High Prevalence of Hormonal Changes and Hepatic Osteodystrophy in Frail Patients with Cirrhosis-An Observational Study.

Background/Aim: Hormonal changes and hepatic osteodystrophy are less often studied complications of cirrhosis. This study describes the variance in hormones and osteodystrophy between Frail and Not frail patients with cirrhosis. Methods: 116 outpatients with cirrhosis were prospectively enrolled in this study. Frailty assessment was done using Liver Frailty Index (LFI). Sociodemographic assessment, anthropometry, nutritional assessment, hormone profile, and dual-energy X-ray absorptiometry scan were done in all patients. Results: 116 patients, predominantly males (100 (86.2%) with mean age of 50.16 years (95% CI, 48.43-51.89) were included. Malnutrition was more common in Frail group as compared to Not frail group. Subjective global assessment (SGA) class-B patients were significantly more in Frail group (37 (74%) vs 3 (4.5%), P = 0.001). The prevalence of lower parathyroid hormone (PTH) (14 (28%) vs 2 (3%)), testosterone (33 (66%) vs 15 (22.7%)), vitamin D3 (44 (88%) vs 39 (59.1%)), and cortisol (37 (74%) vs 37 (56.1) levels was higher in Frail group (P < 0.05). The number of patients diagnosed with osteodystrophy (34 (68%) vs 21 (31.8%), P = 0.001) was significantly higher in Frail group. The marker of osteoclastic activity, ß-cross laps, was significantly elevated in the Frail group both in males (736 (655-818) vs 380 (329-432), P = 0.001) and (females 619 (479-758) vs 313 (83-543), P = 0.02). Bone mineral density (BMD) at lumbar spine (LS) and neck of femur (NF) had significant correlation with LFI (ρ = 0.60, P = 0.001 for LS and ρ = 0.59, P = 0.001 for NF), serum testosterone (ρ = 0.58, P = 0.001 for LS and ρ = 0.53, P = 0.001 for NF), ß-cross laps (ρ = 0.38, P = 0.001for LS and ρ = 0.35, P = 0.000 for NF), vitamin D3 (ρ = 0.23, P = 0.04 for LS and ρ = 0.25, P = 0.01 for NF), PTH (ρ = 0.52, P = 0.001 for LS and ρ = 0.48. P = 0.001 for NF), and cortisol (ρ = 0.50, P = 0.001 for LS and ρ = 0.45, P = 0.001 for NF) levels. Conclusion: This is the first study that highlights the high prevalence of hormonal changes and hepatic osteodystrophy in frail patients with cirrhosis and opens a new dimension for research and target of therapy in this field.

Metabolic syndrome and female gender, but not methotrexate, are the important associations of significant liver fibrosis in patients with moderate-to-severe psoriasis as detected by transient elastography.

BACKGROUND: Many international guidelines on psoriasis management have emphasized upon the need to identify risk factors for liver fibrosis and that the risk may be increased after a certain total cumulative dose of methotrexate. METHODS: Consecutive patients with moderate-to-severe psoriasis were assessed for liver fibrosis using transient elastography and noninvasive scores. Based on the presence of significant liver fibrosis, the Odds ratio associated with various factors was calculated using logistic regression analysis. Receiver operating characteristic curves were calculated to find maximal cutoff values of noninvasive tests to detect fibrosis. RESULTS: In this cross-sectional study, 134 patients completed the study. Significant fibrosis (liver stiffness measurement ≥7, corresponding to F2 fibrosis or higher) was seen in 33 (24.6%) patients. Neither methotrexate exposure nor total cumulative dose of ≥1.5 was associated with significant fibrosis. Female sex (P = 0.024) and the presence of metabolic syndrome (P = 0.034) were the two variables associated with significant liver fibrosis. On logistic regression analysis, the odds ratio for the female gender and metabolic syndrome was estimated to be 2.51 (95% confidence interval - 1.09-5.81) and 2.33 (95% confidence interval - 1.03-5.27), respectively. Aspartate transaminase to platelet ratio index, nonalcoholic fatty liver disease score and the fibrosis-4 index had low sensitivity in comparison to transient elastography. LIMITATIONS: These included small sample size, small number of patients with a total cumulative methotrexate dose of >3-4.5 g, and lack of control group consisting of healthy persons. Another is the absence of liver biopsies considered as the gold standard in the diagnosis of liver fibrosis. CONCLUSIONS: Metabolic syndrome and female sex are associated with the development of significant liver fibrosis in patients with psoriasis. Methotrexate exposure does not seem to be significantly associated with significant liver fibrosis.

