Parenteral nutrition-associated liver disease in extremely low-birthweight infants with intestinal disease.
BACKGROUND
The aim of this study was to investigate factors associated with the development of parenteral nutrition-associated liver disease (PNALD) and to examine the clinicopathological relationship of PNALD in extremely low-birthweight infants (ELBWI).


METHODS
The subjects were 13 ELBWI who had received PN because of intestinal perforation or functional ileus between 2000 and 2013. We measured the serum levels of biochemical parameters, including aspartate aminotransferase, alanine aminotransferase, and direct bilirubin. Liver histopathology was examined in relation to outcome. The subjects were categorized into two groups on liver histopathology: F(+), development of hepatic fibrosis and necrosis with/without cholestasis; and F(-), no hepatic fibrosis.


RESULTS
Of 13 ELBWI, five died of hepatic failure, five died of sepsis, and the other three were alive at the time of the study. Of the five infants who died of hepatic failure, two developed fulminant hepatitis without cholestasis, and the other three developed chronic cholestasis and finally hepatic failure. Postmortem histopathology in F(+) indicated not only massive hepatic necrosis, but also massive hepatic fibrosis. These histopathological findings explained the clinical presentation of portal hypertension. There were significant differences in the fasting period after intestinal disease onset between the two groups.


CONCLUSIONS
The prolonged fasting with PN is responsible for severe hepatocellular necrosis with fibrosis and consequent lethal portal hypertension.
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Katsuhisa
K
Hirano
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Akio
A
Kubota
Clinical Laboratory Medicine and Anatomic Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Masahiro
M
Nakayama
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Hisayoshi
H
Kawahara
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Akihiro
A
Yoneda
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Yuko
Y
Tazuke
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Gakuto
G
Tani
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Tomohiro
T
Ishii
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Taro
T
Goda
Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Satoshi
S
Umeda
Neonatal Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Shinya
S
Hirno
Neonatal Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Jun
J
Shiraishi
Neonatal Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Hirnoyuki
H
Kitajima
D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007226:Infant Mortality / Q000639:trends; D052577:Infant, Extremely Low Birth Weight; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000175:diagnosis / Q000628:therapy*; D007410:Intestinal Diseases / Q000151:congenital* / Q000628:therapy; D007564:Japan / Q000453:epidemiology; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D010288:Parenteral Nutrition / Q000009:adverse effects*; D012189:Retrospective Studies
extremely low-birthweight infant; functional intestinal obstruction; hepatic failure; hepatic fibrosis; intestinal perforation; parenteral nutrition-associated liver disease