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)

Two children are reported with hemangioendothelioma of the hepatobiliary system. One patient, a 7-month-old girl, presented with classic symptomatology of hepatomegaly and congestive heart failure, but did not have any cutaneous lesions. She was treated with systemic steroids and supportive therapy and did very well. The other patient, a 22-month-old boy, is the first patient reported with exclusive involvement of the extrahepatic biliary tree. His symptoms included hepatomegaly and obstructive jaundice. He was evaluated with an endoscopic retrograde cholangiopancreatogram and a transhepatic cholangiogram. He did very well after resection of the lesion and Roux-en-Y drainage of the proximal biliary tree and remains asymptomatic 2 years after surgery.
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PMID:Hemangioendothelioma of the hepatobiliary system: the classic and the unusual. 218 12

13 patients with extrahepatic bile duct carcinoma treated in our institute from 1960 to 1986 are reported. All were proven by pathology. There were 10 moderately differentiated or mucin adenocarcinomas, 2 poorly differentiated and 1 undifferentiated cancers. There were 9 males and 4 females with an average age of 60.6 years. Progressive obstructive jaundice was the most common presenting symptom (11/13). Hepatomegaly was found in 7 patients, distended gallbladder in 4 and gallstone in 2. Before operation, 10 patients were misdiagnosed as hepatitis, cholecystitis or cholelithiasis. During operation, regional lymph node metastasis was observed in the majority of patients. Palliative operation was performed in 10 patients and radical surgery in 3. Three received operation plus postoperative radiotherapy. None survived more than two years. The lesions occurred frequently in the upper bile duct (8 patients). The middle bile duct and diffuse type carcinomas comprised 2 each. One was not recorded clearly. The prognosis is related to the gross type of the tumor and differentiation degree. Finally, carcinogenesis is discussed briefly.
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PMID:[Carcinoma of the extrahepatic bile duct--report of 13 patients]. 285 Jan 47

Oral administration of lantana leaves (6 g/kg body weight) and isolated toxins (125 mg/kg body weight) to rabbits caused ictericity, anorexia and decrease in fecal output. There was increased size of the kidneys, and the livers were ochre-colored and fragile but there was no hepatomegaly. Histopathologically, lantana-intoxicated rabbits had swellings of hepatic cells, portal fibrosis, dilatation of bile canaliculi and biliary hyperplasia. Kidneys had proliferation of mesenchymal cells in glomerular tufts, degeneration of tubules, swelling of tubular epithelial cells and pyknosis of nuclei. The intoxicated animals had elevated levels of conjugated and unconjugated bilirubin in plasma, the major increase being in the conjugated form (suggestive of obstructive jaundice). There were marginal changes in the activities of acid phosphatase and glutamate oxaloacetate transaminase in the plasma.
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PMID:Toxicity of lantana (Lantana camera L) leaves and isolated toxins to rabbits. 338 48

Every physician managing patients with inflammatory bowel disease should be alert to the possibility of the development of hepatobiliary disorders, especially in patients with extensive colonic involvement. There is the question concerning type of follow-up study to be instituted in patients with IBD. Elevation of the level of serum alkaline phosphatase appears to be the most useful and consistent biochemical indicator of hepatic dysfunction (101). This should be estimated at six monthly intervals. A persistent elevation of the level of serum alkaline phosphatase or more overt clinical manifestations, such as pain in the right upper quadrant, hepatomegaly, obstructive jaundice or weight loss, would all indicate the need for further investigations. This would normally take the form of roentgenologic investigation of the biliary tree and biopsy of the liver. Once a patient with IBD has been diagnosed as having one or more hepatobiliary disorders, what is the appropriate management? Each instance should be treated individually according to the nature of the disorder. In general, most of these conditions are histologic abnormalities and are of little clinical importance. There is the question of whether or not there is a role for prophylactic colectomy. There has been conflicting evidence to both support and refute the rationale that colectomy will prevent the development of, or arrest, existing disease of the liver. In the view of the authors, based upon a large experience with the management of these patients, the indication for colectomy should be based upon the severity and extent of colonic disease and almost never upon the existence of associated hepatobiliary disorders.
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PMID:Hepatobiliary disorders in inflammatory bowel disease. 388 36

A 4-year-old cat was examined because of anorexia and lethargy. The cat became icteric within 3 days of admission. Values for aspartate transaminase, alanine transaminase, total bilirubin, alkaline phosphatase, and cholesterol were higher than normal. Radiography revealed hepatomegaly, with loss of detail in the cranioventral portion of the abdomen. Further diagnostic procedures were not permitted, and the cat was euthanatized. At necropsy, cholecystitis, cholangitis, and numerous choleliths were found. Cholelithiasis is a rare cause of obstructive jaundice in the cat.
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PMID:Cholelithiasis in a cat. 397 77

