Michele D. Bishop

The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis

Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations are associated with cystic fibrosis (CF)-related monosymptomatic conditions, including idiopathic pancreatitis. We evaluated prospectively enrolled patients who had idiopathic recurrent acute pancreatitis or idiopathic chronic pancreatitis, healthy controls, CF heterozygotes, and CF patients (pancreatic insufficient or sufficient) for evidence of CFTR gene mutations and abnormalities of ion transport by sweat chloride and nasal potential difference testing. DNA samples from anonymous blood donors were controls for genotyping. At least one CFTR mutation or variant was carried in 18 of 40 patients (45%) with idiopathic chronic pancreatitis and in 6 of 16 patients (38%) with idiopathic recurrent acute pancreatitis but in only 11 of the 50 controls (22%, P=0.005). Most identified mutations were rare and would not be identified in routine genetic screening. CFTR mutations were identified on both alleles in six patient (11%). Ion transport measurements in patients with pancreatitis showed a wide range of results, from the values in patients with classically diagnosed CF to those in the obligate heterozygotes and healthy controls. In general, ion channel measurements correlated with the number and severity of CFTR mutations. Twelve of 56 patients with pancreatitis (21%) fulfilled current clinical criteria for the diagnosis of CF, but CFTR genotyping alone confirmed the diagnosis in only two of these patients. We concluded that extensive genotyping and ion channel testing are useful to confirm or exclude the diagnosis of CF in the majority of patients with idiopathic pancreatitis.

Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations and risk for pancreatic adenocarcinoma

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are common in white persons and are associated with pancreatic disease. The purpose of this case‐control study was to determine whether CFTR mutations confer a higher risk of pancreatic cancer.

Collagenous Colitis Evolving into Ulcerative Colitis: A Case Report and Review of the literature

Collagenous colitis is a form of microscopic colitis that results in chronic watery diarrhea predominantly affecting middle-aged women. It is characterized by a thick band of collagen beneath the surface epithelium and is asso- ciated with increased lymphocytes in the lamina propria of an otherwise normal-appearing colon. The disease is generally benign, with a course that is characterized by spontaneous relapses and remissions. The disease has no malignant potential, and it is uncommon for it to progress to ulcerative colitis or Crohns disease. Three cases have been reported previously of collagenous colitis evolving into ulcerative colitis. We report one patient with classical symptoms and histological features of collagenous colitis who subsequently developed clinical and histological evi- dence of ulcerative colitis. This process of transformation occurred 12 months after the initial diagnosis. This case, in addition to the other three cases, supports a theory that collagenous colitis may be part of a spectrum of inflam- matory bowel disease that may evolve over the course of time. Therefore, progression to ulcerative colitis should be considered in any patient with known collagenous col- itis whenever there is a change in the clinical picture with the appearance of bloody diarrhea or systemic features of inflammatory bowel disease (IBD).

An Enteral Therapy Containing Medium-Chain Triglycerides and Hydrolyzed Peptides Reduces Postprandial Pain Associated with Chronic Pancreatitis

Background/Aim: Pain in patients with chronic pancreatitis is difficult to manage. We examined if an enteral formulation containing medium-chain triglycerides (MCT) and hydrolyzed peptides would (1) minimally stimulate the exocrine pancreas by blunting cholecystokinin release and (2) decrease pain in patients with chronic pancreatitis. Methods: In the first part of the study, on separate days, 6 healthy controls consumed a standard enteral formulation, an enteral formulation containing MCT and hydrolyzed peptides, and a high-fat meal. Baseline and postprandial plasma cholecystokinin (CCK) concentrations were analyzed. Subsequently, 8 patients with chronic pancreatitis were enrolled and instructed to complete a visual analog pain assessment for a baseline period of 2 weeks followed by three cans per day of the enteral formulation containing MCT and hydrolyzed peptides for 10 weeks. Results: Mean CCK levels for our control subjects were 0.46 ± 0.29 pM at baseline, 10.75 ± 0.45 pM in response to the high-fat meal, and 7.9 ± 1.25 pM in response to the standard enteral formulation. Of note, CCK levels were 1.43 ± 0.72 pM in response to the enteral supplement containing MCT and hydrolyzed peptides. In patients with chronic pancreatitis, the average improvement in pain scores from baseline to the conclusion of the study was 61.8% (p = 0.01). This corresponded to a clinical improvement in 6 of the 8 patients. Conclusions: A complete enteral supplement containing MCT and hydrolyzed peptides minimally increases plasma CCK levels. This therapy may be effective in reducing postprandial pain associated with chronic pancreatitis.