CONTEXT:Previous studies from the U.S. and Canada report deficiencies in informed decision making and a need to improve end-of-life (EoL) care in patients undergoing dialysis. However, there is a paucity of literature on these issues in Pakistani dialysis patients, who differ from Western patients in culture, religion, and available health care services.OBJECTIVES:To study informed dialysis decision-making and EoL attitudes and beliefs in Pakistani patients receiving dialysis.METHODS:We used convenience sampling to collect 522 surveys (90% response rate) from patients in seven different dialysis units in Pakistan. We used an existing dialysis survey tool, translated into Urdu, and backtranslated to English. A facilitator distributed the survey, explained questions, and orally administered it to patients unable to read.RESULTS:Less than one-fourth of the respondents (23%) felt informed about their medical condition, and 45% were hopeful that their condition would improve in the future. More than half (54%) wished to know their prognosis, and 80% reported having no prognostic discussion. Almost 63% deemed EoL planning important, but only 5% recalled discussing EoL decisions with a doctor during the last 12 months. Nearly 62% of the patients regretted their decision to start dialysis. Patients' self-reported knowledge of hospice (5%) and palliative care (7.9%) services was very limited, yet 46% preferred a treatment plan focused on comfort and symptom management rather than life extension.CONCLUSION:Pakistani patients reported a need for better informed dialysis decision making and EoL care and better access to palliative care services. These findings underscore the need for palliative care training of Pakistani physicians and in other developing countries to help address communication and EoL needs of their dialysis patients.

Background: Pakistan has a huge burden of Coronary Artery Disease (CAD) and dearth of resources to fight it, which makes primary prevention of the disease by increasing awareness among the population very important. The objective of this study was to assess awareness of Coronary Artery Disease risk factors among patients in Rawalpindi. Methods: This was a cross-sectional descriptive study conducted at Armed Forces Institute of Cardiology Rawalpindi Pakistan from November 2016 to November 2017. Sample size of 480 was calculated using WHO sample size calculator. Questionnaires were distributed to 480 outdoor patients by convenience sampling, of which 80 (16.5%) did not know about CAD and were not investigated further. Responses only from the remaining 400 patients were included. The questionnaire contained close-ended questions on risk factors for CAD and means of increasing CAD awareness. Indoor patients were excluded from the study. Data was analyzed with SPSSv21. Results: The mean age of patients was 53+6 years and 60% were males. 81.5% patients were able to read and write. Mean score of correctly identified risk factors was (6+2 out of 10). The percentage of patients aware of the different risk factors is as follows; diabetes (59.3%), hypertension (80.7%), dietary fat (79%), smoking (77.5%), stress (75.5%), sedentary lifestyle (68%), obesity (66%), old age (65.2%), male gender (52.7%) and family history (52%). Percentage of patients desirous of increasing their awareness about CAD risk factors through various means is as follows; treating physicians (84.8%), media (76.5%), syllabus and scholars (82.8%). Conclusion: Awareness about modifiable risk factors; hypertension, smoking, high dietary fat intake, obesity, sedentary lifestyle and stress with the exception of diabetes is reasonably good. Awareness about non-modifiable risk factors; family history, male sex and aging is poor.

Upper gastrointestinal bleeding is the most common and potentially life threatening emergency. Despite great advances in the field of medicine, the optimal management of bleeding peptic ulcer with adherent clot on endoscopy is still controversial. The aim of this study is to compare the combined endoscopic and medical therapy with medical therapy alone for bleeding peptic ulcer with adherent clot (Forrest type IIB). During two-year study period, around 342 patients presented to our Tertiary care hospital with acute upper gastrointestinal bleeding. Out of these, 81 patients were noted to have adherent clot (Forrest type IIB) during endoscopy and were included in study. 40 patients received combined endoscopic and medical treatment, whereas 41 patients received medical treatment only. The base line characteristics of patients in two groups were comparable and statistically significant. Primary Outcome being recurrence of bleeding within 7 days of treatment was less in combined therapy group compared to medical therapy group (2.5% vs. 17.1%) This was statistically significant. Secondary outcome like recurrence of bleed in 30 days and need for repeat endoscopy were less in combined group compared to medical therapy group. These were statistically significant as well. Other secondary outcomes like necessity for surgery and mortality were fewer in combined group, but these were not statistically significant. In conclusion combination endoscopic therapy consisting of epinephrine injection, removal of the adherent clot, and treatment of underlying stigmata is more effective than medical therapy alone.

Hypertriglyceridemia is a well-known cause of acute pancreatitis. A serum triglyceride level of more than 1000 mg/dl is the identifiable risk factor. Clinical presentation of hypertriglyceridemic pancreatitis is similar to other causes. Treatment is conservative with measures to rapidly lower triglyceride levels by plasmapheresis; insulin and heparin, or purified apo C II. Once acute attack of pancreatitis resolve, subsequent control of hypertriglyceridemia with dietary restriction of fatty meals, antihyperlipidemic agents and even regular apheresis is required to prevent further episodes of acute pancreatitis. Yet there are no clear guidelines for the management of hypertriglyceridemic pancreatitis to follow. Limited literature is available about the efficacy of intravenous insulin and heparin. We are presenting a case of 17 year old male with recurrent episodes of hypertriglyceridemic pancreatitis, successfully managed with insulin and heparin therapy.

