United States Department of Agriculture/Agricultural Research Service, Children's Nutrition Research Center and Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
JPEN J Parenter Enteral Nutr. 2010 Jan-Feb;34(1):32-7. doi: 10.1177/0148607109332772. Epub 2009 Jul 8.
Extremely few data are available about the natural history of parenteral nutrition (PN)-associated cholestasis. The authors evaluated a cohort of infants at a large center to determine the outcome of PN-associated cholestasis in infants with some gastrointestinal function.
The authors reviewed the records of all infants admitted to a level 3 neonatal intensive care unit over a 16-month period who had the diagnosis of PN-associated cholestasis. Records were reviewed in these infants for course of cholestasis, laboratory values, outcome, and infection rate.
Sixty-six patients were admitted who met the study criteria. There were 10 deaths and 1 referral for liver transplant (Death/TPlant) (17%) in the first year of life. All Death/TPlant infants had at least 1 positive blood culture after the onset of cholestasis. Maximum conjugated bilirubin (MaxCB) in Death/TPlant infants was 15.7 +/- 2.2 (SEM) compared to 8.4 +/- 1.0 mg/dL in babies who recovered. Of 21 infants with a MaxCB > or =10.0, Death/TPlant occurred in 8/21 (38%). Of 40 babies with positive blood cultures, 11 were in the Death/TPlant group vs no deaths among the 25 without positive blood cultures. Average time to resolution from the MaxCB to a CB <2.0 mg/dL was 66 +/- 7 days (n = 49).
Infants with PN-associated cholestasis have high rates of mortality despite the presence of some gastrointestinal function. These data support further evaluation and the development of novel forms of therapy for babies with parenteral-associated CB > or =2 mg/dL with emphasis on interventions for infants with a CB >10 mg/dL.
关于肠外营养（PN）相关胆汁淤积症的自然病程，数据极为有限。作者评估了一个大型中心的婴儿队列，以确定具有一定胃肠道功能的婴儿的 PN 相关胆汁淤积症的结局。
作者回顾了在 16 个月期间入住 3 级新生儿重症监护病房的所有符合 PN 相关胆汁淤积症诊断标准的婴儿的记录。在这些婴儿中，对胆汁淤积症的过程、实验室值、结局和感染率进行了回顾。
符合研究标准的 66 例患儿入院。在生命的第一年，有 10 例死亡和 1 例接受肝移植（死亡/肝移植）（17%）。所有发生死亡/肝移植的婴儿在发生胆汁淤积症后至少有 1 次血培养阳性。死亡/肝移植婴儿的最大结合胆红素（MaxCB）为 15.7 +/- 2.2（SEM），而恢复的婴儿为 8.4 +/- 1.0 mg/dL。在 21 例 MaxCB >或= 10.0 的婴儿中，死亡/肝移植发生在 8/21（38%）。在 40 例血培养阳性的婴儿中，死亡/肝移植组有 11 例，而在 25 例血培养阴性的婴儿中无死亡。从 MaxCB 到 CB <2.0 mg/dL 的平均消退时间为 66 +/- 7 天（n = 49）。
尽管存在一定的胃肠道功能，PN 相关胆汁淤积症的婴儿死亡率仍很高。这些数据支持对肠外相关 CB >或= 2 mg/dL 的婴儿进行进一步评估和开发新型治疗方法，重点关注对 CB > 10 mg/dL 的婴儿进行干预。
