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Mayo Clin Proc

Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities.

Desmet VJ.
Laboratory of Histochemistry and Cytochemistry, Universitair Ziekenhuis Sint Rafael, Leuven, Belgium.
Intrahepatic bile ducts (IHBDs) develop from bipotential liver progenitor cells in contact with the mesenchyme of the portal vein and thus form the "ductal plates." The ductal plates are remodeled into mature tubular ducts. Lack of remodeling results in the persistence of periportal epithelial sleeves or "ductal plate malformation" (DPM). A proposal is that virtually all congenital diseases of IHBDs represent examples of DPM. Some early, severe types of extrahepatic bile duct atresia are characterized by DPM, a suggestion of a prenatal beginning of the disease. Several congenital diseases are characterized by dilatation of segments of IHBDs and variable degrees of fibrosis. Such "fibrocystic diseases" represent DPM at different levels of the biliary tree. Autosomal recessive polycystic kidney disease represents DPM of interlobular bile ducts, associated with tubular dilatation of collecting renal tubules. Congenital hepatic fibrosis may derive from the same type of liver lesion, through a superimposed destructive type of cholangiopathy associated with scarring fibrosis. Caroli's disease represents DPM of the larger IHBDs, whereas Caroli's syndrome combines the lesions of Caroli's disease and congenital hepatic fibrosis. von Meyenburg complexes represent DPM of smaller interlobular ducts; their dilatation gives rise to the liver cysts in autosomal dominant polycystic kidney disease. Finally, DPM is a component of the tissue abnormalities in so-called mesenchymal hamartoma.

Biliary atresia.

Lefkowitch JH.
Department of Pathology, College of Physicians and Surgeons of Columbia University, New York, New York 10032, USA. JHL3@columbia.edu
Extrahepatic biliary atresia is an obliterative cholangiopathy that involves all or part of the extrahepatic biliary tree and, in many cases, the intrahepatic bile ducts. In the United States, from 400 to 600 new cases of biliary atresia are encountered annually. The diagnosis is usually suggested by the persistence of jaundice for 6 weeks or more after birth. Several factors have been considered for the pathogenesis of extrahepatic biliary atresia, including viral infection, metabolic insults, and abnormalities in bile duct morphogenesis. Although selected patients benefit from prompt diagnosis and Kasai portoenterostomy surgical intervention within the first 60 days of life, many ultimately require liver transplantation because of portal hypertension, recurrent cholangitis, and cirrhosis.

Gastroesophageal reflux in infants and children.

Faubion WA Jr. Zein NN.
Section of Pediatric Gastroenterology, Mayo Clinic Rochester, Minnesota 55905, USA.
Gastroesophageal reflux is a common pediatric complaint and a frequent reason for pediatric patients to be referred to a gastroenterologist. The pathophysiology and clinical manifestations of this disorder differ according to patient age. The diagnosis is suggested by the history and can be confirmed by a pH probe. In the appropriate clinical setting, anatomic obstruction may need to be ruled out by contrast study. Endoscopy is used to assess associated complications, including esophagitis, esophageal strictures, Barrett's transformation, and failure to thrive. Other complications are controversial, including pulmonary disease, apnea, and sudden infant death syndrome. Treatment depends on the severity of disease. Conservative therapy includes behavorial modifications, prokinetic agents, and H2 antagonists. Proton pump inhibitors are generally reserved for refractory esophagitis. Surgical treatment may be necessary for gastroesophageal reflux resistant to medical management or for severe complications. Gastroesophageal reflux beyond infancy tends to be chronic; therefore, lifelong behavioral modifications or repeated courses of medical therapy may be necessary. An algorithm for the suggested diagnostic approach to gastroesophageal reflux is presented herein.

Ludwig Symposium on biliary disorders--part II. Pathologic features and evolution of primary biliary cirrhosis and primary sclerosing cholangitis.

