Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report studies of a Croatian boy, a proven case of human S-adenosylhomocysteine (AdoHcy) hydrolase deficiency. Psychomotor development was slow until his fifth month; thereafter, virtually absent until treatment was started. He had marked hypotonia with elevated serum creatine kinase and transaminases, prolonged prothrombin time and low albumin. Electron microscopy of muscle showed numerous abnormal myelin figures; liver biopsy showed mild hepatitis with sparse rough endoplasmic reticulum. Brain MRI at 12.7 months revealed white matter atrophy and abnormally slow myelination. Hypermethioninemia was present in the initial metabolic study at age 8 months, and persisted (up to 784 microM) without tyrosine elevation. Plasma total homocysteine was very slightly elevated for an infant to 14.5-15.9 microM. In plasma, S-adenosylmethionine was 30-fold and AdoHcy 150-fold elevated. Activity of AdoHcy hydrolase was approximately equal to 3% of control in liver and was 5-10% of the control values in red blood cells and cultured fibroblasts. We found no evidence of a soluble inhibitor of the enzyme in extracts of the patient's cultured fibroblasts. Additional pretreatment abnormalities in plasma included low concentrations of phosphatidylcholine and choline, with elevations of guanidinoacetate, betaine, dimethylglycine, and cystathionine. Leukocyte DNA was hypermethylated. Gene analysis revealed two mutations in exon 4: a maternally derived stop codon, and a paternally derived missense mutation. We discuss reasons for biochemical abnormalities and pathophysiological aspects of AdoHcy hydrolase deficiency.
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PMID:S-adenosylhomocysteine hydrolase deficiency in a human: a genetic disorder of methionine metabolism. 1502 24

We report the case of a 49-Year-old-man with primary sclerosis cholangitis (PSC) and ulcerative colitis who developed two acute episodes of pseudo-angiocholitis. Both episodes were triggered by septic hepatitis translocated from ulcerative colonic adenocarcinoma. The biliary MRI did not show any signs of lithiasis or cholangiocarcinoma. cholangiocarcinoma, intra-hepatic lithiasis and colonic cancer are potential diagnoses in patients with PSC who develop angiocholitis.
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PMID:[Acute "pseudo-angiocholitis" due to colonic adenocarcinoma in a man with primary sclerosis cholangitis and ulcerative colitis]. 1503 32

Liver biopsy by needle through skin is safe, simple and valuable method for diagnostic evaluation of liver disease. Diffuse parenchymal diseases as cirrhosis, hepatitis, reactions in drugs could be diagnosed with significant accuracy. With increased usage of CT and MRI as well as ultrasound, it is possible to perform aspiration biopsy of isolated changes with thin needles. In this review, most frequent indications and contraindications for that procedure were described.
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PMID:[Liver biopsy and indications]. 1513 38

Hemangiomatosis of the liver is growing in the last decades, because of the abdominal imaging progresses: brain CT scan, MRI. In the presented case, the onset was with symptoms and biochemical disorders characteristic for acute viral hepatitis, with negative serological markers for the A and B hepatitis viruses. The persistence of the clinical symptoms and of the hepatic and biliary retention tests, determined the abdominal echography; which orient the diagnosis to vascular tumour with liver metastasis. Intraoperatively was found: diffuse hemangiomatosis of the liver. The patient didn't benefit from liver transplantation and died due to subfulminant hepatic failure.
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PMID:[Diffuse hepatic hemangiomatosis--case report]. 1568 16

