Characterization of Diabetes Mellitus in Fibrous Dysplasia/McCune-Albright Syndrome

NCT ID: NCT03520153

Last Updated: 2019-09-04

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

WITHDRAWN

Study Classification

OBSERVATIONAL

Study Start Date

2018-08-07

Study Completion Date

2021-08-31

Brief Summary

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The investigators' objective is to understand the pathogenesis of diabetes mellitus in Fibrous dysplasia/McCune-Albright syndrome (FD/MAS) by: 1) establishing the contributions of insulin resistance versus impaired insulin secretion, 2) investigating presence of excess glucagon signaling by measuring gluconeogenesis and glycogenolysis, and 3) investigating a potential interaction between diabetes and intraductal papillary mucinous neoplasms (IPMNs).

Detailed Description

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Specific project aims include:

Aim 1: Determine insulin secretion and sensitivity in subjects with MAS-associated diabetes.

Aim 2: Measure gluconeogenesis and glycogenolysis in MAS-associated diabetes to investigate a potential role for excess glucagon signaling.

Aim 3: Determine if IPMN development is associated with impairment of insulin secretion prior to development of overt diabetes.

The authors expect that this study will:

1. Establish the etiology of diabetes in FD/MAS
2. Increase understanding of the role of IPMNs in pathogenesis of diabetes
3. Provide critical insights into the pathogenesis of diabetes in FD/MAS

Conditions

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McCune-Albright Syndrome Fibrous Dysplasia Intraductal Papillary Mucinous Neoplasm Diabetes Mellitus

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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FD/MAS with diabetes mellitus

Fibrous Dysplasia/McCune-Albright Syndrome with diabetes mellitus.

Participants will receive the following interventions: Hyperinsulinemic Euglycemic Clamp and 2H20; Hyperglycemic Clamp; and Oral Glucose Tolerance Test.

Hyperinsulinemic Euglycemic Clamp and 2H20

Intervention Type PROCEDURE

Test is used to assess insulin effects on hepatic glucose production.

Hyperglycemic Clamp

Intervention Type PROCEDURE

Test of beta-cell function and insulin secretion. Involves increasing and maintaining blood glucose concentration with IV variable infusion of dextrose.

Oral Glucose Tolerance Test

Intervention Type DIAGNOSTIC_TEST

The oral glucose tolerance test (OGTT) measures the body's ability to use a type of sugar, called glucose, that is the body's main source of energy. An OGTT can be used to diagnose prediabetes and diabetes.

FD/MAS without diabetes mellitus

Fibrous Dysplasia/McCune-Albright Syndrome without diabetes mellitus.

Participants will receive the following interventions: Hyperinsulinemic Euglycemic Clamp and 2H20; Hyperglycemic Clamp; and Oral Glucose Tolerance Test.

Hyperinsulinemic Euglycemic Clamp and 2H20

Intervention Type PROCEDURE

Test is used to assess insulin effects on hepatic glucose production.

Hyperglycemic Clamp

Intervention Type PROCEDURE

Test of beta-cell function and insulin secretion. Involves increasing and maintaining blood glucose concentration with IV variable infusion of dextrose.

Oral Glucose Tolerance Test

Intervention Type DIAGNOSTIC_TEST

The oral glucose tolerance test (OGTT) measures the body's ability to use a type of sugar, called glucose, that is the body's main source of energy. An OGTT can be used to diagnose prediabetes and diabetes.

FD/MAS without diabetes and without IPMN

Fibrous Dysplasia/McCune-Albright Syndrome without diabetes mellitus and without intraductal papillary mucinous neoplasms.

Participants will receive the following interventions: Hyperinsulinemic Euglycemic Clamp and 2H20; Hyperglycemic Clamp; and Oral Glucose Tolerance Test.

Hyperinsulinemic Euglycemic Clamp and 2H20

Intervention Type PROCEDURE

Test is used to assess insulin effects on hepatic glucose production.

