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BACKGROUND: Acute liver failure (ALF) remains a dramatic and unpredictable disease with high morbidity and mortality. Early and accurate prognostic assessment of patients with ALF is critically important for optimum clinical pathway. DATA SOURCES: Five English-language medical databases, MEDLINE, ScienceDirect, OVID, Springer Link and Wiley Interscience were searched for articles on "acute liver failure", "prognosis", and related topics. RESULTS: Multi-variable prognostic models including the Kings College Hospital criteria and the model for end-stage liver disease score have been widely used in determination of the prognosis of ALF, but the results are far from satisfactory. Other prognostic indicators including serum Gc-globulin, arterial blood lactate, serum phosphate, arterial blood ammonia, and serum alpha-fetoprotein are promising but await further assessement. CONCLUSIONS: A reliable prognostic model to be developed in the future should not only have predictive value for poor outcome but also help to predict the survival of patients without a liver transplantation. Further studies are necessary to assess the prognostic accuracy of any new models.
BACKGROUND: Gallbladder cancer (GBC) is a rare disease but a leading cause of cancer-related death worldwide. A number of etiological factors have been implicated in the causation of GBC and pathogenic infection by bacteria is one of these. DATA SOURCES: A PubMed search on "helicobacter", "gallbladder cancer", and "biliary tract malignancies" was done on the topic, and the relevant data were collected, reviewed, and analyzed. RESULTS: Helicobacter is an epsilon proteobacterium that infects the mucosal lining of the human gastrobiliary system. Infection with helicobacter is an important risk factor for the development of cancer and the bacterium has been categorized as a group-Ⅰcarcinogen by the International Agency for Research on Cancer (IARC). These microbes enter the human body by means of contaminated food and water. Thereby they invade the tissues and produce chemical carcinogens that lead to DNA damage and subsequently a series of gene mutations transform normal cells into cancer cells. In this review, we focus our attention on the role of helicobacter in the causation of biliary tract malignancies. CONCLUSIONS: The review attempts to summarize the current available data on the role of helicobacter in the causation of GBC. There are accountable data available to suggest the role of helicobacter species in the causation of GBC although larger studies are urgently required for confirmation.
BACKGROUND: Congestion of the right anterior segment may lead to graft dysfunction in right-lobe living donor liver transplantation (LDLT) without a middle hepatic vein (MHV) trunk. Selective reconstruction of MHV tributaries with the interposition of vascular grafts has been introduced to overcome this problem. However, there is still no consensus on the definite criteria of MHV reconstruction. METHODS: LDLT patients were reviewed to evaluate the effects of MHV reconstruction. From March 2005 to September 2008 in our transplantation center, 120 consecutive LDLTs were performed using a right-lobe graft without a MHV. Excluding 11 patients, among the remainder, 73 (67%) had reconstructed MHV tributaries, and the others 36 (33%) did not. The values of liver functional index and liver graft regeneration ratio were compared between the two groups. RESULTS: There was a prolonged period of liver functional recovery in patients with small-for-size grafts and a graft-recipient weight ratio (GRWR) <1.0%, and without MHV reconstruction. The ratio of liver regeneration 1 month post-operatively in reconstruction cases was 81%, versus 78% in patients without reconstruction (P=0.352), but among small-for-size grafts, there was a significant difference between the two groups (95% vs. 80%). CONCLUSION: Our study shows that reconstruction of MHV tributaries is not necessary in all patients, but is beneficial for patients with GRWR <1.0%.
BACKGROUND: Liver transplantation (LT) is an effective therapy for end-stage hepatitis B virus (HBV) infection. Recurrence of HBV is one of the frequent complications. In the present study, we investigated whether human leukocyte antigen (HLA) matching influences the incidence of HBV recurrence, and the time point of HBV recurrence after LT. METHODS: One hundred and two recipients of LT with end-stage chronic HBV infection were reviewed. The triple-drug immunosuppression regimen consisted of tacrolimus, mycophenolate, and prednisone. All patients were subjected to prophylaxis with hepatitis B immunoglobulin and lamivudine. HLA typing was performed using a sequence-specific primer-polymerase chain reaction kit. Serology for hepatitis B and HBV DNA was examined using a commercial kit. RESULTS: The incidence of recurrent HBV infection post-LT was 6.86%. The recurrent infection of HBV was independent of the degree of HLA matching (P>0.05). The time point of HBV recurrence, however, was prolonged in HLA-A matched patients compared with matchless patients (P=0.049). The recurrence of HBV infection was independent of HLA compatibility. CONCLUSIONS: This retrospective analysis showed that more HLA-A locus compatibility is associated with a prolonged time of recurrence of HBV in patients after LT for end-stage HBV infection. The incidence of HBV recurrence is independent of HLA compatibility.
