Pinto Raquel Borges, Schneider Ana Claudia Reis, da Silveira Themis Reverbel
Raquel Borges Pinto, Pediatric Gastroenterology Unit, Hospital da Criança Conceição, Porto Alegre 91340 480, Rio Grande do Sul, Brazil.
World J Hepatol. 2015 Mar 27;7(3):392-405. doi: 10.4254/wjh.v7.i3.392.
Several conditions, especially chronic liver diseases, can lead to cirrhosis in children and adolescents. Most cases in clinical practice are caused by similar etiologies. In infants, cirrhosis is most often caused by biliary atresia and genetic-metabolic diseases, while in older children, it tends to result from autoimmune hepatitis, Wilson's disease, alpha-1-antitrypsin deficiency and primary sclerosing cholangitis. The symptoms of cirrhosis in children and adolescents are similar to those of adults. However, in pediatric patients, the first sign of cirrhosis is often poor weight gain. The complications of pediatric cirrhosis are similar to those observed in adult patients, and include gastrointestinal bleeding caused by gastroesophageal varices, ascites and spontaneous bacterial peritonitis. In pediatric patients, special attention should be paid to the nutritional alterations caused by cirrhosis, since children and adolescents have higher nutritional requirements for growth and development. Children and adolescents with chronic cholestasis are at risk for several nutritional deficiencies. Malnutrition can have severe consequences for both pre- and post-liver transplant patients. The treatment of cirrhosis-induced portal hypertension in children and adolescents is mostly based on methods developed for adults. The present article will review the diagnostic and differential diagnostic aspects of end-stage liver disease in children, as well as the major treatment options for this condition.
多种病症,尤其是慢性肝病,可导致儿童和青少年肝硬化。临床实践中的大多数病例由相似病因引起。在婴儿中,肝硬化最常见的病因是胆道闭锁和遗传代谢性疾病,而在大龄儿童中,肝硬化往往由自身免疫性肝炎、威尔逊氏病、α-1抗胰蛋白酶缺乏症和原发性硬化性胆管炎导致。儿童和青少年肝硬化的症状与成人相似。然而,在儿科患者中,肝硬化的首个迹象通常是体重增加缓慢。儿科肝硬化的并发症与成人患者所见相似,包括由胃食管静脉曲张引起的胃肠道出血、腹水和自发性细菌性腹膜炎。在儿科患者中,应特别关注肝硬化引起的营养改变,因为儿童和青少年生长发育对营养的需求更高。患有慢性胆汁淤积症的儿童和青少年有多种营养缺乏的风险。营养不良对肝移植术前和术后患者都会产生严重后果。儿童和青少年肝硬化所致门静脉高压的治疗大多基于针对成人开发的方法。本文将综述儿童终末期肝病的诊断和鉴别诊断方面,以及该病症的主要治疗选择。