This is interesting and may explain why my infants would stop breathing so
much and things why my youngest slept for 21 hrs straight one time. I
thought it was so great as I already had 3 others and one screamed nonstop.
I should have been scared. Thanks for sharing this. Linda S.

-----Original Message-----
From: Juhlmann <juhlmann@...>
To: mito <mito@onelist.com>; ca list <mitochondrial@onelist.com>
Cc: iem <iem-family@onelist.com>
Date: Sunday, September 05, 1999 8:14 AM
Subject: [iem-family] FYI - found it interesting


>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.
>
>
>--------------------------- ONElist Sponsor ----------------------------
>
>GET WHAT YOU DESERVE! A NextCard Platinum VISA: DOUBLE Rewards points,
>NO annual fee & rates as low as 9.9 percent FIXED APR. Apply online today!
> <a href=" http://clickme.onelist.com/ad/nextcard5 ">Click Here</a>
>
>------------------------------------------------------------------------
>IEM FAMILY FORUM
> -----------------------
> Travel Savings Alert
> http://bestbuystravel.com
>--------------------------
> Find It Now
> http://clickpick.com/
>-------------------------
> My Weather
> http://clickpick.com/hw2/myweather.cgi
>----------------------------------------
> Stop Smoking
> http://just4u.com/forums/cgi/show.cgi?26/26.html
>