NCT03481738

Brief Summary

This study is an observational (ie, noninterventional), longitudinal, multicenter, global registry for patients with pyruvate kinase (PK) deficiency, a rare nonspherocytic hemolytic anemia. This Registry will be open for enrollment for 7 years and all enrolled participants will be followed prospectively for a minimum of 2 years, and up to 9 years. Data will be collected from participating Registry Physicians, participants, and, where appropriate, parents/guardians who have provided informed consent or assent (where relevant) and authorization pursuant to applicable laws and regulations. Data should include demographic, clinical, and treatment data; and other data of relevance to the management of patients with PK deficiency. Annual chart review and data entry are expected in order to enhance longitudinal understanding of PK deficiency; however, no specific protocol schedule of assessment is required by this Registry protocol.

Trial Health

87
On Track

Trial Health Score

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

Enrollment
500

participants targeted

Target at P75+ for all trials

Timeline
13mo left

Started Apr 2018

Longer than P75 for all trials

Geographic Reach
16 countries

52 active sites

Status
active not recruiting

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 Progress88%
Apr 2018May 2027

First Submitted

Initial submission to the registry

March 22, 2018

Completed
7 days until next milestone

First Posted

Study publicly available on registry

March 29, 2018

Completed
25 days until next milestone

Study Start

First participant enrolled

April 23, 2018

Completed
9.1 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

May 31, 2027

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

May 31, 2027

Last Updated

April 23, 2026

Status Verified

April 1, 2026

Enrollment Period

9.1 years

First QC Date

March 22, 2018

Last Update Submit

April 22, 2026

Conditions

Keywords

Glycolytic enzymopathyCongenital Nonspherocytic hemolytic anemia (CNSA)PKD

Outcome Measures

Primary Outcomes (1)

  • Clinical Course of PK Deficiency

    To develop an understanding of the longitudinal clinical implications of PK deficiency, including disease natural history, treatments and outcomes, and variability in clinical care and disease burden.

    9 years

Secondary Outcomes (4)

  • Severity of Disease

    9 years

  • Disease Impact on Pregnancy

    9 years

  • Clinical Management Assistance

    9 years

  • Global Repository

    9 years

Other Outcomes (1)

  • Genetic

    9 years

Study Arms (1)

PKD Diagnosed

Participants diagnosed with PK deficiency by the presence of 2 or more PKLR gene mutations as well as clinical features.

Eligibility Criteria

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

Patients will be recruited or referred by physicians who treat hemolytic anemias at approximately 60 sites in approximately 20 countries.

You may qualify if:

  • Participants of all ages with a confirmed diagnosis of PK deficiency via genetic testing are eligible to enroll;
  • Participants will be considered for enrollment on the basis of clinical features consistent with PK deficiency together with the presence of 2 or more PKLR gene mutations. For novel or indeterminate PKLR gene mutations, participants will be deemed eligible if, in the opinion of the investigator, the reported PKLR gene mutations are sufficient to support a diagnosis of PK deficiency;
  • The participant or the parent/guardian of the participant must be willing and able to give written informed consent and/or assent. E-consent or remote consent may be utilized where permissible as applicable if country regulations and site policies allow.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (52)

Phoenix Childrens Hospital

Phoenix, Arizona, 85016, United States

Location

Arkansas Children's Hospital

Little Rock, Arkansas, 72202, United States

Location

University of Arkansas for Medical Sciences

Little Rock, Arkansas, 72205, United States

Location

Children's Hospital of Orange County

Orange, California, 92868, United States

Location

Stanford University Medical Center

Palo Alto, California, 94304, United States

Location

Children's Healthcare of Atlanta

Atlanta, Georgia, 30342, United States

Location

Massachusetts General Hospital

Boston, Massachusetts, 02114, United States

Location

Boston Children's Hospital

Boston, Massachusetts, 02115, United States

Location

UMass Memorial Medical Center

Worcester, Massachusetts, 01655-0002, United States

Location

Children's Hospital of Michigan

Detroit, Michigan, 48201, United States

Location

Duke University Medical Center

Durham, North Carolina, 27710, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