Incidental Detection of Urinary Bladder Herniation in 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Mimicking as Metastatic Deposit in the Inguinal Canal.

Although 18F-fluorodeoxyglucose (FDG) is the most extensively used tracer in oncological positron emission tomography/computed tomography (PET/CT) studies, various physiological as well as benign pathological conditions are known to cause false-positive results. This report describes 18F-FDG PET/CT done in an elderly man with primary hepatocellular carcinoma, revealing a metastasis mimicking lesion in the left inguinal canal, which was identified as the herniated portion of the urinary bladder. Though rare, bladder herniation, especially with a narrow neck, can be a pitfall in the evaluation for metastatic disease. The study also highlights the utility of delayed imaging in the evaluation of pelvic pathology.

Detection of Hepatic Encephalopathy on 18F-FDG PET/CT Brain Images in a Patient With Decompensated Liver Cirrhosis.

We present a case of decompensated liver cirrhosis with ascites, which had history of asterixis, impaired balance with swaying gait along with mild irritability since 1 month. F-fluorodeoxyglucose PET/CT (FDG-PET/CT) performed to rule out malignancy did not reveal any abnormal FDG avid lesion suspicious for malignancy but showed hypermetabolism in the bilateral basal ganglia and thalamus with reduced metabolism in cerebral cortices and cerebellum, suggesting hepatic encephalopathy.

Drug idiosyncrasy due to pirfenidone presenting as acute liver failure: Case report and mini-review of the literature.

Idiosyncratic drug-induced liver injury (DILI) is ranked among the top most common etiologies of acute liver failure (ALF). It carries poor transplant-free survival. Pirfenidone is an anti-inflammatory and antifibrotic drug that is commonly used for the treatment of idiopathic pulmonary fibrosis (IPF). Hepatotoxicity due to pirfenidone is rare and generally manifests as a mild rise in serum aminotransferases. In this mini-review, we report an unusual case of idiosyncratic DILI due to pirfenidone presenting as ALF, with emphasis on the definition, classification, diagnostic criteria, histopathology, molecular markers, and treatment options for DILI and related ALF. A 77-year-old man with known Parkinson's disease and IPF presented with jaundice for 7 days and altered mental status for 4 days. His long-term medications included a levodopa/carbidopa combination with a recent addition of pirfenidone over the previous 1 month; there was no monitoring of liver function tests. The evaluation suggested features of acute liver failure with grade III hepatic encephalopathy, acute kidney injury, and metabolic acidosis. The diagnostic workup ruled out viral, toxic, ischemic, and other etiologies for acute liver failure. Based on a Roussel Uclaf Causality Assessment Method score of 7 and possible DILI-ALF, pirfenidone was withdrawn. He was evaluated for liver transplantation but was declined. Despite all supportive measures in intensive care, organ failure progressed and he succumbed to the illness on day 4. Postmortem liver biopsy revealed findings consistent with DILI (final Roussel Uclaf Causality Assessment score, 10). Conclusion: DILI-ALF carries poor prognosis, and liver transplantation should be considered early in the course. Characterization, reporting, monitoring, and labeling of pirfenidone-related hepatotoxicity is vital given its common use in IPF. (Hepatology Communications 2018;2:142-147).

Low-Dose Sofosbuvir Is Safe and Effective in Treating Chronic Hepatitis C in Patients with Severe Renal Impairment or End-Stage Renal Disease.

