120例儿童急性肝功能衰竭的病因及预后分析

Q4 Medicine

中华实用儿科临床杂志 Pub Date : 2020-04-08 DOI:10.3760/CMA.J.CN101070-20191108-01113

T. Jiang, Wen-xian Ou-yang, Y. Tan, Lian Tang, Hui Zhang, Z. Kang, Xi Zheng, Shuangjie Li

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引用次数: 0

摘要

目的探讨小儿急性肝衰竭(PALF)的病因、预后及影响预后的因素，为PALF的临床治疗提供依据。方法收集2008年5月至2018年5月湖南省儿童医院收治的PALF患儿的临床资料，分析其病因及预后。根据预后情况将患者分为死亡组和生存组，比较两组患者的生化指标。之后，对不同资料分别采用t检验、Wilcoxon检验和χ2检验进行统计分析。结果120例PALF中，男68例，女52例，其中婴幼儿36例，幼儿34例，学龄前儿童22例，学龄儿童28例。败血症20例(16.7%)、遗传代谢性疾病19例(15.8%)、中毒18例(15.0%)、病毒感染12例(10.0%)、药物6例(5.0%)、胆汁息肉1例(0.8%)、肿瘤1例(0.8%)。病因不明43例(35.9%)。在病因已知的病例中，遗传代谢性疾病和传染性疾病是婴儿发病的主要原因，幼儿发病以感染性疾病和药物/毒物为主，而药物/毒物和遗传代谢性疾病是学龄儿童和学龄前儿童发病的主要原因。PALF患儿死亡率为50.0%。其中Epstein-Barr病毒相关的噬血细胞综合征、败血症、Citrin缺乏症和酪氨酸血症的死亡率高于其他疾病。与生存组比较，死亡组总胆红素(TB)[159.00(73.05、274.00)μmol/L vs.62.75(2.65、221.75)μmol/L]、直接胆红素(DB)[83.00(41.43、160.00)μmol/L vs.38.74(10.98、128.75)μmol/L]、凝血酶原时间(PT)[39.60(24.93、62.60)s vs.24.65(21.43、29.83)s]、国际标准化比值(INR)[3.40(2.30、6.74)vs.2.09(1.85、2.84)]、血氨(NH3)水平[109.50(85.25、149.75)μmol/L vs.80.00(60.25、102.75)μmol/L]显著升高。差异均有统计学意义(P<0.05);死亡组白蛋白[(28.72±5.88)g/L vs(33.69±4.96)g/L]、丙氨酸转氨酶(ALT) [586.50(223.25, 1 082.00) U/L vs.1 434.00(615.00, 3 334.50) U/L]、天冬氨酸转氨酶(AST) [827.50(545.00, 2 024.00) U/L vs.1 663.50(821.00, 4 886.75) U/L]水平均显著降低，差异均有统计学意义(P<0.05)。然而，两组的血糖和胆固醇水平没有统计学上的显著差异。结论小儿PALF病死率高，不同年龄组有不同的病因。TB、DB、PT、INR、NH3及肝性脑病比例升高，白蛋白、AST、ALT降低提示预后不良。关键词:儿童;急性肝衰竭;病原学;预后

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Analysis of etiology and prognosis of 120 children with pediatric acute liver failure