Scheuer PJ.
Department of Histopathology, Royal Free Hospital School of Medicine, University of London, England.
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) share many clinical and pathologic features. Central to the symptoms and biochemical alterations of both conditions is a substantial loss of intrahepatic bile ducts, leading to interference with bile flow. This pathologic change may ultimately result in cirrhosis of the biliary type. In addition, however, biopsy specimens usually show an element of liver-cell destruction and associated inflammation, mainly interface hepatitis. This finding is more pronounced in PBC than in PSC but can lead in both diseases to features that resemble those of cirrhosis as a result of hepatitis virus infection. The resemblance often leads to diagnostic confusion, which is easily overcome by attention to the clinical, radiologic, serologic, and biochemical context. Histologic staging of PBC and PSC has led to a greater appreciation of their evolution but is hampered in biopsy material by sampling error. Examination of explanted livers at transplantation has demonstrated a wide variation in the maturation of lesions in various parts of the organ.

Ludwig Symposium on biliary disorders. Autoimmune cholangitis: a unique entity?

Sherlock S.
Department of Surgery, Royal Free Hospital School of Medicine, London, England.
Overlap syndromes between cholestatic autoimmune chronic hepatitis and primary biliary cirrhosis are being increasingly discussed. The diagnosis depends particularly on the pattern of serum autoantibodies, whether suggestive of one condition or the other. The autoantibodies have an important diagnostic role, but their contribution in mediating bile duct and hepatocellular injury is uncertain. In this report, five patients (three women and two men) are described with hepatic histologic features resembling primary biliary cirrhosis but with negative results for serum antimitochondrial antibody (M2) tests. Serum antinuclear antibody of diffuse type is strongly positive. The serum transaminase levels are 4 to 6 times the upper limit of normal, and serum gamma-glutamyl transpeptidase values are substantially increased. The response to prednisolone therapy is partial: inflammation is reduced, but the serum gamma-glutamyl transpeptidase level remains high and bile duct lesions persist. This condition, which is an overlap between primary biliary cirrhosis and autoimmune hepatitis, has been termed "autoimmune cholangitis." Treatment with ursodeoxycholic acid is recommended, and prednisolone therapy may be considered even though beneficial results have not been impressive.

Idiopathic adulthood ductopenia: an update.

Year 1998
Ludwig J.
Division of Anatomic Pathology, Mayo Clinic Rochester, MN 55905, USA.
Idiopathic adulthood ductopenia (IAD), named only 10 years ago, is the latest entity to join the group of small-duct biliary diseases. On the basis of published data and my consultation practice, I am aware of 57 cases. Most commonly, IAD affects young or middle-aged adults (overall range of affected patients, 15 to 77 years) and occurs with a distinct male preponderance. The median age of male and female patients among 48 published and unpublished cases was 30 and 36 years, respectively (in 9 cases, the precise age had not been stated). The age range probably encompasses several etiologic groups with different age distributions. Biopsy specimens, by definition, show ductopenia and its complications but no other lesions. Laboratory studies reveal a cholestatic profile, but again, by definition, these cases show no changes that would be diagnostic or suggestive of another biliary disease. In approximately half the published cases in which the outcome was reported, the condition seemed to have a benign course; in contradistinction, the other cases of IAD were fatal or necessitated liver transplantation. Indirect evidence suggests that IAD is a syndrome with several causes, including (1) late-onset nonsyndromic paucity of intrahepatic bile ducts, (2) small-duct primary sclerosing cholangitis ("pericholangitis") without large duct involvement and without evidence of inflammatory bowel disease, (3) nonsuppurative viral cholangitis--for instance, in hepatitis C, and (4) autoimmune cholangitis or cholangitis in autoimmune hepatitis, in the absence of the typical autoantibodies (cryptogenic chronic hepatitis). For progressive cases of IAD, liver transplantation is necessary, whether or not a specific cause is suspected.

Esophagogastric hematoma mimicking a malignant neoplasm: clinical manifestations, diagnosis, and treatment.