E-selectin-targeted contrast enhancement of blood vessels in inflamed tissues was investigated with a new contrast agent, Gd-DTPA-B(sLe(x))A, which was recently obtained by grafting a synthetic mimetic of sialyl-Lewis(x), an E-selectin ligand, onto Gd-DTPA. The pharmacokinetics, biodistribution, and potential to image inflammation by MRI of this E-selectin-targeted contrast agent were evaluated. The inhibition (by 15-34%) produced by Gd-DTPA-B(sLe(x))A on Sialyl Le(x)-PAA-biotin binding to E-selectin confirmed the specific interaction of the new contrast agent with this adhesion molecule. Gd-DTPA-B(sLe(x))A was tested at a dose of 0.1 mmol/kg b.w. on mice and rats in a fulminant hepatitis model induced by the co-administration of D-galactosamine and E. coli lipopolysaccharide. A significant and prolonged contrast enhancement between blood vessels and liver parenchyma was obtained in pathological conditions, which attests to the specificity of the agent for E-selectin. The prolonged vascular residence (48.9 min in hepatitis vs. 29.8 min in healthy animals), as evidenced by the pharmacokinetic characterization, suggests that Gd-DTPA-B(sLe(x))A interacts with the specific receptors expressed during inflammation. The biodistribution of the compound indicates its retention in inflamed liver by both specific mechanisms and nonspecific accumulation due to the necrotic lesions. The same mechanisms are invoked to account for its retention in the spleen.
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PMID:Magnetic resonance imaging of inflammation with a specific selectin-targeted contrast agent. 1579 62

We present a series of 8 patients (6 males, 2 females) with hepatocellular carcinoma (HCC) and glycogen storage disease type Ia (GSD Ia). In this group, the age at which treatment was initiated ranged from birth to 39 years (mean 9.9 years). All patients but one were noncompliant with treatment. Hepatic masses were first detected at an age range of 13-45 years (mean 28.1 years). Age at diagnosis of HCC ranged from 19 to 49 years (mean 36.9 years). Duration between the diagnosis of liver adenomas and the diagnosis of HCC ranged from 0 to 28 years (mean 8.8 years, SD = 11.5). Two patients had positive hepatitis serologies (one hepatitis B, one hepatitis C). Alpha-fetoprotein (AFP) was normal in 6 of the 8 patients. Carcinoembryonic antigen (CEA) was normal in the 5 patients in which it was measured. Current guidelines recommend abdominal ultrasonography with AFP and CEA levels every 3 months once patients develop hepatic lesions. Abdominal CT or MRI is advised when the lesions are large or poorly defined or are growing larger. We question the reliability of AFP and CEA as markers for HCC in GSD Ia. Aggressive interventional management of masses with rapid growth or poorly defined margins may be necessary to prevent the development of HCC in this patient population.
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PMID:Hepatocellular carcinoma in glycogen storage disease type Ia: a case series. 1587 4

We monitored the development of hepatocellular carcinoma due to chronic infection with woodchuck hepatitis virus by using monthly serum samples, physical examination, and magnetic resonance imaging. The same woodchucks can be imaged repeatedly over a 1-y period by allowing the animals to recover after each experiment, thus reducing the number of animals required without compromising the quality of the data obtained. Age- and sex-matched uninfected control (n = 5) and chronically infected (n = 5) woodchucks were group-housed according to sex and infection status. Woodchucks were anaesthetized using an inhalation anesthetic (isoflurane) without premedication. During imaging, we regularly monitored heart rate, body temperature, and respiration. Tumor growth was observed using MRI, whereas the extent of hepatocyte injury was followed using serum liver enzymes. Elevated serum gamma glutamyltransferase and aspartate aminotransferase levels indicated hepatocyte injury due to tumor growth. On magnetic resonance images, the liver should appear as a well-defined, homogenous organ with defined regions of hyperintensities from larger blood vessels. Within tumor nodules, the liver appeared irregularly shaped, having heterogeneous intensity from unregulated cellular proliferation. Changes in tumor size can be monitored by imaging infected woodchucks on a regular basis. Using the imaging techniques we describe, the development of hepatocellular carcinoma can be visualized using magnetic resonance imaging, correlated to serum tests, and compared with the results from uninfected control woodchucks, thereby improving the understanding of the disease progress.
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PMID:Chronic care and monitoring of woodchucks (Marmota monax) during repeated magnetic resonance imaging of the liver. 1654 39