BACKGROUND: Interleukin 10 (IL-10), a Th2 type cytokine, modulates inflammatory responses by inhibiting the production of proinflammatory cytokines. This study was designed to investigate the protective effects of adenovirus-mediated human IL-10 (Ad-hIL-10) gene transfer on protecting grafts from cold ischemia-reperfusion injury following orthotopic liver transplantation in rats. METHODS: Adenoviruses encoding hIL-10 or β-galactosidase (Ad-lacZ) were injected via the superior mesenteric vein into prospective donor animals. The donor liver was harvested 48 hours after transduction, and stored for 12 hours at 4 ℃in lactated Ringer's solution prior to transplantation. The rats were divided into saline, Ad-lacZ, and Ad-hIL-10 groups. Liver function test, histopathological examination, reverse transcriptase-polymerase chain reaction (RT-PCR), and Western blotting were performed at 24 hours after transplantation in the three groups. RESULTS: Liver function (ALT and AST) was significantly improved, and the Suzuki score was significantly decreased in the Ad-hIL-10 group. The levels of hepatic TNF-α, MIP-2, ICAM-1 mRNA, and NF-κB protein in the Ad-hIL-10 group were significantly decreased. The expression of hIL-10 mRNA was detected by RT-PCR in Ad-hIL-10-treated grafts but not in controls treated with saline or Ad-lacZ. CONCLUSIONS: Donor pretreatment with Ad-hIL-10 down-regulates the expression of proinflammatory cytokines TNF-α, MIP-2, and ICAM-1 mRNA. hIL-10 protects against hepatic cold ischemia-reperfusion injury, at least in part, by suppressing NF-κB activation and subsequent expression of proinflammatory mediators.
BACKGROUND: Early diagnosis of liver metastasis of colorectal carcinoma is very important for the appropriate treatment of such patients. However, there has been no effective approach available for clinical application. The present study aimed to investigate the differential expression of proteins in patients with liver metastasis of colorectal carcinomas using proteomic analysis and evaluate its potentiality in clinical diagnosis. METHODS: Fluorescence two-dimensional differential in-gel electrophoresis (2-D DIGE) was used to analyze and compare the protein expression between normal mucosa, the primary focus, and liver metastases. Proteomic analysis was made to identify the differentially expressed proteins. Immunohistological staining was used to confirm the expression of differentially expressed proteins in colorectal carcinomas and areas of liver metastasis. RESULTS: A 1.5-fold difference was found with 46 differentially expressed proteins. In 20 differentially expressed proteins, 3 were down-regulated and 17 up-regulated in liver metastases. Proteomic analysis showed that the S-adenosylmethionine transgelin variant was down-regulated in liver metastasis tissues. Zinc finger protein 64 homolog (Zfp64), guanine nucleotide exchange factor 4 (GEF4), human arginase, glutathione S-transferases (GSTs) A3, and tumor necrosis factor α (TNF-α)-induced protein 9 were up-regulated in liver metastasis tissues. Immunohistochemical staining confirmed that human arginase expression was higher in liver metastases than in the primary focus. CONCLUSIONS: There was a significant difference in protein expression between the primary focus of colorectal carcinoma and liver metastases. The differentially regulated proteins were closely related to liver metastasis of colorectal carcinoma. Elevated human arginase may be an important molecular marker for liver metastasis from colorectal carcinoma.
BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Although its unusual imaging and pathologic findings are being recognized with increasing frequency, diagnosis is still difficult. This study aimed to analyze the CT and MRI features of hepatic EHE with a pathological study in order to improve the diagnostic accuracy and knowledge of this disease in daily practice. METHODS: Nine patients with hepatic EHE confirmed pathologically underwent plain and dynamic contrast-enhanced multidetector row CT examination. Of these patients, four underwent additional MRI (plain T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), and dynamic contrast-enhanced scanning) and one had selective hepatic arteriography. The imaging findings were reviewed retrospectively together with the pathological results. RESULTS: A total of 79 lesions, ranging from 3.0 to 44.6 mm in maximum diameter, with an average of 16.8±7.1 mm, were found in various segments of the liver. Thirty of the 79 lesions grew adjacent to the hepatic capsule. In the 4 patients receiving MRI, 39 lesions were found with low signal intensity on unenhanced T1WI and intermediate to high signal intensity on T2WI. The "capsular retraction" sign was found in all the 4 patients. Nine of the 39 lesions showed the "halo" sign after contrast enhancement on MRI. Of the 79 lesions (hypodense nodules) in the 9 patients shown by unenhanced plain CT, 26 were confluent. Calcification was found in 2 patients and the "capsular retraction" sign in 7. Thirty-eight of the 79 lesions demonstrated the "halo" sign after contrast enhancement on CT, and this sign was more clearly demonstrated in the portal venous phase. In one patient, selective hepatic arteriography showed patchy stain in the peripheral liver parenchyma with small vessels around them. Histology in all patients revealed proliferation of abnormal fibrous tissue and vessel-like structures scattered with irregular epithelioid cells having a signet ring-like structure. Immunohistochemically, all patients were positive for CD34, 4 were positive for CD31, and 3 were positive for factor Ⅷ-related antigen. CONCLUSIONS: Hepatic EHE may manifest as solitary or diffuse nodular lesions with a predilection for peripheral subcapsular growth and nodular confluence, together with the "halo" and "capsular retraction" signs. These imaging findings can help to improve the diagnostic accuracy of this rare hepatic tumor.
BACKGROUND: Primary liver cancer (PLC) is one of the common malignant tumors. Liver acquisition with acceleration volume acquisition (LAVA), which allows simultaneous dynamic enhancement of the hepatic parenchyma and vasculature imaging, is of great help in the diagnosis of PLC. This study aimed to evaluate application of the fluoroscopic triggering 3D LAVA technique in the imaging of PLC and liver vasculature. METHODS: The clinical data and imaging findings of 38 adults with PLC (22 men and 16 women; average age 52 years), pathologically confirmed by surgical resection or biopsy, were collected and analyzed. All magnetic resonance images were obtained with a 1.5-T system (General Electrics Medical Systems) with an eight-element body array coil and application of the fluoroscopic triggering 3D LAVA technique. Overall image quality was assessed on a 5-point scale by two experienced radiologists. All the nodules and blood vessel were recorded and compared. The diagnostic accuracy and feasibility of LAVA were evaluated. RESULTS: Thirty-eight patients gave high quality images of 72 nodules in the liver for diagnosis. The accuracy of LAVA was 97.2% (70/72), and the coincidence rate between the extent of tumor judged by dynamic enhancement and pathological examination was 87.5% (63/72). Displayed by the maximum intensity projection reconstruction, nearly all cases gave satisfactory images of branches Ⅲ and Ⅳ of the hepatic artery. Furthermore, small early-stage enhancing hepatic lesions and the parallel portal vein were also well displayed. CONCLUSIONS: Sequence of LAVA provides good multi-phase dynamic enhancement scanning of hepatic lesions. Combined with conventional scanning technology, LAVA effectively and safely displays focal hepatic lesions and the relationship between tumor and normal tissues, especially blood vessels.
BACKGROUND: Conventional drugs used in the treatment and prevention of liver diseases often have side effects, therefore research into natural substances are of significance. This study examined the effects of blueberry on liver protection and cellular immune functions. METHODS: To determine the effects of blueberry on liver protective function, male mice were orally administered blueberry (0.6 g/10 g) or normal saline for 21 days. Hepatic RNA was extracted by Trizol reagent, and the expression of Nrf2, HO-1, and Nqo1 was determined by real-time RT-PCR. Superoxide dismutase (SOD) and malondialdehyde (MDA) in liver homogenate were determined, and liver index was measured. To assess the effects of blueberry on cellular immune function, male mice received blueberry (0.4, 0.6, or 0.8 g/10 g) for 35 days, and the percentages of CD3+, CD4+, and CD8+ T lymphocyte subgroups in peripheral blood were detected by flow cytometry, the index of the thymus and spleen was measured, and lymphocyte proliferation in the spleen was determined by MTT assay. RESULTS: Blueberry treatment significantly increased the expression of Nrf2, HO-1, and Nqo1, the important antioxidant components in the liver. Hepatic SOD in the blueberry group was higher and MDA was lower than that in the control group (P<0.05). Blueberry also increased the index of the spleen and enhanced the proliferation of lymphocytes of the spleen (P<0.05). The percentages of the CD3+ and CD4+ T lymphocyte subsets and the CD4+/CD8+ ratio were also increased by blueberry (P<0.05). CONCLUSIONS: Blueberry induces expression of Nrf2, HO-1, and Nqo1, which can protect hepatocytes from oxidative stress. In addition, blueberry can modulate T-cell function in mice.
