Thank you, I found this interesting. I know this relates to SIDS, but is it
something that continues as they get older? I have never forced my children
to go without milk so I could teach them to sleep through the night, but
what happens when they do?

Kathy
----- Original Message -----

> From: "Juhlmann" <juhlmann@...>
>
> Enzyme Deficiency Responsible for Some Sudden Death Cases
> August 30, 1999
> Pediatric and Developmental Pathology/MedscapeWire
>
> A deficiency in the SCHAD short-chain L-3-hydroxyacyl coenzyme A
> dehydrogenase enzyme is responsible for some infant deaths attributed to
> Sudden Infant Death Syndrome (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 clinical
> chemistry at Children's Medical Center of Dallas. The clinical
consequences
> of SCHAD deficiency -- an inborn error of metabolism -- occur when fatty
> acids from the stored fats enter the liver and fail to generate energy or
> to produce ketones. Ketones are a vital energy source for the brain
because
> it cannot use fatty acids, unlike the heart and skeletal muscle, which use
> fat directly.
> "In these infants, fat can get into the liver, but it can't be metabolized
> and released," Bennett said. "Hence infants' brains starve from the
deficit
> of ketones, and they become comatose." The researchers examined 150 cases
> for this study. While only 2 percent were due to SCHAD deficiency, the
> researchers hope their findings will prevent at least a small portion of
> the unexpected fatalities previously described as SIDS cases by developing
> immediate postnatal testing that would identify infants with the enzyme
> deficiency. According to the U.S. National Center for Infectious Diseases
> Centers for Disease Control, SIDS is the leading cause of death of
children
> 1 month to 1 year of age. In the United States, 5,000 to 6,000 infant
> deaths are attributed to SIDS each year.
> 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.
> "The knowledge of this diagnosis, along with other fatty-acid metabolic
> defects, may be very important to the families of affected infants because
> of the genetic nature of these abnormalities," Bennett said. "The clinical
> features of these disorders may be at least partially controlled if the
> diagnosis is made early in life, possibly preventing deaths due to these
> disorders."
> The full findings were published in the July/August issue of Pediatric and
> Developmental Pathology.
>