Understanding the Long-Term Management of Organic Acidemia Patients With CARBAGLU®: A Mixed Methods Approach

NCT ID: NCT04176523

Last Updated: 2024-02-05

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

RECRUITING

Total Enrollment

95 participants

Study Classification

OBSERVATIONAL

Study Start Date

2019-01-15

Study Completion Date

2029-07-30

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

This is a prospective mixed-design study focused on the long-term management of propionic aciduria (PA) and methylmalonic aciduria (MMA) with N-carbamylglutamate (NCG) maintenance therapy. Treatment characteristics, clinical outcomes, and healthcare utilization data of patients diagnosed PA or MMA treated \>6 months therapy with NCG are collected at baseline, 12 months, 18 months, 36 months and 54 months. Qualitative interviews with adult patients and caregivers are conducted \>6 months after study enrollment to gain a better understanding of the disease burden and the treatment burden of patients and their families.

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

Study Design: This is a prospective, longitudinal, observational study of patients diagnosed with organic acidemia that has been on treatment with carglumic acid for a minimum of six months for the long-term management of their disease. This prospective observational study population will include up to 85 diagnosed MMA or PA patients with current carglumic acid treatment experience from 6 European countries: Sweden, the United Kingdom (UK), the Netherlands, Germany, Norway, Italy, Spain and France. The patients (or caregivers) included in this prospective observational study will also be invited to participate in a one-on-one phone interview.

Study Procedures: After Institutional Review Board (IRB)/Independent Ethics Committee (IEC) approval is obtained for the protocol and all supporting protocol documents, clinicians caring for patients with MMA or PA will provide their patients (or caregivers) with the opportunity to participate in the study. Patients (or caregivers) will receive an Information Letter (PIL) (Appendices A and B) inviting them (or their caregiver) to take part in a prospective observational study and in a one-on-one phone interview.

Patients (or caregivers) who are interested in participating in this observational/interview study will review the informed consent form (Appendices C and D) with the clinician and his/her research team. If agreeable, patients (or caregivers) will be asked to sign the consent form and a countersigned copy will be provided to them for their records. Clinicians and their research team at each site will then review patient charts and record demographic and medical history information for each patient. Patient information from medical charts will be collected at the start of the study (baseline), and 12 months and 18 months, and 36 months, and 54 months after baseline. All information derived from medical chart reviews will be recorded through web-based Electronic Data Capture system (EDC).

Patients (or caregivers) who agree to participate in a one-on-one phone interview will provide contact information for their clinicians and the respective research team to include in the Contact Information Form (CIF) (Appendix E). Researchers will then contact patients (or caregivers) directly to schedule a one-hour phone interview. An interview guide (Appendices F and G) including open-ended questions and probes will be used to elicit concepts and orient the discussion during the interview. Patient (or caregiver) interviews will focus on describing the symptoms and impacts of MMA or PA, as well as patient treatment experiences with carglumic acid, their treatment preferences, and their satisfaction with treatment. At the end of the interview, patients (or caregivers) will be asked to complete a Demographic and Health Information Form (DHIF; Appendices H and I). Phone interviews will be audio-recorded with the patient's (or caregiver) permission, transcribed and translated (as needed) in preparation for qualitative analysis.

Data Analysis: The primary prospective data analysis will involve a comparison of the incidence and duration of decompensation episodes pre- and post- initiation of carglumic acid. Additional analysis will include healthcare resource utilization around individual decompensation events, patient/caregiver burden, and patient/caregiver satisfaction with treatment. All prospective data will be undertaken using SAS® software, version 9.4 of the SAS® system for Windows (Cary, NC, USA). All qualitative data from the interviews will be analyzed using ATLAS.ti, a software package uniquely designed to analyze qualitative data. The audio-recorded verbatim transcripts of the interviews will be coded to identify patterns in participants' responses concerning patient experiences with the symptoms and impacts of MMA or PA, treatment experience with carglumic acid, patient treatment preferences, and treatment satisfaction. Findings will be summarized in a final report and may be published or presented by the investigator(s) after the review by, and in consultation and agreement with, the sponsor. If published or presented, the results will be presented in such a way that confidential or proprietary information is not disclosed.

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

Methylmalonic Acidemia Propionic Acidemia

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Observational Model Type

CASE_ONLY

Study Time Perspective

PROSPECTIVE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

Methylmalonic_acidemia

Patients with confirmed diagnosis of methylmalonic acidemia, and treated with carglumic acid, at any dose form, any dosage,

Carglumic Acid

Intervention Type DRUG

Maintenance therapy with carglumic acid

Propionic_Acidemia

Patients with confirmed diagnosis of propionic acidemia, and treated with carglumic acid, at any dose

Carglumic Acid

Intervention Type DRUG

Maintenance therapy with carglumic acid

Interventions

Learn about the drugs, procedures, or behavioral strategies being tested and how they are applied within this trial.

Carglumic Acid

Maintenance therapy with carglumic acid

Intervention Type DRUG

Other Intervention Names

Discover alternative or legacy names that may be used to describe the listed interventions across different sources.

