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)

Adult polycystic liver disease is an entity that is striking for the presence of extraordinary hepatomegaly. Very rarely do patients under 40 years of age experience problems and only a minority of middle-aged patients manifest symptoms due to organ compression or biliary tree obstruction. The clinical course is usually benign, and no specific therapy is required. The onset of right upper quadrant pain, back pain, and shortness of breath prompted evaluation of a 32-year-old woman with progressive hepatomegaly. CT scan revealed a massive cyst-riddled liver with posterior displacement of the spleen, compression of the pancreas, and renal cysts. Prothrombin time, albumin, and liver enzyme values were normal except for a mildly elevated gamma-glutamyl transpeptidase.
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PMID:Symptomatic adult polycystic liver disease in a young woman. 175 23

The preceding discussions outline the various forms of cirrhosis that may be encountered in the elderly population. Cirrhosis is not uncommon in older patients. Although it has been stated that most cirrhosis in the elderly is due to alcohol, these assumptions are perhaps overestimations. In the authors' experience, many older patients are inappropriately labeled with alcoholic liver disease--presumed guilty until proven otherwise--and have subsequently been shown to have nonalcoholic liver disease. Careful investigation is required. Hepatotoxic drug exposure (e.g., to alpha methyldopa, nitrofurantoin, or isoniazid) should be ruled out, and hepatitis B and hepatitis C serology obtained. Primary biliary cirrhosis may occur in both sexes, and thus antimitochondrial antibody should be assayed. Severe heart disease may result in cardiac cirrhosis in the elderly, with ascites and hepatomegaly. Alpha 1-antitrypsin deficiency, primary sclerosing cholangitis, idiopathic hemochromatosis, and chronic autoimmune hepatitis may result in advanced cirrhosis in the elderly; appropriate serum studies should be obtained. If questions remain and if therapy may be changed, liver biopsy can be performed. A recent study suggested, however, that the risk of hemorrhage from liver biopsy in the elderly may be increased, especially if malignancy is present. The era of treatment for liver diseases has arrived. Colchicine, methotrexate, ursodeoxycholic acid, and others have shown promise in the treatment of PBC, primary sclerosing cholangitis, and alcoholic liver disease. Corticosteroids may be lifesaving in autoimmune liver disease. Phlebotomy remains the treatment of choice for hemochromatosis in any age group. Interferons and other antiviral agents are being used in chronic type B and type C hepatitis. Treatment of the complications of cirrhosis in the elderly may be safely accomplished. Advanced age is not a contraindication to variceal sclerotherapy. Vasopressin, however, may be contraindicated in the elderly patient if there is an underlying history of atherosclerotic coronary or peripheral vascular disease. Large-volume paracentesis and peritoneal venous shunting can afford symptomatic relief of ascites, even in the geriatric population. Finally, as noted previously, advanced age is no longer to be considered an absolute contraindication for liver transplantation. The evaluation of liver disease in the elderly may be diagnostically challenging, and its treatment rewarding.
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PMID:Liver diseases in the elderly. 185 64

Alpers disease consists of diffuse cerebral degeneration manifested as developmental delay, seizures, vomiting, and progressive neuromuscular deterioration, with liver disease and death. We report the clinical course of the liver disease, histologic progression of the hepatic lesions, and etiologic investigations in five patients (four girls, three kinships). All had grown and developed normally until seen at 6 to 36 months of age (mean 20 months), with vomiting (n = 5), progressive hypotonia (n = 3), or seizures (n = 2). All had been given anticonvulsants, including valproic acid in three. Liver disease was noted at a mean age of 35 months (range 9 to 67 months), with hepatomegaly (two patients), abnormal hepatic synthetic function (three) or transaminase values (three), and cirrhosis in one. Patients survived for a mean of 4.6 weeks (range 1 to 8 weeks) after the identification of liver disease; all died of hepatic failure. Results of evaluation for infectious and metabolic causes of liver disease and causes of degenerative neuromuscular disease were negative in all patients. Premortem liver biopsy specimens (n = 3) demonstrated an early lesion consisting of lobular disarray, microvesicular steatosis, periportal acute and chronic inflammation, and individual hepatocyte necrosis. Autopsy findings (n = 5) consisted of macrovesicular steatosis, massive hepatocyte dropout, and proliferation of bile ductular elements, with almost complete replacement of hepatocytes by proliferating bile ductular elements in two patients. Brain showed characteristic neuronal degeneration. We conclude that Alpers disease can be a cause of rapidly progressive liver failure in early childhood. Although the cause of this autosomal recessive disease is not known, it does not appear to be related to peroxisomal dysfunction.
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PMID:Liver involvement in Alpers disease. 186 Dec 11