BACKGROUND: The active form of nuclear factor-kappa B (NF-κB) is involved in the initiation, generation, and development of hepatocellular carcinoma (HCC), and is up-regulated in inflammation-associated malignancies. We investigated the dynamic expression of NF-κB and its influences on the occurrence of HCC through antiangiogenic (thalidomide) intervention in NF-κB activation. METHODS: Hepatoma models were induced with 2-fluorenylacetamide (2-FAA, 0.05%) in male Sprague-Dawley rats, and thalidomide (100 mg/kg body weight) was administered intragastrically to intervene in NF-κB activation. The pathological changes in the liver of sacrificed rats were assessed after hematoxylin and eosin staining. NF-κB mRNA was amplified by RT-nested PCR. The alterations of NF-κB and vascular endothelial growth factor (VEGF) expression were analyzed by enzyme-linked immunosorbent assay, immunohistochemistry, and Western blotting. RESULTS: Rat hepatocytes showed denatured, precancerous, and cancerous stages in hepatocarcinogenesis, with an increasing tendency of hepatic NF-κB, NF-κB mRNA, and VEGF expression, and their values in the HCC group were higher than those in controls (P<0.001). In the thalidomide-treated group, the morphologic changes generated only punctiform denaturation and necrosis at the early or middle stages, and nodular hyperplasia or a little atypical hyperplasia at the final stages, with the expression of NF-κB (χ2=9.93, P<0.001) and VEGF (χ2=8.024, P<0.001) lower than that in the 2-FAA group. CONCLUSION: NF-κB is overexpressed in hepatocarcinogenesis and antiangiogenic treatment down-regulates the expression of NF-κB and VEGF, and delays the occurrence of HCC.
BACKGROUND: Sarcomatoid carcinoma of the gallbladder is rare and its characteristics are poorly understood. This study aimed to understand the behavior and prognosis of sarcomatoid carcinoma of the gallbladder as well as its clinical manifestations and survival rate of patients after radical or palliative surgery, and to review the reported data worldwide and our 10 patients. METHODS: From 2004 to 2009, ten patients were pathologically diagnosed with sarcomatoid carcinoma of the gallbladder and underwent operation at our center. These characteristics, clinical presentations, tumor-node-metastasis (TNM) staging, surgical modes, and prognosis were reviewed, retrospectively. We collected the data of 46 patients reported in the English-language literature worldwide and analyzed the survival with ours. The survival rate was estimated using the Kaplan-Meier method, and was compared using the log-rank test. RESULTS: The median age of the 10 patients was 67 years (inter-quartile range 59-74 years), and the size of tumor inter-quartile ranged from 3.1 to 7.9 cm. In this series, 9 patients received radical surgery, and one undewent palliative surgery. There was no surgical mortality, and one patient underwent a second operation because of liver metastasis. The median survival time of the patients was 9 months (inter-quartile range 6-12 months), with 3 patients still being alive until follow-up; however, two patients had tumor recurrence. The data from the 56 patients (10 patients in our series and 46 reported elsewhere) statistically indicated that the median age was 66 years (inter-quartile range 61-74.5 years) and the overall median survival was 5.5 months (inter-quartile range 2.5-10 months). The survival time in the patients undergoing radical surgery (n=42) was significantly longer than that in the patients undergoing palliative surgery (n=14) (P=0.031). CONCLUSIONS: The survival of the patients with sarcomatoid carcinoma of the gallbladder is poor. Some patients may die shortly after the surgery because of recurrence or metastasis. However, radical surgery is still necessary if possible.
