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Hereditary AngioedemaBOISTERNNCT04957641

A Study of the Burden of Illness and Treatment Patterns in Teenagers and Adults With Hereditary Angioedema

An observational study of Hereditary Angioedema, run by Takeda at 36 sites in 22 countries.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
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Trial summary

This study is about teenagers and adults with hereditary angioedema (HAE) type I and type II. In this study, the burden of illness means the impact of HAE in terms of long-term health outcomes and the financial cost. Read more about this study

Who is it for?
Ages 12 years and older
Conditions the study is for
Hereditary Angioedema
How many people
221 participants
Where
36 sitesin 22 countries
Study dates
21 Apr 2022to 13 Jan 2023
Phase
Observational
Are there results?
None posted in the registry

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 12 years and older
  • All sexes
  • No healthy volunteers
  • Child, Adult, Older adult

Study population: Participants aged greater than or equal to (>=) 12 years with HAE type I or type II suffering from recurrent attacks will be enrolled in this study.

Who may be able to join

  • Participant aged >= 12 years at the time of the last documented HAE attack in the eligibility period.
  • Physician-confirmed diagnosis (or confirmation in medical records) of HAE type I or type II.
  • Participant had at least one documented HAE attack during the eligibility period.
  • Participant is not adequately or sub-optimally controlled according to treating physician.
  • Participant (or parent/legal guardian) is willing and able to comply with the study requirements and provides informed consent or assent.

Who cannot join

  • Participant enrolled in a therapeutic investigational drug or device trial during the observation period.
  • Participant initiated long-term prophylaxis with Takhzyro® at any time since diagnosis.

Trial locations and contacts

36 sites in 22 countries.

36 sites in 22 countries

Israel4 sites
  • Barzilai Medical Center

    Ashkelon

  • Bnai Zion Medical Center

    Haifa

  • Sheba Medical Center - PPDS

    Ramat Gan

  • Tel Aviv Sourasky Medical Center PPDS

    Tel Aviv

Other trials in Israel

Greece3 sites
  • Navy Hospital of Athens

    Athens, 115 21

  • Laiko General Hospital of Athens

    Athens, 115 27

  • University General Hospital of Larissa

    Larissa, 412 21

Other trials in Greece

Portugal3 sites
  • Centro Hospitalar E Universitário de Coimbra EPE

    Coimbra, 3004-561

  • Centro Hospitalar Lisboa Norte, E.P.E. - Hospital de Santa Maria

    Lisbon, 1649-028

  • Centro Hospitalar de São João, E.P.E.

    Porto, 4200-319

Other trials in Portugal

Spain3 sites
  • Hospital Universitario Cruces

    Barakaldo, 48903

  • Hospital Universitario Vall d'Hebron - PPDS

    Barcelona, 08035

  • Hospital Universitari i Politecnic La Fe de Valencia

    Valencia, 46026

Other trials in Spain

Canada2 sites
  • McMaster University Medical Centre

    Hamilton, ON L8N 3Z5

  • Vancouver Allergy Clinic

    Vancouver, British Columbia, BCV5Y1K2

Other trials in Canada

Croatia2 sites
  • General Hospital Sibenik

    Šibenik, 22000

  • Klinicki bolnicki centar Zagreb

    Zagreb, 10000

Other trials in Croatia

Czechia2 sites
  • Fakultni nemocnice Hradec Kralove

    Hradec Králové, 500 05

  • Fakultni nemocnice v Motole

    Prague, 150 06

Other trials in Czechia

Latvia2 sites
  • Pauls Stradins Clinical University Hospital

    Riga, LV-1002

  • Centre of Investigation and Treatment of Allergic Diseases

    Riga, LV-1003

Other trials in Latvia

Lithuania2 sites
  • Hospital of Lithuanian University of Health Sciences Kauno klinikos

    Kaunas, 50161

  • Vilnius University Hospital Santaros Klinikos

    Vilnius, 08410

Other trials in Lithuania

Austria1 site
  • Medizinische Universitat Wien (Medical University of Vienna)

    Vienna, 1090

Other trials in Austria

Belgium1 site
  • UZ Antwerpen

    Edegem, 2650

Other trials in Belgium

Bulgaria1 site
  • University Multiprofile Hospital for Active Treatment Aleksandrovska EAD

