Barrett's esophagus: prevalence-incidence and etiology-origins.

Although the prevalence of Barrett's esophagus (BE) is rising no data exist for racial minorities on prevalence in the general population. Minorities have a lower prevalence than Caucasians, and yet age, smoking, abdominal obesity, and Helicobacter pylori are all risk factors. Metabolic changes induced by adipocytokines and the apparently strong association between obesity, central adiposity, and BE may lead to reconsideration of some aspects of the natural history of BE. There is lack of experimental evidence on acid sensitivity and BE, which is hyposensitive compared to esophageal reflux disease. Reactive nitrogen and oxygen species lead to impaired expression of tumor suppressor genes, which can lead to cancer development; thus, antioxidants may be protective. Gastroesophageal reflux disease may be considered an immune-mediated disease starting at the submucosal layer; the cytokine profile of the mucosal immune response may explain the different outcome of gastroesophageal reflux.

Pulmonary sequestration with CCAM type II appearing as adrenal tumor protruding through a Bochdalek hernia.

A case of a pulmonary sequestration, which almost exclusively consisted of a congenital cystic adenomatoid malformation type II located subdiaphragmatically in the left retroperitoneal area, is reported. This case, in a 24-year-old male patient, is unique in that it appeared as an adrenal incidentaloma and extended through a Bochdalek hernia into the pleural space. It was discovered upon routine ultrasound screening for hepatocellular carcinoma in a patient with a carrier state for hepatitis B. Diagnosis was established only upon histological analysis of the surgically removed tumor after staining with hematoxylin and eosin as well as surfactant A and B. The location of the tumor may indicate that it was formed by an entrapment of a lung bud by the developing diaphragm. This appearance may give us insight into the formation of such tumors. It also highlights the difficulty of diagnosing subdiaphragmatic retroperitoneal tumors without histological examination.

Unilateral ischemic preconditioning and heterologous preconditioning in living donor liver transplantation.

Ischemic preconditioning (IP) exerts a protective effect on tissues undergoing prolonged ischemia. No studies have been performed to assess the clinical impact of IP on normal human liver used for living donor transplantation (LDLT). Heterologous preconditioning (HP) protects liver tissue as demonstrated in a rat model. Our study investigates the impact that IP and HP have on the donor and recipient liver in LDLT. Twenty candidates for living donor right hepatectomy were divided in two groups. The study group underwent 10' unilateral ischemia by clamping the right portal vein and hepatic artery at the end of the parenchymal transection. Demographics, laboratory values, biopsy studies, IL-1Ra, Ki-67, and CytoDEATH stains were compared. The results show that 10' ischemia does not exert significant clinical and laboratory changes in living donor hepatectomy candidates.

Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases.

The aim of this study is to report the clinicopathologic characteristics of 3 cases of alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation. Specimens of 3 cases of alveolar rhabdomyosarcoma were studied using histologic, immunohistochemical, ultrastructural, and molecular genetic techniques. The patients were a 19-year-old man with metastatic alveolar rhabdomyosarcoma in a groin lymph node, a 16-year-old girl with alveolar rhabdomyosarcoma of the perineum, and a 20-year-old man with recurrent orbital alveolar rhabdomyosarcoma. Microscopically, case 1 was composed of compact sheets of medium to large tumor cells. Cases 2 and 3 were small blue round cell tumors. Cases 1 and 3 were solid throughout, whereas case 2 demonstrated alveolar and solid architecture. By immunohistochemistry, the following markers were positive: desmin (3/3), myogenin (3/3), synaptophysin (3/3), and chromogranin (2/3). Ultrastructurally, sarcomeric filaments were seen in all cases, while neuroendocrine granules were detected only in case 1. PAX:FKHR fusion transcript was identified in case 2, case 3 had a variant PAX3 transcript, and case 1 was negative. The data presented expands the known differentiation of alveolar rhabdomyosarcoma.

Does nonalcoholic fatty liver disease predispose patients to hepatocellular carcinoma in the absence of cirrhosis?

