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PN-associated Liver Disease after Intestinal Perforation in ELBW Infants: Consequent Lethal Portal Hypertension.
MedLine Citation:
PMID:  23240986     Owner:  NLM     Status:  Publisher    
BACKGROUND: Parenteral nutrition (PN)-associated liver dysfunction (PNALD) in term infants usually manifests as intrahepatic cholestasis (IHC), which recovers with enteral nutrition (EN) in most cases. However, as the number of extremely-low-birth-weight infants (ELBWI) has been increasing, and consequently intestinal diseases associated with ELBWI has been increasing, more intractable PNALD has been encountered after surgical treatment in ELBWI, which does not resolve or rather worsens with EN. PATIENTS AND METHODS: 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 birth weight ranged from 23 to 26 weeks, and 434 to 968 grams, respectively. The intestinal diseases included necrotizing enteritis (NEC) in two and meconium-related ileus (MRI) with focal intestinal perforation (FIP) in one. RESULTS: The duration of total PN without EN in the three cases was17, 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 NEC. Histopathologial 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. CONCLUSIONS: PN after gastrointestinal disorders in ELBW infants 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.
Kio Kubota; Narutaka Mochizuki; Jun Shiraishi; Masahiro Nakayama; Hisayoshi Kawahara; Akihiro Yoneda; Yuko Tazuke; Taro Goda; Kengo Nakahata; Hiroyuki Sano; Shinya Hirano; Hiroyuki Kitajima
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-12-13
Journal Detail:
Title:  Pediatrics international : official journal of the Japan Pediatric Society     Volume:  -     ISSN:  1442-200X     ISO Abbreviation:  Pediatr Int     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-12-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100886002     Medline TA:  Pediatr Int     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.
Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
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