BACKGROUND AND AIMS: There is sparse data on the use of Sofosbuvir based directly acting antiviral (DAA) drug regimens in chronic hepatitis C (CHC) patients with chronic kidney disease (CKD) with estimated glomerular filtration rate (eGFR) less than 30 mL/min/1.73 m2. We evaluated the safety and efficacy of low-dose Sofosbuvir plus full-dose Daclatasvir in CHC patients with CKD. METHODS: Sixty-five CHC patients with CKD with eGFR less than 30 mL/min/1.73 m2 [54 (83%) patients with ESRD on hemodialysis] were included. All patients irrespective of genotype were treated with half-dose Sofosbuvir [200 mg (half tablet of 400 mg)] plus full-dose Daclatasvir (60 mg) given daily for either 12 or 24 weeks given in patients with genotype 3 cirrhosis. The efficacy was assessed by the sustained virological response (SVR12) with negative HCV RNA 12 weeks after the end of treatment (ETR). RESULTS: The median HCV RNA level in 65 patients (Males 40, mean age 42.9 ± 13 years) was 1.65 × 106 (1.2 × 103-1.73 × 108) IU/mL with 42 (64.6%) patients having HCV genotype 1, followed by genotype 3 and 2 in 22 (34%) and 1 (1.4%) patients, respectively. Twenty-one (32%) patients had evidence of cirrhosis, and ten (15.4%) patients were treatment experienced. Sixty-four (98.5%) patients achieved ETR, and 65 (100%) patients attained SVR12. All patients tolerated the DAAs well with none of the patients reporting any serious adverse events. Minor side effects noted were nausea seen in five (7.7%) patients, insomnia and headache in four (6.2%) patients each, and pruritus in one (1.5%) patient. CONCLUSION: Low-dose Sofosbuvir and full-dose Daclatasvir are safe and effective in treating CHC in patients with CKD with eGFR less than 30 mL/min/1.73 m2.

Comparison of Contrast Enhanced Ultrasound With Contrast Enhanced Computed Tomography for the Diagnosis of Hepatocellular Carcinoma.

BACKGROUND/AIMS: The most common primary malignant tumor of liver is hepatocellular carcinoma (HCC). The highest risk of developing HCC is seen in patients of cirrhosis. Ultrasound is used for surveillance in these patients. This study evaluates the role of contrast enhanced ultrasound (CEUS) in the diagnosis of HCC and compares CEUS to contrast enhanced computed tomography (CECT). MATERIALS AND METHODS: This prospective study included 22 patients with cirrhosis and suspected to have HCC on the basis of gray scale ultrasound or elevated Alpha-fetoprotein. Multiphasic CECT and CEUS were done. On both CECT and CEUS, arterial phase enhancement patterns of the lesions were classified as heterogeneously hyperenhancing, homogeneously hyperenhancing, isoenhancing or nonenhancing. The enhancement patterns of the lesions in portal venous phase were classified as hyperenhancing, isoenhancing, washout or nonenhancing. Presence or absence of neovascularity and peripheral capsule were also noted. The diagnosis of HCC was made as per American Association for the Study of Liver Diseases (AASLD) guidelines. RESULTS: There was moderate degree of agreement between the two modalities in characterizing the enhancement pattern in arterial phase, as calculated by using kappa test (k = 0.59, P < 0.05). Substantial agreement between them, for demonstrating the neovascularity, was also seen (k = 0.772, P < 0.05). CEUS was found to be superior to CECT in demonstrating portal venous phase wash out and peripheral capsule. Only fair agreement was seen between them, with kappa value for portal venous washout being k = 0.38 (P < 0.05) and for peripheral capsule being k = 0.328 (P < 0.05). CONCLUSION: CEUS is comparable to CECT in demonstrating the arterial phase enhancement pattern of HCC and the neovascularity. CEUS was found to be better than CECT in demonstrating the portal venous phase washout and peripheral capsule.

Noninvasive Assessment of Liver Fibrosis By Transient Elastography and FIB4/APRI for Prediction of Treatment Response in Chronic Hepatitis C-An Experience from a Tertiary Care Hospital.

