Kubota Akio, Mochizuki Narutaka, Shiraishi Jun, Nakayama Masahiro, Kawahara Hisayoshi, Yoneda Akihiro, Tazuke Yuko, Goda Taro, Nakahata Kengo, Sano Hiroyuki, Hirano Shinya, Kitajima Hiroyuki
Department of Pediatric Surgery, Osaka, Japan.
Pediatr Int. 2013 Feb;55(1):39-43. doi: 10.1111/ped.12026.
Parenteral nutrition (PN)-associated liver dysfunction (PNALD) in term infants usually manifests as intrahepatic cholestasis, which recovers with enteral nutrition (EN) in most cases; however, as the number of extremely low-birthweight infants (ELBWI) has been increasing, and consequently intestinal diseases associated with ELBWI have been increasing, more intractable PNALD has been encountered after surgical treatment in ELBWI, which does not resolve or rather worsens with EN.
Three cases of ELBWI with intestinal perforation, which developed PNALD and eventually died of hepatic failure with intractable portal hypertension, were reviewed. Their gestational age and birthweight ranged from 23 to 26 weeks, and from 434 to 968 g, respectively. The intestinal diseases included necrotizing enteritis in two and meconium-related ileus with focal intestinal perforation in one.
The duration of total PN without EN in the three cases was 17, 24 and 24 days, respectively. The interval between the introduction of PN and the onset of PNALD was 14, 4 and 18 days, respectively. A marked elevation of serum endotoxin level was detected in both cases of necrotizing enteritis. Histopathological study of the liver revealed marked cholestasis, significant hepatic necrosis with fibrosis, and proliferation of ductules in all these cases, which was responsible for portal hypertension.
PN after gastrointestinal disorders in ELBWI may cause refractory PNALD, which does not resolve, or rather worsens with the resumption of EN. Portal hypertension secondary to hepatic necrosis may be responsible for the exacerbation with the resumption of EN.
足月儿肠外营养（PN）相关肝功能障碍（PNALD）通常表现为肝内胆汁淤积，多数情况下通过肠内营养（EN）可恢复；然而，随着极低出生体重儿（ELBWI）数量的增加，与之相关的肠道疾病也在增多，ELBWI术后出现了更多难治性PNALD，采用EN治疗后病情未缓解甚至加重。
回顾了3例ELBWI合并肠穿孔病例，这些患儿均发生了PNALD，最终死于肝衰竭及难治性门静脉高压。其胎龄和出生体重分别为23至26周和434至968克。肠道疾病包括2例坏死性小肠结肠炎和1例胎粪性肠梗阻伴局灶性肠穿孔。
3例患儿在未进行EN的情况下，全肠外营养的持续时间分别为17天、24天和24天。开始肠外营养至发生PNALD的间隔时间分别为14天、4天和18天。2例坏死性小肠结肠炎患儿均检测到血清内毒素水平显著升高。肝脏组织病理学研究显示，所有这些病例均有明显的胆汁淤积、伴有纤维化的显著肝坏死以及小胆管增生，这是导致门静脉高压的原因。
ELBWI胃肠道疾病后进行肠外营养可能导致难治性PNALD，病情不会缓解，甚至在恢复肠内营养后会加重。肝坏死继发的门静脉高压可能是恢复肠内营养后病情加重的原因。
