NCT01889862

Brief Summary

The BMN 165 clinical development program has been designed to demonstrate the safety and efficacy of BMN 165 in reducing blood Phe concentrations in adults with PKU.

Trial Health

87
On Track

Trial Health Score

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

Enrollment
215

participants targeted

Target at P25-P50 for phase_3

Timeline
Completed

Started Jul 2013

Longer than P75 for phase_3

Geographic Reach
1 country

29 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

First Submitted

Initial submission to the registry

June 18, 2013

Completed
13 days until next milestone

First Posted

Study publicly available on registry

July 1, 2013

Completed
28 days until next milestone

Study Start

First participant enrolled

July 29, 2013

Completed
2.5 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

January 13, 2016

Completed
3.1 years until next milestone

Study Completion

Last participant's last visit for all outcomes

February 5, 2019

Completed
5 months until next milestone

Results Posted

Study results publicly available

July 12, 2019

Completed
Last Updated

May 21, 2021

Status Verified

April 1, 2021

Enrollment Period

2.5 years

First QC Date

June 18, 2013

Results QC Date

June 22, 2018

Last Update Submit

April 29, 2021

Conditions

Keywords

PKUPEG-PALPrismBioMarinrAvPAL-PEGBMN165

Outcome Measures

Primary Outcomes (1)

  • Change From Baseline in the Blood Plasma Phenylalanine (Phe) Concentration at Part 2

    Blood phe concentration in subjects previously exposed to BMN165 who were administered BMN165 20 or 40 mg/day compared with those who were administered a matching placebo.

    At Part 2 baseline and change from baseline at Part 2 week 8

Secondary Outcomes (5)

  • Change From Baseline in the Cognitive and Mood Symptoms Measured by ADHD Rating Scale (RS)-IV Inattention Subscale at Part 2

    At Part 2 baseline and change from baseline at Part 2 week 8

  • Change From Baseline in the Blood Plasma Phenylalanine (Phe) Concentration at Part 4

    At Part 4 week 57, 105, 161, 209 and 249

  • Change From Baseline in the Cognitive and Mood Symptoms Measured by PKU POMS TMD at Part 2

    At Part 2 baseline and change from baseline at Part 2 week 8

  • Change From Baseline in the Cognitive and Mood Symptoms Measured by PKU POMS Confusion Subscale at Part 2

    At Part 2 baseline and change from baseline at Part 2 week 8

  • Change From Baseline in the Cognitive and Mood Symptoms Measured by POMS TMD at Part 2

    At Part 2 baseline and change from baseline at Part 2 week 8

Study Arms (4)

BMN165 20mg/day

ACTIVE COMPARATOR

BMN165 20mg/day self-administered daily

Drug: BMN165 20mg/day

20 mg/day Placebo

PLACEBO COMPARATOR

20 mg/day Placebo self-administered daily

Drug: Placebo

BMN165 40mg/day

ACTIVE COMPARATOR

BMN165 40mg/day self-administered daily

Drug: BMN165 40mg/day

40 mg/day Placebo

PLACEBO COMPARATOR

40 mg/day Placebo self-administered daily

Drug: Placebo

Interventions

BMN165 20mg/day self-administered daily

Also known as: PEG-PAL
BMN165 20mg/day

BMN165 40mg/day self-administered daily

Also known as: PEG-PAL
BMN165 40mg/day

Non-drug placebo, self-administered daily

Also known as: Dextran 40
20 mg/day Placebo40 mg/day Placebo

Eligibility Criteria

Age18 Years - 70 Years
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • Individuals eligible to participate in this study must meet all of the following criteria:
  • Have completed a prior BMN 165 study (PAL-003, 165-205, or 165-301) prior to screening
  • Have had a stable BMN 165 dose regimen for at least 14 days prior to screening
  • Are at least 18 y/o and no older than 70 y/o at screening
  • Subjects who are \< 18 y/o and are already enrolled into Study 165-301 under Amendment #1 (10JAN2014) may enroll into this study
  • Has identified a person who is \> 18 y/o who has the neurocognitive and linguistic capacities to comprehend and complete the Profile of Mood States (POMS)-Observer rated scale
  • Has identified a competent person(s) \> 18 y/o who can observe the subject during study drug administration at certain points in the study
  • A home healthcare nurse may perform the study drug observations
  • Are willing and able to provide written, signed informed consent after the nature of the study has been explained and prior to any research-related procedures; for minors, parent or guardian provides written consent and assent may be requested
  • Are willing and able to comply with all study procedures
  • For females of childbearing potential, a negative pregnancy test at screening and willing to have additional pregnancy tests during the study
  • If sexually active, willing to use two acceptable methods of contraception during and for 4 weeks after the study
  • Males post vasectomy for 2 years with no known pregnancies do not need to use any other forms of contraception during the study.
  • Females who have been in menopause for at least 2 years, have had a tubal ligation at least 1 year prior to screening, or have had a total hysterectomy do not need to use any other forms of contraception during the study.
  • Have received documented approval from a study dietitian confirming that the subject is capable of maintaining their diet
  • +3 more criteria

You may not qualify if:

  • Use of any investigational product (except BMN 165) or investigational medical device within 30 days prior to screening or requirement for any investigational agent prior to completion of all scheduled study assessments
  • Use of any medication (except BMN 165) intended to treat PKU, including the use of large neutral amino acids, within 2 days prior to the administration of study drug
  • Have known hypersensitivity to Dextran® or components of Dextran
  • Use or planned use of any injectable drugs containing PEG (except for BMN 165), including medroxyprogesterone injection, within 3 months prior to screening and during study participation
  • Current use of levodopa
  • A positive test for HIV antibody, hepatitis B surface antigen, or hepatitis C antibody
  • A history of organ transplantation or taking chronic immunosuppressive therapy
  • A history of substance abuse in the past 12 months or current alcohol or drug abuse
  • Current participation in the Kuvan registry study (PKUDOS). Patients may discontinue the PKUDOS registry trial to allow enrollment in this study
  • Pregnant or breastfeeding at screening or planning to become pregnant (self or partner) or breastfeed at any time during the study
  • Concurrent disease or condition that would interfere with study participation or safety.
  • Major surgery planned during the study period
  • Any condition that in the view of the investigator, places the subject at high risk of poor treatment compliance or terminating early from the study
  • Alanine aminotransferase (ALT) concentration at least 2x the upper limit of normal
  • Creatinine at least 1.5x the upper limit of normal

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (29)

University of California, San Diego Clinical and Translational Research Institute

La Jolla, California, 92093, United States

Location

UCSF Benioff Children's Hospital Oakland

Oakland, California, 94609, United States

Location

Children's Hospital Colorado

Aurora, Colorado, 80045, United States

Location

University of Florida Clinical Research Center

Gainesville, Florida, 32610, United States

Location

University of South Florida

Tampa, Florida, 33606, United States

Location

Emory Universty, Department of Human Genetics, Division of Medical Genetics

Atlanta, Georgia, 30322, United States

Location

Ann and Robert H Lurie Children's Hospital of Chicago

Chicago, Illinois, 60611, United States

Location

Indiana CTSI Clinical Research Center

Indianapolis, Indiana, 46202, United States

Location

University of Kentucky Medical Center

Lexington, Kentucky, 40536, United States

Location

University of Louisville, Kosair Charities Pediatric Clinical Research Unit

Louisville, Kentucky, 40202, United States

Location

Boston Children's Hospital

Boston, Massachusetts, 02115, United States

Location

Wayne State University Clinical Research Center at the Mott Center

Detroit, Michigan, 48202, United States

Location

University of Missouri Health Center

Columbia, Missouri, 65212, United States

Location

Washington University Center for Applied Research Sciences

St Louis, Missouri, 63110, United States

Location

University of Nebraska Medical Center

Omaha, Nebraska, 68198, United States

Location

Cooper Health Systems

Camden, New Jersey, 08103, United States

Location

Atlantic Health System - Morristown Medical Center

Morristown, New Jersey, 07960, United States

Location

Albany Medical Center

Albany, New York, 12208, United States

Location

Icahn School of Medicine at Mount Sinai Medical Center

New York, New York, 10029, United States

Location

University Hospitals Case Medical Center

Cleveland, Ohio, 44106, United States

Location

University of Oklahoma Health Sciences Center

Oklahoma City, Oklahoma, 73104, United States

Location

Oregon Health and Science University

Portland, Oregon, 97239, United States

Location

St. Christopher's Hospital for Children

Philadelphia, Pennsylvania, 19134, United States

Location

Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center

Pittsburgh, Pennsylvania, 15224, United States

Location

Vanderbilt Children's Hospital

Nashville, Tennessee, 37232, United States

Location

University of Texas Houston Medical School

Houston, Texas, 77030, United States

Location

University of Utah Hospital

Salt Lake City, Utah, 84108, United States

Location

University of Washinton

Seattle, Washington, 98195, United States

Location

Medical College of Wisconsin

Milwaukee, Wisconsin, 53226, United States

Location

Related Publications (1)

  • Bilder DA, Arnold GL, Dimmock D, Grant ML, Janzen D, Longo N, Nguyen-Driver M, Jurecki E, Merilainen M, Amato G, Waisbren S. Improved attention linked to sustained phenylalanine reduction in adults with early-treated phenylketonuria. Am J Med Genet A. 2022 Mar;188(3):768-778. doi: 10.1002/ajmg.a.62574. Epub 2021 Nov 26.

Related Links

MeSH Terms

Conditions

Phenylketonurias

Interventions

pegvaliaseDextrans

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

Intervention Hierarchy (Ancestors)

GlucansBiopolymersPolymersMacromolecular SubstancesPolysaccharidesCarbohydrates

Results Point of Contact

Title
Debra Lounsbury/Sr Principle Scientist, Clinical Sciences
Organization
BioMarin Pharmaceutical Inc

Study Officials

  • Clinical Trial Specialist

    BioMarin Pharmaceutical

    STUDY DIRECTOR

Publication Agreements

PI is Sponsor Employee
No
Restriction Type
GT60
Restrictive Agreement
Yes

Study Design

Study Type
interventional
Phase
phase 3
Allocation
RANDOMIZED
Masking
TRIPLE
Who Masked
PARTICIPANT, CARE PROVIDER, INVESTIGATOR
Purpose
TREATMENT
Intervention Model
PARALLEL
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

June 18, 2013

First Posted

July 1, 2013

Study Start

July 29, 2013

Primary Completion

January 13, 2016

Study Completion

February 5, 2019

Last Updated

May 21, 2021

Results First Posted

July 12, 2019

Record last verified: 2021-04

Data Sharing

IPD Sharing
Will not share

Locations