CONTEXT: Hepatocellular carcinoma (HCC) is recognized as a complication of cirrhosis related to nonalcoholic fatty liver disease (NAFLD). Diabetes and the metabolic syndrome are also associated with HCC. However, it is not clear whether NAFLD predisposes patients to HCC in the absence of cirrhosis. OBJECTIVE: To seek evidence that HCC can develop in NAFLD unaccompanied by cirrhosis. DESIGN: Retrospective case study was performed on cases from 2004 to 2007 at the University of Illinois at Chicago Medical Center, using the key words hepatocellular carcinoma, liver explant, and liver resection. The diagnosis of HCC was identified and confirmed by hematoxylin-eosin-stained slides in 50 cases. Cause of liver disease was determined by review of liver histology, clinical history, and laboratory data. RESULTS: Three patients presented with advanced HCC with features of metabolic syndrome, including an elevated body mass index. Each patient had bland steatosis on liver biopsy, without fibrosis or cirrhosis. None of the 3 patients had evidence of any cause for liver disease other than NAFLD. CONCLUSIONS: The cases presented here suggest that NAFLD may predispose patients to HCC in the absence of cirrhosis. Further studies are needed to confirm this potentially important observation.

Endovascular treatment of intimomedial mucoid degeneration.

Intimomedial degeneration is a rare and poorly understood vascular disorder involving the circumferential deposition of large amounts of mucoid material within the intima and media of the arterial wall, causing weakening that results in aneurysm formation of the involved segment. The cause of the disease is unknown at this time. The authors describe the endovascular treatment of a large symptomatic superior gluteal artery aneurysm in a patient with multiple arterial aneurysms and the histologic diagnosis of intimomedial mucoid degeneration. In addition, they perform a review of the literature on this unusual vasculopathy.

Adult living-donor liver transplantation with ABO-incompatible grafts.

BACKGROUND: Liver transplantation using ABO-incompatible grafts is rarely performed because the reported outcome is poorer than with compatible grafts. We report our positive experience with adult-to-adult living-donor liver transplant (LDLT) using ABO-incompatible grafts. METHODS: The immunosuppressive protocol consisted of plasmapheresis/intravenous immunoglobulin infusion before LDLT followed by thymoglobulin induction and splenectomy, maintenance with tacrolimus/cyclosporine (FK/CSA), mycophenolate mofetil, and a rapid steroid taper. Plasmapheresis was planned for up to 3 months after LDLT aiming at maintaining the anti-ABO titers level below 1:16. Liver biopsies were routinely stained for humoral rejection with complement 4d (C4d) and for biliary damage with cytokeratin 7. RESULTS: Between January 2003 and September 2004, five patients, mean age 59 years, received an ABO-incompatible LDLT. Patient and graft survival was 80% at mean follow-up of 43 months (range, 34-54) for the four surviving patients. One patient died 4 months after LDLT. Humoral rejection occurred in one patient whereas acute cellular rejection was diagnosed in four patients. CONCLUSIONS: ABO-incompatible LDLT can be performed with patient and graft survival similar to compatible LDLT. Minimization of immunosuppression is possible, and chronic biliary damage is not the norm. Better tools than complement 4d staining must be researched to diagnose the features of immunologic damage to the graft. If these results will be confirmed in a greater number of patients, ABO-incompatible LDLT may be proposed when ABO-compatible donors are not available or when the ABO-incompatible donor is the better candidate.

Five-year colon surveillance after screening colonoscopy.

