1/41. Hepatic inflammatory pseudotumor: case report, review of the literature, and a proposal for morphologic classification.Inflammatory pseudotumors (IPT) are uncommon mass lesions arising most typically in the lungs of young adults. These tumors are so named because of the difficulty in distinguishing them preoperatively from malignant lesions. IPT are characterized histologically by localized fibrous proliferations with infiltration by mononuclear leukocytes, particularly plasma cells. Seventeen previous cases of IPT involving the liver and biliary tree have been reported in children. In this location, IPT may lead to biliary obstruction, portal hypertension, cirrhosis and eventually hepatic failure. We describe the youngest patient ever reported with hepatic IPT (HIPT) and biliary obstruction, who was successfully managed with a left hepatic lobectomy and Kasai portoenterostomy. Based on all previous cases of HIPT in both adults and children (74 cases), we propose a morphologic classification of these lesions based on the presence of single versus multiple lesions, with individualized management. Type 1 lesions are large, solitary lesions, often with central necrosis, giving a characteristic radiographic appearance. Type 2 lesions are multiple smaller, solid nodules indistinguishable from metastatic malignancy.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
2/41. light chain deposition disease of the liver without renal involvement in a patient with multiple myeloma related to liver failure and rapid fatal outcome.We describe a 36-year-old man with advanced multiple myeloma (salmon and Durie stage III) who developed jaundice and severe cholestasis after a first cure with systemic chemotherapy of vincristine, doxorubicin, and oral dexamethasone (VAD). serology for hepatitis a, B, and C and for CMV was negative. A liver ultrasound and CT scan showed mild hepatomegaly without evidence of extrahepatic or intrahepatic biliary tree dilatation. A percutaneous liver biopsy revealed perisinusoidal deposits of an abundant slightly eosinophilic, PAS-positive amorphous substance. immunohistochemistry showed positivity for kappa-light chains and was negative for lambda-light chains, for IgA, IgG, IgM, and IgD immunoglobulins as well as for AA and AL proteins and for amyloid P component. A diagnosis of light chain deposition disease (LCDD) of the liver was made. The patient developed rapid deterioration of liver function, leading to a multisystem dysfunction and death. The occurrence of LCDD in multiple myeloma is close to 5% and myeloma is the underlying disease in two thirds of patients with LCDD. The kidneys are involved in almost all cases of LCDD and renal dysfunction usually reveals the disease. Only three patients with LCDD of the liver without overt renal involvement have been reported so far. This is the first observation of LCDD presenting with jaundice and severe cholestasis shortly after the diagnosis of high tumor mass myeloma, without overt renal involvement, leading rapidly to the patient's death.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
3/41. Squamous cell carcinoma arising in a hepatic forgut cyst.We report the second case of squamous cell carcinoma arising in a hepatic foregut cyst (CHFC) in a 40-year-old woman. Microscopically, the lining of the cyst was composed of ciliated columnar epithelium, gastric and squamous epithelium. The squamous epithelium showed areas with dysplastic changes and other areas with carcinomatous transformation. In this congenital lesion, it was not surprising to find squamous and gastric mucosa because oesophagus, stomach, and tracheobronchic tree derive from the embryologic foregut. Squamous carcinoma might develop in a context of inflammation as in biliary cyst. In agreement with the first case described in the literature, this report also suggests that a large-sized symptomatic hepatic cyst should be excised.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
4/41. The pathology of acute hepatic disintegration in hereditary haemorrhagic telangiectasia.AIMS: Hereditary haemorrhagic telangiectasia is a rare inherited disease in which telangiectases affect skin, mucous membranes and the gastrointestinal tract. Hepatic involvement is common but usually asymptomatic. We report a case of acute hepatic disintegration in hereditary haemorrhagic telangiectasia, document the histopathological findings and present a hypothesis to explain them. methods AND RESULTS: The patient presented at the age of 34 years with abdominal pain, leading to the surgical removal of a severely inflamed gallbladder. Signs of liver damage became increasingly apparent over the next few weeks, with disruption of the intrahepatic biliary tree and marked vascular shunting, necessitating liver transplantation. Six months after the transplant a diagnosis of hepatic hereditary haemorrhagic telangiectasia was made. The principal features of hepatic hereditary haemorrhagic telangiectasia are periportal telangiectases and sinusoidal congestion and dilatation. Acute hepatic disintegration is characterized by disruption of liver structure, hepatocyte necrosis, haemorrhage and extravasation of bile. CONCLUSIONS: Periportal telangiectases in a liver biopsy are highly suggestive of hereditary haemorrhagic telangiectasia. Acute hepatic disintegration is likely to be a consequence of rupture of telangiectases and ischaemic necrosis of intrahepatic bile ducts. patients with hereditary haemorrhagic telangiectasia are at risk of acute hepatic disintegration following intra-abdominal sepsis.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
