Study of a Phenylalanine Restricted Diet During Pregnancy to Prevent Symptoms in Offspring of Patients With Phenylketonuria

This study has been completed.

First Received: August 3, 2000 Last Updated: June 23, 2005 History of Changes

Sponsor:	National Center for Research Resources (NCRR)
Collaborator:	University of Texas
Information provided by:	Office of Rare Diseases (ORD)
ClinicalTrials.gov Identifier:	NCT00006142

Purpose

OBJECTIVES:

I. Assess the impact of a phenylalanine restricted diet during pregnancy on symptoms in offspring of patients with phenylketonuria.

Condition	Intervention
Phenylketonuria	Behavioral: phenylalanine restricted diet

Study Type:	Interventional
Study Design:	Prevention

Resource links provided by NLM:

Genetics Home Reference related topics: argininosuccinic aciduria citrullinemia N-acetylglutamate synthase deficiency ornithine translocase deficiency phenylketonuria succinic semialdehyde dehydrogenase deficiency tetrahydrobiopterin deficiency

MedlinePlus related topics: Diets Phenylketonuria

Drug Information available for: Phenylalanine

U.S. FDA Resources

Further study details as provided by Office of Rare Diseases (ORD):

Study Start Date:

December 1983

Detailed Description:

PROTOCOL OUTLINE: This is a multicenter study.

Patients receive a phenylalanine restricted diet based on a medical food such as Phenyl-free, Lofenalac, PKU 3, or Maximum XP (may be supplemented with tyrosine as needed) to achieve a target blood phenylalanine level of 2-6 mg/dL. The diet begins approximately 3 months prior to conception and continues until delivery of the baby. Patients are evaluated once a month before conception and weekly during pregnancy. Patients also undergo sonographic examination at 8, 20, 28, and 34 weeks gestation. At birth, the cord blood is evaluated for plasma amino acids. If the baby is found to have an elevated phenylalanine level, blood and urine are obtained to determine the baby's genetic status. Phenylalanine and tyrosine levels are checked in the baby daily for three days after birth.

The baby is followed for physical and mental development at 3 and 6 months and then annually thereafter.

Eligibility

Genders Eligible for Study:	Female
Accepts Healthy Volunteers:	No

Criteria

Women with diagnosis of phenylketonuria who are planning on becoming pregnant Slightly elevated phenylalanine level (hyperphe variant) allowed

Contacts and Locations

Please refer to this study by its ClinicalTrials.gov identifier: NCT00006142

Sponsors and Collaborators

National Center for Research Resources (NCRR)

University of Texas

Investigators

Study Chair:

Bobbye M. Rouse

University of Texas

More Information

No publications provided

Study ID Numbers:	199/15326, UTMB-83-188
Study First Received:	August 3, 2000
Last Updated:	June 23, 2005
ClinicalTrials.gov Identifier:	NCT00006142 History of Changes
Health Authority:	United States: Federal Government

Keywords provided by Office of Rare Diseases (ORD):

inborn errors of metabolism
phenylketonuria
rare disease

Additional relevant MeSH terms:

Metabolism, Inborn Errors
Metabolic Diseases
Genetic Diseases, Inborn
Amino Acid Metabolism, Inborn Errors
Nervous System Diseases

Central Nervous System Diseases
Brain Diseases, Metabolic, Inborn
Phenylketonurias
Brain Diseases
Brain Diseases, Metabolic

ClinicalTrials.gov processed this record on November 27, 2009