Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019163 (hepatitis B)
 38,309 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Papillon Lefevre syndrome (PLS) is a rare autosomal recessive disorder, which is characterized by palmar-plantar hyperkeratosis, periodontitis, and premature loss of dentition. We report a 16 years old girl with PLS. The patient presented at 08 years of age with complaints of corn on the feet and hands, and failure to thrive. On examination, her upper primarily canines were loose, she had severe periodontitis, eruption of permanent teeth, diffuse eritematous and hyperkeratotic palms and soles that suggested the syndrome. During the follow-up, the patient was diagnosed to have congenital hepatic fibrosis (CHF) when she was 16 years old, while she was being investigated for the etiology of her splenomegaly and pancytopenia. We report a patient with PLS associated with CHF, an association that has not been previously described. Abbreviations-HbsAg: Hepatitis B virus surface antigen, Anti Hbs: Antibody against Hepatitis B surface antigen, Anti Hbc IgM: Antibody against Hepatitis B cor antigen immunglobulin M, Anti dsDNA: Antibody against double stranded deoksiribonucleic acid, Anti HCV: Antibody against Hepatit C virus, Anti HIV: Antibody against human immun deficiency virus, AST: Aspartat amino transferase, ALT: Alanin amino transferase, Gamma-GT: Gamma glutamyl transferase, LDH: Lactate dehydrogenase & MRI: Magnetic resonance imaging.
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PMID:Papillon-lefevre syndrome with congenital hepatic fibrosis. 1791 35

An 11-year-old girl was admitted with backpain, weight loss, fatigue and behavioural disturbances, starting seven weeks before admission. Physical examination showed acrodynia, tremor, cachexia, hypertension and extensive gingival ulceration. Routine laboratory tests were normal, except for a CRP of 98 mg/l. Screening tests for recreational drugs as well as antibody assays for HIV, hepatitis B and borrelia burgdorferia were negative. Chest X-ray, brain CAT and MRI scan were all normal. Lumbar puncture didn't show any abnormalities. Eventually a 24-hour urine test confirmed the diagnosis that was suspected by further questioning.
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PMID:A previously healthy 11-year-old girl with behavioural disturbances, desquamation of the skin and loss of teeth. 1904 36

Hepatocellular carcinoma (HCC) is a malignant tumor which is becoming more prevalent worldwide. Patients at high risk of developing HCC, namely hepatitis B- and C-related liver cirrhosis patients, should be entered into surveillance programs, which should be performed using both ultrasonography and 3 tumor markers (AFP, PIVKA-II, AFP-L3). The surveillance interval needs to be shortened for patients at higher risk of HCC. Therefore, super-high-risk patients should be screened at 3- to 4-month intervals based on their risk of developing HCC. Sonazoid-enhanced US is extremely useful to characterize hepatic tumors when compared with multidetector-row computed tomography (MDCT). Moreover, Sonazoid-enhanced US with defect reperfusion imaging is a breakthrough approach in the treatment of HCC. This technique will markedly change the therapeutic strategy for liver cancer. Furthermore, diagnostic capability using the new imaging technique Gd-EOB-DTPA MRI is promising. A reduced uptake (low intensity) in the hepatobiliary phase of Gd-EOB-DTPA MRI strongly suggests HCC (including early-stage HCC) or a high-grade dysplastic nodule with high malignant potential. Empirically, intrahepatic arterial infusion chemotherapy using implanted reservoir port is known to be effective for advanced HCC with vascular invasion; however, no randomized study exists to prove its efficacy. Further controlled study is necessary to establish this treatment option as a standard of care in a treatment algorithm for HCC. In contrast, sorafenib was established as the first choice of treatment as a standard of care in advanced HCC patients with preserved liver function and vascular invasion/extrahepatic spread. Furthermore, global clinical trials are now ongoing using sorafenib as an adjuvant setting after resection, ablation or TACE. Efficacy of combined use of sorafenib with TACE or intra-arterial infusion chemotherapy is not clear. In order to clarify this issue a randomized clinical trial for intermediate and advanced HCC comparing sorafenib alone versus sorafenib combined with maintenance TACE/intra-arterial infusion chemotherapy and/or intra-arterial infusion chemotherapy is scheduled to be initiated in Japan in 2009. If positive results are obtained by these trials, its impact on treatment strategy for HCC will be drastically changed.
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PMID:Hepatocellular carcinoma 2009 and beyond: from the surveillance to molecular targeted therapy. 1909 66