St Jude Children's Research Hospital

Memphis, Tennessee, 38105, United States

Location

Primary Children's Hospital

Salt Lake City, Utah, 84113, United States

Location

University of Vermont Medical Center

Burlington, Vermont, 05401, United States

Location

Saint Josephs Healthcare System

Hamilton, Ontario, L8N 4A6, Canada

Location

Toronto General Hospital

Toronto, Ontario, M5G 2C4, Canada

Location

St. Justine Hospital

Montreal, Quebec, H3T 1C5, Canada

Location

Fakultni nemocnice Olomouc

Olomouc, 779 00, Czechia

Location

Ustav hematologie a krevni transfuze

Prague, 128 20, Czechia

Location

Fakultni nemocnice v Motole

Prague, 150 06, Czechia

Location

Copenhagen University Hospital

Herlev, 2730, Denmark

Location

Hopital Necker

Paris, 75743, France

Location

Charite - Universitatsmedizin Berlin

Berlin, 13353, Germany

Location

Evangelisches Krankenhaus Bielefeld gGmbH

Bielefeld, 33617, Germany

Location

Universitatsklinikum Heidelberg

Heidelberg, 69120, Germany

Location

Kinder- und Jugendarztpraxis

Munich, 81377, Germany

Location

Universitatsklinikum Wurzburg

Würzburg, 97080, Germany

Location

St James's Hospital

Dublin, D08 NHY1, Ireland

Location

Presidio Ospedaliero di Pescara

Pescara, Abruzzo, 65125, Italy

Location

AOU dell'Universita degli Studi della Campania Luigi Vanvitelli

Naples, Campania, Italy

Location

E O Ospedali Galliera

Genoa, Liguria, 16128, Italy

Location

Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico

Milan, 20122, Italy

Location

Ospedale S Eugenio

Roma, 144, Italy

Location

Universitair Medisch Centrum Utrecht

Utrecht, 3508 GA, Netherlands

Location

Centro Hospitalar E Universitario de Coimbra EPE

Coimbra, 3041-853, Portugal

Location

Centro Hospitalar Lisboa Central- Hospital Dona Estefania

Lisbon, 1169-045, Portugal

Location

Centro Hospitalar de Vila Nova de Gaia / Espinho E.P.E

Porto, 4200-072, Portugal

Location

The Catholic University of Korea, Seoul St. Mary's Hospital

Seoul, South Korea

Location

Hospital Universitario Germans Trias i Pujol

Badalona, Barcelona, 08916, Spain

Location

Hospital Sant Joan de Deu - PIN

Esplugues de Llobregat, Barcelona, 08950, Spain

Location

Hospital Universitario Vall d'Hebron - PPDS

Barcelona, 08035, Spain

Location

Hospital de La Santa Creu i Sant Pau

Barcelona, 8041, Spain

Location

Hospital Infantil Universitario Nino Jesus

Madrid, 28009, Spain

Location

Hospital Universitario La Paz

Madrid, 28046, Spain

Location

Hospital de Tortosa Verge de la Cinta

Tortosa, 43500, Spain

Location

Centre Hospitalier Universitaire Vaudois

Lausanne, CH 1011, Switzerland

Location

Siriraj Hospital Mahidol University

Bangkok, Thailand

Location

Hacettepe University Medical Faculty

Ankara, Turkey (Türkiye)

Location

Hammersmith Hospital

London, London, City of, W12 0HS, United Kingdom

Location

Kings College Hospital

London, SE5 9RS, United Kingdom

Location

The Newcastle Upon Tyne Hospitals NHS Foundation Trust

Newcastle upon Tyne, NE1 4LP, United Kingdom

Location

Related Publications (1)

  • Grace RF, van Beers EJ, Vives Corrons JL, Glader B, Glenthoj A, Kanno H, Kuo KHM, Lander C, Layton DM, Pospisilova D, Viprakasit V, Li J, Yan Y, Boscoe AN, Bowden C, Bianchi P. The Pyruvate Kinase Deficiency Global Longitudinal (Peak) Registry: rationale and study design. BMJ Open. 2023 Mar 23;13(3):e063605. doi: 10.1136/bmjopen-2022-063605.

Related Links

MeSH Terms

Conditions

Pyruvate Kinase Deficiency of Red CellsAnemia, Hemolytic, Congenital Nonspherocytic

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, CongenitalAnemia, HemolyticAnemiaHematologic DiseasesHemic and Lymphatic DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

  • Eva Gallagher, VP, Medical Affairs

    Agios Pharmaceuticals, Inc.

    STUDY CHAIR

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Target Duration
2 Years
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

March 22, 2018

First Posted

March 29, 2018

Study Start

April 23, 2018

Primary Completion (Estimated)

May 31, 2027

Study Completion (Estimated)

May 31, 2027

Last Updated

April 23, 2026

Record last verified: 2026-04

Locations