Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lipoprotein-X (LP-X) in the serum of infants with persistent jaundice is indicative of cholestasis. In early infancy biliary atresia and biliary agenesis are the most common cause of cholestasis, whereas neonatal hepatitis is a less frequent cause of cholestasis. The authors introduced and described the qualitative and quantitative methods of LP-X determination for diagnostic purposes. LP-X estimations were carried out in 9 children with persistent jaundice. LP-X was found to be present in 4 infants-in 2 with complete absence of extrahepatic biliary tracts, in 1 with extrahepatic biliary atresia and in 1 with hypoplastic extrahepatic biliary tract. LP-X was also found in a 5 year old boy with mechanical occlusion of bile ducts caused by a malignant tumor ( rhabdomyoblastoma ), and in 3 year old girl with inborn enzymatic liver dysfunction. In this case LP-X concentration was estimated before and after 3 week course of cholestyramine, after which there was a 35% decrease in the LP-X concentration. In a 4 month old child LP-X was not found in spite of the absence of extra and intrahepatic biliary tracts. This finding may be explained by the far advanced hepatic cirrhosis. The authors stress the importance of introducing of LP-X estimation in the differential diagnosis of jaundice in early infancy.
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PMID:[Lipoprotein X (LP-X) in the differential diagnosis of cholestasis in children, with special reference to biliary atresia]. 26 31

The influence of cirrhosis on superparamagnetic iron oxide (SPIO)-enhanced magnetic resonance (MR) imaging of the liver was studied in 31 rats. Experimental models included carbon tetrachloride-induced cirrhosis and liver engrafting of rhabdomyosarcoma S4T cells. Hepatic uptake of SPIO measured with relaxometry decreased dramatically with histologic grade, while splenic uptake increased; the same results were achieved by calculating K values. Imaging of 13 tumorous cirrhotic rats confirmed these results by showing a muted decrease in liver signal intensity on spin-echo images after injection of SPIO. Nevertheless, all intrahepatic tumors could be visualized, as confirmed by postmortem examination. On gradient-echo images, postinjection contrast between the tumor and the liver was not affected by cirrhosis. Thus, despite strong impairment of hepatic uptake in cirrhosis, the diagnostic efficacy of MR imaging with SPIO did not seem to be significantly affected.
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PMID:Liver tumors in cirrhosis: experimental study with SPIO-enhanced MR imaging. 205 18

Congenital defects and other disorders have been reported in association with malignant liver tumours. In order to assess their significance, a population-based survey was undertaken on children aged less than 15 years diagnosed with malignant liver tumours during the 30 years 1957-1986. The cases were identified from information collected by the West Midlands Regional Children's Tumour Registry. Pertinent data were extracted from their clinical records, and the original biopsy and any necropsy material were reviewed by a panel of three paediatric pathologists. Of the 50 eligible cases registered, eight were excluded because histology review showed that they had non-malignant conditions (3) or malignancies of extrahepatic origin (4) or because no pathological material was available (1). The diagnoses in the remaining 42 cases were hepatoblastoma (27), hepatocellular carcinoma (3), rhabdomyosarcoma (6), rhabdoid tumour (3) and yolk sac tumour (3). The incidence of primary malignant liver tumours was 1.20 per 10(6) person years and that of the hepatoblastoma sub-group was 0.77 (average childhood population of the West Midlands for the time period being 1,166,500). The presenting clinical, radiological and biochemical features were similar to those reported in other series and the ethnic and social class distributions were unremarkable compared with the local population. Congenital defects or other possibly related features were present in nine (21%) patients. Our results, taken with other reports, suggest that hepatoblastoma is a malignant tumour related to maldevelopment, possibly associated with 11p or 5q mutations, whereas hepatocellular carcinoma is more usually a complication of metabolic and other disorders which lead to cirrhosis.
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PMID:Malignant hepatic tumours in children: incidence, clinical features and aetiology. 237 47

From March 1991 through 31st December 2007, 2042 patients underwent stem cell transplantation at the Hematology-Oncology and Stem Cell Transplantation Research Center, affiliated to Tehran University of Medical Sciences. These transplantations included 1405 allogeneic stem cell transplantation, 624 autologous stem cell transplantation, and 13 syngeneic stem cell transplantation. Stem cell transplantation was performed for various diseases including acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, thalassemia major, sickle cell thalassemia, sickle cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combine immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. We had 105 cellular therapies for postmyocardial infarction, multiple sclerosis, cirrhosis, head of femur necrosis, and renal cell carcinoma. About 30 patients were retransplanted in this center. About 74.9% of the patients (1530 of 2042) remained alive between one to 168 months after stem cell transplantation. Nearly 25.1% (512 of 2042) of our patients died after stem cell transplantation. The causes of deaths were relapse, infections, hemorrhagic cystitis, graft versus host disease, and others.
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PMID:Stem cell transplantation; Iranian experience. 1911 Oct 33

We report a rare case of a hepatic carcinosarcoma with rabdomyosarcomatous differentiation in its sarcomatous component. A 71-year old Caucasian female patient underwent a liver resection for a 4-cm lesion developed on an underlying HCV-related cirrhosis. Post-operative course was uneventful and the patient was discharged 5 days after surgery. At pathology, the tumor presented the features of hepatocellular carcinoma and rhabdomyosarcoma Three months later the patient experienced a liver recurrence, dying 2 months later for systemic disease. The reported case presents several peculiarities, i.e. the female gender, the HCV-related cirrhotic status, and the European origin of the patient. However, the outcome of our case confirms that this neoplasm pursues a highly aggressive course with poor prognosis.
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PMID:An unusual case of hepatic carcinosarcoma. 2201 59