BACKGROUND: Liver fibrosis and its sequel cirrhosis represent a major health care burden, and assessment of fibrosis by biopsy is gradually being replaced by noninvasive methods. In clinical practice, the determination of fibrosis stage is important, since patients with advanced fibrosis have faster progression to cirrhosis and antiviral therapy is indicated in these patients. AIMS: To assess the role of transient elastography (TE) and compare it with APRI and FIB4 for predicting liver fibrosis and assessing the effect of host and viral factors on fibrosis and treatment outcome in CHC patients. METHODS: In a retrospective analysis, 330 CHC patients underwent liver stiffness measurement (LSM) by TE and tests needed for calculating APRI and FIB4 scores at baseline. 228 patients received a combination of Pegylated IFN-based antiviral therapy and were analyzed for therapeutic response. RESULTS: The study included 330 patients (median age 39 years [range 18-67]), predominantly males (n = 227, 68.8%) with baseline LSMs. The median liver stiffness was 7.8 kPa (range 3.2-69.1 kPa). LSMs and its thresholds for severe fibrosis progression (≥9.5 kPa) and cirrhosis (≥12.5 kPa) were significantly higher in patients with age ≥40 years, diabetes mellitus, and patients with significant alcohol intake (P = 0.003 to P < 0.001). By taking TE as a reference, the diagnostic accuracy of FIB4 scores for predicting cirrhosis (AUROC 0.896) was good (+LR 13.4) compared to APRI (AUROC 0.823) with moderate likelihood ratio (+LR 6.9). Among 228 treated patients the SVR rate in genotype 3 was 70% versus 57.8% in genotype 1. Fibrosis score F4 (P = 0.023) and HCV genotype (P = 0.008) were independent predictors of SVR. CONCLUSION: The study shows that LSM by TE and fibrosis assessment by FIB4/APRI scores can be used with fair reliability to predict fibrosis and treatment response in patients with CHC infection.

Angiogenic and anti-angiogenic factor gene transcript level quantitation by quantitative real time PCR in patients with hepatocellular carcinoma.

Tumor angiogenesis, a major requirement for tumor growth and metastasis, is regulated by pro- and anti-angiogenic factors. The aim of this study was to quantify the expression of angiogenic (VEGF, HIF-1α, Angiopiotein-2) and anti-angiogenic (endostatin, angiostatin and Thrombospondin-1) factors and to discern their clinical relevance. A total 90 patients (67 HCC, 9 cirrhosis and 14 chronic hepatitis) were enrolled in the study. Tissue transcript levels of angiogenic (VEGF, HIF-1α, Ang-2) and anti-angiogenic (endostatin, angiostatin and TSP-1) factors were analyzed by quantitative real time-polymerase chain reaction (qRT-PCR) in the tissue samples. The tissue transcript levels of VEGF, HIF-1α and endostatin were found to be significantly higher in HCC in comparison to cirrhosis and chronic hepatitis. Although Ang-2, angiostatin and TSP-1 tissue transcript levels were higher in HCC group than the others groups but the difference was not statistically significant. In univariate analysis both VEGF and HIF-1α were found to be associated with poor survival of HCC patients. Multivariate analysis by the cox proportional hazard model revealed only VEGF as an independent factor predicting poor survival of the HCC patients. Angiogenic and anti-angiogenic factors are all highly expressed in HCC patients. Upregulation of tissue anti-angiogenic factors indicates the urgency for the alternative of anti-angiogenic therapies.

Serum levels of angiogenic and anti-angiogenic factors: their prognostic relevance in locally advanced hepatocellular carcinoma.