The purpose of this study was to evaluate the diagnostic ability of the expanded gallbladder fossa and right posterior hepatic notch signs for hepatic fibrosis determined by double contrast-enhanced MRI. For patients with chronic viral hepatitis B (n = 96) or hepatitis C (n = 13) who underwent gadopentate dimeglumine-enhanced dynamic MRI followed by ferucarbotran-enhanced gradient-echo imaging, the degree of parenchymal fibrosis was categorised into three groups based on the extent of reticulation and nodularity: (1) pre-cirrhotic or minimal fibrosis; (2) mild to moderate fibrosis; (3) advanced cirrhosis. Each group was evaluated for the presence of a sharp notch in the posterior-medial surface of the right lobe of the liver and expanded gallbladder fossa. The expanded gallbladder fossa sign gradually increased with an increasing degree of fibrosis (Group 1, 50%; Group 2, 61%; Group 3, 78%), and there was no significant difference (p>0.5) between hepatitis B (67%) and C (73%). In the case of the right posterior hepatic notch sign, only 6% of Group 1 and Group 2 patients were positive; 27% of hepatitis B patients and 90% of hepatitis C patients in Group 3 exhibited the sign (p<0.05). Owing to its low prevalence, even in advanced cirrhosis, the right posterior hepatic notch sign is of little value in the diagnosis of cirrhosis due to chronic hepatitis B virus infection, whereas an expanded gallbladder fossa could be used as a non-specific indicator of early fibrosis before the gross appearance of advanced hepatic fibrosis.
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PMID:Double contrast-enhanced MRI of viral hepatitis-induced cirrhosis: correlation of gross morphological signs with hepatic fibrosis. 1950 65

We evaluated the frequency, demographic, clinical, disability evolution and genetic association of HLA DRB1*1501, DRB1*1503, DQA1*0102, DQB1*0602 and DPA1*0301 alleles in patients diagnosed as acute disseminated encephalomyelitis (ADEM) among a population of CNS demyelinating diseases. Fifteen patients (8.4%) of our series were diagnosed as ADEM. The mean age onset was 35.23 years (range 12 to 77), 53.3% were male and follow-up range was 8.5 to 16 years. Two cases (13.3%) had a preceding infection before neurological symptoms, one presented a parainfectious demyelinating, and one case had been submitted to hepatitis B vaccination four weeks before the clinical onset. The EDSS range was 3.0 to 9.5. Eight patients (53.3%) presented MRI with multiple large lesions. CSF was normal in 73.3%. The severe disability observed at EDSS onset improved in 86.66% patients. The genetic susceptibility for ADEM was significantly associated with the HLA DQB1*0602, DRB1*1501 and DRB1*1503 alleles (<0.05) in monophasic ADEM.
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PMID:Acute disseminated encephalomyelitis: clinical features, HLA DRB1*1501, HLA DRB1*1503, HLA DQA1*0102, HLA DQB1*0602, and HLA DPA1*0301 allelic association study. 1972 42

A 47-year-old otherwise healthy woman, presented elevation of alpha fetoprotein (AFP) and des-gamma-carboxy prothrombin (DCP) levels at a general health checkup. Both HCV antibody and hepatitis B surface antigen were negative. A screening abdominal CT revealed no abnormal change. An abdominal MRI and repeated CT, however, revealed a 20-mm tumor adjacent to the inferior vena cava and adjacent to or involving the liver. A surgical resection of the tumor was performed. The tumor was adjacent to, but distinct from, the liver. The Capsule of the tumor was connected to the liver but it was distinct from hepatic, renal, and adrenal tissue. A histological examination yielded a diagnosis of moderately differentiated hepatocellular carcinoma, with positive staining of hepatocyte-specific antigen and AFP.
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PMID:[A case of ectopic hepatocellular carcinoma]. 1996 20

A 36-year-old man was hospitalized because of subacutely progressive gait disturbance. Neurological examination disclosed severe ataxia of gait and trunk and moderate ataxia of the four limbs, without signs of cognitive impairment. There were no manifestations of systemic infections. Brain MRI showed mild atrophy of the cerebellar vermis and hemispheres. Extensive laboratory search failed to disclose the cause of subacute ataxia. Cerebellar ataxia progressed, leading to the patient becoming wheelchair-bound two months after admission, when PCR analysis of the cerebrospinal fluid was positive for Epstein-Barr, JC, and hepatitis B viruses. In addition, the quantity of serum HIV1-RNA was 2.9 x 10(4) copies, the absolute count of CD4+ lymphocyte was 28/mm3, and the CD4/CD8 ratio was 0.04, despite clear denials by both the patient and his wife regarding any apparent infectious opportunities. Accordingly thereafter, highly active antiretroviral therapy was initiated. Several weeks after the initiation of therapy, ataxia stabilized with disappearance of serum HIV and cerebrospinal fluid JCV viral load. He returned to his occupation 20 months after disease onset without progression of ataxia or development of other neurological dysfunctions including dementia. We could not establish the exact pathogenesis of ataxia in this patient It could have been primary cerebellar degeneration caused by HIV, or the other viruses detected (EBV, JCV) or autoimmune mechanisms caused by these viruses. However, HIV infection should be considered as an etiology in clinical setting of subacute ataxia, particularly in a young or immunocompromised patient.
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PMID:[A case of cerebellar syndrome associated with HIV infection]. 1999 47

