaging of the abdomen was suggestive of perinatal hemochromatosis. Liver biopsy showed histiocytic infiltration with an absence of hepatocytes. No hemosiderin deposition was identified in a buccal mucosa biopsy.
Neonatal liver failure in the absence of hepatocellular regeneration potentially reflects an acquired or inborn defect in the regulation of hepatic regeneration.
急性暴发性肝衰竭在新生儿期很少发生。病因包括病毒感染（15%）、代谢/遗传疾病（10%）、血液系统疾病（15%）和缺血性损伤（5%）。妊娠性同种免疫性肝病通常表现为严重的新生儿肝衰竭，伴有广泛的肝脏和肝外铁过载，网状内皮系统未受累。空肝衰竭是肝衰竭的一种罕见病因，患者在新生儿期出现肝衰竭，肝活检未见肝细胞。
一名5周大的男性出现黄疸。体格检查发现是一名警觉但黄疸严重的婴儿。实验室检查显示直接胆红素血症、凝血功能严重紊乱、转氨酶正常、血氨正常。腹部磁共振成像提示围生期血色素沉着症。肝活检显示组织细胞浸润，未见肝细胞。颊黏膜活检未发现含铁血黄素沉积。
缺乏肝细胞再生的新生儿肝衰竭可能反映了肝脏再生调节方面的后天或先天性缺陷。