Hepatocellular carcinoma (HCC) is a prototype tumor wherein angiogenesis plays a vital role in its progression. The role of VEGF, a major angiogenic factor in HCC is known; however, the role of anti-angiogenic factors simultaneously with the angiogenic factors has not been studied before. Hence, in this study, the serum levels of major angiogenic [Vascular Endothelial Growth Factor (VEGF), angiopoietin-2 (Ang-2)] and anti-angiogenic (endostatin, angiostatin) factors were analyzed and correlated with clinico-radiological features and with outcome. A total of 150 patients (50 HCC, 50 cirrhosis and 50 chronic hepatitis) and 50 healthy controls were enrolled in this study. Serum levels of VEGF, Ang-2, endostatin, and angiostatin were estimated by enzyme-linked immunosorbent assay. HCC shows significantly elevated serum levels of angiogenic factors VEGF and Ang-2 and of anti-angiogenic factors endostatin and angiostatin. ROC curve analysis for serum VEGF yielded an optimal cut-off value of 225.14 pg/ml, with a sensitivity of 78 % and specificity of 84.7 % for a diagnosis of HCC and its distinction from other group. Using this value, the univariate and multivariate analysis revealed significantly poor outcome in patients with higher levels of serum VEGF (p = 0.009). Combinatorial analysis revealed that patients with higher levels of both angiogenic and anti-angiogenic factors showed poor outcome. Serum VEGF correlates with poor survival of HCC patients and, therefore, serves as a non-invasive biomarker of poor prognosis. Moreover, elevated levels of anti-angiogenic factors occur endogenously in HCC patients.

Hepatic arteriovenous fistulae: role of interventional radiology.

INTRODUCTION: Hepatic arterial venous fistulae are abnormal communications between the hepatic artery and portal or hepatic vein and commonly occur either secondary to iatrogenic causes like liver biopsy, transhepatic biliary drainage, transhepatic cholangiogram and surgery, or following mechanical insult like blunt or penetrating trauma. Congenital fistulae are rare. Treatment is warranted as an emergency management or in the development of portal hypertension/heart failure in chronic cases. Both surgical and endovascular occlusion of the fistula can be attempted with the latter carrying low intra and post-procedure morbidity. Endovascular treatment has thus currently emerged as a minimally invasive reliable treatment option in such individuals. METHODS AND RESULTS: We describe a short series consisting of four cases of acquired hepatic arterioportal/venous fistulae, which were referred to interventional radiology for endovascular management over the last 2 years. Three patients had arterio-portal communication and one patient had communication between the hepatic artery and middle hepatic vein. Successful embolization through the transarterial route was achieved in all four patients. A brief discussion of these cases is presented along with a relevant review of literature. CONCLUSIONS: Endovascular techniques currently form less invasive and first line treatment options in arterioportal/venous fistulae, surgery being reserved only for unsuccessful embolizations/complex fistulae.

Clinical utility of prothrombin induced by vitamin K absence in the detection of hepatocellular carcinoma in Indian population.

BACKGROUND: Alpha-fetoprotein (AFP) is a well known widely used biomarker for the detection of hepatocellular carcinoma (HCC); however, it suffers from a low sensitivity and specificity. Protein or prothrombin induced by vitamin K absence or antagonist II (PIVKA-II) is another tumor marker elevated in HCC but not extensively used. AIM: Evaluation of PIVKA-II and AFP in diagnosing HCC in India. PATIENTS AND METHODS: The study group consisted of 70 consecutive HCC patients, 38 patients with cirrhosis, 30 patients with chronic hepatitis, and 30 normal healthy subjects. All patients were evaluated for PIVKA-II and AFP levels by ELISA. RESULT: The mean plasma concentration of PIVKA-II in HCC, cirrhotic, chronic hepatitis patients and healthy controls was 101.07 ± 78.30 ng/ml, 2.45 ± 4.25 ng/ml, 1.50 ± 0.98 ng/ml and 0.79 ± 0.75 ng/ml, respectively. Receiver operating characteristic (ROC) curve was plotted for PIVKA-II and AFP. At a cutoff level of 9.2 ng/ml for PIVKA-II a sensitivity of 80% and a specificity of 92.1% was found, whereas AFP at a cutoff level of 13.02 ng/ml showed 72.9% sensitivity and 65.8% specificity. No significant relationship of plasma levels of PIVKA-II was observed in HCC with HBsAg/antiHCV positivity and associated portal vein thrombosis, but a positive correlation was seen with the tumor size (P = 0.001). However, no such significant association was found with AFP. CONCLUSION: PIVKA-II was more sensitive and specific than AFP for diagnosing HCC in the Indian population.