BACKGROUND AND OBJECTIVE:Steatohepatitis is a common cause of liver disease due to alcohol (ALD) or non-alcoholic fatty liver disease (NAFLD). We performed this study to compare natural history of ALD and NAFLD.MATERIAL AND METHODS:Retrospective analysis of ALD or NAFLD patients managed at our center (2007-2011). ALD diagnosed by excluding other liver diseases (except HCV) and alcohol abuse of > 40 g/d in women and > 60 g/d in men for > 5 years. NAFLD diagnosed by excluding other liver diseases and a history of alcohol use of < 10 g/d. Cirrhosis was diagnosed using biopsy for uncertain clinical diagnosis.RESULTS:Compared to patients with NAFLD (n = 365; mean age 50 yrs; 43% males; 53% diabetic), ALD patients (n = 206; mean age 51 yrs; 68% males; 24% diabetic) presented more often with cirrhosis or complications(46vs. 12%; P< 0.0001) with a higher MELD score (13 ± 7 vs. 8 ± 8; P<0.0001). On logistic regression, ALD diagnosis was associated with presence of cirrhosis by over 4-fold (4.1 [1.8-9.1]) even after excluding 23 patients with concomitant HCV. Over median follow up of about 3 and 4 yrs among ALD and NAFLD patients respectively, ALD patients more frequently developed cirrhosis or its complications including HCC with worse transplant free survival (90 vs. 95%; P = 0.038).CONCLUSIONS:Compared to NAFLD, ALD patients present at an advanced stage of liver disease with a faster progression on follow-up. Prospective multicenter studies are needed to identify potential barriers to early referral of ALD patients as basis for development of strategies to improve outcome of patients with ALD.

Diarrheal diseases are a major cause of morbidity and mortality in children. However, with appropriate knowledge of treatment and care it is not only treatable but preventable. This article aims to assess maternal knowledge, attitude and practice towards diarrhea and management regarding role of oral rehydration therapy, rotavirus vaccination and zinc supplementation. This descriptive cross sectional study was conducted in pediatric OPD of Military Hospital Rawalpindi, Pakistan from September 2014 to March 2015.

Hodgkin's lymphoma(HL) must be included as a differential in cases of fever of unknown origin and idiopathic cholestasis with non specific hepatitis on liver biopsy. Early bone marrow examination in such cases may allow timely therapy and improve morbidity and mortality. A 60-year-old African American man presented with a two month history of recurrent high grade fever and progressive jaundice. He was treated multiple times with empiric antibiotics. Past history was significant for hypertension, alcohol and tobacco use. He denied any history of travel, new medication intake or prior malignancy. Examination revealed pale, emaciated and weak old man with no lymphade-nopathy and normal chest, cardiovascular and abdominal examination. Initial lab results revealed mild normochromic anemia and cholestatic pattern of hepatic injury with normal hepatitis serology. Elevated 5-Nucleotidase levels confirmed alkaline phosphatase to be of hepatic origin. MRCP showed normal hepatobiliary system. Enhanced CT chest and abdomen revealed small confluent lymphadenopathy with calcifications in subcarinal and peri-hilar regions. On liver biopsy we found nonspecific chronic hepatitis with mild portal fibrosis and no steatosis or granulomas with no features suggestive of autoimmune hepatitis, Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis or viral hepatitis. On further evaluation, bone marrow biopsy revealed hypercellular marrow with fibrohistiocytic infiltrates and large atypical cells suggestive of lymphoma. Immuno-staining of marrow confirmed large atypical cells to be Reed-Sternberg cells (CD 30+, CD15+, CD 45-, CD20-) confirming classical HL. Patient was started on chemotherapy and following two cycles, LFTs started to improve and normalized. Patient initially tolerated chemotherapy well until he developed septic shock and multiorgan failure and eventually passed away. HL has bimodal incidence affecting ages 15-35 and >50 years. It is associated with a diverse array of paraneoplastic syndromes ranging from complex neurological syndromes to rare entities like vanishing bile duct syndrome and idiopathic cholestasis. However, other etiologies of intrahepatic cholestasis should be ruled out like alcohol, viral, drug induced, PBC and PSC. Mortality can be as high as 65% from liver involvement. Intrahepatic cholestasis is an uncommon presentation of HL (3-13% of cases), and even more so rare are cases with negative liver biopsy despite clinical picture consistent with a cholestatic pattern of liver injury. Thus, liver biopsy may not necessary reflect the underlying etiology as highlighted by our case. Although treatment of underlying HL results in normalization of liver enzymes, however, its prognostic value remains undetermined.

