Koul Roshan, Lal Bikrant Bihari, Pamecha Viniyendra, Sarin Shiv, Alam Seema
Pediatric Neurology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi, India.
Pediatric Hepatology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi, India.
Child Neurol Open. 2021 Jan 11;8:2329048X20983763. doi: 10.1177/2329048X20983763. eCollection 2021 Jan-Dec.
To report 2 children with acute hepatic myelopathy after hepatitis A infection who recovered completely after living donor liver transplantation.
All the children admitted into liver intensive care unit (LICU) from November 1st 2018 to 31st October 2019, were evaluated for the neurological features. The data was collected from the admission register of the LICU unit in children below 15 years age. Medical records of these children were reviewed and data collected. Established clinical criteria were used to categorize the various grades of hepatic encephalopathy/myelopathy.
37 children were seen over 1-year period between 6 months to 15 years age. There were 24 male(64.9%) and 13 females. Acute liver failure was seen in 19 (51.3%) and acute on chronic liver failure in 18 (48.7%). There were 10 cases of hepatitis A in acute liver failure group,10 of 19 cases (52.6%), while Wilson's disease and undetermined etiology group formed the chronic group. 2 cases of hepatic myelopathy were seen in acute liver failure following hepatitis A infection. Both these children underwent live liver donor transplantation and recovered completely. Further in hepatitis A group,3 children had spontaneous recovery, 4 died and 1 child was discharged with end of life care. Overall out of all 37 children with liver failure,20 (54%) were discharged, 6 (16.2%) were advised end of life care and 11 (29.8%) died.
Two cases (10.5%) of reversible hepatic myelopathy were seen in acute liver failure group of 19 cases. 18 out of 37 (48.6%) children had residual neurological features at discharge time.
报告2例甲型肝炎感染后发生急性肝性脊髓病的儿童,他们在活体肝移植后完全康复。
对2018年11月1日至2019年10月31日入住肝脏重症监护病房(LICU)的所有儿童进行神经学特征评估。数据从LICU病房15岁以下儿童的入院登记册中收集。回顾了这些儿童的病历并收集了数据。采用既定的临床标准对肝性脑病/脊髓病的不同等级进行分类。
在1年期间共诊治了37例6个月至15岁的儿童。其中男性24例(64.9%),女性13例。19例(51.3%)为急性肝衰竭,18例(48.7%)为慢性肝衰竭急性发作。急性肝衰竭组中有10例甲型肝炎病例,占19例中的10例(52.6%),而威尔逊病和病因不明组构成慢性组。在甲型肝炎感染后的急性肝衰竭中发现2例肝性脊髓病。这两名儿童均接受了活体肝移植并完全康复。此外,在甲型肝炎组中,3名儿童自发康复,4名死亡,1名儿童在临终关怀下出院。在所有37例肝衰竭儿童中,20例(54%)出院,6例(16.2%)接受临终关怀,11例(29.8%)死亡。
在19例急性肝衰竭组中,有2例(10.5%)出现可逆性肝性脊髓病。37例(48.6%)儿童中有18例在出院时仍有残留神经学特征。