Aanpreung Prapun, Laohapansang Mongkol, Ruangtrakool Ravit, Kimhan Jongkolrat
Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
J Med Assoc Thai. 2005 Nov;88 Suppl 8:S9-15.
The objective of this study was to study etiologies and outcome of neonatal cholestasis in Thai infants. The medical records of infants aged less than 3 months with the diagnosis of neonatal cholestasis in Department of Pediatrics, Siriraj Hospital from 1993 to 2004 were retrospectively reviewed. The etiologies were diagnosed by history, physical examination, and proper investigations. There were 252 infants, including 135 males (53.6%) and 117 females (46.4%). The etiologies of cholestasis were idiopathic neonatal hepatitis (INH) 23%, extrahepatic biliary atresia (EHBA) 22.2%, total parenteral nutrition (TPN)-related cholestasis 18.3%, infection 9.9%, endocrine causes 6%, choledochal cyst 5.6%, Down syndrome 4.4%, hemolytic anemia 1.6%, and miscellaneous causes 9.1%, respectively. TPN-related cholestasis was increasingly found due to advance management of critically ill premature infants. Inborn error of metabolism were suspected in 8 patients (3.21%). Seventeen cases (6.7%) developed cholestasis during the first week of life due to hemolytic anemia, intrauterine infection, hypoxia and others. During the 3 month follow-up period, 6 cases died of progressive dysfunction of liver and one case with idiopathic neonatal hepatitis died from intracranial bleeding from vitamin K deficiency. In conclusion, INH and EHBA are the most common causes of neonatal cholestasis. Due to advance management and nutritional support in critically ill premature infants, TPN-related cholestasis is found more often. Inborn error of metabolism related to neonatal cholestases is uncommon in Thai infants. Overall short-term prognosis of neonatal cholestases is good.
本研究的目的是探讨泰国婴儿新生儿胆汁淤积的病因及预后。回顾性分析了1993年至2004年在诗里拉吉医院儿科诊断为新生儿胆汁淤积的3个月龄以下婴儿的病历。通过病史、体格检查和适当的检查来诊断病因。共有252名婴儿，其中男性135名（53.6%），女性117名（46.4%）。胆汁淤积的病因分别为特发性新生儿肝炎（INH）23%、肝外胆管闭锁（EHBA）22.2%、全胃肠外营养（TPN）相关胆汁淤积18.3%、感染9.9%、内分泌原因6%、胆总管囊肿5.6%、唐氏综合征4.4%、溶血性贫血1.6%和其他原因9.1%。由于对危重症早产儿的先进管理，TPN相关胆汁淤积越来越常见。8例患者（3.21%）怀疑存在先天性代谢缺陷。17例（6.7%）在出生后第一周因溶血性贫血、宫内感染、缺氧等原因出现胆汁淤积。在3个月的随访期内，6例死于进行性肝功能衰竭，1例特发性新生儿肝炎患者死于维生素K缺乏引起的颅内出血。总之，INH和EHBA是新生儿胆汁淤积最常见的原因。由于对危重症早产儿的先进管理和营养支持，TPN相关胆汁淤积更为常见。在泰国婴儿中，与新生儿胆汁淤积相关的先天性代谢缺陷并不常见。新生儿胆汁淤积的总体短期预后良好。
