Ng Vicky L, Li Ruosha, Loomes Kathleen M, Leonis Mike A, Rudnick David A, Belle Steven H, Squires Robert H
*Division of Paediatric Gastroenterology, Hepatology and Nutrition, Transplant and Regenerative Medicine Center, The Hospital for Sick Children, University of Toronto, Toronto, Canada †Department of Biostatistics, University of Texas School of Public Health, Houston ‡Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia §Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia ||Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center ¶Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH #Department of Pediatrics and Developmental Biology, Washington University School of Medicine, St. Louis, MO **Department of Epidemiology and Biostatistics, Graduate School of Public Health, University of Pittsburgh ††Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.
J Pediatr Gastroenterol Nutr. 2016 Sep;63(3):357-64. doi: 10.1097/MPG.0000000000001178.
Hepatic encephalopathy (HE) is challenging to identify in children with acute liver failure and was not a requirement for enrollment into the Pediatric Acute Liver Failure Study Group (PALFSG). The outcomes of PALFSG participants presenting with and without HE are presented.
PALFSG participants were classified based on daily assessment of HE during the first 7 days following study enrollment: group 1-never developed HE; group 2-no HE at enrollment with subsequent HE development; and group 3-HE at study enrollment. Clinical and biochemical parameters and outcomes of death, spontaneous recovery, or liver transplantation were compared between groups.
Data from 769 PALFSG (54% boys; median age 4.2 years; range 0-17.9 years) participants were analyzed, with 277 in group 1 (36%), 83 in group 2 (11%), and 409 in group 3 (53%). Mortality occurred in 11% of all participants and was highest among group 3 participants who demonstrated persistent grade III-IV HE (55%) or showed progression of HE (26%). Eleven (4%) group 1 participants died within 21 days of enrollment. Spontaneous recovery was highest in group 1 (79%) and lowest in group 2 (25%; P < 0.001).
Mortality 21 days after enrollment was highest in participants enrolled with severe HE (grades III or IV) or demonstrating HE progression. Four percent of participants without recorded clinical HE in the 7 days after enrollment, however, died within 21 days. Improved assessment of neurological injury and pediatric acute liver failure prognostication schema are needed.
肝性脑病(HE)在急性肝衰竭患儿中难以识别,且并非加入儿科急性肝衰竭研究组(PALFSG)的必要条件。本文呈现了PALFSG参与者中出现和未出现HE的情况。
PALFSG参与者在研究入组后的前7天根据HE的每日评估进行分类:第1组——从未发生HE;第2组——入组时无HE,随后发生HE;第3组——研究入组时即有HE。比较了各组的临床和生化参数以及死亡、自发恢复或肝移植的结局。
分析了769名PALFSG参与者(54%为男孩;中位年龄4.2岁;范围0 - 17.9岁)的数据,第1组277人(36%),第2组83人(11%),第3组409人(53%)。所有参与者中有11%死亡,在第3组参与者中死亡率最高,这些参与者表现为持续性III - IV级HE(55%)或HE进展(26%)。11名(4%)第1组参与者在入组后21天内死亡。自发恢复率在第1组最高(79%),在第2组最低(25%;P<0.001)。
入组时患有严重HE(III级或IV级)或表现出HE进展的参与者在入组21天后死亡率最高。然而,入组后7天内无临床HE记录的参与者中有4%在21天内死亡。需要改进对神经损伤的评估和儿科急性肝衰竭的预后方案。