Background and Aim Autoimmune (AI) markers are reported in patients with steatohepatitis-related liver disease. However, their clinical significance is unclear. Methods Charts of patients due to alcoholic liver disease (ALD) or nonalcoholic fatty liver disease (NAFLD) were stratified for antinuclear antigen (ANA > 1:80), antismooth muscle antibody (ASMA > 1:40), or antimitochondrial antibody (AMA > 1:20). Study outcomes were patient survival and complications of liver disease. Results Of 607 patients (401 NAFLD), information about AI markers was available for 398 (mean age 50 ± 15 year; 52 % males; median body mass index (BMI) 38; 44 % diabetic; 62 % nonalcoholic steatohepatitis (NASH) as type of steatohepatitis; median MELD score 9). A total of 78 (19.6 %) patients were positive for AI markers without differences for ALD versus NAFLD, cirrhosis versus no cirrhosis, and NASH versus no NASH. There were no differences for age, gender, BMI, cirrhosis at presentation, MELD score, endoscopic findings, and histology based on AI markers. Serum ALT was higher among patients with AI markers (65 ± 46 vs. 59 ± 66 IU/l; P = 0.048). Data remained unchanged on analyzing NAFLD patients. None of the 11 ANA-positive patients (1:640 in 4) showed findings of AI hepatitis. Biopsy in three AMA-positive patients showed mild bile duct damage in one patient. On median follow-up of about 3 years, there were no differences in liver disease outcomes (ascites, encephalopathy, variceal bleeding), hepatocellular carcinoma, transplantation, and survival. Conclusions Autoimmune markers are frequently present in steatohepatitis-related liver disease patients. Their presence is an epiphenomenon without histological changes of autoimmune hepatitis. Further, their presence does not impact clinical presentation and follow-up outcomes.

Background and Aims: Alcohol abuse and nonalcoholic fatty liver disease (NAFLD) are common causes of liver disease. Diabetes mellitus (DM) is a common comorbidity among NAFLD patients. We performed this study with the specific aim to examine the impact of DM on progression of alcoholic liver disease (ALD) liver and NAFLD. Methods: Medical charts of 480 patients with ALD or NAFLD (2004-2011) managed at a tertiary center were retrospectively reviewed. NAFLD was diagnosed based on exclusion of other causes of liver disease and alcohol use of <10 g/d. ALD was diagnosed based on alcohol use of >40 g/d in women or >60 g/d in men for >5 years. Results: Of 480 patients (307 NAFLD), 200 diabetics differed from nondiabetics for: age (52 +/- 11 vs. 49 +/- 11 years; p=0.004); male gender (48% vs. 57%; p=0.03); metabolic syndrome (49% vs. 30%; p=0.0002); NAFLD (80% vs. 56%; p<0.0001); cirrhosis (70% vs. 59%; p=0.005); and hepatocellular carcinoma (HCC; 8% vs. 3%; p=0.009). Over a 3 year median follow-up period, diabetics relative to nondiabetics had a higher probability to develop cirrhosis (60% vs. 41%; p=0.022) and HCC (27% vs. 10%; p=0.045). There was a trend for increased development of hepatic encephalopathy in diabetics compared to nondiabetics (55% vs. 39%; p=0.053), and there was no difference between the two groups in survival or other liver disease complications. Conclusions: DM increased risk for cirrhosis and HCC among patients with ALD and NAFLD. Prospective studies with longer follow-up periods are needed to examine the impact of DM on survival and the role of aggressive HCC screening in diabetic cirrhotics. (C) 2015 The Second Affiliated Hospital of Chongqing Medical University. Published by XIA & HE Publishing Ltd. All rights reserved.