BACKGROUND & AIMS: Outcomes of colon surveillance after colorectal cancer screening with colonoscopy are uncertain. We conducted a prospective study to measure incidence of advanced neoplasia in patients within 5.5 years of screening colonoscopy. METHODS: Three thousand one hundred twenty-one asymptomatic subjects, age 50 to 75 years, had screening colonoscopy between 1994 and 1997 in the Department of Veterans Affairs. One thousand one hundred seventy-one subjects with neoplasia and 501 neoplasia-free controls were assigned to colonoscopic surveillance over 5 years. Cohorts were defined by baseline findings. Relative risks for advanced neoplasia within 5.5 years were calculated. Advanced neoplasia was defined as tubular adenoma greater than > or =10 mm, adenoma with villous histology, adenoma with high-grade dysplasia, or invasive cancer. RESULTS: Eight hundred ninety-five (76.4%) patients with neoplasia and 298 subjects (59.5%) without neoplasia at baseline had colonoscopy within 5.5 years; 2.4% of patients with no neoplasia had interval advanced neoplasia. The relative risk in patients with baseline neoplasia was 1.92 (95% CI: 0.83-4.42) with 1 or 2 tubular adenomas <10 mm, 5.01 (95% CI: 2.10-11.96) with 3 or more tubular adenomas <10 mm, 6.40 (95% CI: 2.74-14.94) with tubular adenoma > or =10 mm, 6.05 (95% CI: 2.48-14.71) for villous adenoma, and 6.87 (95% CI: 2.61-18.07) for adenoma with high-grade dysplasia. CONCLUSIONS: There is a strong association between results of baseline screening colonoscopy and rate of serious incident lesions during 5.5 years of surveillance. Patients with 1 or 2 tubular adenomas less than 10 mm represent a low-risk group compared with other patients with colon neoplasia.

Primary biliary B-cell lymphoma of the cystic duct causing obstructive jaundice.

A distinctive case of primary biliary lymphoma occurring in the cystic duct causing extrinsic compression of the porta hepatis in a 48-year-old woman is presented. Imaging studies revealed stricture of the common hepatic duct with a 2.5-cm nonhomogeneous mass at the porta hepatis, mimicking a Klatskin tumor. Exploratory laparotomy revealed a mass in the gallbladder neck with extension into the cystic duct akin to Mirizzi's syndrome. A soft, tan-yellow 1.0-cm mass was removed from the cystic duct, and infiltrating atypical lymphocytic nodules were identified with a final diagnosis of an anaplastic variant of diffuse large B-cell lymphoma.

Peliosis hepatis in a living related liver transplantation donor candidate.

Peliosis hepatis is a rare benign condition histologically characterized by multiple cystic blood-filled spaces distributed throughout the liver parenchyma. Peliosis hepatis has been associated with malignancies, immunosuppression, infections and medications. We report a case of peliosis hepatis in a candidate for living liver donation, which regressed with restitutio ad integrum, after the noxious stimulus was stopped. We conclude that after diagnosis of peliosis hepatis is established and its cause is removed, simple radiographic imaging is sufficient to document the restitutio ad integrum of the parenchyma, avoiding repeat histological confirmation.

Thymoglobulin induction protects liver allografts from ischemia/reperfusion injury.

BACKGROUND: Interventions that minimize hepatic ischemia/reperfusion injury (IRI) can expand the donor organ pool. Thymoglobulin (TG) induction therapy has been shown to ameliorate delayed graft function and possibly decrease IRI in cadaver renal transplants recipients. This controlled randomized trial was designated to assess the ability of TG to protect against IRI in liver transplant recipients. PATIENTS AND METHODS: Twenty-two cadaveric liver transplant recipients were randomized to receive either TG (1.5 mg/kg/dose) during the anhepatic period and QOD x2 doses or no TG. No differences in recipients' demographics were present and donor characteristics were similar in terms of age, cause of death, and cold ischemia time. Maintenance immunosupression consisted of Tacrolimus (or Cyclosporine) and steroids for both groups. Donor biopsies were obtained during organ procurement, cold storage and 1 h after re-vascularization. Post-operative liver function tests were monitored. Early graft function, length of stay, patient and graft survival rates, incidence of primary non-function and rate of rejection were assessed. RESULTS: Patient and graft survival at 3 months was 100%. There was no incidence of primary graft non-function and no need for re-transplantation. The incidence of acute rejection was similar between the two groups. Patients in the TG group had significant decreases in alanine aminotransferase test at day 1 compared to the control group (p = 0.02). There were also near significant decreases of total bilirubin at day 5 and shorter length of hospitalization. Liver biopsy (at procurement, when cold, and post-reperfusion) of TG group demonstrated a trend for increased central ballooning. CONCLUSION: The TG allowed for more compromised liver grafts to be transplanted with less clinical evidence of IRI and improved function. Further studies on the degree of apoptosis in the liver biopsy post-reperfusion are underway.

