RT Book, Section
A1 O'Connor, Judith A.
A2 Bishop, Warren P.
SR Print(0)
ID 55943675
T1 Chapter 28. Metabolic and Drug-Induced Liver Disease
T2 Pediatric Practice: Gastroenterology
YR 2010
FD 2010
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-163379-6
LK accesspediatrics.mhmedical.com/content.aspx?aid=55943675
RD 2024/04/23
AB Metabolic  liver  disease  has  traditionally  referred  to  diseases  that  result  from  inborn  errors  of  metabolism.  These  disorders  are  due  to  a  single  enzyme  defect  that  affects  the  synthesis  or  catabolism  of  a  carbohydrate  (CHO),  protein,  or  lipid.  These  defects  in  metabolism  can  result  in  either  liver  failure  or  cirrhosis,  with  or  without  injury  to  other  tissues,  or  relative  sparing  of  the  liver  with  primary  toxicity  to  other  organ  systems  (Table  28–1).  Metabolic  disorders  that  arise  in  the  liver  with  primary  toxicity  to  other  organ  systems  are  not  addressed  in  this  chapter.  This  chapter  approaches  metabolic-induced  liver  disease  not  only  from  the  traditional  approach,  those  resulting  from  a  single  enzyme  defect,  but  also  as  a  genetic  susceptibility  induced  by  a  trigger,  such  as  a  drug  or  a  metabolic  derangement  associated  with  visceral  obesity  (nonalcoholic  fatty  liver  disease  (NAFLD)).