Introduction: Celiac crisis is an uncommon presentation in adults. While serology is sensitive and specific, it can be negative in some patients, which make intestinal biopsy indispensable in diagnosis. An 80-year-old woman presented to the hospital with loose stools; 15 episodes /day for 1 week, and increasing weakness. She had 10-year history of 3-4 loose stools/day. She has no significant past medical or family history, nor recent travel history. Labs showed low sodium, potassium, chloride, metabolic acidosis, and acute renal failure. She was initially treated with intravenous normal saline and bicarbonate; however, diarrhea continued. Investigations showed elevated stool osmotic gap and negative stool cultures for bacteria, ova, and parasites. Anti-endomysial (EMA) and anti-tissue transglutaminase (TTG) antibodies and 5HIAA were normal. Colonoscopy demonstrated normal colonic mucosa; however, random biopsies demonstrated lymphocytic colitis and increased intraepithelial lymphocytes. Small bowel biopsy showed partial villous atrophy, crypt hyperplasia, and increased inflammatory infiltrate consistent with celiac disease. She was started on a gluten-free diet, and over the subsequent weeks, she had resolution of symptoms. Two interesting aspects of this case are that she presented with celiac crisis, which is rare in adults, and diagnosis depended solely on biopsy because she did not have antibodies to EMA and TTG. Celiac crisis is a life-threatening syndrome of unclear etiology. Manifestations include diarrhea and electrolyte disturbances. It was thought to occur exclusively in patients younger than 2 years of age. The association between microscopic colitis and celiac disease may be 1 factor that contributes to the development celiac crisis due to defective active and passive absorption of sodium and chloride, and reduced chloride-bicarbonate exchange. Diagnosis of celiac disease is based on serologic tests of specific antibodies, including EMA and TTG antibodies, which are highly specific and sensitive (95% and 96%). It was found that 7% of patients were negative for both auto-antibodies despite having normal IgA levels. Intestinal biopsy is the gold standard for diagnosis because EMA and TTG antibodies decrease in patients with partial villous atrophy. Histological criteria are increased intraepithelial lymphocyte infiltrate, crypt hyperplasia, and villous atrophy. Gluten-free diet is the mainstay of treatment with dramatic response and improvement in symptoms. Although celiac crisis is an uncommon presentation in adults, it should be considered in patients presenting with unexplained severe diarrhea and metabolic acidosis, even if EMA and TTG antibodies are negative.

Background and Aims: Fructose overconsumption in Western diet is linked to non-alcoholic fatty liver disease (NAFLD).In animal models fructose overconsumption leads to chronic metabolic low-grade inflammation and was also linked to cardiovascular disease (CVD) as well as development of type-II-Diabetes (T2DM).Until now, the impact of fructose overconsumption is often studied in overweight, T2DM or NAFLD patients, but is not as well described in healthy subjects.To test our hypothesis that fructose overconsumption may impact on the gut-liver axis by increasing intestinal permeability, bacterial translocation, and consecutively metabolic inflammation as important mechanisms in the pathogenesis of NAFLD, CVD and T2DM, we examined the impact of a high oral fructose challenge (150g/ day for 4 weeks) on intestinal permeability and inflammation.Methods: Ten healthy volunteers were enrolled (m:f=5:5; median[range] age=24.5[21-37][years]; p=n.s.).Intestinal permeability was assessed by sucrose-lactulose-mannitol (SLM) test using high performance liquid chromatography of urine collected over 5 hours.Interleukin-6 (IL-6) was assessed by ELISA.C-reactive Protein (CRP) and Fibronectin were assessed using nephelometry.Results: After the oral fructose challenge changes in energy metabolism reflected by increased BMI (mean±SD 21.11±2.68vs 21.51±2.77[kg/m^2]; p<0.001) as well as fasting glucose levels (mean±SD 83.2±8.37 vs 88.4±5.48 [mg/dL]; p=0.035) were observed.Gastroduodenal permeability, reflected by recovered sucrose (median[range] 0.075[0-0.1]vs 0.089[0-0.1][mmol/L]; p=0.68) as well as small intestinal permeability reflected by lactulose/mannitolratio (mean±SD 0.021±0.006vs 0.019±0.008[%]; p=0.622) remained stable.In line, inflammatory parameters CRP (median[range] 0.5[0-3] vs 0[0-1] [mg/dL]; p=0.066) and Fibronectin (mean±SD 35.3±8.63 vs 35.3±7.73 [mg/dL]; p=1.0) remained unchanged.IL-6 was below the detection limit prior and after fructose challenge, suggesting a high oral fructose challenge does not affect intestinal permeability in healthy young volunteers.Conclusion: Healthy young volunteers are capable of handling fructose overconsumption (150g/day for 4 weeks) on top of their normal diet without any increase in intestinal permeability or inflammation.This further supports the hypothesis that fructose overconsumption may act as "hepatotoxin" as a second hit in pre-existing metabolic diseases and/or on top of genetically predisposing backgrounds such as PNPLA3 genotype.

Although splenic involvement alone in hydatid disease is very rare, spleen is the third most common organ involved in hydatid disease. The rarity of splenic hydatid disease poses a diagnostic challenge for clinicians, particularly in non-endemic areas. As the hydatid cyst can present as a simple cyst without having the classic serological and imaging features, and later can lead to life-threatening complications like anaphylaxis, hydatid disease of spleen should be considered in differential in every patient in endemic areas with cystic lesion of spleen until proved otherwise. The author used the keyword “splenic hydatid cyst” in PubMed and reviewed the scientific literatures published from January 1965 to June 2012. The present review is to accentuate the incidence, classification, clinical and pathophysiological features, differential diagnosis, diagnostic modalities, and treatment choices of hydatid cyst of spleen along with follow-up strategy and newer treatment approaches.