Early experience employing a linear hepatic parenchyma coagulation device.

BACKGROUND: In recent years, hepatic resection for primary and metastatic disease has been facilitated by improved anesthetic and surgical techniques, as well as by the application of new technologies. Historically, the major complications associated with hepatic resection have been postoperative bleeding, bile leak, and liver failure. Resection techniques and devices that minimize hemorrhage and bile leak, and enable the preservation of functional hepatic parenchyma, have been useful in the surgical management of liver tumors. METHODS: Herein, the use of a radiofrequency powered device for the pretransection coagulation of hepatic tissue that simultaneously seals blood vessels and bile ducts 3 mm in diameter or smaller is described. RESULTS: Early results from our single-center experience with the use of this linear radiofrequency device are reported. Seven patients underwent liver resection for either hepatocellular carcinoma or colorectal cancer metastases. There were no postoperative bile leaks, hemorrhage, or hepatic insufficiency. No transfusions were required, and the mean estimated blood loss for the parenchymal transection phase was less than 165 cc. CONCLUSIONS: The linear radiofrequency device is expedient for the pretransection coagulation of hepatic tissue and, thus, facilitates liver resection.

Asthmatics with gastroesophageal reflux: long term results of a randomized trial of medical and surgical antireflux therapies.

OBJECTIVE: In short term studies, asthma symptoms and pulmonary function have been reported to improve during and after medical treatment or surgical correction of gastroesophageal reflux (GER). In this study, we aimed to determine whether prolonged treatment of GER altered the long term natural history of asthma in asthmatics with GER. METHODS: A total of 62 patients with both GER and asthma entered a randomized study of antireflux treatments for at least 2 yr: 24 controls (antacids as needed); 22 medical (ranitidine 150 mg t.i.d.); and 16 surgical (Nissen fundoplication). Asthma was defined as a previous diagnosis of asthma with discrete attacks of wheezing and 20% reversibility in airway disease. GER was defined as an abnormal ambulatory 24-h esophageal pH test and macroscopic or microscopic evidence of GER disease. Overall clinical status, asthma symptom scores, and pulmonary medication requirements were recorded monthly. Peak expiratory flow rates were recorded up to seven times per day for 1 wk of each month throughout the years. Pulmonary function, esophageal manometry, and endoscopy with biopsy were repeated yearly. RESULTS: The 62 patients were followed for up to 19.1 yr. In the surgical group, but not in the medical or control groups, there was an immediate and sustained reduction in acute nocturnal exacerbations of wheezing, coughing, and dyspnea. By the end of 2 yr, improvement, marked improvement, or cure in the overall asthma status occurred in 74.9% of the surgical group, 9.1% of the medical group and 4.2% of the control group, whereas the overall status worsened in 47.8% of the control group, 36.4% of the medical group, and 12.5% of the surgical group (p < 0.001, surgical vs medical and control). The mean asthma symptom score of the surgical group improved 43%, compared with less than 10% in the medical and control groups (p = 0.0009). As determined by changes in peak expiratory flow rates, there was no statistically significant difference in pulmonary function during the 2-yr period or during regularly scheduled follow-up. There was no difference in medication requirements among the groups. There was no difference between the groups in overall survival. CONCLUSION: In patients with both GER and asthma, antireflux surgery (but not medical therapy with ranitidine 150 mg t.i.d.) has minimal effect on pulmonary function, pulmonary medication requirements, or survival, but significantly improves asthma symptoms and overall clinical status.

