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

From December 1964 to November 1989, 71 children from 3 to 17 years of age with the eventual diagnosis of hepatocellular carcinoma (HCC) presented at the National Taiwan University Hospital (Taipei, Taiwan, Republic of China). Forty-three of them had pathologic proof, whereas 28 were diagnosed on a clinical and laboratory basis. A male predominance (M:F = 3.2:1) was noticed. Most patients presented in a late, advanced stage. Abdominal pain and abdominal mass were the major symptoms and signs, followed by anorexia, fever, and internal bleeding. Hydrocele, purpura, and obstructive jaundice were rare presenting symptoms. Hepatosplenomegaly, superficial venous engorgement, and ascites were the main physical signs. The prognosis for such children with HCC was very poor. Only 10% of the patients survived longer than 1 year after the onset of the initial symptom. Among 49 patients who could be followed, only two had long-term survival of over 5 years. One patient had a small HCC with internal bleeding, whereas the other had a large HCC with abdominal distention. Both received surgical resection, and a resection was repeated for tumor recurrence in the patient with the large mass. The resectability of these 71 patients was low (9.8%). Resectability and nonicterus seemed to be the factors indicating favourable prognosis. Observation indicated that the prognosis for children with symptomatic HCC is grave but surgical resection, whenever possible, should be carried out.
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PMID:Hepatocellular carcinoma in childhood. Clinical manifestations and prognosis. 165 24

The acute form of tyrosinemia type I usually causes severe hepatocellular dysfunction. We report a 4-month-old infant with hepatosplenomegaly, ascites, and multiple intrahepatic mass lesions mimicking hepatoma. A marked increase of serum alpha-fetoprotein (97.6 micrograms/ml) and multiple small low-density lesions in the liver demonstrated by computed tomography suggested the presence of hepatoma. The liver specimens obtained at laparatomy showed mixed nodular cirrhosis with fatty metamorphosis. Serum levels of tyrosine (6.6 mg/100 ml) and methionine (5.9 mg/100 ml) were increased. Urinary organic acid analyses disclosed increased excretions of succinylacetone (1,330 mg/g creatinine) and delta-amino-levulinic acid (113.6 mg/g creatinine). Histological abnormalities and biochemical evidences led to the correct diagnosis. This case emphasizes the need for complete investigations of puzzling cases and warns against undue reliance on noninvasive imaging techniques.
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PMID:An acute form of tyrosinemia type I with multiple intrahepatic mass lesions. 169 41

Membranous obstruction of the inferior vena cava (IVC) is a curable cause of a primary type of Budd-Chiari syndrome. Magnetic resonance (MR) imaging and vena cavography were performed on nine patients with membranous obstruction of the IVC. The MR findings were retrospectively analyzed and compared with computed tomographic findings in seven patients. The morphologic features of membranous obstruction of the IVC on spin-echo MR images were a curvilinear soft-tissue membrane (five cases) or an obliterated lumen of a hepatic segment of the IVC (four cases) in transverse or sagittal views. The lumen below the obstruction revealed flow-related signal (seven cases), intraluminal thrombus (one case), and thrombotic occlusion (one case). The hepatic veins were narrow and disoriented without connection to the hepatic segment of the IVC just below the diaphragm. On T2-weighted images, inhomogeneity with high signal intensity was shown more prominently in the hepatic parenchyma in Simson type II or III membranous obstruction. Other findings were hepatosplenomegaly, enlarged caudate lobe, cirrhotic liver, associated hepatoma, and presence of various collaterals.
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PMID:Membranous obstruction of the inferior vena cava with Budd-Chiari syndrome: MR imaging findings. 179 12

This study presents the results of abdominal ultrasonic scanning in 108 patients attending a tropical referral hospital. Clinical diagnoses included hepatocellular carcinoma, metastatic liver disease, amoebic liver abscess, hydatid disease, obstructive jaundice, hepatosplenomegaly of uncertain aetiology and renal cysts and tumours. Because of its ability to distinguish solid from fluid-filled lesions, we found ultrasonic scanning the most useful initial investigation for the differentiation of hepatic masses. Ultrasonography is also ideal for the diagnosis of abdominal cysts and is extremely reliable in differentiating extrahepatic from intrahepatic obstructive jaundice. It is a non-invasive procedure, quick and easily repeatable and has great potential in tropical medical practice.
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PMID:Ultrasonic diagnosis of abdominal disease in Kenya. 627 48

A survey of all patients in whom liver biopsy showed epithelioid granulomas was undertaken at two major teaching hospitals in Glasgow for the period 1970-1979. Seventy-seven patients with hepatic granulomas were studied retrospectively. In 53 cases (69 per cent) a clear-cut clinical diagnosis was established, which included sarcoidosis (eight cases), tuberculosis (eight), extrahepatic biliary obstruction (seven), primary liver diseases (11), neoplasm (six), bacterial infection (five) and miscellaneous (eight). In 24 patients (31 percent) no cause was found. Seventeen patients from this idiopathic group were studied prospectively and single examples of the following conditions were subsequently diagnosed; pulmonary tuberculosis, primary biliary cirrhosis, ulcerative colitis, adenocarcinoma of rectum, primary hepatocellular carcinoma, alpha-one antitrypsin deficiency and pulmonary fibrosis, sarcoidosis, pulmonary fibrosis alone, gallstones, rheumatic heart disease, unexplained hepatosplenomegaly and one death from mesenteric artery thrombosis. Only six cases remained truly idiopathic. Three of these patients recovered and in two liver biopsy became normal. The other three have persistent granulomas associated with continuing illness.
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PMID:Hepatic granulomas: experience over a 10-year period in the West of Scotland. 711 78