Dear Editor, Although epidemic, vitamin D deficiency is still under-diagnosed.[1] In 30% of patients it can present as proximal muscle weakness before the biochemical signs of vitamin D deficiency appear leading to unnecessary investigative work up. So in at-risk individuals it should be kept as one of the differential diagnosis for muscle weakness, as the condition is reversible and easily treated with vitamin D and calcium supplementation. A 33-year-old black female, was referred to our hospital because of elevated Creatinine kinase. She has been having difficulty in getting up from chair over last couple of months. She also complained of having generalized aches and pains while doing her routine work. She is a mother of four children and has been on orlistat and following a diet for losing weight. On physical examination, muscle bulk and muscle tone was normal; the muscle power was 3/5 in the flexors and extensors of the hip and shoulder. There was no other neurological deficit. Laboratory parameters [Table 1] revealed hypochromic microcytic anemia due to severe iron deficiency. vitamin B12, thyroid function tests, erythrocyte sedimentation rate, serum magnesium, potassium and phosphate levels were normal. A search for inflammation or other causes of myopathy was negative which included an auto-antibody screen such as ANA, Rheumatoid factor and anti JO-1 antibodies. Serum ionized calcium was low with normal alkaline phosphatase but borderline high Parathyroid hormone (PTH) levels. Her serum 25-hydroxy vitamin D was extremely low (4 ng/ml, reference range > 30-100). Levels of Creatinine Kinase, Aldolase, myoglobin and Lactate Dehydrogenase (LDH) were elevated. Electromyography was also normal. To rule out myositis, muscle biopsy of the left deltoid was done which showed non-specific minimal muscle fiber atrophy [Figure ​[Figure1a1a–c]. Keeping in consideration her risk factors which included Orlistat intake, less sun exposure, high melanin pigment, and poor dietary intake diagnosis of proximal myopathy due to severe vitamin D Deficiency associated with rhabydomyolsis was made based on the clinical and biochemical findings. Medical treatment was initiated, and orlistat was withdrawn. Her serum biochemistry values along with proximal myopathy and muscle tenderness improved on follow-up in 2 months. Table 1 Laboratory parameters Figure 1 (a) A small cluster of atrophic cells (small arrow) with some more normal myocytes in the bottom right. They are separated by fat (×4). (b) Centered on the atrophic fibers which are ragged and irregular (small white arrow). One myocyte (small ... Vitamin D deficiency is defined as a 25-hydroxy vitamin D level of less than 20 ng/ml and a level between 21 ng and 29 ng/ml is considered as relative insufficiency.[2] Lack of sunlight exposure, higher skin melanin content, dietary insufficiency of vitamin D and Orlistat led to vitamin D deficiency in our patient. Orlistat affects body fat as well as interfere with absorption of vitamin D. McDuddie demonstrated that mean vitamin D levels were significantly reduced compared with baseline after 1 month of Orlistat, despite multivitamin supplementation including vitamin D.[3] In 30% it can present mainly as proximal myopathy. A serum 25-hydroxy vitamin D level below 20 ng/ml causes increased body sway and a level below 10 ng/ml leads to difficulties in rising from a chair, inability to ascend stairs and pain and discomfort due to muscular effort as in our patient.[4] Clement et al. highlighted the importance of vitamin D in patients with multiple myeloma, and showed that it is common and can cause generalized musculoskeletal pain and increase the risk of falls in such patients yet it often goes unrecognized.[5] Furthermore, Glucecek et al demonstrated that hypercholesterolemic patients with vitamin D deficiency are intolerant to statins due to myositis-myalgia more tahn general population without deficiency. And after supplementation with vitamin D, statin could be successfully reintroduced in 90% of patients without having recurrent myositis-myalgia, reflecting a reversible interaction between vitamin D and statins on skeletal muscles.[6] Basis of this hypothesis is skeletal muscle contains vitamin D receptors that modulate various transcription factors in muscle cells,[7] mediating muscle cell proliferation and differentiation into mature type II muscle fibers. Furthermore, vitamin D is responsible for the active transportation of calcium into sarcoplasmic reticulum necessary for sarcomeric muscular contraction which has been shown to play a role in maintenance of postural equilibrium.[8] The assessment of serum 25 OHD (25 hydroxy vitamin D) is the only reliable test as clinical myopathy may be present before the development of biochemical signs (low calcium and increased Alkaline phosphatase) of bone disease.[2] Elevation of muscle enzyme creatinine kinase level has been reported in a minority of patients with vitamin D related muscle weakness,[9] but was significantly elevated in our patient pointing towards the significant muscle damage. Muscle biopsy is not indicated and if done, shows non-specific muscle fiber atrophy and no signs of inflammatory reaction. The ultimate evidence of the diagnosis rests on the response to therapy. Proximal muscle strength strikingly improves when 25-hydroxy vitamin D levels increases from 4 ng to 16 ng/ml and continues to improve as the levels increase to more than 40 ng/ml.[10] We conclude that in patients with proximal muscle weakness, finding a typical constellation of biochemical alterations should limit additional costly and invasive neuromuscular workup for other causes of muscle dysfunction. In such a patient, an early therapeutic trial of vitamin D is warranted. A lack of objective improvement in proximal muscle strength after about a month on an adequate dosage of vitamin D indicates a need for reevaluation of the diagnosis.

