Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019204 (hepatocellular carcinoma)
 71,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X describes a disease characterized by histiocytic infiltration of the reticuloendothelial system, skin, bones, and pituitary gland. The disseminated form frequently occurs in infants and children. Chemotherapy has significantly improved the prognosis in this disorder. Sixty-three per cent of survivors, however, have some residual disability related to fibrosis of tissues previously infiltrated by histiocytes. In instances of liver involvement, healing by fibrosis may result in cirrhosis with portal hypertension and bleeding esophageal varices. Clinical findings include hepatosplenomegaly, jaundice, ascites, hypoalbuminemia, prolonged prothrombin time, and Bromsulphalein retention. Histologic examination of the liver shows a characteristic dense "macronodular" periportal cirrhotic pattern. Three children with portal hypertension and bleeding varices due to healed histiocytosis X were sucessfully managed by portosystemic shunt procedures. Portacaval, mesocaval, and central splenorenal shunts were equally effective in relieving poral hypertension. These children had neither recurrence of bleeding nor evidence of encephalopathy. Two children remain well whereas in one patient a primary hepatoma developed fourteen years posthung and he died of pulmonary metastases. Portosystemic shunt procedures effectively relieve the threat of potentially fatal variceal hemorrhage and improve the opportunity for long-term survival in children with cirrhosis and portal hypertension due to healed histiocytosis X.
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PMID:Portal hypertension in infants and children with histiocytosis X. 108 50

In this chapter the surgical management of bleeding oesophageal varices, ruptured hepatocellular carcinoma and fulminant liver failure have been discussed. Bleeding oesophageal varices can usually be successfully treated with vasopressin, balloon tamponade and injection sclerotherapy. Emergency surgery should be considered if two courses of injection sclerotherapy have failed to achieve haemostasis. Stapled oesophageal transection and portosystemic shunting are currently the two most popular procedures. The former is associated with a lower morbidity and mortality as well as a lower incidence of subsequent encephalopathy. Ruptured hepatocellular carcinomas are usually associated with liver cirrhosis and impaired liver function. Selective coeliac axis cannulation followed by embolization of the hepatic artery branches supplying the tumour is an effective method of achieving haemostasis and is associated with a lower morbidity and mortality than emergency hepatic artery ligation or liver resection. If haemostasis is achieved by embolization the patient may subsequently be assessed for an elective resection of the tumour. Fulminant liver failure may be managed by supportive medical therapy or orthotopic liver transplantation. Patients whose liver failure is graded as mild (grade I) should be treated by medical therapy, whereas those with severe liver damage (grades III and IV) should be assessed for transplantation. Accurate monitoring of the patient's clinical progress and prognostic indicators are vital in deciding whether conservative treatment should be continued or liver transplantation performed.
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PMID:Surgical emergencies in liver disease. 166 53

We describe a patient with a silent hepatocellular carcinoma in whom a progressive dysfunction developed in the brainstem, cerebrum, and cerebellum. This clinical manifestation was also associated with polyradiculopathy. The changes were microscopically unlike those seen in hepatic encephalopathy, progressive multifocal leukoencephalopathy and other metabolic encephalopathy. The findings seem to have occurred as a paraneoplastic syndrome of the nervous system. Although in the present case, the cause-and-effect relationship between the hepatocellular carcinoma and the encephaloradiculopathy is only hypothetical, we suggest that the demyelinating process which involves both central and peripheral nervous system in our case resulted from interaction of tumor with the host-immunological mechanism. The neurological presentation in our case is unique and has never been reported before.
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PMID:Encephaloradiculopathy: a non-metastatic complication of hepatocellular carcinoma. 166 47