Objective To investigate the etiology, prognosis and prognostic factors of pediatric acute liver failure(PALF), in order to provide the basis for clinical treatment of PALF. Methods The clinical data of children with PALF hospitalized at Hunan Children′s Hospital from May 2008 to May 2018 were collected, and the causes and prognosis were analyzed.According to the prognosis, the patients were divided into the death group and the survival group, whose biochemical indexes were then compared.After that, the statistical analysis of different data were carried out by using t-test, Wilcoxon test and χ2 test separately. Results In 120 PALF cases, there were 68 males and 52 females, and there were 36 infants, 34 toddlers, 22 preschoolers and 28 school-age children.Twenty cases (16.7%) were caused by sepsis, 19 cases (15.8%) by genetic metabolic diseases, 18 cases (15.0%) by poisoning, 12 cases (10.0%) by viral infection, 6 cases (5.0%) by drugs, 1 case (0.8%) by bile polyp, and 1 case (0.8%) by tumor disease.Besides, the etiology of 43 cases (35.9%) was unknown.Among the cases with known etiologies, genetic metabolic and infectious diseases were the main cause of disease in infants, toddler patients were mostly caused by infectious diseases and drug/toxicants, and drug/toxicants and hereditary metabolic diseases were the dominant cause of disease in school-age children and preschoolers.Mortality rate of children with PALF was 50.0%.Among them, the mortality of Epstein-Barr virus-associated hemophagocytic syndrome, sepsis, Citrin deficiency and Tyrosinemia was higher than that of other diseases.Compared with the survival group, the total bilirubin (TB)[159.00(73.05, 274.00) μmol/L vs.62.75(2.65, 221.75)μmol/L], direct bilirubin(DB)[83.00(41.43, 160.00) μmol/L vs.38.74(10.98, 128.75) μmol/L], prothrombin time (PT)[39.60(24.93, 62.60) s vs.24.65(21.43, 29.83) s], international standardized ratio (INR)[3.40(2.30, 6.74) vs.2.09(1.85, 2.84)], and blood ammonia (NH3) levels [109.50(85.25, 149.75) μmol/L vs.80.00(60.25, 102.75) μmol/L] in the death group were significantly increased, and the diffe-rences were statistically significant(all P<0.05); while the levels of albumin[(28.72±5.88) g/L vs.(33.69±4.96) g/L], alanine aminotransferase (ALT) [586.50(223.25, 1 082.00) U/L vs.1 434.00(615.00, 3 334.50) U/L]and aspartate aminotransferase (AST) [827.50(545.00, 2 024.00) U/L vs.1 663.50(821.00, 4 886.75) U/L]in the death group were significantly decreased, and the differences were statistically significant(all P<0.05). However, the blood glucose and cholesterol levels in both groups had no statistically significant difference. Conclusion The mortality of children with PALF is high, and different age groups have different etiologies.The increase of TB, DB, PT, INR, NH3 and the ratio of hepatic encephalopathy, and the decrease of albumin, AST and ALT suggest poor prognosis. Key words: Child; Acute liver failure; Etiology; Prognosis

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来源期刊

中华实用儿科临床杂志 Medicine-Pediatrics, Perinatology and Child Health

CiteScore

0.60

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0.00%

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14243

期刊介绍： Chinese Journal of Applied Clinical Pediatrics ( semi-monthly ) is a core journal of paediatrics under the supervision of China Association for Science and Technology, sponsored by Chinese Medical Association and undertaken by Xinxiang Medical College. Founded in 1986, it is openly circulated both at home and abroad. The journal has several columns, such as Expert Forum, Experimental Research and Paediatric Surgery, which are mainly for paediatric medical workers and medical researchers in hospitals. Its purpose is to reflect the new theories and technologies in paediatric medicine and scientific research at home and abroad, and to promote academic exchanges. Chinese Journal of Applied Clinical Pediatrics is a source journal of China Science Citation Database (CSCD), a core journal of Peking University, a source journal of Chinese science and technology paper statistics (China Science and Technology Core Journals), a core academic journal of RCCSE, a high-quality scientific and technical journal of China, a high-quality scientific and technical journal of China Association for Science and Technology, and a high-quality scientific and technical journal of China Biomedical Science and Technology Association. We have been published in China Biomedical Literature Database (SinoMed), China Knowledge Network, Wanfang Data Knowledge Service Platform, China Academic Journal Abstracts, Scopus Database, Chemical Abstracts (USA), Japan Science and Technology Agency (JSTA) Database, Copernicus Abstracts (Poland), Abstracts of the Centre for Agricultural and Biological Sciences (CABS) of the United Kingdom, Cambridge Scientific Abstracts ProQuest Database, WHO Medical Journal of the Western Pacific Region (WMPR), and WHO Medical Journal of the Western Pacific Region (WMPR) of the United States. We have been included in dozens of authoritative databases at home and abroad, such as WHO Western Pacific Region Index of Medicine (WPRIM), Ullrich's Guide to Periodicals, and so on.