Carbaglu

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

Inclusion Criteria

1. Patient has confirmed diagnosis of an organic acidemia (e.g., MMA or PA)
2. Patient initiated treatment with carglumic acid for long-term management of MMA or PA
3. Patient has been treated with carglumic acid for a minimum of 6 months
4. Patient (or caregiver) is able to comply with all prospective study procedures
5. Patient (or caregiver) is able to provide informed consent

Exclusion Criteria

* None
Minimum Eligible Age

6 Months

Maximum Eligible Age

99 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

Recordati Rare Diseases

INDUSTRY

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Responsibility Role SPONSOR

Principal Investigators

Learn about the lead researchers overseeing the trial and their institutional affiliations.

Sufin Yap, MD

Role: PRINCIPAL_INVESTIGATOR

Sheffield Children's NHS Trust

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

Hopital Des Enfants, CHU de Bordeaux-GH Pellegrin

Bordeaux, , France

Site Status RECRUITING

Hopital de la Conception

Marseille, , France

Site Status RECRUITING

Hôpital de la Timone

Marseille, , France

Site Status RECRUITING

CHU de Nancy - Hopitaux de Brabois

Nancy, , France

Site Status RECRUITING

Hôpital Necker Enfants Malades

Paris, , France

Site Status RECRUITING

CHU de Rouen

Rouen, , France

Site Status RECRUITING

Nouvel Hopital Civil

Strasbourg, , France

Site Status RECRUITING

Hopital de Hautepierre

Strasbourg, , France

Site Status RECRUITING

Klinikum Bremen Mitte, Prof. Hess Kinderklinik

Bremen, , Germany

Site Status RECRUITING

Azienda Ospedaliero Universitaria Meyer

Florence, , Italy

Site Status RECRUITING

San Paola Hospital, ASST Santi Paolo e Carlo

Milan, , Italy

Site Status RECRUITING

Milan Policlinico University

Milan, , Italy

Site Status RECRUITING

Azienda Ospedaliera San Gerardo Monza

Monza, , Italy

Site Status RECRUITING

Azienda Ospedaliera Universitaria Federico II

Napoli, , Italy

Site Status RECRUITING

University Hospital of Padova

Padua, , Italy

Site Status RECRUITING

Turin University Hospital

Turin, , Italy

Site Status RECRUITING

Oslo University Hospital

Oslo, , Norway

Site Status RECRUITING

AMM hospital val d'hebron

Barcelona, , Spain

Site Status RECRUITING

Sant Joan de Deu

Barcelona, , Spain

Site Status RECRUITING

Universitario de Cruces

Bilbao, , Spain

Site Status RECRUITING

Hospital Infantil Universitario Nino Jesus

Madrid, , Spain

Site Status RECRUITING

Hospital 12 de Octubre

Madrid, , Spain

Site Status RECRUITING

Hospital Universitario La Paz

Madrid, , Spain

Site Status RECRUITING

Universitario Ramon y Cajal Madrid

Madrid, , Spain

Site Status RECRUITING

Hospital Universitario Virgen de la Arrixaca

Murcia, , Spain

Site Status RECRUITING

Hospital Universitario Son Espases

Palma de Mallorca, , Spain

Site Status RECRUITING

Hospital Universitario Virgen del Rocio

Seville, , Spain

Site Status RECRUITING

Universitario Miguel Servet

Zaragoza, , Spain

Site Status RECRUITING

NÄL

Trollhättan, , Sweden

Site Status TERMINATED

Guy's and St. Thomas' Hospital NHS Foundation Trust

London, , United Kingdom

Site Status RECRUITING

Great Ormond Street Hospital

London, , United Kingdom

Site Status RECRUITING

Sheffield Children's Hospital NHS Foundation Trust

Sheffield, , United Kingdom

Site Status RECRUITING

Countries

Review the countries where the study has at least one active or historical site.

France Germany Italy Norway Spain Sweden United Kingdom

Central Contacts

Reach out to these primary contacts for questions about participation or study logistics.

Vincenzo Giordano, MD

Role: CONTACT

33 1 79911282

Facility Contacts

Find local site contact details for specific facilities participating in the trial.

