Monogenic Diabetes Misdiagnosed as Type 1

NCT ID: NCT03988764

Last Updated: 2024-12-10

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

5000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2019-09-24

Study Completion Date

2025-12-31

Brief Summary

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The study has two aims:

1. To (1a) determine the frequency of monogenic diabetes misdiagnosed as type 1 diabetes (T1D) and (2) to define an algorithm for case selection.
2. To discover novel genes whose mutations cause monogenic diabetes misdiagnosed as T1D.

Detailed Description

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Aim 1. The investigators will recruit 5,000 cases diagnosed as T1D under the age of 25, from 17 participating clinics across Canada. All cases will be tested for four antibodies (against proinsulin, GAD65, islet antigen 2 (IA-2), and ZnT8). Cases negative for all four will be exome-sequenced.

1. Variant annotation will be focused on known monogenic diabetes genes. Variants rated as pathogenic, likely pathogenic or of unknown significance whose zygosity fits the genetic model, will be confirmed in a clinically certified laboratory and communicated to the treating health care team. End-point is the frequency of such variants compared to their frequency in control, non-T1D exomes.
2. The following variables will be examined for the ability to predict monogenic diabetes: Negativity for all autoantibodies tested, family history, polygenic T1D risk score, age of onset, sex, glycosylated hemoglobin (HbA1c), insulin dose, and presence of syndromic features. Predictors will be analyzed by multiple regression and results subjected to jackknife (leave-one-out) validation. Machine-learning techniques may be used.

Aim 2. Variants outside known genes in non-diagnostic exomes will be annotated and examined under autosomal dominant, recessive, X-linked and mitochondrial inheritance models. Corresponding frequency cutoffs will be 0.0005, 0.01, 0.001 and 0.0005 (if heteroplasmy \>70%). Formal mutation-burden analysis will be based on depth-adjusted data from the Genome Aggregation Database (gnomAD). Genes mutated in more than one unrelated proband will be examined by a statistical approach taking into account the presence of a large number of phenocopies (Akawi et al., Nat Genet. 2015;47:1363-1369). Genes that achieve statistical significance will be tested in additional cohorts with international collaborations.

Conditions

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Diabetes Mellitus, Type 1 Monogenic Diabetes Neonatal Diabetes Maturity-onset Diabetes in the Young (MODY) Wolfram Syndrome Wolcott-Rallison Syndrome Mitochondrial Diabetes

Study Design

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Observational Model Type

CASE_ONLY

Study Time Perspective

PROSPECTIVE

Study Groups

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Antibody-negative

Patient has been found negative for at least three T1D antibodies.

The investigators will proceed with whole exome sequencing

None AHT

Intervention Type OTHER

No further intervention planned for either group as part of the current study.

Antibody-positive

Patient has been found to be positive for at least one T1D autoantibody.

No further studies will be performed as part of the main study.

None AHT

Intervention Type OTHER

No further intervention planned for either group as part of the current study.

Interventions

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None AHT

No further intervention planned for either group as part of the current study.

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of diabetes under the age of 25 as either type 1 or undetermined type.

Exclusion Criteria

* Existing T1D autoantibody testing with a positive result
Minimum Eligible Age

1 Day

Maximum Eligible Age

25 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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McGill University Health Centre/Research Institute of the McGill University Health Centre

OTHER

Sponsor Role lead

Responsible Party

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Constantin Polychronakos

Senior investigator

Responsibility Role PRINCIPAL_INVESTIGATOR

Locations

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The Montreal Children's Hospital

Montreal, Quebec, Canada

Site Status RECRUITING

Countries

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Canada

Central Contacts

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Constantin Polychronakos, MD

Role: CONTACT

Phone: 5144124400

Email: [email protected]

Angeliki Makri, MD

Role: CONTACT

Phone: 5144124400

Email: [email protected]

Facility Contacts

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Natalija Popovic, PhD

Role: primary

Angeliki Makri, MD

Role: backup

Other Identifiers

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Canscreen

Identifier Type: OTHER

Identifier Source: secondary_id

ADDAM

Identifier Type: -

Identifier Source: org_study_id