    Sofia, 1431

Other trials in Bulgaria

Estonia1 site
  • East Tallinn Central Hospital

    Tallinn, 10138

Other trials in Estonia

Germany1 site
  • Universitätsklinikum Frankfurt

    Frankfurt, 60590

Other trials in Germany

Hungary1 site
  • Semmelweis Egyetem

    Budapest, 1085

Other trials in Hungary

Ireland1 site
  • St James's Hospital

    Dublin, D08 NHY1

Other trials in Ireland

North Macedonia1 site
  • Medical Faculty of Ss. Cyril and Methodius Univers

    Skopje, 1000

Other trials in North Macedonia

Poland1 site
  • Wojskowy Instytut Medyczny

    Warsaw, 04-349

Other trials in Poland

Romania1 site
  • MediQuest Clinical Research Center

    Sângeorgiu de Mures, 547530

Other trials in Romania

Serbia1 site
  • University Clinical Center of Serbia - PPDS

    Belgrade, 11000

Other trials in Serbia

Slovakia1 site
  • Univerzitna nemocnica Martin

    Martin, 036 01

Other trials in Slovakia

Slovenia1 site
  • Bolnisnica Golnik - Klinicni Oddelek za Pljucne Bolezni In Alergijo

    Golnik, 4204

Other trials in Slovenia

About this study

Official title: A Retrospective Observational Chart Review Study Evaluating the Burden of Illness and Treatment Patterns in Hereditary Angioedema Type I and II

This study is about teenagers and adults with hereditary angioedema (HAE) type I and type II. In this study, the burden of illness means the impact of HAE in terms of long-term health outcomes and the financial cost.

The main aims of this study are as follows:

Read more
  • to learn how often, how severe and where on the body HAE attacks occur.
  • to describe how HAE prophylaxis and on-demand medicines are prescribed and used. (Prophylaxis medicines prevent a bleed from happening and on-demand medicines treat a bleed when it occurs.)

This study is about collecting data only; participants will not receive treatment as part of this study. Existing data available in the participant's medical records will be collected. Participants will be asked to complete an electronic questionnaire either on a website or by using an app on their mobile phone; data from this questionnaire will also be collected.

Participants do not need to visit their doctor in addition to their normal visits.

Medical subject headings
Angioedemas, Hereditary

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

Study groups in NCT04957641
Study groupRoleWhat they receive
Participants with HAEParticipants' data will be collected retrospectively from electronic medical records using chart review of de-identified data on participant demographics, HAE medical history and information on diagnostics, treatments and disease course assessments that are routinely performed in accordance with current guidelines and/or local standard of care which are entered into an Electronic data capture (EDC) system over multiple data collection waves up to 6 months.

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Number of Participants With HAE Attacks Occurrence During Study Period

    HAE attack is defined as the symptoms or signs consistent with an attack in at least 1 of the following locations: peripheral angioedema (cutaneous swelling involving an extremity, the face, neck, torso, and/or genitourinary region), abdominal angioedema (abdominal pain, with or without abdominal distention, nausea, vomiting, or diarrhea), laryngeal angioedema (stridor, dyspnea, difficulty speaking, difficulty swallowing, throat tightening, or swelling of the tongue, palate, uvula, or larynx). Number of participants with HAE attacks occurrence (start and end date of each HAE attack) during study period will be reported.

    Measured: Throughout the study period (up to 6 months)

  2. Number of Participants Categorized Based on Characteristics of HAE Attacks During Study Period

    HAE attack characteristics will be based on prodromal symptoms, severity and location, whether attack was life-threatening will be reported.

    Measured: Throughout the study period (up to 6 months)

  3. Time Interval Between HAE Attacks During Study Period

    Time interval between HAE attacks during the study period will be reported.

    Measured: Throughout the study period (up to 6 months)

  4. Duration of Each HAE Attacks During Study Period

    Duration of each HAE attacks during study period will be reported.

    Measured: Throughout the study period (up to 6 months)

  5. Number of Participants Based on Type of HAE Prophylactic Treatments

    Number of participants based on type of HAE prophylactic treatment will be reported.

    Measured: Throughout the study period (up to 6 months)

  6. Number of Participants Based on Long-term or Short-term HAE Prophylactic Treatment

    Number of participants based on long-term or short-term HAE prophylactic treatment will be reported.

    Measured: Throughout the study period (up to 6 months)

  7. Number of Participants Categorized Based on Reasons for HAE Prophylactic Treatment Initiation

    Number of participants categorized based on reasons for HAE prophylactic treatment initiation will be reported.

