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Background: Women with Extra hepatic portal vein obstruction (EHPVO) are mostly young and belong to Asian countries. In the Indian subcontinent, 20–30% variceal bleeds are caused by EHPVO. Hence pregnancy is a concern in such patients. The objective of this study is to observe the maternal and neonatal outcomes in women with EHPVO. Materials and Method: Extra hepatic portal vein obstruction was studied retrospectively in 28 pregnancies in 20 women from Jan 2011 to July 2018 at a tertiary hospital in South India and the pregnancy outcomes were observed during this period. Institutional Review Board approval obtained. Results: The mean age of the women was 24.3 years and the mean age of diagnosis was 18.5 years. Splenomegaly, thrombocytopenia and anaemia were seen in 22 (78.5%), 17 (60.7%) and 8 (28.5%) of pregnancies, respectively. Rate of abortions and preterm deliveries were 2 (7.1%,n=28) and 10 (35.7%,n=28). There was one stillbirth (3.6%) in the study group. EHPVO was diagnosed in 25 (89.3%) women prenatally in our series. During pregnancy only one woman had variceal bleed, which was managed conservatively. Blood and blood product transfusion was required in 7(25%) of women and there was no maternal mortality. Conclusion: Pregnancies in EHPVO have good maternal and neonatal outcomes, provided they are taken care of by a multidisciplinary approach in a tertiary care centre.

The role of vasoactive chemicals in the pathogenesis of hepatopulmonary syndrome (HPS), a disorder characterized by intrapulmonary vascular dilation (IPVD), is only vaguely elucidated. We aimed to study the association between plasma HConsecutive adult patients with cryptogenic cirrhosis were evaluated for IPVD (by contrast echocardiography) and for hypoxemia (by arterial blood gas analysis). Plasma HFifty-eight patients with cryptogenic cirrhosis (male, 45; median age, range, 45, 16-74 years; Child's class; A, 30; B, 18; C, 10) were enrolled in this study. Thirty-four of the 58 (59%) patients had IPVD and 13 (22%) had HPS (mild, 4; moderate, 5; severe, 2; very severe, 2). Plasma HRaised plasma H

Acute fatty liver of pregnancy (AFLP), characterized by hepatic microvesicular steatosis, is a sudden catastrophic illness occurring almost exclusively in the third trimester of pregnancy. Defective fatty acid oxidation in the fetus has been shown to be associated with this disease. Since the placenta has the same genetic makeup as the fetus and as AFLP patients generally recover following delivery, we hypothesized that the placenta might be involved in pathogenesis of this disease. In an animal model of hepatic microvesicular steatosis (using sodium valproate), we found that microvesicular steatosis results in mitochondrial structural alterations and oxidative stress in subcellular organelles of the liver. In placentas from patients with AFLP, we observed placental mitochondrial dysfunction and oxidative stress in subcellular organelles. In addition, defective placental fatty acid oxidation results in accumulation of toxic mediators such as arachidonic acid. Escape of these mediators into the maternal circulation might affect the maternal liver resulting in microvesicular steatosis.

Background & objectives: There are only a few studies on aetiology of portal hypertension among adults presenting to tertiary care centres in India; hence we conducted this study to assess the aetiological reasons for portal hypertension in adult patients attending a tertiary care centre in southern India. Methods: Causes of portal hypertension were studied in consecutive new adult patients with portal hypertension attending department of Hepatatology at a tertiary care centre in south India during July 2009 to July 2010. Results: A total of 583 adult patients (>18 yr old) were enrolled in the study. After non-invasive testing, commonest causes of portal hypertension were cryptogenic chronic liver disease (35%), chronic liver disease due to alcohol (29%), hepatitis B (17%) or hepatitis C (9%). Of the 203 patients with cryptogenic chronic liver disease, 39 had liver biopsy - amongst the latter, idiopathic non cirrhotic intrahepatic portal hypertension (NCIPH) was seen in 16 patients (41%), while five patients had cirrhosis due to non alcoholic fatty liver disease. Fifty six (10%) adult patients with portal hypertension had vascular liver disorders. Predominant causes of portal hypertension in elderly (>60 yrs; n=83) were cryptogenic chronic liver disease (54%) and alcohol related chronic liver disease (16%). Interpretation & conclusions: Cryptogenic chronic liver disease was the commonest cause of portal hypertension in adults, followed by alcohol or hepatitis B related chronic liver disease. Of patients with cryptogenic chronic liver disease who had liver biopsy, NCIPH was the commonest cause identified. Vascular liver disorders caused portal hypertension in 10 per cent of adult patients. Cryptogenic chronic liver disease was also the commonest cause in elderly patients.

