NCT00778206

Brief Summary

The objective of this study is to evaluate the safety of long-term treatment with Kuvan.

Trial Health

87
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
1,887

participants targeted

Target at P75+ for all trials

Timeline
Completed

Started Sep 2008

Longer than P75 for all trials

Geographic Reach
1 country

45 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Start

First participant enrolled

September 1, 2008

Completed
2 months until next milestone

First Submitted

Initial submission to the registry

October 21, 2008

Completed
2 days until next milestone

First Posted

Study publicly available on registry

October 23, 2008

Completed
12.3 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

January 29, 2021

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

January 29, 2021

Completed
Last Updated

October 12, 2022

Status Verified

September 1, 2022

Enrollment Period

12.4 years

First QC Date

October 21, 2008

Last Update Submit

October 11, 2022

Conditions

Keywords

PhenylketonuriaHyperphenylalaninemiaPKUPhenylalanine

Outcome Measures

Primary Outcomes (1)

  • Observational Data Only

    Registry data including demographic and baseline characteristics

    15 years

Study Arms (2)

1. PKUDOS Registry

Patients with a confirmed diagnosis of Phenylketonuria (PKU) with hyperphenylalaninemia who have either received Kuvan therapy, or currently receive Kuvan therapy, or intend to begin receiving Kuvan therapy within 90 days of entering the registry.

2. PKU MOMS Subregistry

Patients with PKU who are pregnant at enrollment in the registry or who become pregnant while participating in the registry.

Drug: Kuvan

Interventions

KuvanDRUG

-Recommended post-partum Assessments include: collection of baby's plasma at Birth, 1 Month and 6 months, and collection of mother's plasma and expressed breast milk sample at 1 Month.

Also known as: sapropterin dihydrochloride
2. PKU MOMS Subregistry

Eligibility Criteria

Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

Diagnosis of Phenylketonuria with hyperphenylalaninemia

You may qualify if:

  • Patient has confirmed diagnosis of PKU with hyperphenylalaninemia documented by a Phenylalanine level of greater than or equal to 360 umol/L (6 mg/dL)
  • Patient has previously received Kuvan
  • Patient is currently receiving Kuvan
  • Patient intends to receive Kuvan therapy within 90 days of enrollment into the registry
  • The Patient is being followed at a PKUDOS participating center
  • Willing and able to provide written authorization or, if under the age of 18 years, provide written assent (if required) and written patient authorization by a parent or legal guardian
  • Willing to provide personal health information

You may not qualify if:

  • Patients are not eligible to participate in PKUDOS if they are participating in a BioMarin-sponsored clinical study of Kuvan
  • Patients not previously treated with Kuvan and patients that are unwilling to begin Kuvan therapy within 90 days of entry into the registry
  • PKU MOMS Subregistry
  • Willing to enroll in (or are already enrolled in) PKUDOS
  • Agree to follow the standard of care for pregnant women with PKU in the United States (NIH, 200, NIH Consensus Statement)
  • Agree to be followed by a hospital or PKU clinic offering the standard of care for maternal PKU
  • Are within 10 weeks of their last menstrual period
  • Patients who have not adhered to the standard of care for pregnant women with PKU in the United States are not eligible to participate in PKU MOMS

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (45)

Childrens Hospital of Los Angeles

Los Angeles, California, 90027, United States

Location

LAC and USC Medical Center

Los Angeles, California, 90033, United States

Location

UCLA

Los Angeles, California, 90095, United States

Location

Children's Hospital of Orange County

Orange, California, 92868, United States

Location

University of California, San Diego

San Diego, California, 92093, United States

Location

Stanford University, Pediatrics

Stanford, California, 94305, United States

Location

Children's Hospital, University of Colorado School of Medicine

Aurora, Colorado, 80045, United States

Location

Children's National Medical Center

Washington D.C., District of Columbia, 20010, United States

Location

University of Florida

Gainesville, Florida, 32611, United States

Location

University of Miami, Miller School of Medicine, Department of Human Genetics

Miami, Florida, 33136, United States

Location

University of South Florida

Tampa, Florida, 33606, United States

Location

Tampa Children's Hospital, St. Joseph's Pediatric Endocrine Associates

Tampa, Florida, 33607, United States

Location

Emory University

Decatur, Georgia, 30033, United States

Location

Ann and Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, 60611, United States