Comparison of 7 staging systems in north Indian cohort of hepatocellular carcinoma.

BACKGROUND & AIM: Many liver staging systems have been proposed for patients with hepatocellular carcinoma (HCC); however it is still controversial which staging system is best. The aim of this study was to compare the ability of 7 different staging systems in predicting survival in an Indian cohort of patients with HCC. METHODS: In this prospective study, 101 HCC patients were diagnosed and stratified according to 7 different staging systems; along with analysis of independent predictors of survival and their correlation with it (Kaplan-Meier analysis). RESULTS: CLIP, Tokyo score and BCLC staging system showed a significant difference in the probability of survival. All other staging systems failed to show a significant difference in survival. Age, portal vein thrombosis, serum bilirubin, MELD score showed a significant difference with survival in univariate analysis. However, serum bilirubin was the independent predictor of survival with a hazard ratio of 1.609 (95% CI 1.015-2.553, p = 0.043). CONCLUSION: The CLIP, Tokyo score and BCLC are the most useful staging systems in an Indian cohort.

Elevation of interleukin-18 in chronic hepatitis C: implications for hepatitis C virus pathogenesis.

The outcome of hepatitis C virus (HCV) infection is determined by the interplay between the virus and the host immune response. Interleukin (IL)-18, an interferon-gamma-inducing factor, plays a critical role in the T helper type 1 (Th1) response required for host defence against viruses, and antibodies to IL-18 have been found to prevent liver damage in a murine model. The present study was conducted to investigate the possible role of IL-18 in the pathogenesis and persistence of HCV. IL-18 levels were measured in sera of 50 patients at various stages of HCV infection (resolved, chronic and cirrhosis) and compared with those of normal controls. IL-18 gene expression was studied in peripheral blood mononuclear cells (PBMC) from each group, and in liver biopsy tissue from patients with chronic hepatitis C. The mean levels of IL-18 in sera were markedly elevated in patients with chronic hepatitis and cirrhosis, and were reduced in patients with resolved HCV infection. The serum IL-18 concentrations were related to the Child-Pugh severity of liver disease in cirrhotic patients. There also existed a strong positive correlation of IL-18 levels with histological activity score and necrosis. IL-18 mRNA expression was significantly up-regulated in the PBMC of cirrhotic patients when compared with other groups, while in the liver, higher levels of IL-18 transcripts were expressed in patients with chronic hepatitis C. The results of our study indicate that IL-18 levels reflect the severity and activity of HCV infection, and may contribute to the pathogenesis and progression of liver disease associated with HCV.

Chemical shift magnetic resonance imaging is helpful in detecting hepatic steatosis but not fibrosis in patients with nonalcoholic fatty liver disease (NAFLD).

BACKGROUND & AIM: Imaging modalities have a role in the diagnosis of patients with nonalcoholic fatty liver disease. Aim of the present study was to evaluate the role of chemical shift magnetic resonance imaging in assessing hepatic steatosis and fibrosis in patients with nonalcoholic fatty liver disease. METHODS: Chemical shift magnetic resonance imaging was done in 10 biopsy proven patients (7 females, mean age 41 +/- 9.2 years) with nonalcoholic fatty liver disease. Objective measurements of signal intensity (SI) were done and a ratio was calculated (SI out-of- phase liver/ SI out-of- phase kidney)/ (SI in- phase liver/ SI in-phase kidney). A lower ratio indicated a higher signal drop and hence higher fat content. The ratio was correlated with hepatic steatosis on histology (< 33% and > 33%). Patients were classified as having histological NASH or no NASH and MRI was assessed in diagnosing hepatic fibrosis as seen on liver histology. RESULTS: Six patients had > 33% hepatic steatosis on histology. Five patients (50%) had evidence of histological NASH. MRI was not helpful in differentiating patients with and without histological NASH. One patient amongst NASH patients did not have fibrosis, one had stage 1, 2 had stage 2 and one had stage 4 fibrosis. SI ratio ranged between 0.35-0.69 in 6 patients with steatosis > 33% and was in the range of 0.69-1.20 in four patients with steatosis < 33% on histology. Fibrotic changes seen in 4 patients on biopsy were not detected on MRI. CONCLUSION: Chemical shift MRI provides objective data on fat infiltration in patients with NAFLD without giving information about hepatic fibrosis.