Alcoholic cirrhosis remains the second most common indication for liver transplantation. A comprehensive medical and psychosocial evaluation is needed when making a decision to place such patients on the transplant list. Most transplant centers worldwide need a minimum of 6 mo of alcohol abstinence for listing these patients. Patients with alcohol dependence are at high risk for relapse to alcohol use after transplantation (recidivism). These patients need to be identified and require alcohol rehabilitation treatment before transplantation. Recidivism to the level of harmful drinking is reported in about 15%-20% cases. Although, recurrent cirrhosis and graft loss from recidivism is rare, occurring in less than 5% of all alcoholic cirrhosis-related transplants, harmful drinking in the post-transplant period does impact the long-term outcome. The development of metabolic syndrome with cardiovascular events and de novo malignancy are important contributors to non liver-related mortality amongst transplants for alcoholic liver disease. Surveillance protocols for earlier detection of de novo malignancy are needed to improve the long-term outcome. The need for a minimum of 6 mo of abstinence before listing makes transplant a nonviable option for patients with severe alcoholic hepatitis who do not respond to corticosteroids. Emerging data from retrospective and prospective studies has challenged the 6 mo rule, and beneficial effects of liver transplantation have been reported in select patients with a first episode of severe alcoholic hepatitis who are unresponsive to steroids.

Purpose: Steatohepatitis (SH)-related liver disease due to either alcohol use (ASH) or non-alcoholic steatohepatitis (NASH) is a common cause of cirrhosis and liver transplantation in the US. Although imperfect, liver biopsy is a definitive method of diagnosing SH and assessing its severity. However, liver biopsy is not always performed in clinical practice, and often, a diagnosis of SH is made clinically by exclusion of other causes of liver disease and history of alcohol use. We performed retrospective analysis to determine factors predictive of taking a liver biopsy among SH-related liver disease patients. Methods: Patients with SH-related liver disease, including ASH and NASH, managed at a single tertiary care center (2007-2011) were stratified based on receipt of liver biopsy for confirming diagnosis. Chisquare and t-tests were used for comparing categorical and continuous variables, respectively. Logistic regression model was built to assess factors associated with taking liver biopsy for diagnosing SH, its etiology, and assess its severity. Data were reported as odds ratio (OR) with 95% confidence interval (CI). Results: ASH patients (N=226) presented more often to clinician with symptoms of cirrhosis or its complications compared to NASH (69 vs. 27%; P<0.0001). Comparing 110 (22%) patients receiving liver biopsy to 391 non-biopsied cases, those undergoing liver biopsy differed for: female gender (53 vs. 42%; P=0.03), history of cholecystectomy (49 vs. 29%; P=0.0001), NASH diagnosis (73 vs. 50%; P<0.0001), asymptomatic presentation (71 vs. 49%; P<0.0001), median ALT (61 vs. 48; P=0.03), and MELD score (8 vs. 11; P=0.005). Age, race, and proportion of metabolic syndrome components, steatosis on imaging, cirrhosis, and HCC were similar. After controlling for significantly different and clinically relevant variables, patients undergoing liver biopsy were over two-fold more likely to have had cholecystectomy: OR [95% CI] of 2.1 [1.3-3.5]. Histology details available on 95 (70 NASH) biopsies showed NASH patients to differ from ASH for: steatosis >33% of hepatocytes (62 vs. 41%; P=0.03), lobular inflammation (94 vs. 72%; P=0.03), trend for higher NASH activity score (3.7±1.5 vs. 2.8±1.9; P=0.058), and lower stage 3 or 4 fibrosis (54 vs. 72%; P=0.15). Conclusion: About 2/3 of biopsies in SH-related liver disease is taken at the time of cholecystectomy. Most of these biopsies are taken for NASH patients. Studies are needed to examine whether taking routine liver biopsy at laparotomy for alcoholics is helpful in improving patient adherence to alcohol abstinence and in early diagnosis of liver disease related to ASH.

