Status:

RECRUITING

Beta-cell Response to Incretin Hormones in Cystic Fibrosis

Lead Sponsor:

University of Pennsylvania

Collaborating Sponsors:

Children's Hospital of Philadelphia

Conditions:

Cystic Fibrosis

Pancreatic Insufficiency

Eligibility:

All Genders

18+ years

Phase:

NA

Brief Summary

In recent years, diabetes has emerged as one of the most significant co-diseases that many Cystic Fibrosis (CF) patients develop. Type 1 (T1D) and Type 2 (T2D) diabetes results when either the body do...

Detailed Description

Previously, cystic fibrosis related diabetes (CFRD) was considered to be a consequence of damage to the pancreas therefore the cells contained in the pancreas--i.e.--islets that house beta cells, whic...

Eligibility Criteria

Inclusion

  • Confirmed diagnosis of cystic fibrosis, defined by positive sweat test or CFTR mutation analysis according to CFF diagnostic criteria,
  • Age greater than or equal to 18y on date of consent
  • Pancreatic insufficiency
  • Recent OGTT consistent with Indeterminate-GT, IGT, CFRD w/o fasting hyperglycemia, or an established diagnosis of CFRD without fasting hyperglycemia
  • For female subjects, negative urine pregnancy test at enrollment.
  • Control Subjects:
  • No history of cystic fibrosis.
  • Age ≥ 18y on date of consent.
  • Recent OGTT consistent with NGT.
  • For female subjects, negative urine pregnancy test at enrollment.

Exclusion

  • Established diagnosis of non-CF diabetes (i.e. T1D) or CFRD with fasting hyperglycemia (fasting glucose greater than126 mg/dL)
  • History of clinically symptomatic pancreatitis within last year
  • Prior lung or liver transplant
  • Severe CF liver disease, as defined by portal hypertension
  • Fundoplication-related dumping syndrome
  • Medical co-morbidities that are not CF-related or are unstable per investigator opinion (i.e. history of bleeding disorders, immunodeficiency)
  • Acute illness or changes in therapy (including antibiotics) within 6 weeks prior to study procedures
  • Treatment with oral or intravenous corticosteroids within 6 weeks of study
  • Hemoglobin less than10g/dL, within 90 days of Visit 1 or at Screening
  • Abnormal renal function, within 90 days of Visit 1 or at Screening; defined as Creatinine greater than 2x upper limit of normal (ULN) or potassium greater than 5.5mEq/L on non-hemolyzed specimen
  • Inability to perform study specific procedures (MMTT, GPA)
  • Subjects, who in study team opinion, may be non-compliant with study procedures.
  • Control Subjects who will be exposed to GIP only:
  • History of clinically symptomatic pancreatitis.
  • History of liver disease.
  • History of any illness or condition that, in the opinion of the investigator might confound the results of the study or pose an additional risk to the subject.
  • Hemoglobin \<10g/dL, within 90 days of GPA test or at Screening.
  • Abnormal renal function, within 90 days of GPA test or at Screening; defined as creatinine \> 2x upper limit of normal (ULN) or potassium \> 5.5mEq/L on non-hemolyzed specimen.
  • Inability to perform study specific procedures (MMTT, GPA).
  • subjects, who in study team opinion, may be non-compliant with study procedures.
  • elevation of serum amylase or lipase \> 1.5x ULN within 90 days of GPA test.

Key Trial Info

Start Date :

May 1 2013

Trial Type :

INTERVENTIONAL

Allocation :

ESTIMATED

End Date :

December 1 2025

Estimated Enrollment :

45 Patients enrolled

Trial Details

Trial ID

NCT01851694

Start Date

May 1 2013

End Date

December 1 2025

Last Update

January 31 2025

Active Locations (1)

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1

Children's Hospital of Philadelphia and University of Pennsylvania

Philadelphia, Pennsylvania, United States, 19060