Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Carolis disease is a rare communicating segmental or diffuse dilatation of the intrahepatic biliary tree. Cholangitis, liver cirrhosis and cholangiocarcinoma are its potential complications. A case of Carolis disease in a boy of 6 years with bilobal involvement presenting with intermittent abdominal pain, fever and hepatomegaly is reported here.
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PMID:Caroli's disease. 1534 76

A 7-year-old neutered male Somali cat, bred in Western Australia, was presented for investigation of jaundice and severe anaemia. Splenomegaly and hepatomegaly were evident on physical examination. Severe anaemia, along with leukopenia and increased liver enzymes, were present on laboratory evaluation. Clinical investigation identified cholangitis and treatment for this resolved the jaundice but failed to resolve the anaemia. Treatment for Mycoplamsa haemofelis was administered concurrently. Genetic testing was then performed and pyruvate kinase deficiency was identified, the first time this has been reported in an Australian cat. Treatment with immunosuppressive medication was not successful.
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PMID:Pyruvate kinase deficiency in a Somali cat in Australia. 1611 20

Opisthorchiasis caused by Opisthorchis viverrini remains a major public health problem in many parts of Southeast Asia, including Thailand, Lao PDR, Vietnam and Cambodia. The infection is associated with a number of hepatobiliary diseases, including cholangitis, obstructive jaundice, hepatomegaly, cholecystitis and cholelithiasis. Multi-factorial etiology of cholangiocarcinoma, mechanical damage, parasite secretions, and immunopathology may enhance cholangiocarcinogenesis. Moreover, both experimental and epidemiological evidences strongly implicate liver fluke infection as the major risk factor in cholangiocarcinoma, cancer of the bile ducts. The liver fluke infection is induced by eating raw or uncooked fish products that is the tradition and popular in the northeastern and northern region, particularly in rural areas, of Thailand. The health education programs to prevent and control opisthorchiasis are still required in the high-risk areas.
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PMID:Opisthorchis viverrini: the carcinogenic human liver fluke. 1820 54

Opisthorchiasis caused by Opisthorchis viverrini (O. viverrini) remains a major public health problem in many parts of Southeast Asia including Thailand, Lao PDR, Vietnam and Cambodia. The infection is associated with a number of hepatobiliary diseases, including cholangitis, obstructive jaundice, hepatomegaly, cholecystitis, chole-lithiasis and cholangiocarcinoma. The liver fluke infection was induced by eating raw or uncooked fish products that is the tradition and popular in the northeastern and northern region, particularly in rural areas of Thailand. Health education programs to prevent and control opisthorchiasis are still required in high-risk areas.
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PMID:Opisthorchiasis in Thailand: review and current status. 1841 53

Alveolar echinococcosis, a parasitic disease due to the larval stage of the cestode Echinococcus multilocularis, is initially located in the liver in 97% of cases. Progression is very slow and the disease remains silent for many years. The developing larva behaves like a slow-growing liver tumor that gradually invades the liver parenchyma, vessels and bile ducts. Marked granulomatosis around the larva, and the subsequent strong reactive fibrosis, contribute to the severity of the disease. Gradual extension to adjacent organs and distant metastases due to haematogenous spread can also occur. Purely extrahepatic alveolar echinococcosis is rare, but physicians in endemic areas should be aware of this possibility. Diagnostic methods have dramatically improved over the past twenty years. The clinical presentation used to be similar to that of liver cancer, with slowly progressivejaundice (due to involvement of the hilum), huge, hard and irregular hepatomegaly, and a chronic Budd-Chiari syndrome due to hepatic vein involvement. Currently, with extensive use of abdominal ultrasonography, alveolar echinococcosis is commonly diagnosed when still asymptomatic. Alveolar echinococcosis may also be revealed by a complication, such as cholangitis due to communication between the parasite mass and the lumen of a bile duct or to pigment stones accumulating above a parasitic biliary stenosis; liver abscess related to centro-parasitic necrosis; or hematemesis due to esophagal varices in case of portal vein involvement. Metastases, especially in the lungs, reveal the disease in 5% of cases.
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PMID:[Alveolar echinococcosis: a disease comparable to a slow growing cancer]. 1923 77

Among adults, liver involvement is relatively frequent in Langerhans' cell histiocytosis (LCH), even though it is often overlooked. In fact, the liver involvement may be missed in apparently localized LCH or when it is the sole site of involvement. We present 23 cases of liver involvement in LCH out of a cohort study of 85 adult patients included in the French Histiocytosis Study Group Registry. The most frequent clinical setting was multiorgan involvement (87% of our cases). The main histological pattern in liver LCH was sclerosing cholangitis (56% of the cases). The symptoms included hepatomegaly (48%) and/or liver biochemistry abnormalities (61%, including cholestasis associated with increased transaminases levels in 35% of cases, cholestasis only in 22% and increased transaminases levels only in 4% of the cases). Particularly suggestive of the diagnosis was the observation of biliary tree abnormalities through magnetic resonance imaging (MRI). The natural history of liver LCH fits into two stages: early infiltration by histiocytes and late sclerosis of the biliary tree. We found that liver involvement had a significant impact on survival. Thus we suggest that clinical and biological liver evaluation must be performed regularly onwards to screen every LCH adult patient from the time of the initial diagnosis. MRI and liver biopsy should be considered as soon as the data point to a possible liver localization. If this diagnosis is confirmed, we suggest a treatment with ursodesoxycholic acid, as in other cholestatic diseases, together with treatments specifically directed towards LCH. However, the ideal treatment of liver LCH remains to be found, and in advanced cases transplantation is the sole option.
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PMID:Langerhans' cell histiocytosis of the liver in adults. 2155 Mar 30

