Clissold RL, et al. - In this current investigation, faecal elastase-1 was measured in patients with hepatocyte nuclear factor 1β (HNF1B)-associated renal disease regardless of diabetes status and the degree of symptoms associated with pancreatic exocrine deficiency was assessed. Faecal elastase-1 deficiency was shown to be a common feature of HNF1B-associated renal disease even in the absence of diabetes. Also, pancreatic exocrine deficiency may be more symptomatic than previously suggested. Chronic abdominal pain, loose stools or unintentional weight loss were reported to be the indications for measuring faecal elastase-1 in patients with known HNF1B-associated disease. Notably, the finding of a low faecal elastase-1 concentration in individuals with developmental kidney disease of uncertain cause should prompt referral for HNF1B genetic testing.