Psacharopoulos H T, Mowat A P, Davies M, Portmann B, Silk D B, Williams R
Arch Dis Child. 1980 Apr;55(4):252-8. doi: 10.1136/adc.55.4.252.
To document the clinical features and complications of fulminant hepatic failure in childhood, 31 consecutive cases (of whom only 9 survived) were reviewed. Of 26 children with acute hepatitis (HbSAg-negative), liver function steadily deteriorated in all but 2, and encephalopathy occurred within 3 weeks of the onset of symptoms in all except 3 of them. Eight of these patients survived, as did one of 3 in which this deterioration was caused by paracetamol overdosage. Single cases due to and halothane died. Encephalopathy lasted from 2 to 16 days in the survivors, and from one to 20 days in the fatal cases. The severity fluctuated by more than one grade in 9 patients. The outcome was not related to the age or sex of patient, clinical or biochemical abnormalities at presentation, or to the duration of the encephalopathy. Prothrombin time was prolonged by more than 90 seconds in 10 fatal cases, but in none of the survivors. The outcome was related to the severity of the encephalopathy, only one (6%) of 19 children in grade 4 coma surviving, and to the occurrence of neurological complications—particularly brain stem dysfunction (9 cases), decerebrate posturing (12 cases), and convulsions (7 cases). Massive gastrointestinal bleeding (14 cases) and renal failure (10 cases) were confined to the fatal group. At necropsy 7 (54%) of 13 had cerebral oedema. Hypoglycaemia, septicaemia, respiratory tract infections, ascites, and haemopoietic complications occurred both in fatal cases and survivors. Although liver function tests and liver biopsy appearances remained abnormal in survivors for 24 and 30 months respectively, these children developed normally without evident disease during or after this period. Children with fulminant hepatic failure and severe encephalopathy develop major pathophysiological complications affecting almost every system. Such complications must be prevented or vigorously treated. The mortality is no lower than in adults. Effective treatment must be instituted before grade 4 coma is established.
为记录儿童暴发性肝衰竭的临床特征及并发症，对连续31例病例（其中仅9例存活）进行了回顾。26例急性肝炎（乙肝表面抗原阴性）患儿中，除2例肝功能稳定外，其余均逐渐恶化，除3例之外，所有患儿均在症状出现后3周内发生脑病。这些患儿中有8例存活，3例因对乙酰氨基酚过量导致肝功能恶化的患儿中有1例存活。因异烟肼和氟烷导致的单例患儿死亡。存活患儿的脑病持续2至16天，死亡患儿则持续1至20天。9例患儿的病情严重程度波动超过1级。预后与患儿的年龄、性别、就诊时的临床或生化异常情况以及脑病持续时间无关。10例死亡病例的凝血酶原时间延长超过90秒，但存活患儿均未出现这种情况。预后与脑病严重程度有关，4级昏迷的19例患儿中仅1例（6%）存活，还与神经系统并发症的发生有关，尤其是脑干功能障碍（9例）、去大脑强直姿势（12例）和惊厥（7例）。致命组患儿出现大量胃肠道出血（14例）和肾衰竭（10例）。尸检时，13例患儿中有7例（54%）出现脑水肿。低血糖、败血症、呼吸道感染、腹水和血液系统并发症在死亡病例和存活患儿中均有发生。尽管存活患儿的肝功能检查和肝活检表现分别在24个月和30个月内仍异常，但这些患儿在此期间及之后均正常发育，未出现明显疾病。患有暴发性肝衰竭和严重脑病的儿童会出现影响几乎每个系统的主要病理生理并发症。必须预防或积极治疗这些并发症。其死亡率并不低于成人。必须在4级昏迷确立之前开始有效治疗。
