Diabetes mellitus and profound insulin resistance in Johanson-Blizzard syndrome

Pancreatic exocrine insufficiency in Johanson-Blizzard syndrome (JBS) is we ll described but only two previous patient reports document pancreatic endo crine insufficiency manifested as diabetes mellitus, and each patient requi red only a modest dose of insulin to control hyperglycemia. We report a pat ient with JBS and new-onset diabetes mellitus with profound insulin resista nce, with no clinical or laboratory evidence of pancreatic exocrine insuffi ciency.