Autoimmune hepatitis.
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Biomedical subjects
Publications and source records attributed to C E Eapen.
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BACKGROUND: We report our experience with management of patients with Budd Chiari syndrome over the past two decades. In 1996 we described a novel approach involving recanalisation of hepatic veins by combined percutaneous and transvenous approaches. This was incorporated into an algorithm published in 1999 in which our preferred treatment for all cases of Budd Chiari syndrome with short segment occlusion or stenosis of the hepatic veins involves recanalisation of the hepatic veins by transvenous or combined percutaneous-transvenous approaches. In symptomatic Budd Chiari syndrome where recanalisation is not possible, we perform transjugular intrahepatic portosystemic shunts (TIPS) because TIPS decompresses the portal circulation directly in an adjustable way. In this series of patients with Budd Chiari syndrome treated with radiological interventions alone, we assess their medium term outcome using two independent objective prognostic indices. METHODS: We retrospectively studied 61 patients with non-malignant Budd Chiari syndrome treated by radiological intervention alone in our centre. RESULTS: Actuarial survival for the entire cohort at one year and five years was 94% and 87%, respectively. Survival of our patients with mild disease (according to the Murad classification) was 100% at one year and at five years, with intermediate disease severity 94% at one year and 86% at five years, and with severe disease 85% at one year and 77% at five years. CONCLUSION: Management of Budd Chiari syndrome by interventional radiology resulted in excellent medium term survival for patients in all categories of disease severity.
Progressive hepatocellular dysfunction in a neonate, resulting in elevated serum alpha-fetoprotein together with raised blood levels of tyrosine and methionine, a generalized amino aciduria and the absence of urinary delta-aminolevulinic acid and succinylacetone, suggests a diagnosis of tyrosinemia type Ib. Classical tyrosinemia type I arises from a deficiency of fumarylacetoacetate hydrolase while the variant tyrosinemia type Ib results from a deficiency of maleylacetoacetate isomerase.
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BACKGROUND AND AIMS: The adverse effect of acute hepatitis A in chronic liver disease is well known. The outcome of acute hepatitis E in chronic liver disease has not been extensively studied. The present study aimed to examine the clinical profile and outcome of patients with chronic liver disease and hepatitis E virus (HEV) superinfection, and the seroprevalence of hepatitis A and E infections in patients with chronic liver disease and controls in India. METHODS: A retrospective study of patients with chronic liver disease and acute icteric hepatitis E was performed. Acute hepatitis E was diagnosed by immunoglobulin (Ig)M ELISA. Seroprevalence studies were carried out using IgG ELISA in 100 patients with chronic liver disease and 79 age- and sex-matched controls. RESULTS: From June 2001 to December 2002, nine patients with chronic liver disease were found to have superinfection with HEV. Out of these, six patients died of advanced liver failure. The etiology of liver disease was Wilson's disease in six, hepatitis B virus in one, autoimmune in one and cryptogenic in one case. The seroprevalence of hepatitis A was 99 and 100% and 56 and 21% for HEV in cases and controls, respectively. CONCLUSIONS: Acute HEV in patients with chronic liver disease has a grave prognosis. Wilson's disease was the most common cause of chronic liver disease complicated by acute HEV. Seroprevalence studies showed that 44% of patients with chronic liver disease were at risk of developing hepatitis E. Hepatitis E vaccine, when available, is indicated for use in this group.
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We present three siblings (out of four) with intrahepatic cholestatic disease and cirrhosis. Two of the siblings, a 33-year-old woman and a 34-year-old man had advanced liver disease- with the liver histology showing established cirrhosis with chronic cholestasis and excess copper accumulation. Both died two years later due to hepatic encephalopathy. The third sibling, a 37-year-old man on routine check-up was found to have abnormal liver functions. The liver biopsy showed marked bile ductular proliferation with bridging fibrosis, reduction in interlobular bile ducts, and excess copper accumulation. The presence of antimitochondrial antibody in the serum in 1 in 320 dilutions in all three patients and 1 in 80 dilutions in the oldest healthy sibling and hypergammaglobulinemia in all the siblings confirmed the diagnosis of familial primary biliary cirrhosis. Antinuclear and smooth muscle antibodies were not present. Clinical and biochemical improvement has been noted in the third sibling after therapy with ursodeoxycholic acid.
