R. K. Dhiman

Review article: the modern management of portal vein thrombosis.

Background  Portal vein thrombosis (PVT) is an important cause of portal hypertension. It may occur as such with or without associated cirrhosis and hepatocellular carcinoma. Information on its management is scanty.

The Clinicopathological Profile of Indian Patients with Nonalcoholic Fatty Liver Disease (NAFLD) is Different from That in the West

There are limited data on nonalcoholic fatty liver disease (NAFLD) from India. The clinicopathological profile of Indian patients with NAFLD may be different from that of Western patients. One hundred NAFLD patients with increased liver enzymes were prospectively evaluated for clinical presentation, associated diseases, overweight/obesity, central obesity (n=54), presence of diabetes mellitus, lipid abnormalities, insulin resistance (n=39), metabolic syndrome (n=54), serum iron, serum ferritin, and transferrin saturation (n=60), and HFE gene mutations (n=30). Risk factors for the grade and stage of the disease on histology were studied in 38 biopsy-proven patients. Patients were treated with lifestyle modifications and ursodeoxycholic acid (UDCA). Seventeen nonresponder patients were treated with metformin. The majority of patients were males (n=70). Twenty percent of patients were overweight, 68% had obesity, and 78% had central obesity. Abnormal cholesterol, HDL, and triglycerides were present in 36%, 66%, and 53% of patients, respectively. Twelve percent of patients had diabetes mellitus and 16% patients had various associated diseases. All 22 (100%) patients studied by ITT and all but 1 (98%) studied by HOMA-IR were found to have reduced insulin sensitivity and 50% were found to have metabolic syndrome by the modified ATP III criteria. Two (3%) patients were found to have high serum iron, 4 (7%) patients had high ferritin, 5 (8%) patients had increased transferrin saturation, and 4 (13%) patients were found to be heterozygotes for H63D HFE gene mutation. Twenty patients of 38 (53%) had histological evidence of NASH (class 3=6, class 4=14). The other 18 (47%) qualified for class I (n=1) or class II (n=17) NAFLD. Four (10.5%) patients had bridging fibrosis and none had evidence of cirrhosis liver. Seventy-four (74%) patients achieved a biochemical response to lifestyle modification and UDCA. All 17 patients treated with metformin had a reduction in ALT level and 10 (59%) of them had normalization of their enzymes. We conclude that the clinicopathological profile of NAFLD in Indian patients is different from that in the West.

Pregnancy outcome in women with autoimmune hepatitis

IntroductionAutoimmune hepatitis (AIH) is a chronic disease of unknown cause, characterized by progressive hepatocellular inflammation and necrosis, frequently leading to cirrhosis. AIH usually affects young women and may influence fertility and pregnancy in them. Nearly 200 pregnancies have been reported so far in the world literature in women with AIH, and no definite guidelines have been established. We hereby report our experience of four pregnancies in women with definite diagnosis of AIH.Materials and methodsWe studied the maternal and perinatal outcome of four women with AIH. The medical history was recorded in detail. The pregnancies were allowed to continue with careful fetal and maternal surveillance. Antenatal complications, disease course during pregnancy, intrapartum maternal fetal events, mode of delivery, and perinatal outcome were noted.ResultsAll four patients had preterm labor: three had vaginal delivery and cesarean was done in one due to breech presentation with preterm rupture of membranes. All the infants were live born with birth weight appropriate for gestational age and were born with good Apgar scores and no congenital malformation. The incidence of post partum hemorrhage was 25% and so was the maternal mortality.ConclusionSuccessful pregnancy outcome in patients with well-controlled AIH seems to be a realistic expectation with careful monitoring jointly by the Obstetricians and Hepatologists, especially in the post partum period. Steroids seem to be the safe alternative as immunosuppressant therapy although azathioprine has also been seen to be generally safe for mother or baby.

Eosinophilic Cholangiopathy—A Case Report

Eosinophilic cholangiopathy is a rare benign disorder of the biliary tract occurring in a setting of peripheral or tissue eosinophilia. It is a broader term and comprises eosinophilic cholecystitis when it involves only the gallbladder (GB) and eosinophilic cholangitis when it involves the bile ducts (1, 2). There are only a few case reports in the literature and most often they describe the combination of GB and bile duct involvement (Table 1). Isolated involvement of bile ducts without involvement of GB is very rare. Eosinophilic cholangiopathy occurs most often in patients with idiopathic hypereosinophilic syndrome, and to our knowledge, there are only two previous reports of bile duct involvement (eosinophilic cholangitis) in patients with eosinophilic gastroenteritis (3, 4). We report a patient who had eosinophilic gastroenteritis with eosinophilic cholangitis.