Clinical features of severe opisthorchiasis were studied in 88 patients. The ratio of males to females was 6.3 : 1; 75% were over 40 years old. The presenting symptoms were obstructive jaundice, 25% associated with secondary infection of biliary system; cholangitis and cholecystitis; intraabdominal mass, which was enlarged liver; 18% had palpable gallbladder; 18% had adenocarcinoma of the bile duct. High bilirubin was found in 46% of cases, high alkaline phosphatase in 80%; elevation of serum transaminase in 78% and low serum albumin in 62% of patients. There was no correlation between severity of the disease and the faecal egg output. The obstructive jaundice patients had low or no egg output. Two patients had no eggs in stool, but numerous Opisthorchis viverrini eggs and flukes were found in the gallbladder and bile ducts at operation.
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PMID:Clinical features in severe opisthorchiasis viverrini. 409 5

Between 1978 and 1982 14 patients were treated for bile-duct cysts. The predominant clinical signs were obstructive jaundice and hepatomegaly in the younger patients, while pain was the most common symptom in the older patients. Ultrasonography was a valuable diagnostic technic but percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde cholangiopancreatography (ERCP) were associated with septic complications; as an alternative, routine preoperative cholangiography is recommended to visualize the type of bile-duct cyst. Excision of the bile-duct cyst was considered the treatment of choice. Excision was performed in 11, and internal drainage in three patients. Cholangitis developed in four children after excision of the cyst. In our series the prognosis was good following surgical treatment, even in younger patients.
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PMID:Bile-duct cysts--diagnosis and treatment in 14 patients. 663 26

A female newborn weighing 2650 g had hepatomegaly and deepening obstructive jaundice. She died of hepatic failure three weeks later. There were grayish white periportal histiocytic nodules of 1-2 mm in diameter. Histiocytes contained a small amount of neutral lipids and PAS-positive material. Single elements had multiple nuclei arranged in circles like in Touton cells. Bile duct and ductules were among them. The picture resembled the C variant of Niemann-Pick's disease but basic histochemical tests on fixed tissue excluded this possibility. The pathogenesis of unique disease remained obscure.
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PMID:[Congenital tumor-like histiocytosis of the liver with cholestasis in a newborn infant]. 687 48

We report the clinical and pathologic features of five children wih a botyroid rhabdomyosarcoma of the biliary tract. There were three boys and two girls ranging in age from 11/2 to 51/2 years at diagnosis. Obstructive jaundice was the usual presenting sign, often with fever and hepatomegaly. Of four tumors involving the common bile ducts, two extended into porta hepatis and adjacent liver parenchyma; the fifth tumor arose in the wall of the cystic duct. Each had a typical botryoid (grape-like) configuration with a cambium layer of tumor cells beneath biliary epithelium. Rhabdomyoblasts were identified in all cases and confirmed by ultrastructure in one. Our data, along with review of other cases reported in the English literature, indicate a rather guarded prognosis. Although metastases have developed in 40% of cases, death was usually due to the effects of local invasion by tumor. Aggressive adjuvant therapy currently in use for childhood rhabdomyosarcomas may lessen the high mortality associated with this tumor.
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PMID:Botryoid rhabdomyosarcoma of the biliary tract. 733 57

We report the use of a nasobiliary catheter in the management of a 55-yr-old female with autosomal dominant polycystic kidney disease who developed obstructive jaundice from a hepatic cyst. The patient presented with a 2-wk history of fatigue, jaundice, nausea, vomiting, and abdominal pain. Physical examination was remarkable for tender hepatomegaly. Computerized tomography revealed multiple hepatic cysts and dilated intrahepatic biliary radicles. Endoscopic stent placement failed to relieve the obstruction. Computerized tomography guided percutaneous aspiration of the obstructing hepatic cyst was successful with the aid of a nasobiliary cholangiogram allowing visualization of the biliary tree and identification of the obstructing hepatic cyst. However, the cyst rapidly accumulated fluid, and the obstruction recurred within 1 wk of simple aspiration. Relief of symptoms was maintained only after alcohol sclerosis of the obstructing hepatic cyst. Review of the literature shows that alcohol sclerotherapy is a safe and effective nonsurgical means of treating symptomatic hepatic cysts.
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PMID:Alcohol sclerosis for polycystic liver disease and obstructive jaundice: use of a nasobiliary catheter. 807 38


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