TY  - CHAP
M1  - Book, Section
TI  - Metabolic and Genetic Disorders of the Liver
A1  - Feldman, Amy G.
A1  - Suchy, Frederick J.
A2  - Kline, Mark W.
Y1  - 2018
N1  - 
T2  - Rudolph's Pediatrics, 23e
AB  - The  liver  plays  a  central  role  in  the  biosynthesis,  degradation,  and  regulation  of  carbohydrates,  lipids,  amino  acids,  trace  elements,  and  vitamins  (see  Chapter  413).  Thus,  the  liver  is  involved  primarily  or  secondarily  in  many  inborn  errors  of  metabolism.  In  certain  inborn  errors  of  metabolism  (such  as  hereditary  tyrosinemia),  the  absence  of  a  critical  enzyme  may  cause  an  accumulation  of  toxic  metabolites.  In  other  disorders  (such  as  inborn  error  of  bile  acid  metabolism),  progressive  liver  injury  may  occur  because  of  failure  to  produce  essential  compounds  such  as  bile  acids.  Severe  liver  injury  may  also  result  from  a  third  mechanism,  sequestration  of  an  abnormally  synthesized  product  within  the  liver,  as  observed  in  α1-antitrypsin  deficiency.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/24
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1182909131
ER  -