I seem to remember that, quite a long time ago, there was a question on
TIPS about the need for people with phenylketonuria to remain on a low-phenyalanine
diet for their entire lives.

There is an article in the current JAMA about the need for pregnant women
born with PKU to continue the diet during the pregnancy in order to prevent
mental retardation and microcephaly in the developing fetus (who is often
heterozygous with respect to the PKU gene). The article also discusses "barriers"
to continuing on the diet.

Jeff

------------------------

http://jama.ama-assn.org/issues/v287n10/ffull/jwr0313-2.html

Barriers to Dietary Control Among Pregnant Women With Phenylketonuria United
States, 1998-2000

MMWR. 2002;51:117-120

2 tables omitted

Newborns in the United States are screened for phenylketonuria (PKU), a
metabolic disorder that when left untreated is characterized by elevated
blood phenylalanine (phe) levels and severe mental retardation (MR). An
estimated 3,000-4,000 U.S.-born women of reproductive age with PKU have
not gotten severe MR because as newborns their diets were severely restricted
in the intake of protein-containing foods and were supplemented with medical
foods (e.g., amino acid-modified formula and modified low-protein foods).1-4
When women with PKU do not adhere to their diet before and during pregnancy,
infants born to them have a 93% risk for MR and a 72% risk for microcephaly.5,6
These risks result from the toxic effects of high maternal blood phe levels
during pregnancy, not because the infant has PKU.5, 6 The restricted diet,
which should be maintained for life, often is discontinued during adolescence.5-10
This report describes the pregnancies of three women with PKU and underscores
the importance of overcoming the barriers to maintaining the recommended
dietary control of blood phe levels before and during pregnancy. For maternal
PKU-associated MR to be prevented, studies are needed to determine effective
approaches to overcoming barriers to dietary control.....

[for rest of article, go to: http://jama.ama-assn.org/issues/v287n10/fful=
l/jwr0313-2.html]

--
Jeffry P. Ricker, Ph.D.      Office Phone: (480) 423-6213
9000 E. Chaparral Rd.      FAX Number: (480) 423-6298
Psychology Department    [EMAIL PROTECTED]
Scottsdale Community College
Scottsdale, AZ 85256-2626

Psychologists Educating Students to Think Skeptically (PESTS)
http://www.sc.maricopa.edu/sbscience/pests/index.html

The Psychology Student: Learning About The Science Of Psychology
http://www.sc.maricopa.edu/sbscience/psychscience/index.html














---
You are currently subscribed to tips as: [EMAIL PROTECTED]
To unsubscribe send a blank email to [EMAIL PROTECTED]

Reply via email to