To determine the incidence of acute hepatitis because of hepatitis E virus (HEV) and the source of the epidemic in Nellore in south India in 2008.Anti-HEV IgM ELISA and RT-PCR for HEV-RNA were carried out on blood and stool samples from patients with acute hepatitis presenting to different hospitals in the city. The city was divided into 33 clusters, and 20 families from each cluster were systematically interviewed to determine the incidence of hepatitis E in the city. The survey was conducted on 2685 residents of 673 households from 24th November to 4th December 2008.The overall incidence of hepatitis was 5.7% (152/2685), i.e. an estimated 23,915 persons in the city were affected. There were two deaths because of acute hepatitis in the population surveyed translating to an estimated 315 deaths. Men had higher attack rates than women (7.8%vs. 3.5%) and young adults compared to children under 5 years (6.9%vs. 2.9%). Families drinking water from the pumping station at Bujjamarevu had the highest attack rate of 54.5% (39.8-69.2%). HEV IgM antibodies were present in 80/100 plasma samples tested. HEV-RNA was detected in 43/100 individuals tested, and isolates were characterized as genotype 1 by sequencing.Sewage draining into the river close to the pumping stations and broken pipelines crossing sewage drains may have triggered this large outbreak.

Mannose binding lectin (MBL) is an important innate immune system pattern recognition molecule. The MBL gene polymorphisms are reported to play a crucial role in outcome of hepatitis B virus (HBV) infection. In this study, we ascertained the association of MBL genotypes with HBV outcome in a South Indian population. The MBL gene polymorphisms at codons 52, 54 and 57 of exon I, and promoter polymorphisms at -221 were typed by polymerase chain reaction-sequence specific primer in spontaneously recovered and in chronic HBV group. The allele frequency of codon 52 'C' was significantly higher in chronic HBV group than in the recovered group (98.5% vs. 93.6%; P = 0.003) and codon 52 'T' was significantly higher in recovered group than in the chronic group (6.4% vs. 1.5%; P = 0.003). In multivariate analysis, after adjusting for age, sex and state of origin, codon 52 'CC' and 'CT' genotypes were significantly associated with chronicity and recovery respectively [odds ratio (OR), 0.25; 95% confidence interval (CI), 0.08-0.80, P = 0.02] in co-dominant analyzing models. This was re-affirmed in analysis performed exclusively on Tamil Nadu subjects (OR, 0.23; 95% CI, 0.06-0.93, P = 0.039). The frequency of low/none haplotype (XY/O) was significantly higher in recovered group than in chronic group (15.6% vs 7.5%) and associated with spontaneous recovery (OR, 2.28; 95% CI, 1.04-4.99, P = 0.035). Our results provide preliminary evidence that inheritance of codon 52 genotypes and XY/O haplotype associated with low MBL level substantially determine the outcome of HBV infection in a sympatrically isolated South Indian population.

To analyze ATP7B mutations in Wilson's disease (WD) patients from the Indian subcontinent and to correlate these with WD phenotype.We studied 27 WD patients from 25 unrelated families. Twenty-two families were from three southern Indian states - Tamil Nadu andhra Pradesh and Kerala. We applied conformation- sensitive gel electrophoresis (CSGE) to screen for the mutations in patients and their families. PCR products exhibiting aberrant patterns in CSGE were subjected to direct DNA sequencing. As siblings affected by WD within a family share identical ATP7B genotype, we compared WD phenotype among affected siblings within families.ATP7B mutations were detected in 22 of the 25 probands -13 were homozygotes and 9 were compound heterozygotes. Eleven novel mutations were detected. Only two common mutations were found: G3182A in 4 (16%) and C813A in 3 (12%) probands. 'Hot spots' for ATP7B mutations were exons 18 and 13. Lack of common dominant mutations prevented correlation of individual ATP7B mutations with WD phenotype. Symptomatic WD in a live sibling was not found in any family. In 8 families, a sibling died of presumed WD - in 6 of these, WD phenotype was identical to that in the proband.We describe the spectrum of ATP7B mutations including 11 novel mutations in Indian WD patients and document lack of a single dominant mutation. Identical WD phenotype among siblings in only 6 of 8 families with1 child affected by WD suggests that factors other than ATP7B mutations influence WD phenotype.