    Measured: Throughout the study period (up to 6 months)

  8. Duration of HAE Prophylactic Treatment

    Duration of HAE prophylactic treatment includes treatment start and end dates will be reported.

    Measured: Throughout the study period (up to 6 months)

  9. Number of Participants Categorized Based on Dose and Route of Administration for HAE Prophylactic Treatment

    Number of participants categorized based on dose and route of administration for HAE prophylactic treatment will be reported.

    Measured: Throughout the study period (up to 6 months)

  10. Number of Participants Categorized Based on Reasons for HAE Prophylactic Treatment Discontinuation

    Number of participants categorized based on reasons for HAE prophylactic treatment discontinuation will be reported.

    Measured: Throughout the study period (up to 6 months)

  11. Number of Participants Based on Type of HAE On-demand Treatment

    Number of participants based on type of HAE on-demand treatment will be reported.

    Measured: Throughout the study period (up to 6 months)

  12. Duration of HAE On-demand Treatment

    Duration of HAE on-demand treatment includes treatment start and end dates will be reported.

    Measured: Throughout the study period (up to 6 months)

  13. Number of Participants Categorized Based on Dose and Route of Administration for HAE On-demand Treatment

    Number of participants categorized based on dose, route of administration and setting of administration for HAE on-demand treatment will be reported.

    Measured: Throughout the study period (up to 6 months)

  14. Number of Participants Associated Between Prescriptions Patterns and HAE Attacks

    Number of participants with association between prescriptions patterns and HAE attacks related to frequency or severity. Generalized linear models (GLM) will be used to evaluate the association of outcomes of interest with relevant covariates between prescription pattern, HAE attacks, frequency and severity of HAE attacks will be reported.

    Measured: Throughout the study period (up to 6 months)

Secondary outcomes (11)
  1. Healthcare Resource Utilization (HRU): Number of Visits to Healthcare Professionals (HCP)

    HRU is measured by the number of visits to HCP will be reported.

    Measured: Throughout the study period (up to 6 months)

  2. HRU: Number of Participants With Emergency Room/Emergency Departments (ER/ED) Visits due to HAE Attacks

    Number of participants with ER/ED visits due to HAE attacks will be reported.

    Measured: Throughout the study period (up to 6 months)

  3. HRU: Number of Participants With Hospitalizations due to HAE Attacks

    Number of participants with hospitalizations due to HAE attacks will be reported.

    Measured: Throughout the study period (up to 6 months)

  4. Health-related Quality of Life (HRQoL): Angioedema Quality of Life (AE-QoL) Questionnaire

    The AE-QoL was developed to measure HRQoL in participants with recurrent angioedema. It is a self-administered patient-reported outcome instrument with a recall period of four weeks. There are 17 items across four domains: functioning (four items), fatigue/mood (five items), fears/shame (six items), and food (two items). Responses use a 5-point Likert scale ranging from 1 (Never) to 5 (Very Often). Global scores range from 0 to 100 and scores by domains range from 0 to 100. An overall score is calculated and a higher score indicates lower quality of life.

    Measured: Throughout the study period (up to 6 months)

  5. HRQoL: EuroQoL Group 5-Dimension 5-Level (EQ-5D-5L) Questionnaire

    The EQ-5D-5L descriptive system assesses health in five dimensions (Mobility, Self-Care, Usual Activities, Pain / Discomfort, Anxiety / Depression), each of which has five levels of response (no problems, slight problems, moderate problems, severe problems, extreme problems/unable to). This part of the EQ-5D questionnaire provides a descriptive profile that can be used to generate a health state profile. Health state index scores generally range from less than 0 (where 0 is the value of a health state equivalent to dead; negative values representing values as worse than dead) to 1 (the value of full health), with higher scores indicating higher health utility. The second part of the questionnaire consists of a visual analogue scale (VAS) on which the participant rates his/her perceived health from 0 (the worst imaginable health) to 100 (the best imaginable health).

    Measured: Throughout the study period (up to 6 months)

  6. HRQoL: EuroQoL-five Dimensions, Youth Version (EQ-5D-Y)

    The EQ-5D-Y is composed of two sections. The first section contains one question for each of the five dimensions: mobility, looking after myself, doing usual activities, having pain or discomfort, and feeling worried, sad, or unhappy. Participants select from three response levels (no problems, some problems, a lot of problems) for each dimension. A health state profile score can be calculated from the responses on these five questions. The second section includes the same visual analogue scale as the EQ-5D-5L. The scale is scored from 0-100. The reference to a high score indicates a better outcome of quality of life.