We report a 6-year-old Iranian boy with silvery-gray hair, eyelashes and the eyebrows who was admitted because of seizures and subsequent stupor. He had previous history of acute hemiparesis at 1 year of age and hepatitis-like syndrome 3 months ago. Microscopic examination of the patient's hair shaft revealed different sized clumps of melanin seen in the center of the shafts. Bone marrow aspiration revealed erythroid hyperplasia and erythrophagocytic cells. Bilateral frontal cortical and subcortical high signal lesions, dirty white matter, high signal areas in the upper pons and in both caudates and lentiform nuclei in T2 WI were the brain MRI findings of the patient. He died in the accelerated phase of Griscelli Syndrome (GS) type 2. To our knowledge we report the first case of GS from Iran.
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PMID:Bilateral basal ganglia involvement in a patient with Griscelli syndrome. 1695 71

Gemcitabine is the only cytotoxic agent approved by FDA for the treatment of pancreatic carcinoma. Gemcitabine has a relatively safe profile. Major side effects include bone marrow suppression and flu-like syndrome. Transient abnormalities of liver transaminase enzymes are seen in two third of patients: elevations of alkaline phosphatase and bilirubin are less common, but severe hepatic toxicity is uncommon. Four case reports regarding severe hepatic toxicity of gemcitabine leading to rapid deterioration in patients' health status and death have been reported. We report the fifth case in which liver functions were within normal limits but liver toxicity was preceded by radiological findings on the MRI. We describe a 61-year-old male with stage T4N1M0 who initially received gemcitabine-oxaliplatin (GemOx) regimen was switched to gemcitabine-capecitabine (every two weeks schedule) after four months of therapy due to lack of response. Restaging CT scan after eight-weeks showed new multiple foci of low attenuation resembling simple cysts. MRI of the abdomen was performed which revealed early and active fibrosis. Hepatitis panel were negative. Subsequently the patient developed nausea, vomiting, abdominal pain and weight loss and was referred for palliative radiotherapy. Gemcitabine was discontinued and follow-up CT scan two months later showed stable lesions in the liver. In conclusions, four cases of gemcitabine-induced liver toxicity has been reported in the literature. Such toxicity is manifested by elevated liver transaminases and more common in the presence of liver metastasis. However, our case showed that gemcitabine-induced liver toxicity can be detected by MRI, before liver enzymes start to rise and discontinuation of gemcitabine can prevent further liver toxicity and fibrosis. Report of such cases is encouraged as it will bring awareness among clinicians caring for such patients receiving gemcitabine.
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PMID:Gemcitabine-induced liver fibrosis in a patient with pancreatic cancer. 1762 1

A 36-year-old woman was admitted to our department for close examination of a liver tumor that was found during a medical checkup. Abdominal US, CT and MRI showed a tumor in segment 7 (S7) of the liver. Although imaging suggested hepatocellular carcinoma, laboratory tests showed no abnormality in liver function, hepatitis virus markers were negative, and tumor markers including protein induced by vitamin K absence or antagonist II (PIVKA-II), alpha-fetoprotein (AFP), carbohydrate antigen 19-9 (CA19-9), and carcinoembryonic antigen (CEA) were all within normal ranges. Upon aspiration biopsy of the liver, the histopathological diagnosis was moderately differentiated hepatocellular carcinoma. Therefore, right hepatectomy was performed. Although a part of the tumor was necrotic, about 60% of the viable part showed a clear-cell variant. Consequently, it was diagnosed as clear-cell hepatocellular carcinoma. It was noted that the background liver tissue was normal. This case is worthy of reporting because development of clear-cell hepatocellular carcinoma in the normal liver of a middle-aged woman is rarely seen.
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PMID:Case of clear-cell hepatocellular carcinoma that developed in the normal liver of a middle-aged woman. 1817 75


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