Intrahepatic portal venopathy and related disorders of the liver.

Intrahepatic portal venopathy leads to various entities that are important causes of portal hypertension. Noncirrhotic portal fibrosis (NCPF) occurs in the Indian subcontinent, whereas idiopathic portal hypertension (IPH) occurs in Japan although the pathogenesis and presentation of both are similar. NCPF presents mainly with upper gastrointestinal bleeding; IPH presents with massive splenomegaly. The liver functions are preserved. Wedged hepatic venous pressure is normal, but portal venous pressure is high indicating a presinusoidal block. Patients are best managed with endoscopic therapy or surgery, with better results than in patients with cirrhosis. Nodular regenerative hyperplasia is a histological diagnosis characterized by development of nodules in the liver due to uneven perfusion of the portal venous blood. These patients may develop portal hypertension and if they bleed would require treatment as in NCPF/IPH. Schistosomiasis produces portal hypertension by the development of fibrous tissue around the portal veins as a response to schistosome eggs. Gratifying results have been reported with praziquantel therapy. Rarely sarcoidosis and chronic biliary obstruction may also produce portal venopathy.

Pancreatic cysts causing biliary obstruction in von Hippel Lindau syndrome.

CONTEXT: Extrahepatic biliary obstruction secondary to pancreatic cysts is rare in patients with von Hippel Lindau syndrome. We describe a patient with von Hippel Lindau syndrome who had biliary obstruction due to pancreatic cysts who was initially managed endoscopically and then surgically. CASE REPORT: A female patient with von Hippel Lindau syndrome which had been diagnosed ten years earlier based on the presence of pancreatic and renal cysts with retinal hemangiomas, presented with cholestatic jaundice of two months duration. On investigation, she was found to have lower end biliary obstruction caused by pancreatic cysts. The patient was initially managed with endoscopic retrograde cholangiography and a 7 French/10F/12F biliary plastic stent placement. Her cholestatic symptoms improved but required frequent stent exchange due to stent block; she finally underwent a hepaticojejunostomy and is doing well on follow-up. CONCLUSION: This case highlights the fact that pancreatic involvement leading to biliary obstruction, although uncommon, can occur in patients with von Hippel Lindau syndrome. Endoscopic biliary stent placement and surgery are helpful in these patients.

Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature.

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non-cirrhotic portal fibrosis are presented. METHODS: The disease is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction (EHPVO). Retrospective analysis of records of 151 patients with NCPF was analyzed for the clinical presentation, physical findings, laboratory tests, radiological and histological findings, and for the outcome of treatment. RESULTS: The disease is characterized by massive splenomegaly with anemia, preserved liver function and benign prognosis in a majority of patients. Splenoportovenography (SPV) showed massive dilatation of the portal and splenic veins, and the presence of collaterals. Twenty-four (15.9%) patients showed evidence of natural/spontaneous shunts (splenorenal 15, umbilical nine) on SPV; these patients had a lower incidence of variceal bleeding. Liver histology demonstrated maintained lobular architecture, portal fibrosis of variable degree, sclerosis and obliteration of small-sized portal vein radicles, and subcapsular scarring with the collapse of the underlying parenchyma. Piecemeal or hepatocytic necrosis was absent in all histology specimens. Three patients showed nodular transformation along with abnormal liver functions, and may represent late manifestation of NCPF where features are similar to those seen in patients with incomplete septal cirrhosis. In the initial part of the study, surgery (side-to-side lieno-renal shunt) was the preferred modality of treatment, however, endoscopic sclerotherapy or variceal ligation has now become the preferred first line of management of variceal bleeding. CONCLUSIONS: The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.