Characterization of Diabetes Mellitus in Fibrous Dysplasia/McCune-Albright Syndrome
试验速览
- 阶段
- 不适用
- 状态
- 撤回
- 试验地点
- 1
- 主要终点
- Glucose tolerance status
研究概览
简要总结
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).
详细描述
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:
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •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
排除标准
- •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
结局指标
主要结局
Glucose tolerance status
时间窗: Baseline
An oral glucose tolerance test will be performed to assess glucose tolerance status to determine if subjects have pre-IGT, IGT or diabetes
Insulin sensitivity
时间窗: Baseline
This primary outcome will be obtained from the oral glucose tolerance test
Insulin secretion
时间窗: Baseline
This primary outcome will be obtained from the hyperglycemic clamp
Beta cell capacity
时间窗: Baseline
AIRmax stimulation test during the hyperglycemic clamp to ascertain the maximal acute insulin response (AIR) to arginine, which is a measure of functional beta cell capacity.
Hepatic glucose fluxes (gluconeogenesis and glycogenolysis)
时间窗: At least 2 weeks post baseline testing
Measurements from the Hyperinsulinemic Euglycemic Clamp/ 2H20 Study will be used to assess insulin effects on hepatic glucose production and glycerol kinetics isotopes and the deuterium enrichment at carbons 2 and 5 (C2 and C5) of plasma glucose providing information on glucose fluxes
次要结局
- Zinc transporter 8 (ZnT8)(Baseline)
- Fasting lipid panel(Baseline)
- Growth hormone(Baseline)
- Islet antigen-2 antibodies (IA-2A)(Baseline)
- Renal function (BUN and creatinine)(Baseline)
- Islet cell antibodies (ICA),(Baseline)
- Glutamic acid decarboxylase antibodies (GAD65)(Baseline)
- Fasting incretins(Baseline)
- Prandial incretins(Baseline)
- Free fatty acids(Baseline)
- Insulin-like growth factor-1 (IGF-1)(Baseline)
- C-reactive protein (CRP)(Baseline)
- Liver function tests (AST and ALT)(Baseline)
- Tumor necrosis factor alpha (TNF-alpha)(Baseline)
- Hemoglobin A1c(Baseline)
- Urinalysis(Baseline)
- Glycerol(Baseline)
- Leptin(Baseline)
- Adiponectin(Baseline)
- Interleukin 6 (IL-6)(Baseline)