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BACKGROUND: Cancer cells have alterations in energy metabolism due to defective mitochondrial function. This may be due to generation of excessive free radicals and/or defective antioxidant enzyme systems. The aim of the present study was to assess mitochondrial function and antioxidant defences in the gastric mucosa of patients with gastric carcinoma (CA). METHODS: Gastric mucosal mitochondrial function was assessed by means of the reduction of tetrazolium dye (MTT), and levels of antioxidants such as glutathione S-transferase (GST), catalase, superoxide dismutase (SOD), and thiols were measured in biopsy specimens taken from the tumour mucosa (TM) and tumour-free (TF) mucosa, 2 cm away from the tumour, in 49 patients with gastric CA and compared with that in 54 controls. In a further 10 patients with gastric CA, these studies were done on TM and TF mucosa 2 cm and > or = 5 cm away from the tumour. In 10 patients and 5 controls, specimens were obtained for electron microscopy as well. Helicobacter pylori infection was diagnosed by means of histology. RESULTS: MTT reduction and GST and SOD activities were significantly decreased in TM and TF mucosa in patients with CA compared with controls (P < 0.01). The levels of thiols and catalase activity were significantly increased in CA as compared with controls (P < 0.01). H. pylori positivity did not influence most of these variables but did give significant decrease in MTT reduction in CA (TF) mucosa (P=0.01) and significant increase in thiol levels in CA (TM) mucosa (P=0.04). Electron microscopy showed mitochondrial alterations in tumour cells in all patients and in adjacent mucosa of 10%-50% of the cells. CONCLUSIONS: 1) In gastric CA the cancer mucosal cells and the non-involved cells adjacent to the tumour have defective mitochondrial function, which may be due to altered antioxidant defences and possibly altered free radical formation. 2) Ultrastructural mitochondrial abnormalities are shown to parallel these biochemical abnormalities.
Vesical varices in portal hypertension are rare. We report a patient with portal hypertension who developed recurrent painless hematuria. Cystoscopy was normal. Doppler ultrasound and MR angiography showed a dilated paraumbilical vein within the falciform ligament coursing down to the urinary bladder wall and draining into the right internal iliac vein. He underwent liver transplantation for decompensated chronic liver disease. He is in good health and has not had further episodes of hematuria.
We report a 35-year-old man, a renal allograft recipient, who presented with toxic megacolon. Segmental biopsies from the colon were consistent with cytomegalovirus colitis. Serum polymerase chain reaction for cytomegalovirus DNA confirmed the diagnosis. He was treated with ganciclovir but, though his abdominal condition improved initially, he worsened later and succumbed to his illness.
OBJECTIVE: To investigate the occurrence of silent hepatitis B virus (HBV) infection among patients with chronic liver disease (CLD). METHODS: Plasma samples from 71 CLD patients including 9 HBsAg-positive individuals were tested for HBV DNA by nested polymerase chain reaction (nPCR), and for HBV serum markers, i.e., anti-HBc antibody, HBeAg and anti-HBe antibody. The individuals were also tested for hepatitis C virus (HCV) RNA and anti-HCV antibody. RESULTS: Among 62 HBsAg-negative patients, silent HBV infection was seen in only two (3.2%). Silent HBV infection was not found in any of the 26 patients who had evidence of HCV infection. One HBsAg-positive patient was positive for anti-HCV in the absence of HCV RNA. CONCLUSIONS: There is a low rate of silent HBV infection among patients with CLD in India, where HBV is moderately endemic. Silent HBV infection is not associated with HCV-related CLD, which is in contrast to reports from other HBV-endemic areas in Asia.