Location

Indiana University School of Medicine

Indianapolis, Indiana, 46202, United States

Location

University of Iowa

Iowa City, Iowa, 52242, United States

Location

Wesley Pediatric Faculty Clinic

Wichita, Kansas, 67208, United States

Location

University of Kentucky

Lexington, Kentucky, 40536, United States

Location

Kosair Charities Pediatric Clinical Research Unit

Louisville, Kentucky, 40202, United States

Location

Tulane University Medical School

New Orleans, Louisiana, 70112, United States

Location

Johns Hopkins University School of Medicine

Baltimore, Maryland, 21287, United States

Location

Children's Hosptial of Boston

Boston, Massachusetts, 02115, United States

Location

University of Minnesota

Minneapolis, Minnesota, 55455, United States

Location

University of Missouri Health Care

Columbia, Missouri, 65212, United States

Location

St. Louis Children's Hospital

St Louis, Missouri, 63110, United States

Location

University of Nebraska

Omaha, Nebraska, 68182, United States

Location

AHS Hospital Corp

Morristown, New Jersey, 07960, United States

Location

Albany Medical Center

Albany, New York, 12208, United States

Location

University of Rochester Medical Center

Rochester, New York, 14642, United States

Location

Westchester Medical Center

Valhalla, New York, 10595, United States

Location

University of North Carolina

Chapel Hill, North Carolina, 27599, United States

Location

Akron Children's Hospital

Akron, Ohio, 44308, United States

Location

Nationwide Children's Hospital

Columbus, Ohio, 43205, United States

Location

University of Oklahoma

Oklahoma City, Oklahoma, 73104, United States

Location

Oregon Health and Science University

Portland, Oregon, 97239, United States

Location

Penn State, Milton S. Hershey Medical Center

Hershey, Pennsylvania, 17033, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

St. Christophers Hospital for Children

Philadelphia, Pennsylvania, 19134, United States

Location

Children's Hospital of Pittsburgh

Pittsburgh, Pennsylvania, 15224, United States

Location

Greenwood Genetic Center

Greenwood, South Carolina, 29646, United States

Location

Sanford Children's Specialty Clinic

Sioux Falls, South Dakota, 57117, United States

Location

University of Utah

Salt Lake City, Utah, 84132, United States

Location

Children's Hospital of the King's Daughters

Norfolk, Virginia, 23507, United States

Location

West Virginia Genetics Center, Department of Pediatrics

Morgantown, West Virginia, 26506, United States

Location

Children's Hospital of Wisconsin

Milwaukee, Wisconsin, 53226, United States

Location

MeSH Terms

Conditions

Phenylketonurias

Interventions

sapropterin

Condition Hierarchy (Ancestors)

Brain Diseases, Metabolic, InbornBrain Diseases, MetabolicBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesAmino Acid Metabolism, Inborn ErrorsMetabolism, Inborn ErrorsGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesMetabolic DiseasesNutritional and Metabolic Diseases

Study Officials

  • Kristin Lindstrom, MD

    BioMarin Pharmaceutical

    STUDY DIRECTOR

Study Design

Study Type
observational
Observational Model
CASE ONLY
Time Perspective
PROSPECTIVE
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

October 21, 2008

First Posted

October 23, 2008

Study Start

September 1, 2008

Primary Completion

January 29, 2021

Study Completion

January 29, 2021

Last Updated

October 12, 2022

Record last verified: 2022-09

Locations