HoFH, the International Clinical Collaborators Registry

NCT ID: NCT04815005

Last Updated: 2025-12-18

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

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Recruitment Status

RECRUITING

Total Enrollment

1000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2017-01-24

Study Completion Date

2026-12-31

Brief Summary

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Homozygous familial hypercholesterolemia (HoFH), a rare inherited disorder caused by bi-allelic mutations in the LDL Receptor pathway, is characterized by extremely elevated levels of low-density lipoprotein cholesterol (LDL-C) from birth and premature atherosclerotic cardiovascular disease (ASCVD). Our current knowledge about HoFH is disjointed and largely stems from relatively small case series and expert opinion. HICC (Homozygous FH International Clinical Collaborators) is a global consortium of clinicians who are contributing de-identified data of patients diagnosed with HoFH with the goal to advance our understanding of this rare disease.

Detailed Description

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The HICC registry is an observational, multicenter, international registry collecting de-identified clinical and genetic information from patients with homozygous Familial Hypercholesterolemia (HoFH) worldwide.

Patients are eligible to be enrolled in the registry based on the diagnosis of HoFH by the treating clinician, irrespective of how the diagnosis was made. To generate up-to-date data reflecting current rather than historic practice, patients who died or were lost to follow-up prior to 2010 are excluded.

Anonymized data on demographics, type of HoFH diagnosis (clinical and/or based on the results of a genetic test), genetic results, (cardiovascular) medical history, relevant family history, physical examination, laboratory measurements, lipid lowering treatment and cardiovascular imaging are collected for 3 different time points: at diagnosis, at enrolment and at time of best lipid profile (if this is different from time at enrolment). Data are collected using pre-definite electronic case report forms to ensure uniformity of data collected. Primary analysis will be cross-sectional (e.g. based on country of residence, age, etc)

Conditions

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Homozygous Familial Hypercholesterolemia

Keywords

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Homozygous Familial Hypercholesterolemia Autosomal Dominant Hypercholesterolemia Autosomal Recessive Hypercholesterolemia Registry

Study Design

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Observational Model Type

CASE_ONLY

Study Time Perspective

CROSS_SECTIONAL

Study Groups

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HoFH

Patients diagnosed with HoFH by their physicians, either based on clinical or genetic criteria.

Demographics, diagnosis type, genotype, lipid profile, treatment allocation, country of residence.

Intervention Type OTHER

Differences in diagnosis, genotype, lipid profile treatment allocation among HoFH patients worldwide.

Interventions

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Demographics, diagnosis type, genotype, lipid profile, treatment allocation, country of residence.

Differences in diagnosis, genotype, lipid profile treatment allocation among HoFH patients worldwide.

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of homozygous familial hypercholesterolemia (HoFH) clinically of genetically determined

Exclusion Criteria

* No diagnosis of HoFH
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University of Amsterdam

OTHER

Sponsor Role collaborator

University of Cape Town

OTHER

Sponsor Role collaborator

University of Witwatersrand, South Africa

OTHER

Sponsor Role collaborator

University of Pennsylvania

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Marina Cuchel, MD, PhD

Role: PRINCIPAL_INVESTIGATOR

University of Pennsylvania

Locations

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University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

Department of Vascular Medicine, Amsterdam UMC

Amsterdam, , Netherlands

Site Status RECRUITING

Department of Medicine, Division of Lipidology and Hatter Institute for Cardiovascular Research in Africa, University of Cape Town

Cape Town, , South Africa

Site Status RECRUITING

c. Carbohydrate and Lipid Metabolism Research Unit, Faculty of Health Sciences, University of Witwatersrand

Johannesburg, , South Africa

Site Status RECRUITING

Countries

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United States Netherlands South Africa

Central Contacts

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Marina Cuchel, MD, PhD

Role: CONTACT

Phone: 2156627188

Email: [email protected]

Lauren Vincent, MRA

Role: CONTACT

Phone: 2156155448

Email: [email protected]

Facility Contacts

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Marina Cuchel, MD, PhD

Role: primary

Lauren Vincent, MRA

Role: backup

G. Kees Hovingh, MD, PhD

Role: primary

Dirk J Blom

Role: primary

Frederick J Raal

Role: primary

References

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Mulder JWCM, Schonck WAM, Tromp TR, Reijman MD, Reeskamp LF, Hovingh GK, Blom DJ, Roeters van Lennep JE. Real-world family planning and pregnancy practices in women with homozygous familial hypercholesterolemia. Atherosclerosis. 2025 May;404:119187. doi: 10.1016/j.atherosclerosis.2025.119187. Epub 2025 Apr 3.

Reference Type DERIVED
PMID: 40250039 (View on PubMed)

Tromp TR, Hartgers ML, Hovingh GK, Vallejo-Vaz AJ, Ray KK, Soran H, Freiberger T, Bertolini S, Harada-Shiba M, Blom DJ, Raal FJ, Cuchel M; Homozygous Familial Hypercholesterolaemia International Clinical Collaborators. Worldwide experience of homozygous familial hypercholesterolaemia: retrospective cohort study. Lancet. 2022 Feb 19;399(10326):719-728. doi: 10.1016/S0140-6736(21)02001-8. Epub 2022 Jan 28.

Reference Type DERIVED
PMID: 35101175 (View on PubMed)

Other Identifiers

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HICC

Identifier Type: -

Identifier Source: org_study_id