The relationship between hepatocyte expression of hepatitis B virus (HBV) antigens, liver histology and viral replication in asymptomatic subjects with incidental detection of hepatitis B surface antigen (HBsAg) remains unclear. We evaluated the histological activity index (HAI) and hepatocyte expression of viral antigens with replicative status in asymptomatic chronic HBV infection.Asymptomatic subjects with incidental detection of HBsAg and ALT levels less than twice the upper limit of normal were grouped as follows: Group A - negative for HBeAg and HBV DNA (no HBV replication); B - HBeAg negative, HBV DNA positive (low HBV replication or pre-core mutant); C - positive HBeAg and HBV DNA (high viral replication). Liver biopsies were assessed for HAI (Ishak's scoring system). These were also subjected to immunohistochemistry for expression of HBsAg and hepatitis B core antigen (HBcAg); distribution, staining pattern and quantitative measurement of antigen expression were assessed.Median HAI was similar in the three groups (1.0, 2.0 and 2.0 in groups A, B and C, respectively). All subjects in Group C showed discrete cytoplasmic expression of HBsAg, whereas the other two groups showed heterogeneity in distribution and pattern of HBsAg staining. Quantitative measurement of cytoplasmic HBsAg revealed similar results in the three groups. Core antigen (nuclear) was detected in 4 of 5 subjects in Group C and none of those in Groups A and B. Ground-glass hepatocytes were seen in 20 and orcein-positive cells in 26 cases. HBsAg was detected by immunohistochemistry in 37 biopsies.Among asymptomatic subjects with chronic HBV infection, those with high rate of viral replication had discrete cytoplasmic HBsAg expression and nuclear expression of core antigen; these findings were uncommon in subjects with low or no viral replication.

Spontaneous rupture of amyloid liver is a fatal complication. A 48-year-old man with systemic amyloidosis secondary to multiple myeloma presented with acute hemoperitoneum. Emergency angiogram showed extravasation of contrast from the liver into the sub-hepatic space, which was successfully stopped by embolization of the right hepatic artery.

Penicillamine is the standard therapy for Wilson's disease in children. We report an 8-year-old-girl with liver disease due to Wilson's disease who developed extrapyramidal symptoms following administration of penicillamine. Symptoms resolved within 20 hours of stopping the drug but recurred within 24 hours when gradually increasing small doses were recommenced.

Intussusception is the most common cause of intestinal obstruction in young children and has been reported as a complication of a recently withdrawn tetravalent reassortant rotavirus vaccine.We studied the history, clinical presentation, management and outcome of intussusception presenting to a tertiary care hospital in southern India over a 10-year period, in order to assess potential association with diarrheal disease and immunization.Data from 137 index cases and 280 control subjects indicated that the risk of diarrheal disease or oral polio vaccine administration in the month prior to presentation was similar in the index cases and controls. Mean time to presentation to hospital after developing symptoms was 1.8 days, and 77.3% of patients required surgery, with 47.4% undergoing intestinal resection. Mortality was 0.006%.No association could be demonstrated between gastroenteritis or oral poliovirus vaccine immunization and intussusception in southern Indian children. These children presented later and required operative intervention more frequently than has been reported in other studies, but had a good outcome with low mortality.

Acute fatty liver of pregnancy is an uncommon, potentially fatal disorder. Between 1998 and 2000, two patients with acute fatty liver of pregnancy presented at the Christian Medical College Hospital, Vellore. Both patients were in the thirty-sixth week of pregnancy. jaundice and encephalopathy were the predominant symptoms. Both the mothers died after they delivered a stillborn Infant each. The maternal deaths were due to multiorgan failure and/or postpartum haemorrhage and sepsis. The route of delivery was vaginal in both the patients. Extrahepatic and metabolic complications in both cases Included renal failure, sepsis, hypoglycaemia, disseminated intravascular coagulation and gastrointestinal bleeding. Liver biopsy done in both patients was consistent with the diagnosis of acute fatty liver of pregnancy. To the best of our knowledge, this is the first report from India on acute fatty liver of pregnancy.

