Excerpt
Methods: Patients with idiopathic recurrent pancreatitis were referred for electrophysiological investigation.
Results: 18 patients (10 males) aged 22±11 years (9 below the age of 18 years) with recurrent pancreatitis were studied. 1 patient had mild asthma and one patient had Crohn’s disease. There was no family history of CF. All patients had normal spirometry and negative sputum cultures. Most patients had imaging of the pancreatic duct (ERCP/MRCP) and in 2 patients mild pancreatic duct stenosis was found. No patient was Pancreatic Insufficient. Mean sweat chloride values were 41±13 meq/L (range 20–66). Nasal potental difference (NPD) was pathological and diagnostic for CF in 4 patients (sweat chloride 44,60,43,45 meq/L). Mean basal potential difference (PD) in these 4 patients was −32.5±13 mV and there was an abnormal response to chloride-free and isoproterenol solutions. In the other 14 patients the mean sweat chloride concentration was 38±14 meq/L. Basal PD was in the normal range (−15±5 mV) and the chloride transport was normal. Mutation analysis revealed W1282X/5T and W1282X/— in 2 patients with abnormal NPD and one W1282X allele was found in one patient with normal NPD.
Conclusion: In this series, a fifth of patients with recurrent pancreatitis have abnormalities of CFTR function. Larger studies are warranted to firmly establish the role of NPD as an aid to diagnosis of CF in otherwise idiopathic recurrent pancreatitis.