RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164070991
T1 Glycogen Storage Disease Type 0
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accesspediatrics.mhmedical.com/content.aspx?aid=1164070991
RD 2020/04/03
AB Inborn  error  of  metabolism  of  glucose  caused  by  decreased  glycogen  synthetase  activity  and  characterized  by  fasting  hypoglycemia,  high  blood  ketones,  increased  free  fatty  acids,  and  low  levels  of  alanine  and  lactate  beginning  in  early  infancy.  Conversely,  feeding  results  in  hyperglycemia  and  hyperlactatemia.  Unlike  other  GSDs,  GSD  0  does  not  result  in  tissue  accumulation  of  normal  or  abnormal  glycogen.  Although  glycogen  synthase  deficiency  does  not  result  in  storage  of  extra  glycogen  in  the  liver,  it  is  often  classified  as  a  glycogen  storage  disease  because  it  is  another  defect  of  glycogen  storage  and  can  cause  similar  problems.