Year 1998
Geller A. Gostout CJ.
Division of Gastroenterology and Hepatology, Mayo Clinic Rochester, Minnesota 55905, USA.
Esophagogastric hematoma is a rare condition occurring spontaneously or after esophageal instrumentation. In this report, we describe a patient with acute dysphagia in whom a lower esophageal mass was detected radiographically. Upper endoscopy revealed an esophageal mass that extended from the mid-esophagus to the gastroesophageal junction and was associated with a malignant-appearing ulcerated mass (5 to 6 cm) in the cardia. Gastric cancer with esophageal extension was the presumptive diagnosis. Computed tomography showed that the esophageal mass had a density similar to blood, a finding suggesting the presence of an esophageal hematoma. Biopsy specimens of the ulcer revealed acute inflammation but no malignant involvement. The patient was treated conservatively, and the initial symptoms resolved. Esophagogastric hematomas can mimic a neoplasm; thus, establishing the correct diagnosis is important because this condition has a favorable prognosis, and only conservative treatment is needed.

Clinicians guide to hepatitis C.

Year 1998
Gross JB Jr.
Division of Gastroenterology and Hepatology, Mayo Clinic Rochester, MN 55905, USA.
Hepatitis C virus infection is common, often silent, and almost always chronic and can lead to cirrhosis and hepatocellular cancer. Deaths related to chronic hepatitis C are expected to increase dramatically in the future. Many cases of infection are asymptomatic and are undiagnosed because of a lack of recognition by patients and physicians. All patients currently or previously at risk of infection should undergo screening, including those who received blood transfusions before 1992. Interferon is the only effective therapy, but disappearance of virus is sustained in only 10 to 15% of patients. The combination of interferon and oral ribavirin therapy may increase the sustained response rate to about 40%. New agents such as hepatitis C virus-specific protease inhibitors may be available in the next 5 to 10 years, and treatment is evolving toward multiple-drug regimens analogous to those used for human immunodeficiency virus (HIV) infection. In contrast to public funding for drug development in HIV, such funding for hepatitis C has been limited.

Immune cholangitis: liver allograft rejection and graft-versus-host disease.

Year 1998
Demetris AJ.
Division of Transplantation Pathology, University of Pittsburgh Medical Center, Pennsylvania, USA.
A pronounced similarity exists between liver allograft rejection and graft-versus-host disease (GVHD) in the damage and eventual destruction of small intrahepatic bile ducts. Although an immunologic reaction has an important role, precisely identifying the target antigens or reason for persistence of the immune response has been difficult. An important difference between GVHD and liver rejection is the development of obliterative arteriopathy only in rejection. The three main histopathologic features of acute rejection are a predominantly mononuclear but mixed portal inflammation, subendothelial inflammation of portal or terminal hepatic veins (or both), and bile duct inflammation and damage. In acute rejection, a controversial issue is determining when therapeutic intervention is needed. The recommended approach is to base treatment on a combination of histopathologic changes and liver injury or dysfunction. Chronic rejection, which usually does not occur before 2 months after transplantation, is characterized by two main histopathologic features: (1) damage and loss of small bile ducts and (2) obliterative arteriopathy. Acute GVHD begins within the first month after transplantation and most commonly involves the skin, gastrointestinal tract, and liver, whereas chronic GVHD usually develops more than 80 to 100 days after liver transplantation and affects 30 to 50% of long-term survivors. Recognition of the early, cellular stages of chronic GVHD is important in preventing irreversible damage.

Ischemic cholangitis.

