John J. Fromkes

Familial Barrett's Esophagus Associated With Adenocarcinoma

A family with six cases of Barretts esophagus over three generations is described. The Barretts esophagus affected only males, and there were three associated adenocarcinomas. The mechanism of inheritance is compatible with an autosomal dominant pattern with nearly complete penetrance. Other case reports in the literature are compared with this familial case of Barretts esophagus with associated adenocarcinomas.

Pseudomonas aeruginosa sepsis following retrograde cholangiopancreatography (ERCP).

SummaryEndoscopic retrograde cholangiopancreatography (ERCP) has become a routine examination in a number of medical centers within the past several years. We report a life-threatening case of acute pancreatitis withPseudomonas aeruginosa sepsis immediately following ERCP. Cultures of the blood, the inner channel of the duodenoscope, and irrigating water bottles all were positive forPseudomonas aeruginosa. ThePseudomonas aeruginosa isolated from the blood and endoscope both reacted to three common antisera: serotypes 2, 15, and 16, suggesting a common source of infection. Although it is obvious that the ERCP procedure cannot be sterile, attempts should be made to prevent transmission of noscomial pathogens by this procedure.

Unique mechanism of inhibition of Na+-amino acid cotransport during chronic ileal inflammation

In the chronically inflamed ileum, unique mechanisms of alteration of transport processes suggest regulation by different immune-inflammatory mediator pathways. We previously demonstrated that Na+-glucose cotransport in the chronically inflamed ileum was inhibited by a decrease in cotransporter number without a change in glucose affinity. The aim of this study was to determine the alterations in Na+-amino acid cotransport in chronically inflamed ileum produced by coccidial infection in rabbits. [3H]alanine uptake was performed in cells and vesicles by rapid filtration. In villus cells from chronically inflamed ileum, Na+-K+-ATPase was reduced 50% and Na+-alanine cotransport was also reduced (5.8 ± 1.2 in normal and 1.4 ± 0.5 nmol/mg protein in inflamed; n = 6, P < 0.05). [3H]alanine uptake in brush-border membrane vesicles was reduced in chronically inflamed ileum (73.2 ± 1.2 in normal and 21.5 ± 3.2 pmol/mg protein in inflamed; n = 3, P < 0.05), suggesting a direct effect on the cotransporter itself. Na+-amino acid cotransport in chronically inflamed ileum was inhibited by a decrease in affinity without a change in the maximal rate of uptake, and unaltered steady-state mRNA levels also suggested that the number of cotransporters was unchanged. Thus the mechanisms of inhibition of Na+-amino acid cotransport and Na+-glucose cotransport in chronically inflamed ileum are different. These observations suggest that different immune-inflammatory mediators may regulate different transport pathways during chronic ileitis.

Surgical management of pancreas divisum

Pancreas divisum is a congenital anatomic variant characterized by nonunion of dorsal and ventral pancreatic ducts in an otherwise fused pancreas. Of 21 patients with divisum documented by endoscopic retrograde cholangiopancreatography, 6 (28 percent) were found to have no reason other than divisum to account for multiple attacks of pancreatitis. Cholelithiasis was present in one patient, who remains free of recurrent pancreatitis after cholecystectomy only. The remaining five patients underwent surgical treatment directed at pancreas divisum in the belief that stenosis of the duct of Santorini at the entrance into the duodenum is responsible for recurrent attacks of pancreatitis. Four of five have done well with follow-up of 12, 13, 18 and 28 months. Successful sphincteroplasty of the duct of Santorini appears to prevent recurrent attacks of pancreatitis due to pancreas divisum. Pancreaticojejunostomy is reserved for those with markedly dilated ducts secondary to chronic pancreatitis.

Pancreatic cystadenoma in Von Hippel-Lindau disease: an unusual cause of pancreatic and common bile duct obstruction.

Pancreatic cystadenoma is an unusual lesion of the pancreas, typically occurring as an isolated lesion in a middle-aged woman with abdominal pain or an asymptomatic epigastric mass. Jaundice is unusual. Two types of pancreatic cystadenoma are distinguished: microcystic and mucinous; the latter has a tendency to malignant degeneration. A patient with Von Hippel-Lindau disease and pancreatic cystadenoma developed jaundice due to common bile and pancreatic duct obstruction. Cystic and adenomatous lesions of the pancreas are common in Von Hippel-Lindau disease, but jaundice due to common bile duct obstruction has not previously been reported.