Gastrointestinal stromal tumors: a spectrum of disease.

The majority of gastrointestinal stromal tumors (GIST) express c-kit, a growth factor receptor with tyrosine kinase activity. Mutations in the c-kit proto-oncogene may lead to constitutive ligand-independent activation of c-kit and subsequent neoplastic transformation. Selective tyrosine kinase inhibitors target this property of GIST and have become the standard chemotherapy for metastatic or unresectable tumors. The mainstay of treatment, however, continues to be complete surgical resection. Tyrosine kinase inhibitors may prove expedient for adjuvant therapy, and are currently the focus of clinical trials conducted by the ACOSOG, RTOG, and ACRIN. It is important to distinguish GISTs from other mesenchymal tumors of the GI tract because of differences in natural history, as well as the efficacy of treatments targeting the GIST tyrosine kinase.

Hemostatic modulation with the liver dialysis device in humans with advanced liver disease.

BACKGROUND/AIMS: To determine the alterations in coagulation and fibrinolysis occurring in patients treated with the liver dialysis device. METHODOLOGY: Patients with advanced liver disease treated with the liver dialysis device were studied immediately before and after the liver dialysis device treatment. SETTING: A university hospital intensive care unit. PATIENTS: Thirteen consecutive patients with advanced liver disease being evaluated for or awaiting liver transplantation. INTERVENTION: 4-6 hours of liver dialysis treatment for management of hepatic encephalopathy. OUTCOME MEASURES: A panel of coagulation and anticoagulation factors, as well as fibrinolytic and anti-fibrinolytic factors plus measures of activation of inflammation and soluble adhesion factors. RESULTS: The liver dialysis device used was found to be associated with activation of both coagulation and fibrinolytic pathways, activation of inflammation reactants, and an increase in sL-selectin levels. CONCLUSIONS: Liver dialysis device activates both coagulant and fibrinolytic pathways, activates inflammatory response, but these responses are limited to the vascular compartment by an increase in sL-selectin levels.

Hiatal hernia size, Barrett's length, and severity of acid reflux are all risk factors for esophageal adenocarcinoma.

OBJECTIVE: The reasons for the development of dysplasia and adenocarcinoma in Barrett's mucosa are not well understood. The aims of this study were to characterize risk factors for the transition from Barrett's esophagus without dysplasia to Barrett's esophagus with high-grade dysplasia or esophageal adenocarcinoma. METHODS: A group of 131 patients with high-grade dysplasia or esophageal adenocarcinoma were selected as case subjects. A first population of 2170 patients without gastroesophageal reflux disease (GERD) and a second population of 1189 patients with Barrett's esophagus served as two control groups. Logistic regression analyses were used to compare the risk factors associated with the occurrence of high-grade dysplasia or esophageal adenocarcinoma. RESULTS: Patients with high-grade dysplasia or esophageal adenocarcinoma shared many characteristics with other forms of severe GERD, such as older age, male gender, and white ethnicity. The length of Barrett's esophagus and the size of hiatus hernia increased the risk for both conditions. Subjects with high-grade dysplasia and adenocarcinoma had more severe acid reflux than patients with other forms of GERD. Smoking and alcohol consumption did not affect the risk for developing high-grade dysplasia or adenocarcinoma in patients with Barrett's esophagus. CONCLUSIONS: High-grade dysplasia and esophageal adenocarcinoma seem to stem from an extreme and unfavorable constellation of all risk factors that are generally held responsible for the development of GERD and Barrett's esophagus.

Malakoplakia of liver diagnosed by a needle core biopsy: a case report and review of the literature.

Although malakoplakia of the genitourinary tract and colon is reported frequently in the literature, malakoplakia that occurs primarily in the liver is rare, and only 4 cases have been described thus far. To our knowledge, this is the first case of malakoplakia of the liver diagnosed by a needle core biopsy. This case occurred in a 19-year-old man with small bowel ileus following Klebsiella pneumonia.