Year 1998
Batts KP.
Division of Anatomic Pathology, Mayo Clinic Rochester, Minnesota 55905, USA.
Ischemia-induced bile duct lesions have been collectively labeled as ischemic cholangitis. The biliary epithelium is dependent on arterial blood flow, unlike the hepatic parenchyma with its dual arterial and portal venous blood supply. As such, the biliary epithelium is susceptible to injury when arterial blood flow is compromised. This compromise can occur at the level of the major, named hepatic artery branches or at the microscopic, peribiliary capillary plexus level. Typically, ischemic cholangitis manifests as segmental strictures and cholangiectases with resultant mechanical impairment of bile flow and, occasionally, secondary infection of the biliary system. Ischemic cholangitis after liver transplantation is becoming an important problem and likely is attributable to numerous factors. Hepatic arterial infusion of chemotherapy and systemic vasculitis are other causes of ischemic cholangitis. The role of ischemia in other chronic biliary and ductopenic diseases remains speculative.

The radiodensity of medications seen on x-ray films.

Year 1998
Florez MV. Evans JM. Daly TR.
Mayo Medical School, Mayo Clinic Rochester, Minnesota 55905, USA.
OBJECTIVE: To analyze the radiodensity of commonly used medications and determine their ability to be seen on plan x-ray films. MATERIAL AND METHODS: Under conditions intended to simulate a patient undergoing radiography of the abdomen (including the use of a patient-equivalent phantom), 50 prescription and nonprescription drugs were tested. Their radiodensities were quantified, and their visibility on plain x-ray films was noted. The study drugs were then ranked in order of decreasing radiodensity. In addition, we report an illustrative case of ingested pills in the stomach that mimicked gallstones, a phenomenon we termed "pseudogallstones". RESULTS: In a 71-year-old woman with upper abdominal pain and nausea, a presumptive diagnosis of gallstones based on x-ray findings was subsequently found to be retained iron tablets. This case prompted our assessment of the radiodensity of medications frequently prescribed for elderly patients. Although all 50 medications studied were visible on plain x-ray films, a 13-fold difference was found in their relative radiodensities. Of the medications studied, potassium chloride was the most radiodense, and prednisone was the least radiodense. As a group, minerals were the most radiodense of all medications studied. CONCLUSION: Numerous commonly prescribed medications in their undissolved, undigested state are visible on plain x-ray films, as are mineral supplements, which have high radiodensities. At times, the appearance of these medications and supplements may be confused with organic pathologic conditions.

Diagnostic value of albumin gene expression in liver tumors: case report and review of the literature.

Year 1998
Rajkumar SV. Richardson RL. Goellner JR.
Division of Medical Oncology, Mayo Clinic Rochester, Minnesota 55905, USA.
Management of a solitary liver mass necessitates reliable distinction between primary hepatocellular carcinoma and metastatic lesions. The histologic differentiation can be difficult even with special stains such as alpha-fetoprotein, cytokeratin, and carcinoembryonic antigen. Albumin is a specific product of hepatocytes, and in situ hybridization to reveal albumin messenger RNA (mRNA) is highly specific and sensitive for the diagnosis of primary hepatocellular carcinoma. This technique can be used on histopathologic specimens and in cytologic diagnosis. Herein we describe a patient with synchronous renal and hepatic masses, in whom the distinction had to be made between metastatic renal cell carcinoma and two separate primary tumors--one in the liver and one in the kidney. In situ hybridization for albumin mRNA proved helpful in making this distinction. In addition, we review the literature on the diagnostic use of albumin gene expression in liver tumors.

Liver transplantation for primary biliary cirrhosis and primary sclerosing cholangitis: predicting outcomes with natural history models.

Year 1998
Wiesner RH.
Division of Gastroenterology and Hepatology and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905, USA.
In patients with primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), risk score models that reflect disease severity have been developed and can serve as an objective measurement to assess and evaluate the effect of the severity of liver disease on the outcome of liver transplantation. Thus, using the established Mayo risk scores for PBC and PSC, one not only can estimate survival for the individual patient but can measure disease activity as well. Indeed, several studies have suggested that the optimal timing of liver transplantation with use of the Mayo PBC model may be an important tool to improve survival, decrease morbidity, and decrease overall related costs. Likewise, studies in patients with PSC have yielded similar results. This review explores how prognostic mathematical survival models for PBC and PSC might be applied to individual patients in need of liver transplantation. The following question is addressed: How can the timing of liver transplantation be optimized to increase survival, decrease postoperative morbidity, and ultimately, decrease the overall resource utilization involved in this procedure?