Hyperglycemic Clamp

Intervention Type PROCEDURE

Test of beta-cell function and insulin secretion. Involves increasing and maintaining blood glucose concentration with IV variable infusion of dextrose.

Oral Glucose Tolerance Test

Intervention Type DIAGNOSTIC_TEST

The oral glucose tolerance test (OGTT) measures the body's ability to use a type of sugar, called glucose, that is the body's main source of energy. An OGTT can be used to diagnose prediabetes and diabetes.

FD/MAS without diabetes and with IPMN

Fibrous Dysplasia/McCune-Albright Syndrome without diabetes mellitus and with intraductal papillary mucinous neoplasms.

Participants will receive the following interventions: Hyperinsulinemic Euglycemic Clamp and 2H20; Hyperglycemic Clamp; and Oral Glucose Tolerance Test.

Hyperinsulinemic Euglycemic Clamp and 2H20

Intervention Type PROCEDURE

Test is used to assess insulin effects on hepatic glucose production.

Hyperglycemic Clamp

Intervention Type PROCEDURE

Test of beta-cell function and insulin secretion. Involves increasing and maintaining blood glucose concentration with IV variable infusion of dextrose.

Oral Glucose Tolerance Test

Intervention Type DIAGNOSTIC_TEST

The oral glucose tolerance test (OGTT) measures the body's ability to use a type of sugar, called glucose, that is the body's main source of energy. An OGTT can be used to diagnose prediabetes and diabetes.

Healthy Controls

Participants will receive the following interventions: Hyperinsulinemic Euglycemic Clamp and 2H20; Hyperglycemic Clamp; and Oral Glucose Tolerance Test.

Hyperinsulinemic Euglycemic Clamp and 2H20

Intervention Type PROCEDURE

Test is used to assess insulin effects on hepatic glucose production.

Hyperglycemic Clamp

Intervention Type PROCEDURE

Test of beta-cell function and insulin secretion. Involves increasing and maintaining blood glucose concentration with IV variable infusion of dextrose.

Oral Glucose Tolerance Test

Intervention Type DIAGNOSTIC_TEST

The oral glucose tolerance test (OGTT) measures the body's ability to use a type of sugar, called glucose, that is the body's main source of energy. An OGTT can be used to diagnose prediabetes and diabetes.

Interventions

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Hyperinsulinemic Euglycemic Clamp and 2H20

Test is used to assess insulin effects on hepatic glucose production.

Intervention Type PROCEDURE

Hyperglycemic Clamp

Test of beta-cell function and insulin secretion. Involves increasing and maintaining blood glucose concentration with IV variable infusion of dextrose.

Intervention Type PROCEDURE

Oral Glucose Tolerance Test

The oral glucose tolerance test (OGTT) measures the body's ability to use a type of sugar, called glucose, that is the body's main source of energy. An OGTT can be used to diagnose prediabetes and diabetes.

Intervention Type DIAGNOSTIC_TEST

Eligibility Criteria

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Inclusion Criteria

Fibrous dysplasia/McCune-Albright syndrome (FD/MAS) group:

* Must be diagnosed based on clinical grounds and/or mutation testing on bone and/or affected tissue

Control group:

* Must be at least 18 years old

Exclusion Criteria

Fibrous dysplasia/McCune-Albright syndrome (FD/MAS) group:

* Unwilling to fully cooperate with the evaluation
* Unable to provide informed consent

Control group:

* History of diabetes, insulin resistance, pancreatic disease, pancreatic cysts or amylase/lipase abnormality
* Use of any type of oral diabetes medications and/or insulin
* Unable to provide informed consent
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Yale University

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Cemre Robinson, MD

Role: PRINCIPAL_INVESTIGATOR

Yale University

Locations

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Yale New Haven Hospital

New Haven, Connecticut, United States

Site Status

Countries

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United States

Other Identifiers

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2000022658

Identifier Type: -

Identifier Source: org_study_id

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