BACKGROUND: Biliary interventions during pregnancy are associated with risks to both the pregnancy and developing fetus. In this report we summarize our experience with endoscopic interventions including endoscopic ultrasound (EUS) in the management of biliary disorders during pregnancy. METHODS: Endoscopic retrograde cholangiopancreatographies (ERCPs) performed between May 2003 through January 2010 (n=607) were identified from our database, and cases of interventions during pregnancy were reviewed. All procedures were done using conscious sedation and lead shielding. RESULTS: Nine ERCPs (1.5%) were performed in 8 pregnant patients. Their median gestational period was 22 weeks (range, <2-36 weeks). Two, 5 and 2 patients were in their first, second and third trimester, respectively. Indications for ERCP included obstructive jaundice (6 patients) cholangitis (2), and acute pancreatitis/obstructive jaundice (1). Two patients underwent EUS before ERCP. Fluoroscopy was used in 5 ERCPs (median 12 seconds; range 2-20 seconds), and the overall time for a ERCP ranged from 5 to 25 minutes. During ERCP endoscopic sphincterotomy was performed in 5 patients, stenting in 6, and balloon clearance in 3. One procedure caused complication in induction of labor. During pregnancy, there were 4 non-procedure related complications including acute cholecystitis (1), HELLP syndrome resulting in spontaneous abortion (1) and stent migrations (2). Five pregnancies had uncomplicated term deliveries, whereas 2 required urgent caesarian sections (one for fetal distress and 1 for cholangitis secondary to stent migration). One patient was well in her second trimester during follow-up. Seven babies were well at birth with median APGAR scores of 9, and 10 at 5 and 10 minutes, respectively. One baby died of sudden death syndrome at age of 40 days. CONCLUSIONS: ERCP is a safe procedure for pregnant women. It can be conducted for biliary stenting and subsequent clearance after deliveries. EUS has a complementary role. Different strategies can be applied according to the conditions or expertise of endoscopists.
BACKGROUND: Hilar cholangiocarcinoma is one of the most difficult carcinomas to manage because of the location of the main tumor at the hepatic hilus and the complex anatomy of the biliary, arterial, and portal systems. To plan an operation, it is important to acquire accurate information about the relationship between hilar cholangiocarcinoma and adjacent vessels. This study aimed to evaluate the clinical value of cholangiography combined with spiral CT three-dimensional (3D) angiography for a preoperative assessment of hilar cholangiocarcinoma. METHODS: From March 2007 to August 2009, cholangiography was performed in 13 patients with hilar cholangiocarcinoma. Meanwhile, contrast-enhanced abdominal scanning was performed using 16-slice spiral CT, and the 3D images of the hepatic artery and portal vein were acquired. The level and range of invasion of the hepatic artery, the portal vein, and the bile duct, the preoperative Bismuth classification, and T-staging were recorded and compared with those after surgical exploration. RESULTS: The hepatic artery and portal vein were reconstructed successfully in all these patients. Percutaneous transhepatic cholangiography was performed in 9 patients, endoscopic retrograde cholangiopancreatography in 1, and magnetic resonance cholangiopancreatography in 3. The CT angiography records of invasion of the hepatic artery were consistent with the results of explorations in these patients. The data from 5 of the 13 patients were consistent with those on invasion of the portal vein. The results of the Bismuth classification and the T-staging system were consistent with those of surgical exploration in 12 of the 13 patients. Seven of 8 patients who were estimated to be suitable for operation based on images were curatively treated and 5 who were judged to be unsuitable for curative operation by cholangiography and CT angiography were confirmed intraoperatively and underwent palliative procedures. CONCLUSIONS: Cholangiography combined with multi-slice spiral 3D CT angiography can satisfactorily delineate the local invasion of hilar cholangiocarcinoma and accurately evaluate the resectability. This approach, therefore, contributes to the planning of safe operation.
