TY  - CHAP
M1  - Book, Section
TI  - Chapter 149. Vitamin B6 and Vitamin B1 Responsive Disorders
A1  - Saudubray, Jean-Marie
A2  - Rudolph, Colin D.
A2  - Rudolph, Abraham M.
A2  - Lister, George E.
A2  - First, Lewis R.
A2  - Gershon, Anne A.
Y1  - 2011
N1  - 
T2  - Rudolph's Pediatrics, 22e
AB  - Thiamine  has  long  been  recognized  as  an  essential  component.  Its  minimal  essential  requirement  is  about  0.5  mg/1000  Kcal,  which  is  usually  covered  by  a  normal,  well-balanced  diet.  However,  requirements  are  variable  and  increase  in  parallel  with  carbohydrate  intake,  during  pregnancy,  lactation,  hypermetabolic  states,  and  in  infants.  Thiamine  acts  under  its  phosphorylated  form,  thiamine  pyrophosphate  (TPP),  which  is  the  coenzyme  of  pyruvate  decarboxylase  in  the  pyruvate  dehydrogenase  complex,  α-ketoglutarate  dehydrogenase,  and  oxidative  decarboxylation  of  the  three-branched  chain  alpha-keto  acids.  It  is  also  the  coenzyme  of  the  transketolase  in  the  pentose  phosphate  pathway.  Being  placed  at  these  highly  regulated  enzymatic  steps,  thiamine  plays  a  crucial  role  in  carbohydrate  metabolism  and  in  the  metabolic  switch  from  the  fed  to  the  fasting  state.  Acute  thiamine  deficiency  states  (as  in  total  parenteral  nutrition  without  thiamine  supplement)  are  life-threatening  emergencies  and  present  as  cardiac  failure,  Gayet-Wernicke  encephalopathy,  or  lactic  acidosis.1,2  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/24
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=6725849
ER  -