We report the clinical results of 38 calibrated side-to-side portocaval shunts performed in patients with hemorrhagic liver cirrhosis (alcoholic in 90 percent of cases). The operative mortality (at 2 months) was 10.5 percent. The rate of recurrent bleeding was 2.6 percent; hepatic encephalopathy was encountered in 16 percent (acute encephalopathy: 6.5 percent; chronic encephalopathy: 9.6 percent; this rate decreased to 3.2 percent after anastomotic narrowing). Hepatopedal portal blood flow was maintained in 74.3 percent of cases in the early postoperative period (83.3 percent since the portacaval pressure gradient was maintained at 2/3 of the initial gradient) and disappeared with time in 75 percent of cases. The survival rates at 1 and 4 years were 79.4 percent and 60 percent, respectively, for all patients (94.4 and 83.3 percent for Child A patients) with a normal social activity in 90 percent of cases. Twelve patients developed hepatocellular carcinoma. These clinical results are similar to those observed after selective shunts and suggest that the side-to-side calibrated portacaval shunt is an excellent procedure for the treatment of bleeding esophageal varices in case of failure or contraindication to endoscopic sclerotherapy or in patients with chronic ascites and good liver function.
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PMID:[Calibrated side-to-side portacaval anastomosis in the treatment of bleeding from ruptured esophageal varices. Results in 38 cirrhotic patients]. 226 16

The results of a prospective series using the mesocaval interposition shunt (MIS) over a period of 13 years is reviewed. One hundred patients were selected for the operation using strict criteria, and in 98 cases the operation was performed electively. Selection criteria included a liver volume of between 1,000 and 2,500 ml, portal perfusion of between 15-30%, no active liver disease and no stenosis of hepatic artery or celiac axis, as well as a good functional Child-Pugh classification (A-B). In all the cases preoperative sclerotherapy was performed as many times as needed with the aim of controlling the active bleeding at admission and of diminishing the pre- and postoperative bleeding probability. Intra-operative postshunt measurements showed residual portal perfusion in all patients studied. Early mortality was 10% and the follow-up mortality 38.8%. The main causes of death were liver failure and hepatocellular carcinoma. The five- and ten-year survival rates were 65% and 35%, respectively. The total encephalopathy rate was 12.2%. Rebleeding was observed in 5.5% of the cases, and the long term-shunt patency rate was 90%. Anigography and sequential scintigraphy showed residual portal perfusion in 75% of the cases soon after operation, in 60% after 6 months, and in 38% after 2 years, showing the tendency of the diversion to diminish the portal perfusion rate in the late postoperative period. The results show that MIS still has a place in the treatment of portal hypertension and that it is an excellent alternative choice to the selective shunts and the devasculariaztion procedures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Analysis of a prospective series of 100 mesocaval interposition shunts for bleeding portal hypertension. 231 35

The authors measured alkaline phosphatase isozyme I (ALP-I) in sera of 24 brain-damaged patients and four with disorders other than brain damage. The study population comprised three patients with postresuscitation encephalopathy, four with ruptured cerebral aneurysms, 14 with acute subdural hematoma and cerebral contusion, and three with nontraumatic intracerebral hemorrhage. ALP-I detected in brain damage is physicochemically different from the other known ALP-Is that appear in patients with obstructive jaundice or hepatoma. In the brain-damaged patients, ALP-I became elevated about 7 days after admission and markedly increased as secondary brain damage developed. Excluding patients who died within 9 days of admission, the maximum serum ALP-I concentration was well correlated with the functional outcome. In cases in which barbiturate therapy was effective, the appearance of ALP-I was delayed and its elevation was suppressed. The results of this study suggest that measurement of serum ALP-I is useful not only in the management but also in predicting the prognosis of brain damage.
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PMID:Measurement of serum alkaline phosphatase isozyme I in brain-damaged patients. 248 67

The results of a prospective series using the mesocaval interposition shunt (MIS) over a period of 13 years is reviewed: 100 patients were selected for the operation using strict criteria and in 98 cases the operation was performed electively. Selection criteria included liver volume between 1000-2500 mL, portal perfusion between 15-30%, no active liver disease and no stenosis of hepatic artery or coeliac axis as well as a good functional CHILD-PUGH classification (A-B). In all the cases preoperative sclerotherapy was performed so many times as needed by each individual patient with the goal of controlling the active bleeding episodes at admission and of diminishing the pre and postoperative bleeding probability. Intraoperative postshunt measurements showed residual portal perfusion in all studied patients. Early mortality was 10% and the follow up mortality 38.8%. Main causes of death were liver failure and hepatocellular carcinoma. Five and ten years survival rates were 63.9% and 35.1% respectively. The total encephalopathy rate was 12.2%. Rebleeding was observed in 5.5% of the cases and long term shunt patency rate among survivors was 90%. Angiography and sequential scintigraphy showed residual portal perfusion in 75% of cases soon after operation, in 60% after 6 months and 38% after 2 years, showing the tendency of the derivation to diminish the portal perfusion rate in the late postoperative period. The results show that MIS still has a place in the treatment of portal hypertension and that it is an excellent alternative choice to the selective shunts and the devascularization procedures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:100 meso-caval interposition-shunts for recurrent variceal hemorrhage in portal hypertension. A prospective study. 263 Nov 66