High prevalence rates of hepatitis C virus (HCV) were recently reported among Egyptian blood donors. To confirm these observations and estimate the magnitude of HCV infection in this country, we determined the prevalence of antibodies to HCV (anti-HCV) in samples collected in 1992 from seven different populations of children and adults living in Egypt. Anti-HCV was found in 12.1% of rural primary schoolchildren, 18.1% of residents of a rural village, 22.1% of army recruits, 16.4% of children with hepatosplenomegaly, 54.9% of hospitalized, multitransfused children, 46.2% of adults on hemodialysis, and 47.2% of adults with chronic liver disease or hepatoma. Age-related prevalence of anti-HCV in a random sample of 270 inhabitants of a rural village increased progressively from zero in those 5-10 years of age to 41% in adults greater than the age of 50. Although the increased prevalence of anti-HCV among children and adults with parenteral exposures and chronic liver disease was expected, the prevalence of anti-HCV among persons representing the general population of Egypt was strikingly high. These data demonstrate the magnitude of HCV infection and its importance in chronic liver disease in Egypt. Future studies are needed to determine the routes of transmission of HCV in this country.
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PMID:High seroprevalence of hepatitis C infection among risk groups in Egypt. 752 86

A case of hepatocellular carcinoma complicating biliary cirrhosis caused by biliary atresia is reported. The patient had persistent severe jaundice with hepatosplenomegaly. A liver tumor was suspected because of the elevated serum alpha-fetoprotein and was shown by ultrasonography at 6 years of age. The tumor was treated with percutaneous ethanol injection therapy (PEIT). Nine months after initiation of PEIT, the patient died of massive bleeding from a metastatic tumor.
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PMID:Hepatocellular carcinoma complicating biliary cirrhosis caused by biliary atresia: report of a case. 874 33

Cell mediated immune response (CMIR) was studies in 120 patients having chronic liver diseases. Patients were divided into 6 groups, (20 each). (1) Early hepatosplenic Schistosomiasis. (EHSS), (2) Late hepatosplenic Schistosomiasis. (LHSS), (3) Hepatosplenic Schistosomiasis with hepatitis B and/or C infections, (4) Hepatitis B virus cases. (HBV), (5) Hepatitis C virus cases (HCV), (6) Hepatocellular carcinoma cases. (HCC). Twenty within normal subjects taken as controls. Laboratory investigations revealed significant esinophilia in patients of group (1), haemoglobin level was significantly reduced in patients of group (1, 2, 3, & 6), serum albumin was significantly reduced in group (2). The percentage of positivity of skin testing using purified protein derivative, ranged between 10% of patients with LHSS, HBV, HCC and HSS with HBV and/or HCV, 20% of patients with HCV and 25% of patients with EHSS. Percentage of positivity in control group was 100%. The mean diameter of delayed intradermal reaction (2.2 +/- 0.5-6.1 +/- 2.1 mms.) was significantly lower in patients than controls. The response of lymphocyte transformation test to phytohaemmagglutinin was significantly lower in patients when compared to controls. The association of HBV and/or HCV with hepatosplenomegaly was accompanied with a marked depression in cell mediated immune response. Anaemia, hypoalbuminemia and nutritional status of the patients with chronic liver diseases play a major role in the suppression of cell mediated immune response.
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PMID:Cell mediated immune response in chronic liver diseases: schistosomal, viral and neoplastic. 991 13

Mucopolysaccharidosis type I (MPS I), a deficiency in the lysosomal enzyme alpha-L-iduronidase (IDUA), is characterized by skeletal abnormalities, hepatosplenomegaly and neurological dysfunction. To evaluate the potential for treatment of the disease using a gene delivery approach, recombinant adeno-associated virus (rAAV) vectors were constructed and evaluated for expression of the human IDUA cDNA in transduced cells. 293 cells transduced with these AAV vectors contained IDUA activity at 0.5 to 1.4 micromol/mg x hr, 50- to 140-fold above background (control-transduced) levels. In time course studies of transduced 293 cells, IDUA activity levels peaked 1 week after transduction and persisted at 50% of the peak level for at least 6 weeks. Transduced MPS I fibroblasts also expressed high levels of IDUA activity (114-290 nmol/mg x hr), which persisted for at least 3 weeks in the absence of selection. In addition, transduced MPS I fibroblasts were capable of clearing intracellular radiolabeled glycosaminoglycan (GAG). As a test of the ability of these vectors to mediate metabolic cross-correction, transduced HuH7 human hepatoma cells were demonstrated to release enzyme that was subsequently taken up by nontransduced MPS I fibroblasts. These results illustrate the effectiveness of AAV vectors for delivery and expression of human IDUA gene sequences and for potential treatment of MPS I.
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PMID:Enzymatic correction and cross-correction of mucopolysaccharidosis type I fibroblasts by adeno-associated virus-mediated transduction of the alpha-L-iduronidase gene. 1049 48


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