Enzyme Deficiency Responsible for Some Sudden
Death Cases, August 30, 1999, Pediatric and Developmental
Pathology/MedscapeWire
http://pediatrics.medscape.com/11592.rhtml
A deficiency in the SCHAD short-chain L-3-hydroxyacyl coenzyme A
dehydrogenase enzyme is responsible for some infant deaths attributed to
(SIDS). The deficiency occurs during the breakdown of fatty acids derived
from fat stores in the body. The body uses these fats as an energy source
when its normal energy supply of glucose, which is converted into glycogen,
is used up. In the event of fasting, infants tend to exhaust their limited
glycogen supply quickly and begin using the stored fat, said Dr. Michael J.
Bennett, a professor of pathology and pediatrics at UT Southwestern Medical
Center and director of clinicalchemistry at Children's Medical Center of
Dallas. <snip>
"In these infants, fat can get into the liver, but it can't be metabolized
and released," ... "Hence infants' brains starve from the deficit of
ketones, and they become comatose." <snip>
Based on their results so far, the researchers advocate avoiding any
circumstances that would induce a fast in an infant. When an infant ingests
fewer calories than normally consumed, the liver will not produce the needed
ketones, and glucose will be used up too quickly, precipitating a
potentially fatal coma.
<snip>The full findings were published in the July/August issue of Pediatric
and Developmental Pathology.

Laurie Wheeler, RN, MN, IBCLC
Violet Louisiana, s.e.USA


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