David L. Gang

Fulminant hepatic failure associated with 2',3'-dideoxyinosine (ddI)

Results from recently published phase I trials (1-3) of 2′,3′-dideoxyinosine (didanosine, ddI) show promise in the treatment of patients with the acquired immunodeficiency syndrome (AIDS) and AIDS-...

Sudden death in an infant with aberrant origin of the right coronary artery from the left sinus of Valsalva of the aorta: Case report and review of the literature

SummaryAn infant who had aberrant origin of the right coronary artery from the left sinus of Valsalva of the aorta died suddenly secondary to acute myocardial infarction. Although this coronary artery anomaly has been acknowledged in previous reviews as a potential risk for sudden death, this is the first documented case of its occurrence that we could find in our review of the literature.

The identification of ganglion cells in Hirschsprung disease by the immunohistochemical detection of ret oncoprotein.

The absence of ganglion cells (GCs) is the primary anatomic abnormality in Hirschsprung disease. Light microscopy is the mainstay in establishing this diagnosis. However, establishing a condition of aganglionosis may be challenging on routine H&E-stained sections of colonic biopsies and resections. We studied the identification of GCs by retinoblastoma oncoprotein (ret) immunoreactivity and routine H&E light microscopy by evaluating 53 blocks from 34 patients demonstrating GCs on original H&E-stained sections and 55 blocks from 38 patients lacking GCs on original H&E-stained sections. All blocks demonstrating GCs on H&E-stained sections also were positive for GCs on ret staining (100%). In 3 blocks that were negative for GCs by H&E staining (5%), GCs were shown on ret-stained sections. Immunoreactivity for ret has comparable specificity but slightly higher sensitivity to routine light microscopic evaluation in identifying GCs. GCs are identified more readily by ret immunoreactivity than by routine morphologic examination.

Villous Adenoma of the Duct of Wirsung

SummaryA patient presenting with recurrent pancreatitis was found to have a benign polypoid villous adenoma of the duct of wirsung. The tumor was excised locally via transduodenal access to the duct. The patient has now remained free of pancreatitis for 24 months. Obstruction of the pancreatic duct by this rare tumor appears to have been the cause of pancreatitis. Villous adenomas of the pancreatic duc epithelium may be premalignant.

Gastroduodenal mucosal prostaglandin generation in patients with Helicobacter pylori before and after treatment with bismuth subsalicylate

To determine whetherHelicobacter pylori has an effect on gastroduodenal mucosal prostaglandin generation, mucosal biopsies were obtained from the gastric body, antrum, and duodenal bulb of 30 patients who were undergoing upper gastrointestinal endoscopy for clinical indications. One biopsy from the gastric body and one from the antrum were tested for urease activity (urea broth) and one biopsy from each area including the duodenum was processed for histology. Two other biopsies form each area were incubated and the accumulation of prostaglandin E2 and 6-keto prostaglandin F1α in the incubation medium was measured by radioimmunoassay. Twelve of the 17H. pylori-positive patients and seven of the 13H. pylori-negative patients agreed to take bismuth subsalicylate (Pepto-Bismol) two tablets four times a day for four weeks. One week after treatment, these patients again underwent endoscopy and the above studies. This study indicates that: (1) mucosal PGE2 generation may be increased in the duodenum, gastric body, and antrum inH. pylori-positive patients compared toH. pylori-negative patients, and (2) treatment with bismuth subsalicylate for four weeks results in reduction of mucosal PGE2 in the duodenum, gastric body, and antrum ofH. pylori-positive patients and fails to eradicateH. pylori or reduce gastric inflammation.

Case 27-1983

Presentation of Case A 25-year-old man was admitted to the hospital because of liver disease. He was well until seven weeks earlier, when he experienced the onset of malaise, daily fever, chillines...

Triplet placentas: reference values for weights.

The occurrence of twins, triplets, and other multiple births increased significantly between 1970 and 2000 in the United States and other industrialized countries. The number of triplet placentas submitted for examination as pathologic specimens has also markedly increased, but no reference values are published for triplet weights. We examined 196 normal triplet placentas. Specimens with associated conditions known to affect the weights of the placentas were excluded. The gestational ages ranged between 20 and 38 weeks. Mean weights for different gestational ages are summarized as follows: 253 g for 20 weeks, 319 g for 22 weeks, 406 g for 24 weeks, 509 g for 26 weeks, 621 g for 28 weeks, 738 g for 30 weeks, 855 g for 32 weeks, 965 g for 34 weeks, 1065 g for 36 weeks, and 1147 g for 38 weeks. Weight gain of triplet placentas appears to parallel that of twin placentas. The mean values of placental weights for triplets at each gestational age are less than triple those of singleton weights for the same duration of gestation. The placental weights in multiple gestations do not increase proportionately with the number of fetuses.

Hepatic Dysfunction as the Presenting Feature of Acute Lymphoblastic Leukemia

Purpose Hepatic dysfunction is a rare presentation of leukemia in children. Because most chemotherapy agents are metabolized by the liver, this complication may have major adverse consequences and effective treatment could be compromised. Patients and Methods The MEDLINE database and current management guidelines from the United States Pediatric Cooperative Cancer Groups were reviewed and analyzed. Data from two institutional cases are described. Results Although previous literature is not informative, our experience suggests that children with leukemia and moderate hepatic dysfunction may tolerate aggressive chemotherapy. Conclusion Current protocol guidelines for dose modification for liver disease may be overly stringent and modification may be beneficial.

Primary hepatocellular carcinoma following nonspecific non-B hepatitis with tumor DNA negative for HBV DNA

SummaryAn association between non-A, non-B hepatitis and hepatocellular carcinoma has been made on the basis of negative serological markers for hepatitis B virus (HBV); however, hepatocellular carcinomas have been found to contain hepatitis B virus deoxyribonucleic acid (HBV DNA) in individuals who lack serological markers of HBV infection. Therefore, reports which ascribed a hepatocellular carcinoma to non-A, non-B hepatitis but did not examine the tumor for HBV DNA are open to question. We describe a case of chronic active hepatitis with primary hepatocellular carcinoma that lacked HBV DNA in the tumor, the nontumorous liver tissue, and serum. The individual lacked all serological markers for HBV infection. This report more fairly supports the association between hepatocellular carcinoma, and chronic hepatitis not due to hepatitis B. Whether this is related specifically to a non-A, non-B hepatitis agent requires identification of the viral agents that cause non-A, non-B hepatitis.

Russell body gastritis

Introduction Russell body gastritis, first described in 1998 [1], is a reactive gastric mucosal infiltration of plasma cells filled with cytoplasmic Russell bodies. Russell bodies are immunoglobulins present within dilated rough endoplasmic reticulum cisternae that have accumulated secondary to abnormal secretion [2]. We present a case of Russell body gastritis occurring in a 75-year-old man with a history of alcohol use, renal failure, dyslipidemia, and prior rhabdomyolysis. He was noncompliant with reflux medications and reported Image 1. Regenerative gastric mucosa showing numerous plasma cells filled with bright eosinophilic Russell bodies (H&E stain; original magnification 3200).