Endoscopic retrograde cholangiopancreatography in the management of pancreatic pseudocysts

The value of ERCP was studied in 25 patients with pancreatic pseudocysts. There were no episodes of sepsis; however, acute pancreatitis developed in one patient for an overall complication rate of 4 percent. Results of ERCP were positive in 24 of the 25 patients (96 percent), with filling of the pseudocyst in 17 and pancreatic ductal obstruction in 7. Biliary tract abnormalities were found in seven patients and included common bile duct strictures in four, bile duct dilatation in two, and cystic duct obstruction in one. ERCP also detected six pseudocysts not diagnosed by ultrasonography, five of which were small and resolved with nonoperative therapy. ERCP is a safe diagnostic procedure for patients with pancreatic pseudocysts and may provide important information about coexistent biliary tract disease not otherwise available. It is also sufficiently sensitive to detect small pseudocysts that otherwise would be missed.

Suppression of Gastrin and Gastric Acid Secretion in the Zollinger-Ellison Syndrome by Long-Acting Somatostatin (SMS 201-995)

(1986). Suppression of Gastrin and Gastric Acid Secretion in the Zollinger-Ellison Syndrome by Long-Acting Somatostatin (SMS 201-995) Scandinavian Journal of Gastroenterology: Vol. 21, No. sup119, pp. 206-211.

Acute pancreatitis in elderly patients: Pathogenesis and outcome

Forty patients over 70 years of age with acute pancreatitis were studied. The most common cause of pancreatitis was biliary tract disease (14 patients, 35 percent). Twelve patients (30 percent) were discharged with a diagnosis of idiopathic pancreatitis, but tests such as endoscopic retrograde cholangiopancreatography that might have established the cause of disease were frequently not employed. Eight of the 40 patients died, for a mortality rate of 20 percent. Significant morbidity occurred in an additional seven (17.5 percent). Multisystem failure was the cause of death in all eight patients, and only two patients with multisystem failure survived. The mortality rate was significantly higher in those patients with postoperative pancreatitis. Acute pancreatitis in the elderly carries a grave prognosis. All patients should undergo thorough evaluation, as biliary tract disease is the most common cause. Cholecystectomy should be performed in those with biliary disease to prevent recurrent attacks.

Spontaneous Bacterial Peritonitis Associated with Acute Viral Hepatitis

Spontaneous bacterial peritonitis (SBP) occurs most frequently in patients with cirrhosis and preexistent ascites; SBP has not been previously recognized in association with acute liver disease. We report two patients with acute hepatitis B infection who developed SBP. Patient 1 had Streptococcus pneumoniae peritonitis and bacteremia, but did not have ascites until after the peritoneal infection was evident. Subsequent liver biopsy and follow-up studies confirmed the clinical diagnosis of acute hepatitis. Patient 2 had sub-massive hepatic necrosis due to hepatitis B and developed ascites before Streptococcus fecalis SBP. Although the association of SBP with acute hepatic injury is rare, these two patients illustrate that the syndrome of SBP does occur with acute liver disease.

Esophageal intramural pseudodiverticulosis.

Three cases of esophageal intramural pseudodiverticulosis (EIP) are described and the literature reviewed. We conclude that EIP is an inflammatory disease of unknown etiology characterized by dilatation of the esophageal submucosal glandular elements. The classic radiographic picture which is that of collar-button-shaped outpouchings projecting in a perpendicular fashion from the luminal surface is mainly related to dilatation in the glandular ductal system. It is usually diagnosed in the sixth and seventh decades, but all ages may be affected. Dysphagia is a very common but not universal symptom of the disease process. Approximately one third of these patients have diabetes mellitus. Strictures of the esophagus are frequently seen, and varying degrees of esophagitis may be manifest endoscopically. Esophageal motility disturbances are common and may be severe.Candida infestation with and without tissue invasion has been seen in a number of patients and should be searched for in all cases of EIP.