    Measured: Throughout the study period (up to 6 months)

  7. Number of Participants With HAE Attacks Occurrence in Relevant Subgroups

    HAE attack is defined as the symptoms or signs consistent with an attack in at least 1 of the following locations: peripheral angioedema (cutaneous swelling involving an extremity, the face, neck, torso, and/or genitourinary region), abdominal angioedema (abdominal pain, with or without abdominal distention, nausea, vomiting, or diarrhea), laryngeal angioedema (stridor, dyspnea, difficulty speaking, difficulty swallowing, throat tightening, or swelling of the tongue, palate, uvula, or larynx). Relevant subgroups is defined by disease characteristics (e.g., number of attacks) and treatment patterns (type of treatments for HAE). Number of participants with HAE attacks occurrence (start and end date of each HAE attack) in relevant subgroups will be reported.

    Measured: Throughout the study period (up to 6 months)

  8. Number of Participants Categorized Based on Characteristics of HAE Attacks in Relevant Subgroups

    HAE attack characteristics will be based on prodromal symptoms, severity and location, whether attack was life-threatening in relevant subgroups will be reported. Relevant subgroups is defined by disease characteristics (e.g., number of attacks) and treatment patterns (type of treatments for HAE).

    Measured: Throughout the study period (up to 6 months)

  9. Time Interval Between HAE Attacks in Relevant Subgroups

    Time interval between HAE attacks in relevant subgroups will be reported. Relevant subgroups is defined by disease characteristics (e.g., number of attacks) and treatment patterns (type of treatments for HAE).

    Measured: Throughout the study period (up to 6 months)

  10. Duration of Each HAE Attacks in Relevant Subgroups

    Duration of each HAE attacks in relevant subgroups will be reported. Relevant subgroups is defined by disease characteristics (e.g., number of attacks) and treatment patterns (type of treatments for HAE).

    Measured: Throughout the study period (up to 6 months)

  11. HAE Attacks Prescription Pattern in Relevant Subgroups

    Prescription pattern of HAE attacks medication by drug class in relevant participant subgroups during study period. Relevant subgroups is defined by disease characteristics (e.g., number of attacks) and treatment patterns (type of treatments for HAE).

    Measured: Throughout the study period (up to 6 months)

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted12 Jul 2021
  2. Study start21 Apr 2022
  3. Primary completion13 Jan 2023
  4. Study completion13 Jan 2023

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Observational
Observational model
Cohort
Time perspective
Retrospective
Enrolment
221 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT04957641
Sponsor's protocol ID
TAK-743-4009
Other IDs
MACS-2020-080401
Acronym
BOISTERN
Status last verified
Jun 2023
First submitted
5 Jul 2021
Last updated
26 Jun 2023
Results posted
No
Data monitoring committee
No
FDA-regulated drug
No
FDA-regulated device
No
Individual data sharing
Yes: Takeda provides access to the de-identified individual participant data (IPD) for eligible studies to aid qualified researchers in addressing legitimate scientific objectives (Takeda's data sharing commitment is available on https://clinicaltrials.takeda.com/takedas-commitment?commitment=5). These IPDs will be provided in a secure research environment following approval of a data sharing request, and under the terms of a data sharing agreement.

Publications

  • Farkas H, Aygoren-Pursun E, Ebo DG, Bara N, Psarros F, Gavini F, Bent-Ennakhil N, Sayegh L, Andresen I. Real-world treatment patterns and burden-of-disease of sub-optimally controlled hereditary angioedema. World Allergy Organ J. 2025 Sep 1;18(9):101100. doi: 10.1016/j.waojou.2025.101100. eCollection 2025 Sep.

    PubMed 40949786

Questions about this trial

What is this clinical trial studying?

An observational study of Hereditary Angioedema, run by Takeda at 36 sites in 22 countries.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 5 inclusion and 2 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 26 Jun 2023. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 36 sites in Israel, Greece, Portugal, Spain, Canada and 17 more countries. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

Are results available for this study?

The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Number of Participants With HAE Attacks Occurrence During Study Period (time frame: Throughout the study period (up to 6 months)). The study lists 14 primary outcomes in all. It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT04957641, which also lists 1 linked publication.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 26 Jun 2023.

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