GB virus-C infection in type 1 autoimmune hepatitis.

Year 1998
Czaja AJ. Abdulkarim AS. Carpenter HA. Perez RG. Persing DH. Zein NN.
Division of Gastroenterology and Hepatology and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905, USA.
OBJECTIVE: To assess the frequency and significance of GB virus-C infection in type 1 autoimmune hepatitis. MATERIAL AND METHODS: Serum specimens from 94 patients with type 1 autoimmune hepatitis were tested for GB virus-C RNA by reverse transcription and polymerase chain reaction. Serum samples from 50 normal subjects were also assessed. RESULTS: Three of the 94 specimens from patients with autoimmune hepatitis were positive for GB virus-C RNA in comparison with none of the 50 control samples (3% versus 0%; P = 0.5). Two patients were seropositive after variceal hemorrhage and blood transfusion, including one patient who clearly acquired the infection in this fashion. One patient had no epidemiologic basis for his seropositivity. Viremia was prolonged in all infected patients (mean duration, 69 +/- 23 months; range, 36 to 113); however, no clinical features suggested a concurrent viral infection, and mortality was similar to that among the uninfected counterparts (33% versus 8%; P = 0.2). Liver transplantation was more common in the infected patients (67% versus 9%; P = 0.03), but the duration of disease was also longer in these patients (277 +/- 29 months versus 106 +/- 9 months; P = 0.0008). Clinical features and immediate responses to corticosteroid therapy were similar in both groups. CONCLUSION: GB virus-C RNA is found infrequently in type 1 autoimmune hepatitis, and GB virus-C is unlikely to be an important etiologic agent or prognostic determinant.

Localized lymphoplasmacellular pancreatitis forming a pancreatic inflammatory pseudotumor.

Year 1998
Petter LM. Martin JK Jr. Menke DM.
Department of Surgery, Mayo Clinic Jacksonville, Florida 32224, USA.
Inflammatory pseudotumors (IPTs) of the pancreas are rare. To our knowledge, we report the first case of a pancreatic IPT composed of dense lymphocytic and plasmacellular infiltrates that histologically resembled a primary lymphoplasmacytic lymphoma of the pancreas. In addition, it is the first pancreatic IPT analyzed for latent Epstein-Barr virus, an agent implicated in the pathogenesis of IPTs of the liver and spleen.

Management of Barretts esophagus.

Year 1998
Cameron AJ.
Division of Gastroenterology and Hepatology and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905, USA.
In Barrett's esophagus, the squamous lining of the lower esophagus is replaced by columnar epithelium. Barrett's esophagus is associated with gastroesophageal reflux and an increased risk of the development of esophageal cancer. Endoscopy shows red columnar epithelium in the lower esophagus. Biopsy is needed to confirm intestinal metaplasia. Some cases progress from dysplasia to invasive adenocarcinoma. Medical or surgical antireflux treatment controls symptoms and esophagitis, but Barrett's esophagus remains. Patients are usually followed up by endoscopy for detection of dysplasia or early cancer. For patients with low-grade dysplasia, follow-up is adequate; however, for those with high-grade dysplasia, esophagectomy or experimental endoscopic mucosal ablation is advised.

Sarcoidosis of the liver and bile ducts.

