frequently sampled oral glucose tolerance testing will be performed in a one-time cross sectional visit

frequently sampled oral glucose tolerance testing will be performed in a one-time cross sectional visit + continuous glucose monitor will be placed after the visit and mailed back

frequently sampled oral glucose tolerance testing will be performed in a one-time cross sectional visit + a DXA scan will be done at the same visit

frequently sampled oral glucose tolerance testing will be performed in a one-time cross sectional visit + continuous glucose monitor will be placed after the visit and mailed back + a DXA scan will be done at the same visit

Cystic Fibrosis-related Diabetes

oral glucose solution is given by mouth, blood is drawn prior to the administration of the oral glucose beverage and then at timed intervals afterward. This is the standard test to diagnosis cystic fibrosis related diabetes mellitus. However, this study will have more time points than standard screening for cystic fibrosis related diabetes mellitus

Oral glucose tolerance test

a device is placed on the subject's arm that continuously monitors subcutaneous glucose levels for up to 10 days

Continuous glucose monitoring

low dose x-rays are used to measure the subject's bone density. This is the standard test to diagnose osteoporosis

Dexa scan

University of Colorado

University of Iowa

University of Minnesota

Washington University St. Louis

EnVision CF Multicenter Study of Glucose Tolerance in Cystic Fibrosis

The primary endpoint is relationship of beta cell sensitivity to glucose to age. To assess the primary endpoint, the following method will be used: Quantiles of beta-cell glucose sensitivity will be calculated using quantile regression. The outcome variable of the quantile curve is beta-cell glucose sensitivity (continuous, picomol per minute-1 per meter-2 per millimole-1) and the predictor variable is age (continuous, years). On the basis of the available data, we expect a linear relationship between beta-cell glucose sensitivity and age with no heteroskedasticy

The primary outcome of this study is to establish age- and sex-based nomograms ("growth charts") ranging from the 5th-95th % for OGIS (oral glucose insulin sensitivity)

The relationship of the nomogram for beta cell glucose sensitivity will be assessed by quantile regression with the outcome variable beta cell glucose sensitivity, and the predictor the outcomes BMI Z score

The relationship of the nomogram for beta cell glucose sensitivity will be assessed by quantile regression with the outcome variable beta cell glucose sensitivity, and the predictor the outcomes FEV1 score

The relationship of the nomogram for beta cell glucose sensitivity will be assessed by quantile regression with the outcome variable beta cell glucose sensitivity, and the predictor the outcome pulmonary exacerbations in the previous 12 months

The relationship of the nomogram for OGIS will be assessed by quantile regression with the outcome variable OGIS, and the predictor the outcomes BMI Z score

The relationship of the nomogram for OGIS will be assessed by quantile regression with the outcome variable OGIS, and the predictor the outcomes FEV-1

The relationship of the nomogram for OGIS will be assessed by quantile regression with the outcome variable OGIS, and the predictor the outcome the number of pulmonary exacerbations in the previous 12 months

The relationship of the nomogram for insulin will be assessed by quantile regression with the outcome variable insulin, and the predictor the outcomes BMI Z score

The relationship of the nomogram for insulin will be assessed by quantile regression with the outcome variable insulin, and the predictor the outcomes FEV-1

The relationship of the nomogram for insulin will be assessed by quantile regression with the outcome variable insulin, and the predictor the number of pulmonary exacerbations in the previous 12 months

The relationship of the nomogram for beta c-peptide will be assessed by quantile regression with the outcome variable c-peptide, and the predictor the outcomes BMI Z score

The relationship of the nomogram for beta c-peptide will be assessed by quantile regression with the outcome variable c-peptide, and the predictor the outcomes FEV-1

The relationship of the nomogram for beta c-peptide will be assessed by the same method (quantile regression) with the outcome variable c-peptide, and the predictor the outcomes number of pulmonary exacerbations in the previous 12 months

We will measure the correlation between fat mass and fat free mass as determined by DXA and beta cell function as measured by beta cell sensitivity to glucose. The model will be adjusted for BMI z-score, weight Z score, sex, age, and genotype classification, as well as therapy with CFTR potentiator/correctors.

linear regressions will be used to model the responses We will then determine, in a multivariate model if GLP-1 is a significant predictor of beta cell glucose sensitivity, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors.

linear regressions will be used to model the responses We will then determine, in a multivariate model if GIP is a significant predictor of beta cell glucose sensitivity, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors.

linear regressions will be used to model the responses We will then determine, in a multivariate model if PP is a significant predictor of beta cell glucose sensitivity, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors.

linear regressions will be used to model the responses We will then determine, in a multivariate model if glucagon is a significant predictor of beta cell glucose sensitivity, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors.

linear regressions will be used to model the responses We will then determine, in a multivariate model if GLP-1 is a significant predictor of OGIS, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors

linear regressions will be used to model the responses We will then determine, in a multivariate model if GIP is a significant predictor of OGIS, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors.

linear regressions will be used to model the responses We will then determine, in a multivariate model if PP is a significant predictor of OGIS, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors

linear regressions will be used to model the responses We will then determine, in a multivariate model if glucagon is a significant predictor of OGIS, also controlling for potential confounders such as age, sex, genotype classification and therapy with CFTR potentiator/correctors.

Mean catecholamine levels (epinephrine, norepinephrine) at blood glucose nadir will be compared between subjects who do and do not report symptomatic hypoglycemia.

linear regressions will be used to model the responses We will then determine, in a multivariate model Correlation between CGM Time spent >140 mg/dl, and b-cell glucose sensitivity will be determined, controlling for potential confounders

linear regressions will be used to model the responses We will then determine, in a multivariate model Correlation between CGM Time spent >200 mg/dl, and b-cell glucose sensitivity will be determined, controlling for potential confounders.

linear regressions will be used to model the responses We will then determine, in a multivariate modelCorrelation between CGM peak glucose and b-cell glucose sensitivity will be determined, controlling for potential confounders

linear regressions will be used to model the responses We will then determine, in a multivariate modelCorrelation between CGM standard deviation of sensor glucose values and b-cell glucose sensitivity will be determined, controlling for potential confounders

v insulin/atrofija

EnVision CF Multicenter Study of Glucose Tolerance in Cystic Fibrosis

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