BACKGROUND: Studies have revealed that macrophages play an important role in the development of severe acute pancreatitis (SAP). Activated macrophages can lead to a systemic inflammatory response, induce lipid peroxidation, impair membrane structure, result in injury to the liver and the other extrahepatic organs, and eventually result in multiple organ dysfunction syndrome by promoting excessive secretion of cytokines. Liver injury can further aggravate the systemic inflammatory response and increase mortality by affecting the metabolism of toxins and the release of excessive inflammatory mediators. Clodronate is a synthetic bisphosphonate, which is often used for treating bone changes caused by osteoporosis and other factors. In the current study, we created liposomes containing superparamagnetic iron oxide particles (SPIOs) for macrophage labeling and magnetic resonance imaging, using a novel method that can bind the clodronate to induce apoptosis and deplete macrophages. METHODS: Superparamagnetic Fe3O4 nanoparticles were prepared by chemical coprecipitation. SPIO-containing liposomes and SPIO-clodronate-containing liposomes were prepared by the thin film method. SAP models were prepared by injection of sodium taurocholate (2 ml/kg body weight) into the subcapsular space of the pancreas. Sprague-Dawley rats were randomly divided into a control group, a SAP plus SPIO-liposome group, and a SAP plus SPIO-clodronate-containing group. Two and six hours after SAP models were available, T2-weighted MRI scans (in the same plane) of the livers of rats in each group were performed. At the end of the scans, 2 ml of blood was taken from the superior mesenteric vein to measure the levels of serum amylase, ALT, AST, TNF-α, and IL-6. Pathological changes in the liver and pancreas were assessed. RESULTS: Transmission electron microscopy showed that the liposomes had a uniform size. No pathological changes in the pancreata of rats in the control group were noted. The pathological changes in the pancreata and livers of rats in the SAP plus SPIO-clodronate-containing liposome group were milder than those in the SAP plus SPIO-liposome group. The MRI signal intensity of the livers in the SAP plus SPIO-liposome and SAP plus SPIO-clodronate-containing groups was significantly lower than that in the control group. There were significant changes in the two experimental groups (P<0.01). In addition, the levels of serum amylase, ALT, AST, TNF-α, and IL-6 in rats in the SAP plus SPIO-liposome group were higher than those in the control group (P<0.01), while the corresponding levels in the SPIO-clodronate-containing liposome group were significantly lower than those in the SAP plus SPIO-liposome group (P<0.01). CONCLUSION: Clodronate-containing liposomes protect against liver injury in SAP rats, and SPIO can be used as a tracer for MRI examination following liver injury in SAP rats.
BACKGROUND: Chemokines and their receptors play key roles in the pathogenesis of acute pancreatitis. This study aimed to establish a rat model of severe acute pancreatitis (SAP) for investigating monocyte chemotactic protein-1 (MCP-1) expression in the pathogenesis of the disease. We assessed the effects of the inhibitor of MCP-1, Bindarit, on SAP and explored the mechanisms underlying SAP. METHODS: Seventy-two Sprague-Dawley rats were randomly divided into a saline control group (group S), an SAP group (group P), and a Bindarit group (group T). The SAP model was induced by retrograde infusion of 4% sodium taurocholate into the biliopancreatic duct. Based on the SAP model, Bindarit was injected intraperitoneally in group T, and 0.5% methyl cellulose was injected intraperitoneally in groups S and P. In group S, saline was retrogradely infused into the bilipancreatic duct. Serum amylase levels and the histological changes in the pancreas were assessed at different time-points in each group. Expression of MCP-1 in serum was measured by enzyme-linked immunoadsorbent assay (ELISA). MCP-1 protein and mRNA expression levels were detected by immunohistochemistry, Western blotting, and semi-quantitative reverse transcriptase-polymerase chain reaction (RT-PCR). RESULTS: Serum amylase levels in groups P and T were higher than those in group S. Serum amylase levels were significantly lower in group T than in group P at 6 and 12 hours after operation. The levels of MCP-1 in serum at 6 and 12 hours after operation in group P were significantly higher than in group S, and significantly lower in group T than in group P at 6 and 12 hours after operation. The pathological damage in the pancreas was milder in group T than in group P. MCP-1 protein and mRNA expression levels in the pancreas were higher in groups P and T than in group S. These expression levels were positively correlated with the pathological damage of pancreatic tissues. The activity of MCP-1 in group T was significantly lower than in group P. CONCLUSION: MCP-1 may play important roles in the pathogenesis of SAP. The data suggest that Bindarit ameliorates SAP by inhibiting the activity of MCP-1 in vivo.