Between January 1981 and December 1990, 41 patients were operated on for hepatic hydatid disease, representing 18% of the overall surgery for liver disease performed in our institution during the same period of time. The mean age of the patients was 39.5 years (range 17-71 years), 24 were females and 17 males. The most common clinical sign was abdominal pain (59%) with 3 patients jaundiced at the time of admission. 2 patients suffered from acute rightsided pneumonia, one of them with a very important productive cough due to bronchial perforation of the hydatid cyst of the liver. One patient was admitted due to anaphylactic shock with collapse. For 14 patients (34%) the discovery of the disease was casual, either by clinical hepatomegaly or by X-ray investigations performed for other reasons. We performed 18 pericystectomies, 4 subtotal pericystectomies and 16 liver resections. Only 3 patients were treated by partial cystectomy with removal of the prominent part of the cyst. Several other surgical procedures were performed concomitantly, including 2 pulmonary lobectomies, 1 cystojejunostomy, and pericystectomies for other hepatic or extrahepatic localizations of hydatid cysts. Rupture of the cyst into the bile duct was diagnosed in 13 patients. One patient presented with a peritoneal rupture of the cyst and 2 patients had a fistula through the diaphragm into the pleural cavity. Our postoperative morbidity is acceptable (10 patients, 25%). A bilioma and a biliary leak were successfully treated by percutaneous drainage, a postoperative hemorrhage required surgical treatment, four patients experienced pulmonary complications and the three others minor wound abscesses.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Surgical treatment of hepatic hydatidosis]. 192 52

The purpose of our study was to assess the pattern of liver diseases diagnosed by ultrasonography (US) in Yaounde (Cameroon). From a total of 1,007 consecutive US studies performed over a period of 14 months in subjects over the age of 15 years, we found 322 patients with a sonographic diagnosis of liver disease. 8 diagnoses comprised 83% of the patients. Apart from non specific homogeneous hepatomegaly these diagnoses consisted of: cirrhosis, primary liver malignancies, liver abscess, congestive hepatomegaly, secondary liver malignancies, diffuse steatosis and nodular calcifications. 30 liver abscess drainages were performed with sonographic guidance. We conclude that in our environment where Computed Tomography is scarce and expensive, US is a useful tool in the study of some of the most frequent liver diseases.
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PMID:[Pattern of hepatic pathology diagnosed with echography in Yaounde (Cameroon)]. 192 48

To assess the prevalence, demography, and clinical features of liver disease among patients with cystic fibrosis the case notes of 524 patients of all ages who were attending the cystic fibrosis clinic were studied. Computer databases were used to establish the condition of the liver in a further 576 such patients. The overall prevalence of overt liver disease indicated by the presence of an enlarged liver or spleen (or both) was 4.2%. The age related prevalence rose to a peak in adolescence, and then fell in patients over 20 years old. The implied increase in mortality among those with liver disease was not explained by deaths from liver disease, which were rare. Male patients were significantly more affected than female, the ratio being 3:1 among adolescents. Increasing prevalence of liver disease in patients with cystic fibrosis is, therefore, not just a result of longevity.
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PMID:Prevalence of liver disease in cystic fibrosis. 205 91

A group of 87 adults from a small village in north-east Thailand was chosen to undergo ultrasound investigation based on their intensity of infection with the liver fluke, Opisthorchis viverrini, or clinical status (history of jaundice, current hepatomegaly). From this group, 8 cases of suspected early cholangiocarcinoma were found, and the diagnostic features of 6 of the 8 were confirmed by computerized tomography scan and endoscopic retrograde cholangiopancreatography. In addition, several cases of mild gall-bladder disease, chronic cholecystitis, cholelithiasis and parenchymal liver disease were detected. A highly significant positive relationship between the intensity of liver fluke worm burden and the severity of biliary tract disease within individuals is reported. These results indicate that Opisthorchis is associated with moderate to severe hepatobiliary disease in a considerable proportion of infected individuals.
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PMID:A high frequency of hepatobiliary disease and suspected cholangiocarcinoma associated with heavy Opisthorchis viverrini infection in a small community in north-east Thailand. 217 78