Delphine Lamireau, MD

Role: primary

05.56.79.56.46

Karin Mazodier, MD

Role: primary

04.91.38.23.79

Hôpital de la Timone

Role: primary

Francois Feillet, MD

Role: primary

03.83.15.47.96

Hôpital Necker Enfants Malades

Role: primary

Stephanie Torre, MD

Role: primary

02.32.88.07.66

Esther Noel, MD

Role: primary

03.88.11.62.52

Marie-Therese Abi-Warde, MD

Role: primary

03.88.12.83.28

Alexandra Horbe-Blindt

Role: primary

Maria Alice Donati, MD

Role: primary

Sabrina Paci, MD

Role: primary

Milan Policlinico University

Role: primary

Serena Gasperini, MD

Role: primary

Giancarlo Parenti, MD

Role: primary

University Hospital of Padova

Role: primary

Turin University Hospital

Role: primary

Trine Tangeraas, MD

Role: primary

0047 23 07 78 20

AMM hospital val d'hebron

Role: primary

Sant Joan de Deu

Role: primary

Universitario de Cruces

Role: primary

Elvira Canedo-Villarroya, MD

Role: primary

Consuelo Pedron-Giner, MD

Role: backup

0034 915 03 59 33

Elena Martin-Hernandez, MD

Role: primary

0034 913 90 88 86

Ana Morais-Lopez, MD

Role: primary

0034 917 27 73 01

Universitario Ramon Ramon y Cajal Madrid

Role: primary

David Gil-Ortega, MD

Role: primary

0034 609 08 23 44

Angeles Ruiz-Gomez, MD

Role: primary

0034 659 71 96 81

Maria Bueno-Delgado, MD

Role: primary

0034 616 11 98 97

Universitario Miguel Servet

Role: primary

Roshini Vara, MD

Role: primary

+44 ‭(0)20 7188 9412‬

James Davidson, MD

Role: primary

44 207 4059200

Sufin Yap, MD

Role: primary

Laura Looby

Role: backup

‭‭‭+44 (0)115 ‭‭‭924 9924

References

Explore related publications, articles, or registry entries linked to this study.

Burlina A, Bettocchi I, Biasucci G, Bordugo A, Gasperini S, La Spina L, Maines E, Meli C, Menni F, Paci S, Procopio E, Rossi A, Rubert L, Spada M, Tubili F, Tummolo A. Long-term use of carglumic acid in methylmalonic aciduria, propionic aciduria and isovaleric aciduria in Italy: a qualitative survey. Eur Rev Med Pharmacol Sci. 2022 Jul;26(14):5136-5143. doi: 10.26355/eurrev_202207_29302.

Reference Type BACKGROUND
PMID: 35916811 (View on PubMed)

Alfadhel M, Nashabat M, Saleh M, Elamin M, Alfares A, Al Othaim A, Umair M, Ahmed H, Ababneh F, Al Mutairi F, Eyaid W, Alswaid A, Alohali L, Faqeih E, Almannai M, Aljeraisy M, Albdah B, Hussein MA, Rahbeeni Z, Alasmari A. Long-term effectiveness of carglumic acid in patients with propionic acidemia (PA) and methylmalonic acidemia (MMA): a randomized clinical trial. Orphanet J Rare Dis. 2021 Oct 11;16(1):422. doi: 10.1186/s13023-021-02032-8.

Reference Type BACKGROUND
PMID: 34635114 (View on PubMed)

Kido J, Matsumoto S, Nakamura K. Carglumic Acid Contributes to a Favorable Clinical Course in a Case of Severe Propionic Acidemia. Case Rep Pediatr. 2020 Mar 9;2020:4709548. doi: 10.1155/2020/4709548. eCollection 2020.

Reference Type BACKGROUND
PMID: 32231837 (View on PubMed)

Kiykim E, Oguz O, Duman C, Zubarioglu T, Cansever MS, Zeybek ACA. Long-term N-carbamylglutamate treatment of hyperammonemia in patients with classic organic acidemias. Mol Genet Metab Rep. 2021 Jan 30;26:100715. doi: 10.1016/j.ymgmr.2021.100715. eCollection 2021 Mar.

Reference Type BACKGROUND
PMID: 33552909 (View on PubMed)

Tummolo A, Melpignano L, Carella A, Di Mauro AM, Piccinno E, Vendemiale M, Ortolani F, Fedele S, Masciopinto M, Papadia F. Long-term continuous N-carbamylglutamate treatment in frequently decompensated propionic acidemia: a case report. J Med Case Rep. 2018 Apr 22;12(1):103. doi: 10.1186/s13256-018-1631-1.

Reference Type BACKGROUND
PMID: 29679984 (View on PubMed)

Burlina A, Cazzorla C, Zanonato E, Viggiano E, Fasan I, Polo G. Clinical experience with N-carbamylglutamate in a single-centre cohort of patients with propionic and methylmalonic aciduria. Mol Genet Metab Rep. 2016 Jul 13;8:34-40. doi: 10.1016/j.ymgmr.2016.06.007. eCollection 2016 Sep.

Reference Type BACKGROUND
PMID: 27489777 (View on PubMed)

Yap S, Lamireau D, Feillet F, Ruiz Gomez A, Davison J, Tangeraas T, Giordano V. Real-World Experience of Carglumic Acid for Methylmalonic and Propionic Acidurias: An Interim Analysis of the Multicentre Observational PROTECT Study. Drugs R D. 2024 Mar;24(1):69-80. doi: 10.1007/s40268-023-00449-z. Epub 2024 Jan 10.

Reference Type BACKGROUND
PMID: 38198106 (View on PubMed)

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

17180

Identifier Type: -

Identifier Source: org_study_id

More Related Trials

Additional clinical trials that may be relevant based on similarity analysis.

Miglustat in Niemann-Pick Type C Disease
NCT00517153 COMPLETED PHASE2