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Protocol Details

Maternal Inborn Errors of Metabolism in Pregnancy: A Pregnancy Registry Protocol

This study is NOT currently recruiting participants.

Summary | Eligibility | Citations | Contacts

Summary

Number

15-HG-0049

Sponsoring Institute

National Human Genome Research Institute (NHGRI)

Recruitment Detail

Type: Completed Study; data analyses ongoing
Gender: Female
Min Age: 14
Max Age: 50

Referral Letter Required

No

Population Exclusion(s)

Male

Keywords

Inborn Error of Metabolism;
Pregnancy;
Amino acid disorder;
Fatty acid oxidation disorder;
Organic Acidemia;
Natural History

Recruitment Keyword(s)

None

Condition(s)

Inborn Errors of Metabolism;
Pregnancy;
Acidemias

Investigational Drug(s)

None

Investigational Device(s)

None

Intervention(s)

None

Supporting Site

National Human Genome Research Institute

Background:

- People with inborn errors of metabolism can t turn food into energy the right way. This can affect a person s growth and health. Researchers want to know how this condition affects a pregnant woman and her baby.

Objectives:

- To collect data from the medical records of women with an inborn error of metabolism. Also, to create a pregnancy registry of inborn errors of metabolism.

Eligibility:

- Women with an inborn error of metabolism who either:

- have been pregnant in the past,

- are currently pregnant, or

- have recently talked with their doctor about becoming pregnant.

Design:

- This study will collect data only. No extra tests will be done.

- Participants will be in the study for the length of their pregnancy and for 1 year after delivery.

- Participants will answer questions about their family s health.

- The participant s doctor will send their medical records to researchers. These may include data about:

- Last health care visit before pregnancy

- Blood, urine, ultrasound, or lab results during pregnancy

- Delivery and recovery after delivery

- Researchers will ask for the test(s) used to confirm pregnancy.

- After the participant has her baby, researchers will ask for data about how the baby is doing. This may include when the baby is sitting, walking, talking, etc.

- The data will be placed into a database. The database will not include the participant s name or identifying data.

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Eligibility

INCLUSION CRITERIA:

- Patients clinically diagnosed with methylmalonic acidemia or another inborn error of metabolism are eligible to participate; mutational and enzymatic status is preferred but not required. Biochemical testing is required.

- Women with inborn errors of metabolism who have had a clinically documented prior pregnancy, currently are pregnant or planning a pregnancy.

EXCLUSION CRITERIA:

- Patients with phenylketonuria or hyperphenylalaninemia.

- Anyone unwilling to provide informed consent (for themselves as adults, or on behalf of their children as minors) or assent.

- Medical condition(s) or mental retardation are not in themselves reason for exclusion if in the judgment of the referring physician this would involve no more than minimal risk. We will make every effort to explain the study for the purpose of assent in a manner that the family feels is both age and developmentally appropriate for that child.

-We will review a clinical description from the referring physician about a potential research subject to determine that the subject is appropriate to enter into the study. We reserve the right to exclude cases that are not definitively diagnosed by metabolites and/or molecular genetics or related to our direct research interests. We expect this to be a rare minority.


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Citations:

Kuseyri O, Weissbach A, Bruggemann N, Klein C, Gi ewska M, Karall D, Scholl-B(SqrRoot) rgi S, Romanowska H, Krzywi ska-Zdeb E, Monavari AA, Knerr I, Yap c Z, Leuzzi V, Opladen T. Pregnancy management and outcome in patients with four different tetrahydrobiopterin disorders. J Inherit Metab Dis. 2018 Mar 28. doi: 10.1007/s10545-018-0169-0. [Epub ahead of print]

Raval DB, Merideth M, Sloan JL, Braverman NE, Conway RL, Manoli I, Venditti CP. Methylmalonic acidemia (MMA) in pregnancy: a case series and literature review. J Inherit Metab Dis. 2015 Sep;38(5):839-46. doi: 10.1007/s10545-014-9802-8. Epub 2015 Jan 8.

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Contacts:

Principal Investigator

Referral Contact

For more information:

Charles P. Venditti, M.D.
National Human Genome Research Institute (NHGRI)
NIHBC 10 - CLINICAL CENTER BG RM 7N248A
10 CENTER DR
BETHESDA MD 20892
(301) 496-6213
venditti@mail.nih.gov

Charles P. Venditti, M.D.
National Human Genome Research Institute (NHGRI)
NIHBC 10 - CLINICAL CENTER BG RM 7N248A
10 CENTER DR
BETHESDA MD 20892
(301) 496-6213
venditti@mail.nih.gov

Office of Patient Recruitment
National Institutes of Health Clinical Center (CC)
Building 61, 10 Cloister Court
Bethesda, Maryland 20892
Toll Free: 1-800-411-1222
Local Phone: 301-451-4383
TTY: TTY Users Dial 7-1-1
ccopr@nih.gov

Clinical Trials Number:

NCT02322177

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