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic liver disease, which usually affects young adults and is diagnosed by cholangiography. On a few occasions, the disease either starts in or exclusively involves the small intrahepatic bile ducts, referred to as small-duct PSC. METHODS: A 31-year-old man presented with severe hematemesis secondary to liver cirrhosis. Over a course of 8 years, his liver decompensated and required an orthotopic liver transplantation. In this report we discuss his disease presentation, course of management, and the post-transplantation course of management, and review the morphologic diagnosis, and differential diagnosis of the disease with large-duct type and other diseases that involve small intrahepatic bile ducts. RESULTS: The patient's explanted liver showed changes of PSC affecting only the small- and medium-sized bile ducts in addition to three incidental nodules of hepatocellular carcinoma. CONCLUSIONS: Small-duct PSC has a substantially better prognosis than the large-duct type, with less chance of developing cirrhosis and an equal risk for developing hepatocellular carcinoma, but no increased risk for developing cholangiocarcinoma. Treatment seems to help relieve the symptoms but not necessarily improve survival. Liver transplantation remains the ultimate cure.
BACKGROUND: Douglas described choledochal cysts in 1852 and Todani proposed an anatomy-based classification in 1977. The classification is the most extensively used, but does not include some rare variations. We present a case of hepatic duct diverticulum, one of the variations, and discuss its diagnosis and treatment. METHODS: A 57-year-old woman presented with upper abdominal pain and discomfort associated with nausea and vomiting. She was finally diagnosed with cholelithiasis and right hepatic duct diverticulum. RESULT: The patient underwent resection of the hepatic duct diverticulum and cholecystectomy, and was asymptomatic 26 months after surgery. CONCLUSIONS: Hepatic duct diverticulum is a rare form of choledochal cyst, not included in Todani s classification. Todani s classification including this and other uncommon variations of choledochal cysts must be reviewed. The best diagnostic imaging methods and treatment for choledochal cysts must be defined.
BACKGROUND: Xanthogranulomatosis is an idiopathic, rare process in which lipid-laden histiocytes are deposited at various locations in the body. We present two cases who were treated by duodenum-preserving pancreatic head resection and eventually diagnosed as having xanthogranulomatous pancreatitis. METHODS: A 30-year-old caucasian man was admitted to our clinic for vague abdominal pain and epigastric dullness. Magnetic resonance cholangiopancreatography and endoscopic retrograde pancreatography suggested the existence of chronic pancreatitis. Another 34-year-old caucasian woman was admitted to our clinic because of right upper quadrant pain. Magnetic resonance cholangiopancreatography demonstrated a dilatation and stone of the main pancreatic duct. Based on a diagnosis of chronic pancreatitis, pancreatic head resection was planned and a laparotomy was performed in both of cases. RESULTS: In both cases, duodenum-preserving pancreatic head resection was performed. Macroscopic and microscopic findings revealed xanthogranulomatous inflammation, which led to a diagnosis of xanthogranulomatous pancreatitis. CONCLUSION: Although this type of pancreatitis is extremely rare, it is important to keep it in mind for a differential diagnosis because it may simulate chronic pancreatitis or a malignant tumor on imaging.
BACKGROUND: Inflammatory pseudotumor of the biliary tract is a benign disease, and is extremely rare. Its diagnosis often depends on pathological examination after operation. The histopathological examination shows inflammatory lesions with a polymorphous infiltration and variable amounts of fibrous tissue. This study was undertaken to elucidate that an inflammatory pseudotumor in the right hepatic duct is especially difficult to distinguish from hilar cholangiocarcinoma. METHOD: The clinical data of one patient with inflammatory pseudotumor of the right hepatic duct were analyzed. RESULTS: An occupying lesion of the right hepatic duct was revealed by abdominal ultrasound and magnetic resonance cholangiopancreatography. The right hepatic duct inflammatory pseudotumor was not identified during the operation but was confirmed by postoperative histopathological analysis. The patient recovered well without any serious complication. CONCLUSIONS: The preoperative evaluation for optimizing surgical management is important to the diagnosis of hepatobiliary occupying lesions. The evaluation involves clinical manifestations, imaging appearance and tumor markers. Malignant tumors and possible benign lesions should be considered to avoid aggressive surgical treatment.
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