5/41. Decision analysis in clinical gastroenterology.OBJECTIVES: Although the tools of medical decision analysis have been mostly used to address complex issues of healthcare and management policies, an important role remains for decision analysis to play in solving mundane medical problems of daily practice. The present article aims to restate the origins of decision analysis and to demonstrate the utility of decision models in clinical gastroenterology. methods: Three clinical scenarios are presented to illustrate the applicability of decision analysis in clinical gastroenterology. The examples are modeled as simple decision trees and phrased in terms of threshold analysis. RESULTS: In spite of its striking simplicity, the framework of threshold analysis is able to capture a large variety of heterogeneous and often perplexing medical problems. Rather than calculate a cost-effectiveness or cost-benefit ratio, the result of the analysis is expressed as a probability value, which forms the threshold between the two choices against or in favor of a particular medical action. For most analyses little if any calculation is needed to solve the decision tree, and the final outcome of the analysis can be derived based on comparative estimates and considerations of magnitude only. CONCLUSIONS: The study demonstrates that threshold analysis can serve as a simple and convenient instrument to solve a large variety of problems in clinical gastroenterology.- - - - - - - - - - ranking = 2keywords = tree (Clic here for more details about this article) |
6/41. Hepatic subcapsular biloma: a rare complication of laparoscopic cholecystectomy and common bile duct exploration.PURPOSE: Description of an unusual complication of laparoscopic cholecystectomy and common bile duct exploration. methods: Case report from experience at an air Force tertiary care hospital in a 70-year-old woman undergoing laparoscopic cholecystectomy. literature search for journal articles reporting similar complications using medline, pubmed, and Ovid internet search systems. RESULTS: A hepatic subcapsular biloma was diagnosed on postoperative day 11 from an elective laparoscopic cholecystectomy and common bile duct exploration. review of the literature found only 2 other reports of this complication. CONCLUSIONS: Hepatic subcapsular biloma is a rare complication of laparoscopic cholecystectomy. Computed tomography is highly sensitive for diagnosing bilomas, and percutaneous drainage procedures are appropriate treatment. Imaging of the biliary tree should be performed early to determine the location and extent of bile leaks.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
7/41. Clinical vignette in antiretroviral therapy: jaundice.hiv caregivers face many challenges following initiation of art. The development of jaundice is uncommon but worrisome. In this case, two distinct and contrasting episodes of jaundice were observed. In the first instance, isolated elevation of the indirect bilirubin without elevation of the alkaline phosphatase was noted. The normal PT and serum aminotransferase levels indicate the absence of intrinsic liver dysfunction. Elevations in the indirect bilirubin may result from either impaired uptake/conjugation or excess production. The latter, usually from acquired hemolysis, may be a complication of an occult NHL. A work-up for this AIDS-related malignancy was not initiated since the caregivers recognized jaundice as a complication of IDV, which inhibits UDP-glucuronyl transferase and produces a Gilbert's-like syndrome. physicians can expect to encounter this syndrome even more frequently with ATV. Experienced patients given RTV-boosted ATV have experienced elevations of unconjugated hyper-bilirubinemia in up to 45 percent of cases in clinical trials. However, such elevations do not reflect liver dysfunction and symptomatic jaundice requiring dosage reduction that occurred infrequently (7 to 8 percent of study patients). counseling patients about this syndrome may promote adherence and prevent self-directed interruptions of ATV that compromise efficacy. The second case of jaundice provides a more formidable diagnostic challenge. The triad of LFT abnormalities (mild elevation of aminotransferases, normal PT, and marked cholestatic jaundice) implies an acute process that is mildly toxic to hepatocytes without affecting their synthetic function. The subacute nature of the patient's cholestatic jaundice suggests either intrahepatic infiltrative disease of the liver or extrahepatic obstruction of the biliary tree, most likely due to the patient's relatively modest level of pain and lack of fever. Despite LFT abnormalities occurring 17 months after a switch in his art, cumulative drug-related toxicities must still be considered. ritonavir can produce significant elevations in the AST/ALT, especially with pre-existing chronic liver disease as with hepatitis c virus coinfection. The NRTIs can produce hepatic steatosis, a result of mitochondrial toxicity and impaired fatty acid oxidation. However, jaundice and cholestasis are not typical of the latter syndrome. With a negative contrast CT that excludes parenchymal liver disease, investigation of the biliary tree to assess the presence of AIDS-related cholangitis was the next step. Performing a sphincterotomy or stent placement, and obtaining brushings or biopsy specimens to determine the extent of extrahepatic obstruction may help define a pathogen and be life-saving. The negative results of the ERCP justify the final diagnostic step, a