A Transient Metabolic Recovery from Early Life Glucose Intolerance in Cystic Fibrosis Ferrets Occurs During Pancreatic Remodeling

Endocrinology. 2016 May;157(5):1852-65. doi: 10.1210/en.2015-1935. Epub 2016 Feb 10.

Abstract

Cystic fibrosis (CF)-related diabetes in humans is intimately related to exocrine pancreatic insufficiency, yet little is known about how these 2 disease processes simultaneously evolve in CF. In this context, we examined CF ferrets during the evolution of exocrine pancreatic disease. At 1 month of age, CF ferrets experienced a glycemic crisis with spontaneous diabetic-level hyperglycemia. This occurred during a spike in pancreatic inflammation that was preceded by pancreatic fibrosis and loss of β-cell mass. Surprisingly, there was spontaneous normalization of glucose levels at 2-3 months, with intermediate hyperglycemia thereafter. Mixed meal tolerance was impaired at all ages, but glucose intolerance was not detected until 4 months. Insulin secretion in response to hyperglycemic clamp and to arginine was impaired. Insulin sensitivity, measured by euglycemic hyperinsulinemic clamp, was normal. Pancreatic inflammation rapidly diminished after 2 months of age during a period where β-cell mass rose and gene expression of islet hormones, peroxisome proliferator-activated receptor-γ, and adiponectin increased. We conclude that active CF exocrine pancreatic inflammation adversely affects β-cells but is followed by islet resurgence. We predict that very young humans with CF may experience a transient glycemic crisis and postulate that pancreatic inflammatory to adipogenic remodeling may facilitate islet adaptation in CF.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Blood Glucose / metabolism
  • Cystic Fibrosis / metabolism*
  • Cystic Fibrosis / pathology
  • Cytokines / blood
  • Female
  • Ferrets
  • Glucagon / genetics
  • Glucagon / metabolism
  • Glucose Intolerance / metabolism*
  • Glucose Intolerance / pathology
  • Glucose Tolerance Test
  • Hyperglycemia / metabolism*
  • Hyperglycemia / pathology
  • Insulin / blood
  • Insulin Resistance / physiology*
  • Male
  • Pancreas / metabolism*
  • Pancreas / pathology

Substances

  • Blood Glucose
  • Cytokines
  • Insulin
  • Glucagon