The aim of this retrospective study was to define prognostic factors for cure and survival after spontaneous bacterial peritonitis. In a 4-year period from 1982 to 1986, spontaneous bacterial peritonitis was diagnosed in 38 consecutive hospitalized cirrhotic patients (positive ascites culture and polymorphonuclear cell concentration greater than 250 cells per mm3). Twenty-five patients recovered from their infection (69 p. 100) in a mean time of 9 +/- 7 days. The cumulative survival was 68 p. 100 at one week, 50 p. 100 at one month, and 25 p. 100 at one year. The best independent prognostic factors for lack of cure from peritonitis were a low ascitic pH value (p less than 0.001), an elevated serum creatinine level (p = 0.01) and the presence of hepatocellular carcinoma (p less than 0.05). The best prognostic factors for death were low ascitic pH value (p = 0.001) and gastrointestinal hemorrhage (p = 0.005). A low ascitic pH value was correlated with other signs of severe infection (signs of generalized infection, ongoing infection during the first week after diagnosis), with signs of severe liver disease (encephalopathy, hepatocellular carcinoma) or severe renal dysfunction (high serum creatinine level, low arterial pH value). Because of the late high-death rate associated with spontaneous bacterial peritonitis, liver transplantation should be considered in these patients.
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PMID:[Prognosis of spontaneous ascitic infection in cirrhotic patients]. 275 3

A 40-yr-old man presented with encephalopathy and was found to have hepatocellular carcinoma without cirrhosis. A large vascular hepatic mass was defined by CT scan and angiography; laparoscopy with biopsy confirmed the absence of chronic liver disease. A definitive tissue diagnosis of hepatocellular carcinoma was made at laparotomy; the tumor was unresectable. Peripheral arterial and selective portal and hepatic venous ammonia levels were high, and this finding suggested that the encephalopathy was nitrogenous and hepatic in origin. The proposed mechanisms of the encephalopathy are generation of ammonia from tumor breakdown and portosystemic shunting, a result of partial tumor occlusion of the hepatic veins. An unusually high urinary excretion of orotic acid was found similar to that seen in hereditary orotic aciduria.
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PMID:Hepatic encephalopathy and orotic aciduria associated with hepatocellular carcinoma in a noncirrhotic liver. 282 14

The authors report the clinical and biological features in 197 patients with hepatocellular carcinoma seen in two French hospitals. Mean age was 63 +/- 12 years. Eighty-nine per cent were men. Cirrhosis was present in 88 p. 100. Alcoholic liver disease was associated with hepatocellular carcinoma in 71.5 p. 100. At the time of diagnosis, ascites was present in 62 p. 100 of the patients, jaundice in 49 p. 100, encephalopathy in 20 p. 100 and gastrointestinal bleeding in 12.5 p. 100. Twenty patients (10 p. 100) did not have any of these complications. An increase in serum gammaglutamyl transpeptidase and ASAT was the most frequent biological abnormality observed in 96 and 94 p. 100 of patients respectively. Hypercalcemia and a high hematocrit were present in 5 and 6 p. 100 of patients respectively. Serum HBs Ag (RIA) was present in 17.5 p. 100 of patients, anti-HBc in 50 p. 100 and anti-HBs in 33.5 p. 100; 38.5 p. 100 of patients had no serum HBV marker. Serum alphafetoprotein levels were higher than 20 ng/ml, 250 ng/ml and 1,000 ng/ml in 76.5 p. 100, 43.5 p. 100 and 33 p. 100 of patients respectively. There were no relationships between the presence of serum markers of HBV or high alphafetoprotein levels and clinical and biological data. These results confirm that the clinical, biological and virological aspects of hepatocellular carcinoma in France are similar to those reported in other western countries.
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PMID:[Hepatocellular carcinoma in France. Clinical, biological and virological aspects in 197 patients]. 298 69


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