liver biopsy to evaluate microscopic infiltrative disease that might not have been detected on contrast abdominal CT. Examples might include granulomatous disease (MAC), fungal etiologies (histoplasmosis), carcinomatosis (lymphoma, hepatoma, cholangiocarcinoma), and microvascular disease (bacillary angiomatosis). The failure to observe granulomatous inflammation in the liver does not exclude MAC infection, as MAC may involve other peri-aortic or mesenteric lymph nodes. This form of iris is unlikely given the abdominal CT findings, lack of systemic complaints, and extended persistence of liver aminotransferases. The nonspecific results of the liver biopsy are a common outcome in advanced AIDS patients with elevated alkaline phosphatase levels. Despite not having identified a pathogen, the biopsy establishes chronic liver disease and prompts re-evaluation and change of treatment to NFV. The subsequent normalization of the patient's aminotransferase levels suggests a prior adverse effect of LPV/r in the setting of unexplained, chronic liver disease. Most importantly, this case highlights the importance of hiv caregivers to review art for safety when noting chronic liver dysfunction. patients need to be counseled to minimize acetaminophen use, to consume alcohol in moderation, and to avoid behavior with risk for hepatitis c. Finally, all hiv patients should receive appropriate vaccination against hepatitis a and B if serology shows lack of protective immunity.- - - - - - - - - - ranking = 2keywords = tree (Clic here for more details about this article) |
8/41. Liver involvement in the histiocytic disorders of childhood.The liver can be involved directly, by infiltration, and indirectly--by remote effects--in the histiocytoses of childhood. Langerhans cell disease, the most well recognized of these, infiltrates the liver directly but has a remarkable selectivity for the bile ducts. Early involvement is by Langerhans cell histiocytosis (LCH) infiltration leading to a sclerosing cholangitis and, eventually, biliary cirrhosis. Gamma glutamyl transpeptidase is a sensitive indicator of liver infiltration in a child with LCH. The indirect effects on the liver of LCH elsewhere in the body are mediated through an accompanying macrophage activation syndrome that is most likely responsible for hepatomegaly and hypoalbuminemia but without direct infiltration. These indirect effects are completely reversible. Juvenile xanthogranuloma/xanthoma disseminatum, a related dendritic cell disorder that can have systemic manifestations, has a strikingly different pattern, with a predominantly portal infiltrate spilling over into the adjacent lobule but sparing the biliary tree. The biology of the liver lesions is not clear but regression has been documented. myeloproliferative disorders and myeloid leukemias can express CD1a and/or S100 protein, mimicking LCH but distinguished by their sinusoidal pattern. The primary macrophage histiocytoses such as the familial hemophagocytic syndromes can lead to severe liver damage. Although a portal lymphohistiocytic infiltrate is most characteristic, it is probably cytokine-mediated hepatocellular damage that can cause substantial functional impairment or even hepatic failure as a presenting feature. Liver involvement in other, more unusual histiocytic disorders, is also illustrated.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
9/41. Disappearing cyst of the hepatic hilum in uncorrectable biliary atresia.A 2-month-old boy with biliary atresia (BA) with an extremely rare prenatal history is reported. A cystic lesion in the hepatic hilum was diagnosed during prenatal ultrasonography at 20 weeks' gestation. However, it disappeared during later prenatal observation. He was diagnosed with an uncorrectable type of BA after a massive subdural hemorrhage at the age of 66 days. A fetus suspected of having a cyst of the biliary tree should be followed even after the cyst has disappeared spontaneously.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
10/41. Inflammatory pseudotumor of the liver and peripheral eosinophilia in autoimmune pancreatitis.AIM: Inflammatory pseudotumor (IPT) of the liver is a rare benign lesion, the etiology of which remains obscure. It is not associated with any particular diseases apart from phlebitis and Crohn's disease. methods: A middle-aged male with hepatic IPT and peripheral eosinophilia associated with autoimmune pancreatitis (AIP) was selected for this study and review of literature. RESULTS: A 59-year-old male was admitted with obstructive jaundice, marked eosinophilia (1 343/mm(3)) and hypergammaglobulinemia (4 145 mg/dL). Imaging techniques revealed dilatation of the intrahepatic bile duct, stenosis of the common bile duct with diffuse wall thickening, gallbladder wall thickening, irregular narrowing of the pancreatic duct, and swelling of the pancreatic parenchyma. Multiple liver masses were also demonstrated and diagnosed as IPT by biopsy specimens. Six months later, the abnormal features of the biliary tree remarkably improved by the oral administration of prednisolone, and the liver masses disappeared. The swelling of the pancreatic head also improved. The peripheral eosinophil count normalized. IPT associated with AIP, as we know, has not been reported in the literature. The clinical features of the present case mimicked those of pancreatic cancer with liver metastasis. This case deserves to be documented to prevent misdiagnosis of similar cases.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
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