Dear Editor, Prevalence of hydatid cyst is increasing in developed countries due to immigration. However, complicated hydatid disease of liver causing inferior venacaval and portal vein thrombosis is exceedingly rare. In literature, five similar cases have been reported worldwide; however, most of them have been treated with radical surgery. Our case differs in the fact that it was successfully treated with non-operative approach using sequential endoscopic retrograde cholangiopancreatography (ERCP) and and catheter-directed thrombolytic therapy. A 42-year-old Asian female presented with 6 days history of right upper quadrant pain, progressive jaundice, high grade fever, persistent vomiting, and progressive distention of abdomen. Examination revealed dry mucous membranes, scleral icterus, temperature of 103°F, pulse 120/min, blood pressure 80/60 mm Hg, and tender hepatomegaly. Table 1 demonstrates initial and subsequent laboratory parameters. Sonography of abdomen demonstrated a well defined cyst (6.2 × 6cm) in the left lobe of liver with floating membranes inside the cavity and ascites [Figure 1]. The patient was initially resuscitated with intravenous fluids, vasopressors, blood transfusions, and antibiotics along with albendazole were also started. Emergency ERCP was performed which revealed dilated common bile duct with multiple filling defects [Figure 2a]. A Naso Biliary Drain (NBD) was placed in the left hepatic ductal system, which drained large amount of pus mixed with membranes and bile. NBD cholangiogram revealed large cavity in left lobe of liver communicating with left hepatic duct [Figure 2b]. Culture of blood, urine and ascitic fluid was sterile but pus from NBD came positive for scolices and Escherichia coli. Echinococcus serology assessed by ELISA was also noted to be positive. Table 1 Comparison of laboratory parameters at admission, hospital stay and discharge Figure 1 USG abdomen showing Hydatid Cyst (6.2 × 6.0cm) in left lobe of liver with floating membranes inside (white arrow). Also, dilated CBD with membranes inside (black arrow) Figure 2 (a) Initial ERCP showing dilated common bile duct with multiple filling defects. (b) ENBD cholangiogram revealed large cavity in left lobe of liver communicating with left hepatic duct. (c) CT Abdomen with contrast showing well defined cystic lesion in ... Contrast Enhanced Computerized Tomography of abdomen demonstrated well-defined cystic cavity in left lobe of liver, multiple small hepatic abscesses, ascites and well-defined filling defect within the lumen of inferior venacava suggestive of thrombus [Figures ​[Figures2c2c and ​andd].d]. Doppler study revealed thrombotic occlusion of inferior venacava, right portal vein, and main portal vein along with portal hypertension [Figure 3a]. Patient was started on heparin drip. On day 8th, patient's condition begins to deteriorate again with worsening jaundice, leukocytosis, and increasing alkaline phosphatase [Table 1]. An elective ERCP demonstrated bulging papilla of vater with protruding membranes. The common bile duct was dilated 20 mm with numerous leaf-like filling defects. A papilotomy was performed and copious amount of pus with hydatid material was evacuated [Figure ​[Figure3b3b and ​andcc]. Figure 3 (a) Doppler ultrasound study of abdomen revealing thrombotic occlusion of portal vein. (b) ERCP showing CBD dilated 20 mm with numerous filling defects(white arrows) and filling defects in left hepatic duct (black arrow). (c) Cholangiogram showing dilated ... Although, fever, leukocytosis, and features of chlolestasis improved after ERCP, but her abdominal pain and ascites progressively worsened. A peritoneal tap was performed and ascitic fluid analysis was negative for scolices and hooklets with serum ascitic albumin gradient (SAAG) of 1.3 g/dL suggestive of a transudate likely due to portal hypertension secondary to portal vein thrombosis. As the thrombosis was acute and irreversible even after decompression of liver, and patient was high risk for bleeding due to low platelets and anticoagulant use. It was, therefore, decided to attempt catheter-directed thrombolysis to prevent long-term complications of inferior venacaval and portal vein thrombosis and risk of bleeding. Using transfemoral catheter directed approach, 250,000 units of streptokinase was given followed by infusion of 100,000 units/hour for 36 hours. No complication was encountered during or after the procedure and patient was monitored in intensive care unit. Subsequent to thrombolysis, abdominal pain, ascites and portal hypertension significantly improved and patient was discharged on albendazole for 3 months and oral warfarin for 6 months. On follow up, repeat sonography revealed decrease in the size of hydatid cyst cavity suggestive of patient is improving with no disease recurrence [Figure 3d]. Despite the rise in prevalence of Echinococcosis, it still remains a very rare disease (<1 case per 1 million inhabitants) in United States. With intrabiliary rupture, the classic triad of jaundice, biliary colic, and fever occurs but ascites and lower extremity edema is rare; however, it can be the presentation of thrombus in either portal vein or inferior venacava. The Budd-Chiari syndrome (BCS) indicates an obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava (IVC) and the right antrum. Acute form of BCS is a rare condition and up to 15% of cases are complicated with portal vein thrombosis (PVT). Prognosis in patients with Budd-Chiari syndrome with portal vein thrombosis is poor.[1] In Western countries, its spontaneous mortality is reported to approach 70% at 1 year and 90% at 3 years.[2] Therefore, early diagnosis and treatment is necessary. The initial screening test of choice is ELISA (sensitivity 80%). Sensitivity of Ultrasound abdomenand ERCP for diagnosis of intrabiliary rupture of hydatid cyst is almost 70% and 90%, respectively. ERCP is gold standard for diagnosis.[3] To evaluate inferior venacaval and portal vein thrombosis, ultrasound and contrast enhanced triple phase computerized tomography (CT) are considered primary diagnostic modalities. Unlike venography, they can accurately demonstrate the cephalic extent of thrombus and assessment of other surrounding viscera.[4] USG is best and safest means to follow up such patients. Endoscopic therapy appears to be an effective mode of treatment for biliary fistulas complicating liver abscesses and cysts.[5] Preoperative ERCP is very helpful in patients with cysto-biliary fistula and, in selected cases, it can solve the problem without further surgical intervention.[6] Although most of these cases have been managed with decompression by ERCP or Puncture Aspiration Irrigation and Reaspiration (PAIR) followed by surgical resection of hydatid cyst which also led to the resolution of inferior venacaval thrombosis as well.[4,6] Interestingly, in our case cyst decompression with ERCP alone did not lead to resolution of IVC thrombosis and portal hypertension. Thus, thrombolysis was performed. A number of thrombolytic agents, such as urokinase, streptokinase, and tissue plasminogen activator have been administered. Thrombolysis is more likely to be successful in acute thrombosis and when the thrombolytic agent is locally infused into a hepatic vein and/or inferior venacava.[7] Recently a quick and easy thrombolytic approach; that is, agitation thrombolysis for the treatment of fresh thrombosis have been devised.[8] There are a few case reports in the literature describing the benefits of systemic thrombolysis, whereas, few investigators have used combined local and systemic thrombolysis.[9] Although we used local thrombolysis for inferior venacava thrombosis, possibly its systemic effect led to resolution of portal vein thrombosis. Vascular access may be attempted via the transjugular or transfemoral route when inferior venacava interventions have to be performed. Mostly, the femoral route is preferred as negotiation is easier and risk of bleeding is less compared to transhepatic approach. Transhepatic route has proved to be a safe and effective method in the treatment of portal vein thrombosis. But it carries a risk of post-procedure uncontrollable bleeding through the transparenchymal tract after removal of the catheter and is increased in patients with ascites, coagulopathy or those on anticoagulant therapy.[10] This is the reason transfemoral approach was chosen in our patient. In conclusion, as yet there is no consensus on treatment guidelines for patients with intrabiliary rupture of hepatic hydatid cyst complicated with inferior venacava and portal vein thrombosis. Keeping in view Echinococcosis is not a malignant disease; consideration should be given to all nonsurgical therapeutic approaches before contemplating operative procedures.