Liver fibrosis determines the functional liver reserve. Several studies have reported that the apparent diffusion coefficient (ADC) values of diffusion-weighted magnetic resonance imaging (DW-MRI) can assess liver fibrosis. We investigated whether DW-MRI predicts postoperative hepatic insufficiency and liver fibrosis in patients with hepatitis B virus (HBV)-related hepatocellular carcinoma (HCC). Twenty-six patients with HBV-related HCC who received preoperative DW-MRI on a 3-T MRI system were enrolled between July and December 2008. ADC values were measured twice by two observers. Three "b values" were used: 50, 400 and 800 s/mm(2). Postoperative hepatic insufficiency was defined as persistent hyperbilirubinemia (total bilirubin level >5 mg/dl for more than 5 days after surgery) or postoperative death without other causes. The mean age (21 men and 5 women) was 51.4 years. Three patients experienced postoperative hepatic insufficiency. liver stiffness measurement predicted postoperative hepatic insufficiency, advanced fibrosis (F3-4), and cirrhosis significantly [area under the receiving operator characteristic curve (AUROC)=0.942, 0.771 and 0.818, respectively, with P=.047, 0.048 and 0.006, respectively]; ADC values of DW-MRI, however, did not (AUROC=0.797, 0.648 and 0.491, respectively, with P=.100, 0.313 and 0.938, respectively). Reliability of ADC values between right and left hepatic lobes (rho=0.868 and rho=0.910 in the first and second measures of Observer A; rho=0.865 and rho=0.831 in the first and second measures of Observer B) was high and the intra- and interobserver reliability (rho=0.958 in observer A and rho=0.977 in observer B; rho=0.929 in the first measure and rho=0.978 in the second measure between the two observers) were high. All reliability was significant (P<.001). Our results suggest that DW-MRI on a 3-T MRI system is not suitable for predicting postoperative hepatic insufficiency, advanced liver fibrosis, and cirrhosis in patients with HBV-related HCC, despite significantly high reliability.
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PMID:Can preoperative diffusion-weighted MRI predict postoperative hepatic insufficiency after curative resection of HBV-related hepatocellular carcinoma? A pilot study. 2039

Posterior reversible encephalopathy syndrome (PRES) is a rare complication of nephrotic syndrome and corticosteroid therapy. Here, we discuss an 18 year old man with type 1 membranoproliferative glomerulonephritis (MPGN) secondary to hepatitis B infection who developed posterior leukoencephalopathy while on therapy with lamivudine and prednisone. He developed seizures and vision loss. He also had hypertension, but no sudden elevation was recorded at any time. Magnetic resonance imaging revealed patchy areas of altered signal intensity involving cortical gray and subcortical white matter in the bilateral frontoparietal regions, occipital cortices, temporal cortices and cerebellar hemispheres, and hyperintensity on T2W and FLAIR sequences. Tapering of prednisone and controlling hypertension resulted in clinical improvement within a few days, and in a month MRI was normal. Diagnosing PRES requires a high index of suspicion when treating similarly susceptible patients. PRES as a complication during the treatment of MPGN secondary to hepatitis B has, to our knowledge, never been reported previously in the literature.
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PMID:Posterior reversible encephalopathy syndrome in a patient with hepatitis B induced type 1 membranoproliferative glomerulonephritis. 2073 Apr 70

Solitary necrotic nodule of the liver (SNNL) is rare. Generally thought to be nonmalignant, it is often mistaken for malignancy based on imaging findings alone. We present a case of a hepatitis B carrier who was found to have a new sonographically detected hepatic lesion. The lesion was further evaluated with CT and MRI, and as appearances were suggestive of a hypovascular hepatoma, the lesion was surgically resected. This case is unique in that while it demonstrates several characteristic features of SNNL, several other imaging and histological features have not been previously described.
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PMID:Solitary necrotic nodule of the liver: radiologic-pathologic correlation in a case with unusual imaging features. 2326 24


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