RT Book, Section
A1 Roth, Karl S.
A1 Berry, Gerard T.
A2 Sarafoglou, Kyriakie
A2 Hoffmann, Georg F.
A2 Roth, Karl S.
SR Print(0)
ID 1140315020
T1 Disorders of Fructose Metabolism
T2 Pediatric Endocrinology and Inborn Errors of Metabolism, 2e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9780071773140
LK accesspediatrics.mhmedical.com/content.aspx?aid=1140315020
RD 2024/04/24
AB Fructose  is  a  quantitatively  important  source  of  carbohydrates,  especially  in  the  Western  diet.  It  is  a  component  of  table  sugar  (sucrose,  Glc-Fru  disaccharide)  and  is  contained  in  large  amounts  in  honey,  fruits,  and  various  vegetables.  Fructose,  sucrose,  and  sorbitol  (metabolized  mainly  via  fructose)  are  frequent  food  additives.  A  special  concern  is  sucrose-containing  infant  formulas  which  may  elicit  a  potentially  fatal  response  in  an  affected  newborn.  Fructose  also  constitutes  the  main  carbohydrate  in  seminal  fluid.  The  average  daily  dietary  intake  by  Americans  was  estimated  to  be  100  g.1,2  In  the  last  century  fructose  and  sorbitol  were  used  as  carbohydrates  in  parenteral  nutrition,  for  example,  equimolar  solutions  of  glucose  and  fructose  prepared  by  hydrolysis  of  sucrose  as  “invert  sugar.”  IV  solutions  containing  fructose  or  sorbitol  can  be  toxic  to  patients  with  fructose  intolerance  and  should  no  longer  be  used.  Oral  fructose  is  absorbed  in  the  small  intestine  and  mainly  metabolized  in  the  liver.  Significant  fructose  metabolism  also  takes  place  in  the  kidney  and  the  small  intestine,  explaining  the  organ  manifestations  of  genetic  diseases  of  fructose  metabolism  (see  below).