Purpose: A 47-year-old asymptomatic woman was self-referred for evaluation of diffuse colorectal polyposis. Recent colonoscopy revealed > 100 diffuse 3-7 mm polyps throughout the colon and rectum, reported as mixed hyperplastic polyps and tubular adenomas without high-grade dysplasia; colectomy was recommended by her initial gastroenterologist. Her past medical history included nephrectomy for hypernephroma at age 38, hysterectomy with bilateral oophorectomy for uterine fibroids and ovarian cysts, partial thyroidectomy for multinodular goiter, and removal of oral papillomas and soft cutaneous nodules. Physical examination revealed macrocephaly and flesh-colored cutaneous and gingival nodules. Upper endoscopy revealed multiple small raised plaques 2 mm to 10 mm in size throughout the esophagus, and multiple small sessile 3-5 mm polyps in the gastric fundus and body. Colonoscopy revealed pancolonic polyps ranging in size from 3 mm to 1 cm. Pathologic review demonstrated esophageal glycogenic acanthoses, gastric hyperplastic polyps, and colonic hamartomas. Genetic testing revealed a mutation in the phosphatase and tensin homolog (PTEN) gene, which confirmed the diagnosis of Cowden syndrome (CS). Thus, the patient was advised to undergo appropriate cancer surveillance according to National Cancer Center Network guidelines rather than total colectomy. Cowden syndrome is an autosomal dominant disorder related to germ line mutations in the PTEN gene mapped to the 10q22-23 locus, and is generally reported to be found in 80% of patients with CS. The prevalence of CS has been estimated to be between 1/200,000 and 1/250,000. Typical benign manifestations of the syndrome include trichilemmomas (83%), acral keratoses, lipomas, multinodular goiter (50-70%), macrocephaly (80%) and gastrointestinal polyposis (40%). Co-occurrence of extensive esophageal glycogenic acanthoses and colonic polyposis is rare outside of CS, and can be considered pathognomonic for this disease. Although hamartomatous polyps preponderate in CS (90%), adenomatous, inflammatory, hyperplastic, lymphoid, ganglioneuromatous, and leiomyomatous polyps can also occur throughout the entire gastrointestinal tract. The ambiguity of the prevalence and risks of colorectal polyposis in patients with CS is reflected in the lack of recommendations concerning colorectal screening or surveillance in the National Comprehensive Cancer Network (NCCN) treatment guidelines. The greatest neoplastic risks for CS patients are for breast cancer (25-50%), endometrial cancer (5-10%), and thyroid cancer (3-10%). Careful breast, thyroid, cutaneous, and genitourinary surveillance is indicated. Intervals for GI tract surveillance have not been established.