The occurrence of platynosomiasis and intestinal sparganosis is described in feral cats from Grand Cayman, Cayman Islands. Spirometra spp. was observed within the intestine of 18.18% (10/55) of cats; 1.18% (1/55) of cats demonstrated gross and histological manifestation of parasitism by Platynosomum fastosum, but 14.5% (8/55) of cats had the characteristic pathological manifestations of P. fastosum-induced intrahepatic cholangitis without the concomitant presence of the intraductal trematode. Combined parasitism (Spirometra spp. and P. fastosum) was observed in 9.09% (5/55) of feral cats. Significant pathological findings were only associated with the hepatic fluke, P. fastosum, and were grossly characterized by moderate hepatomegaly with enlarged and dilated bile ducts. Examples of cestodes with morphological features characteristic of Spirometra spp. were observed within the small intestine without any associated pathological lesion. The histopathological evaluation of liver fragments revealed chronic intrahepatic cholangitis with and without the associated intraductal trematode, and was characterized by marked periductal fibrosis, adenomatous proliferation of bile duct epithelium, dilation of intrahepatic bile ducts and portal accumulations of inflammatory cells. The occurrence of the cestode in feral cats coupled with factors that are unique to Grand Cayman makes this island the ideal location for sporadic cases of human sparganosis.
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PMID:Platynosomum fastosum-induced chronic intrahepatic cholangitis and Spirometra spp. infections in feral cats from Grand Cayman. 2172 87

Since opisthorchiasis does not show pathognomonic signs or symptoms, physicians can have serious problems to make a differential diagnosis of this infection in non endemic areas, in particular when there is a simultaneous occurrence with other seasonal infections. Moreover, symptomatic infections due to O. felineus can last a few weeks and then the signs and symptoms disappear, but the worms survive in the bile ducts for years causing hepatobiliary diseases including hepatomegaly, cholangitis, fibrosis of the periportal system, cholecystitis, and gallstones. Consequently, an early diagnosis prevents chronicity and loss of working days. The detection of specific antibodies has been considered as a complementary tool to the fecal examination to establish the definitive diagnosis of this infection and for the follow up. Therefore the aim of this work was the development and validation of an enzyme-linked immunosorbent assay (ELISA) using excretory/secretory antigens (ESA) from O. felineus adult worms to detect anti-Opisthorchis IgG in human sera. A total of 370 human sera were tested: 144 sera from persons with a confirmed diagnosis of opisthorchiasis, 110 sera from healthy Italian people, and 116 sera from people with other parasitic or non-parasitic infections. Results were analyzed by receiver-operator characteristic (ROC) curve analysis. The accuracy of the test, calculated by the area under curve (AUC), yielded a 0.999 value, indicating the high performance of the test. The sensitivity was 100% (95% CI: 97.40% to 100%) and no false-negative sera were detected; the specificity was 99.09% (95% CI: 95.02% to 99.83%). The validated ELISA shows a good performance in terms of sensitivity, repeatability and reproducibility, and it is suitable to detect anti-Opisthorchis IgG in human sera for diagnostic purposes and for the follow up to assess the efficacy of drug treatment.
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PMID:Validation of an excretory/secretory antigen based-ELISA for the diagnosis of Opisthorchis felineus infection in humans from low trematode endemic areas. 2367 89

Inflammatory pseudotumor (IPT) of the liver is a rare, benign lesion that may be mistaken for malignancy. IPTs are difficult to diagnose due to non-specific clinical, laboratory and imaging features. We report the case of a 38-year old Asian male who presented with fatigue, weight loss and hepatomegaly. He was found to have a large hepatic IPT and underwent surgical resection; approximately two and a half years later, he developed acute cholangitis secondary to IPT recurrence. We present the imaging features of hepatic IPT using ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI). We also review the literature on the diagnosis and management of this disease. The unique features of this case include the IPT's recurrence following surgical resection, large size and multiple modalities presented.
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PMID:Inflammatory pseudotumor of the liver: a rare case of recurrence following surgical resection. 2496 25

Fascioliasis is a zoonotic infestation caused by Fasciola hepatica that usually attacks mammals, such as goats, sheep and cattle. The parasites can infect humans via freshwater plants contaminated with encysted metacercariae. In the acute phase, which involves hepatic invasion, the disease may present with abdominal pain, mild fever and hepatomegaly. In the chronic phase, the parasites settle into the biliary tracts, and then cause cholangitis and cholestasis. Sometimes, the disease may mimic malignancies, creating a mass appearance. Endoscopic retrograde cholangiopancreatography (ERCP) is an important diagnostic and treatment method because it allows simultaneous diagnosis and treatment. Here, we present a 44-year-old female patient who presented to our hospital with complaints of abdominal pain, nausea, vomiting, anorexia and weight loss. After diagnostic investigations with laboratory and imaging methods, she was initially hospitalized with a preliminary diagnosis of cholangiocellular carcinoma (CCC). However, after a full work-up, the patient was diagnosed with Fasciola hepatica via ERCP and parasites were extracted with ERCP at the same time and then treated with a single dose of triclabendazole 10 mg/kg. Two months later, the clinical status of the patient had improved markedly, with resolution of all symptoms and all laboratory and imaging tests returning to within normal limits.
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PMID:An incidental case of biliary fascioliasis mimicking cholangiocellular carcinoma. 2555 48


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