Year 1998
Ishak KG.
Department of Hepatic and Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
In sarcoidosis, granulomas are frequently present in multiple organs, including the liver. Typically, epithelioid granulomas (noncaseating) are scattered throughout the liver, but confluent granulomas can be present in cases with severe hepatic involvement. The characteristic inclusions in giant cells (for example, Schaumann bodies and asteroid bodies) are not seen in all cases and are not pathognomonic. The granulomas of sarcoidosis may heal without a trace, but confluent granulomas can result in extensive, irregular scarring. Occlusion of intrahepatic portal vein branches by the granulomatous inflammation probably accounts for the development of portal hypertension in some cases. A granulomatous cholangitis leading to ductopenia seems to be the underlying pathogenetic mechanism of the chronic cholestatic syndrome of sarcoidosis. Recognition of this syndrome is important in the differential diagnosis of other chronic cholestatic diseases, such as primary biliary cirrhosis or primary sclerosing cholangitis. Other rare complications of sarcoidosis are the Budd-Chiari syndrome and obstructive jaundice attributable to hepatic hilar lymphadenopathy or strictures of the bile ducts.

Bacterial and parasitic cholangitis.

Year 1998
Carpenter HA.
Division of Anatomic Pathology, Mayo Clinic Rochester, Minnesota 55905, USA.
Bacterial cholangitis is a clinically defined syndrome caused by the regurgitation of infected bile into the circulation. The pathogenic mechanism is unclear, and systemic sepsis may not occur. Prerequisite conditions are the presence of microorganisms in the bile and increased biliary pressure. Bacteria that commonly cause cholangitis are Escherichia coli, Klebsiella, Enterococcus, Enterobacter, Pseudomonas, and anaerobes. Although most infections are polymicrobial, this situation may not always prevail. Successful treatment depends on relieving biliary obstruction and administering antibiotics effective against bacteria in the circulation and the bile. The causes of biliary obstruction that predispose to bacterial cholangitis are myriad. Common conditions include biliary stones and benign strictures. In many parts of the world, biliary parasites are an important factor. Biliary parasites cause necrosis, inflammation, fibrosis, strictures, and cholangiectasis of the bile ducts by several mechanisms: (1) as a direct result of the irritating chemical composition of the parasite, parasitic secretions, or eggs; (2) physical obstruction of the bile ducts; (3) induction of formation of biliary stones; and (4) introduction of bacteria into the biliary system during migration from the duodenum. Therefore, bacterial cholangitis has an important and frequently dominant role in the pathogenesis and clinical course of biliary disease due to these parasitic infestations. Common biliary parasites include the nematode Ascaris lumbricoides, the trematodes Opisthorchis viverrini and felineus, Clonorchis sinensis, and Fasciola hepatica, and the cestodes Echinococcus granulosus and multilocularis. The epidemiologic, pathologic, and clinical manifestations of these parasitic infestations are reviewed.

Cholangitis in viral disease.

Year 1998
Burgart LJ.
Division of Anatomic Pathology, Mayo Clinic Rochester, Minnesota 55905, USA.
This review of biliary manifestations of viral diseases includes aspects of morphologic diagnosis, therapeutic implications, prognostic effect, and natural history. The viral causes of cholangitis are reviewed, with subclassification on the basis of primary hepatic versus systemic infections and immune competence of the host. Special attention is given to the histopathologic and clinical features of viruses affecting the biliary tree. Among hepatotropic viruses, hepatitis C more frequently is associated with cholangitis than is hepatitis B. In both hepatitis B and hepatitis C, the lymphocytic cholangitis duct damage is reversible and does not adversely influence the course of disease or response to therapy. Hepatitis A and hepatitis E, despite causing clinical cholestasis, do not result in severe cholangitis. The effect of systemic viruses on the biliary tree is primarily dependent on the status of the host immune system. Infants and severely immunosuppressed patients (such as those who have undergone liver transplantation) are at risk for cytomegalovirus cholangitis, whereas patients with late-stage acquired immunodeficiency syndrome (AIDS) are at risk for cholangitis due to numerous organisms. Overall, cholangitis attributable to viral disease encompasses a wide spectrum of clinicopathologic scenarios, depending on the etiologic virus and the immune competence of the host.

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