Acute on chronic liver failure in India: the INASL Consortium experience.

The aim of this study was to analyze etiologies and frequency of hepatic and extrahepatic organ failures (OFs) and outcome of acute‐on‐chronic liver failure (ACLF) at 10 tertiary centers in India.

Functional reconstitution of defective myeloid dendritic cells in chronic hepatitis C infection on successful antiviral treatment.

Poor cellular trafficking and suboptimal T‐cell responses in liver, the hall marks of chronic hepatitis C virus (CHC) infection, might be attributed to defective antigen presentation. Controversy exists regarding role of myeloid dendritic cells (DCs) in CHC and response to antiviral treatment. This study examines functional status of DCs before and after completion of treatment with the aim to find any modulatory effect.

Serum Iron Levels and Hepatic Iron Overload in Patients with Nonalcoholic Steatohepatitis

To the Editor: We read with interest the article by Uraz et al. [1] on the serum iron levels and hepatic iron overload in patients with nonalcoholic hepatitis (NASH) and chronic hepatitis. They studied 25 patients, each with biopsy-proven NASH and chronic viral hepatitis, and found higher serum iron and ferritin levels in patients with chronic viral hepatitis in comparison to those with NASH. Five (20%) patients with chronic viral hepatitis showed positivity for iron on Perls’ Prussian blue staining on liver biopsy compared to none in patients with NASH. We agree with Uraz et al. [1] that patients with nonalcoholic fatty liver disease (NAFLD) do not show evidence of iron overload [2]. We studied iron parameters in 59 patients with NAFLD (38 biopsy proven) (Table 1). Only two (3%) patients had high serum iron (>150 μg/dl), five (8%) had high transferrin saturation (>55%), and four (7%) had high serum ferritin (>250 ng/dl). In contrast to Uraz et al. [1], even though the Perls’ Prussian blue staining for iron on liver biopsy was positive in 10 of 30 (33%) patients in whom this information was available

Pregnancy outcome in Budd Chiari Syndrome-a tertiary care centre experience

BackgroundPregnancy management is a crucial issue in women with Budd-Chiari Syndrome (BCS) and there are no established guidelines on the management.AimTo report our experience of pregnancy outcome with BCS.Material and MethodsWe report outcome of 13 pregnancies in three women, with favourable outcome after the diagnosis of the condition and its treatment using intervention to bypass obstruction and anticoagulant therapy during pregnancy.ResultsThree women had a total of 13 pregnancies; three after the diagnosis and decompressive treatment of the disease. Disease was diagnosed during index pregnancy in two women. Anticoagulation was given in all the three pregnancies (Robertson et al., Br J Haematol, 132:171–196, 2006).Pregnancies prior to diagnosis and treatment resulted in a live birth.ConclusionPregnancy does not seem to be a contraindication in well treated and controlled BCS. Maternal outcome is good with close multidisciplinary surveillance. Foetal outcome, however, may still be poor due to underlying prothrombotic condition.

Primary Leiomyosarcoma of Gallbladder: A Rare Diagnosis

Leiomyosarcoma of the gallbladder is a rare entity, constituting about 1.4 per 1000 gallbladder malignancies. Literature review shows female preponderance in sixth decade of life, due to unknown reasons. We report one such rare case of a 50-year-old female admitted with pain in right upper abdomen. On examination, mass was felt in right hypochondrium. The ultrasound abdomen showed mass with loss of interface with liver and cholelithiasis. CECT abdomen showed polypoidal gallbladder malignancy with ill-defined interface with liver. She was operated upon with diagnosis of carcinoma gallbladder; extended cholecystectomy was done. Histopathological examination revealed spindle-cell proliferation and possibility of malignant tumor of mesenchymal origin was kept. This was later confirmed on immunohistochemistry.

Development of predisposition, injury, response, organ failure model for predicting acute kidney injury in acute on chronic liver failure

There is limited data on predictors of acute kidney injury in acute on chronic liver failure. We developed a PIRO model (Predisposition, Injury, Response, Organ failure) for predicting acute kidney injury in a multicentric cohort of acute on chronic liver failure patients.