Zygomycosis is an infection due to the fungi Zygomycetes, which, despite being ubiquitous, rarely cause disease in humans. Predisposing conditions include diabetic ketoacidosis, hematologic malignancies, iron overload, multiorgan failure, chronic liver disease, renal failure, metabolic acidosis and immunosuppressed state [1–3]. Zygomycosis can rarely occur in pregnancy as a complication of diabetic ketoacidosis [1]. We report zygomycosis occurring in a non-diabetic woman with pregnancy—related liver disease and multi-organ failure. A 20-year-old woman presented to our center at 38 weeks of gestation (primigravida) with history of swelling of feet and fatigue for 10 days, breathlessness, and jaundice (3 days), and abdominal pain (1 day). She had no prior major illness. She had not taken any drug known to cause liver dysfunction. On presentation, she was conscious and oriented, her blood pressure was 140/90 mmHg, pulse rate was 124/min and respiratory rate was 36/min. She was icteric and pale. Abdominal examination showed a gravid uterus; there was no ascites or palpable mass. Urinalysis revealed proteinuria and pelvic ultrasound showed intrauterine death of the fetus. Her laboratory results were: hemoglobin 13 gm%, white cell count 12,800/cmm, platelet count 56,000/cmm, serum bilirubin 16.6 mg/dL, alanine transaminase 130 IU/L, creatinine 3.4 mg/dL, random blood sugar 160 mg/dL, lactate dehydrogenase 892 IU/L, and INR 4.2. Arterial blood gas analysis showed pH 7.2, lactate 7.3 mmol/L, anion gap 12.6 and, base excess −17.5. Etiological work up for liver dysfunction like peripheral smear for malaria and serology for hepatitis viruses (hepatitis A, B, C and E) was non-contributory. Her blood culture was sterile. A diagnosis of pregnancy—related liver dysfunction [Diagnostic criteria for acute fatty liver of pregnancy (AFLP) and pre-eclamptic liver dysfunction and hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome were met] with intra-uterine death was made. She was started on piperacillin with tazobactam and metronidazole intravenously. After a failed attempt to induce vaginal delivery, she underwent emergency cesarean section under blood product coverage 22 h after admission to our hospital. The baby delivered was a macerated stillbirth. There was 750 mL of ascites drained and 500 mL of blood loss during the surgery. She was maintained on the same antibiotics in intensive care unit and was on mechanical ventilation with other supportive measures. Hemodialysis was instituted, with which acidosis resolved partially (pH 7.35), but she had persistent lactic acidemia (lactate >4 mmol/L) with worsening liver functions and recurrent hypoglycemia. A. Goel (*) :U. Zachariah : C. E. Eapen Department of GI Sciences, Christian Medical College, Vellore 632 004, India e-mail: drashish_goel@yahoo.com

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.

To investigate the occurrence of silent hepatitis B virus (HBV) infection among patients with chronic liver disease (CLD).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.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.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.

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.

Scoring systems to predict mortality in intensive care units have been developed in western populations. There is a need to identify and validate prognostic variables in the Indian context. We compared two scoring systems to predict the discharge outcome in patients admitted to a medical intensive care unit.Five hundred patients admitted to a medical intensive care unit were studied prospectively. Modified acute physiology and chronic health evaluation II (APACHE II) score and modified organ system failure (OSF) score were applied on the day of admission to the intensive care unit. The scores obtained by the two systems were compared using the area under the curve approach. The likelihood ratios were calculated for predicting discharge outcome.The modified OSF score predicted discharge outcome better than the modified APACHE II score--receiver operating characteristic curve area (standard error-area) 0.7062 (0.0244) and 0.6068 (0.0267) for the modified OSF and the modified APACHE II scores, respectively. This was statistically significant (p0.001). The likelihood ratio for the modified OSF score for different cut-off points varied from 0.27 to 5.49, while the likelihood ratio for the modified APACHE II score varied from 0.11 to 2.08. This means that for an intensive care unit with a 30% overall mortality, the modified OSF score could separate patients with 10% to 70% mortality, while the modified APACHE II score could predict only 5% to 47% mortality.The modified OSF score was superior to the modified APACHE II score in predicting mortality in patients admitted to the medical intensive care unit.