Cystic fibrosis transmembrane conductance regulator functional evaluations in a G542X+/- IVS8Tn:T7/9 patient with acute recurrent pancreatitis

Figure 1 Results of standardized cystic fibrosis transmembrane conductance regulator functional test. Intestinal current measurement tracings from the controversial clinical case (presented herein), cystic fibrosis (CF) patients with pancreatic insufficiency-CF or pancreatic sufficiency-CF and control (non-CF donor). As shown in the top panel, there were positive tissue responses to forsk/IBMX: Forskolin/3-isobutyl-1-methylxanthine, Carbachol and Histamine clearly visible in acute recurrent pancreatitis and not consistent with CF diagnosis. ARP: Acute recurrent pancreatitis; CF: Cystic fibrosis; PI-CF: Pancreatic insufficiency-cystic fibrosis; PS-CF: Pancreatic sufficiency-cystic fibrosis.