Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado Denver School of Medicine and the Pediatric Liver Center, The Children's Hospital, Aurora, CO, USA.
J Pediatr. 2009 Dec;155(6):801-806.e1. doi: 10.1016/j.jpeds.2009.06.005. Epub 2009 Jul 29.
To describe the frequency of diagnostic testing for the 4 most common causes of pediatric acute liver failure (PALF) (drugs, metabolic disease, autoimmune process, and infections) in indeterminate PALF within the PALF Study Group Database.
PALF was defined by severe hepatic dysfunction within 8 weeks of onset of illness, with no known underlying chronic liver disease in patients from birth through 17 years of age.
Of the 703 patients in the database, 329 (47%) had indeterminate PALF. In this group, a drug history was obtained in 325 (99%) urine toxicology screenings performed in 118 (36%) and acetaminophen level measured in 124 (38%) patients. No testing for common metabolic diseases was done in 179 (54%) patients. Anti-nuclear antibody, anti-smooth muscle antibody, and anti-liver kidney microsomal autoantibodies associated with autoimmunity were determined in 239 (73%), 233 (71%), and 208 (63%) patients, and no tests were obtained in 70 (21%). Testing was performed for hepatitis A virus, hepatitis B virus, and Epstein Barr virus in 80%, 86%, and 68%, respectively.
Current practice indicates that investigation for metabolic and autoimmune causes of PALF are infrequent in patients ultimately given a diagnosis of indeterminate acute liver failure. This offers an opportunity to improve diagnosis and potential treatment options in children with acute liver failure.
描述在 PALF 研究组数据库中，无法明确病因的小儿急性肝衰竭（PALF）中，最常见的 4 种病因（药物、代谢疾病、自身免疫过程和感染）的诊断检测频率。
PALF 的定义为疾病发作后 8 周内出现严重肝功能障碍，且 17 岁以下患者无已知潜在慢性肝病。
在数据库中的 703 名患者中，329 名（47%）为无法明确病因的 PALF。在这一组中，对 325 名（99%）患者进行了药物史询问，对 118 名（36%）患者进行了尿液毒物筛查，对 124 名（38%）患者进行了乙酰氨基酚水平检测。对 179 名（54%）患者未进行常见代谢疾病的检测。对 239 名（73%）、233 名（71%）和 208 名（63%）患者进行了抗核抗体、抗平滑肌抗体和抗肝-肾微粒体自身抗体的检测，对 70 名（21%）患者未进行检测。对甲型肝炎病毒、乙型肝炎病毒和 Epstein-Barr 病毒进行了检测，阳性率分别为 80%、86%和 68%。
目前的实践表明，对于最终被诊断为无法明确的急性肝衰竭的患者，代谢和自身免疫原因的检查并不常见。这为改善急性肝衰竭儿童的诊断和潜在治疗选择提供了机会。