Histiocytosis X rarely disseminates in an adult. The authors describe an unusual patients who presented with multiple areas of cutaneous and bone involvement. During the course of his disease he developed massive hepatomegaly. Aggregates of vacuolated histiocytes were found on liver biopsy. He subsequently developed diabetes mellitus complicated by ketoacidosis. Both his hepatomegaly and diabetes resolved spontaneously. No pancreatic nor pituitary abnormalities were identified. The combination of histiocytosis X, hepatomegaly, and diabetes mellitus has not been previously reported. The medical literature is reviewed with an emphasis on disseminated histiocytosis X in adults and the mechanism of glucose intolerance in liver disease.
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PMID:Reversible hepatomegaly and diabetes mellitus in an adult with disseminated histiocytosis X. 218 Feb 96

Retrospective review of 291 solid tumor and lymphoma patients undergoing autologous bone marrow transplantation (BMT) was performed to determine the influence of pretransplant characteristics and preparative regimen to the development of hepatic venoocclusive disease (VOD). Twelve patients (4.1%) developed a clinical syndrome of right upper quadrant (RUQ) tenderness or hepatomegaly, jaundice, and ascites, with or without encephalopathy, within 40 days of marrow reinfusion. Evidence of metastatic liver disease was the only pretransplant characteristic predictive for VOD (P = .0002). Sex, age, histology, hepatitis B serology, and elevated liver function tests were not predictive. No individual preparative agent had a significant effect on the development of VOD. However, a single 2-hour infusion of carmustine (BCNU) (greater than or equal to 450 mg/m2) led to an increased incidence of VOD when compared with the same dose administered in a fractionated schedule (P = .0258) when given with two other chemotherapeutic agents. Seven of eight autopsy specimens confirmed the clinical diagnosis of VOD. The four patients in whom clinical VOD resolved had lower median peak bilirubins (7.3 v 15.9 mg/dL), lower median peak creatinines (2.1 v 4.1 mg/dL), and relatively quick engraftment of neutrophils (mean, 18.7 days). One of the four patients in whom VOD resolved had other grade 4 (life-threatening) toxicities in contrast to eight of eight who succumbed. In summary, VOD is an uncommon complication in autotransplantation of solid tumors and lymphomas. Our data suggest caution in selecting patients with known metastatic liver disease and consideration of a fractionated BCNU schedule especially in combination with other alkylating agents.
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PMID:Hepatic venoocclusive disease in autologous bone marrow transplantation of solid tumors and lymphomas. 221 5

This analysis of the morphology of suspected amiodarone (AD) liver disease is based on a study of liver specimens from 17 individuals. Changes similar to alcoholic liver injury were commonly seen. Steatosis, both macrovesicular and microvesicular, was the most frequent histopathologic feature. Ballooning of hepatocytes, Mallory bodies, and fibrosis were also common. Other changes included nuclear unrest, acidophilic bodies, foam cells, glycogenated nuclei, and portal inflammation. Characteristic lamellar lysosomal inclusion bodies representing phospholipidosis were found in two of 14 specimens studied ultrastructurally. These changes of pseudoalcoholic hepatitis and/or phospholipidosis were present in liver specimens from asymptomatic, anicteric patients with mild elevations in serum aminotransferase or alkaline phosphatase values with or without hepatomegaly, as well as in patients with clinically overt symptoms of hepatotoxicity. Phospholipidosis appears to be a generalized systemic effect of cationic amphophilic compounds, such as AD. The cytotoxic pseudoalcoholic changes appear to be an independent phenomenon in susceptible patients, whom we speculate may have been unable or less able to metabolize AD through normal pathways. The true incidence of hepatic injury from AD remains to be determined from prospective evaluations of pretreatment and follow-up liver biopsies.
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PMID:Histopathologic analysis of suspected amiodarone hepatotoxicity. 240 75


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