seizure prevention in individuals with epilepsy
control of glucose levels in people with diabetes
Precautions
Liver damage may reduce the rate at whichmag-
nesiumis removed from the body. People with liver
damage (e.g. cirrhosis) may be at higher risk of devel-
oping symptoms of manganese excess.
Interactions
Antacids and laxatives that contain magnesium
(e.g. milk of magnesia) may reduce the amount of
manganese absorbed from food.
Complications
No complications are expected from manganese
acquired through food and water. Individuals who
take multivitamin/mineral supplements containing
manganese are unlikely to have any adverse effects.
People who take manganese or joint supplements
should be alert to how much manganese they are con-
suming, although overdose is extremely rare.
Parental concerns
Parents should have few concerns about children
getting either too much or too little manganese. Sup-
plemental manganese should rarely be necessary.
Parents should encourage their children to eat a diet
high in fruits, vegetables, and whole grains.
Resources
BOOKS
Fragakis, Allison.The Health Professional’s Guide to Popu-
lar Dietary Supplements. Chicago: American Dietetic
Association, 2003
Lieberman, Shari and Nancy Bruning.The Real Vitamin and
Mineral Book: The Definitive Guide to Designing Your
Personal Supplement Program,4th ed. New York:
Avery, 2007.
Pressman, Alan H. and Sheila Buff.The Complete Idiot’s
Guide to Vitamins and Minerals,3rd ed. Indianapolis,
IN: Alpha Books, 2007.
ORGANIZATIONS
Linus Pauling Institute. Oregon State University, 571
Weniger Hall, Corvallis, OR 97331-6512. Telephone:
(541) 717-5075. Fax: (541) 737-5077. Website:http://
lpi.oregonstate.edu
Office of Dietary Supplements, National Institutes of Health.
6100 Executive Blvd., Room 3B01, MSC 7517, Bethesda,
MD 20892-7517 Telephone: (301)435-2920. Fax:
(301)480-1845. Website:http://dietary-supplements.
info.nih.gov
OTHER
Familydoctor.org. ‘‘Vitamins and Minerals: What You
Should Know.’’ American Family Physician, December
2006.<http://familydoctor.org/863.xml>
Higdon, Jane. ‘‘Manganese.’’ Linus Pauling Institute-Oregon
State University, August 8, 2001.<http://lpi.oregonstate.
edu/infocenter/minerals/manganese>
Maryland Medical Center Programs Center for Integrative
Medicine. ‘‘Manganese.’’ University of Maryland
Medical Center, April 2002.<http://www.umm.edu/
altmed/ConsSupplements/manganesecs>
Mayo Clinic Staff. ‘‘Dietary Supplements: Using Vitamin
and Mineral Supplements Wisely.’’ MayoClinic.com,
June 5, 2006.<http://www.mayoclinic.com/health/
supplements/NU00198>Helen M. DavidsonMaple syrup urine disease
Definition
Maple syrup urine disease (MSUD), which is also
known as branched-chain ketoaciduria, branched-
chain alpha-keto acid dehydrogenase deficiency, or
BCKD deficiency, is a rare but potentially fatal inher-
ited metabolic disorder (IMD) passed down in an
autosomal recessive pattern. The special diet associ-
ated with MSUD is alow-protein dietcharacterized
by restriction of a specific amino acid known as leu-
cine; the use of high-calorie liquid or gel formulas that
are free of branched-chain amino acids (BCAAs); and
frequent monitoring of the BCAA levels in the
patient’s blood plasma. Strict adherence to this diet
is necessary to prevent developmental delays, mental
retardation, and recurrent metabolic crises leading to
respiratory failure and death.Origins
MSUD was first reported in 1954 by J. H.
Menkes, a pediatrician, and his colleagues. The family
in Menkes’s case study had lost four infants within the
first 3 months of life to a previously undescribed
degenerative disorder of the nervous system. The
urine of these infants smelled like maple syrup or
burned sugar, whence the disease got its name of
maple syrup urine disease or MSUD. An effective
treatment, however, had to await further biochemical
analysis of the metabolic dysfunction underlying the
disease. In 1960, a researcher named Dancis estab-
lished that the metabolic block in MSUD is caused
by an insufficient supply of an enzyme that helps toMaple syrup urine disease