Primary Hepatic Sarcomatoid Carcinoma Presenting with Dysphagia: A Rare Presentation.

A 50-year male presented with vomiting and dysphagia for 2 weeks. Laboratory workup showed a positive serology for hepatitis C and normal serum α-fetoprotein (AFP) levels. CT abdomen revealed a large lesion in the right lobe of the liver extending upto the lower esophagus causing significant luminal narrowing and dysphagia. The enhancement pattern on the CT scan was not consistent with hepatocellular carcinoma. Liver lesion biopsy showed an infiltrating spindle cell lesion exhibiting fascicles of spindle cells with moderately hyperchromatic nuclei and perinuclear vacuolization. Mitotic count was 2-3/10 HPFs. Immunohistochemical markers were positive for CK AE1/AE3 and vimentin. Thus, a diagnosis of sarcomatoid carcinoma was made on the basis of morphological and immunohistochemical features. Due to unresectable disease and poor functional status, palliative care was opted for. Key Words: Dysphagia, Vomiting, Liver biopsy, Sarcomatoid carcinoma.

Clinical Characteristics and Comparison of Different Prognostic Scores in Wilson's Disease.

Aim: Wilson's disease (WD) is a rare autosomal recessive disease, that can involve any organ of the body, the main ones being the liver and the brain. These patients can have varied presentations, ranging from having no symptoms to having neurological manifestations to features of chronic liver disease (CLD). Those patients that end up having CLD are prognosticated via the Child-Turcotte-Pugh (CTP) score and the Model for End-stage Liver Disease (MELD) score. However, two specific scores exist for prognostication in patients having WD, namely, the Nazar score and the Dhawan score. However, these are yet to be validated nor has their use been implemented in clinical practice. Materials and methods: Our study involved 65 patients with WD, comprising both the pediatric and the adult population. We aimed at evaluating the clinical manifestations the lab parameters and the management of these patients. Furthermore, we tried validating the Nazar and the Dhawan score and later compared them with the CTP and the MELD score, which are well-known prognostic tools in CLD. Results: Our patients were subdivided into the pediatric (more than 50%) and the adult group. The most common presenting complaint noted in both groups was abdominal distension. Values of the urine copper and serum ceruloplasmin did not defer between the pediatric and adult patients. Hepatic involvement is frequently seen in the pediatric age-group. Also, CTP class C was chiefly seen in pediatrics 17/33 (51.5%), while CTP class B was in adults 13/32 (40.6%). The mean Nazar score was 3 ± 3, while the mean Dhawan score was 5 ± 4. The main treatment offered for both groups was zinc along with penicillamine. Conclusion: Our study showed the Dhawan score was comparable to the CTP and the MELD score in terms of predicting the disease severity of WD in our patient population. How to cite this article: Majid Z, Abrar G, Laeeq SM, et al. Clinical Characteristics and Comparison of Different Prognostic Scores in Wilson's Disease. Euroasian J Hepato-Gastroenterol 2022;12(2):69-72.

Safety and Efficacy of Extracorporeal Shock Wave Lithotripsy for Difficult-to-retrieve Common Bile Duct Stones: A Ten-year Experience.

BACKGROUND AND OBJECTIVE: Extracorporeal shock wave lithotripsy (ESWL) for common bile duct (CBD) stones has been used in the past, but experience is limited. We report our experience of ESWL in the management of difficult CBD stones. METHODS: Patients with difficult-to-retrieve CBD stones were enrolled and underwent ESWL. Fluoroscopy is used to target the stones after injection of contrast via nasobiliary drain. CBD clearance was the main outcome of the study. RESULTS: Eighty-three patients were included (mean age 50.5 ± 14.5 years); these patients were mainly females (43; 51.8%). Large stones >15 mm were noted in 64 (77.1%), CBD stricture in 22 (26.5%) and incarcerated stone in 8 (9.6%) patients. Patients needed 2.1 ± 1.2 sessions of lithotripsy and 4266 ± 1881 shock waves per session. In 75 (90.3%) patients, the fragments were extracted endoscopically after ESWL, while spontaneous passage was observed in 8 (9.6%). Total CBD clearance was achieved in 67 (80.6%) patients, partial clearance in 5 (6%) and no response in 11 (13.2%). Failure of the treatment was observed in large stone with size ≥2 cm (P = 0.021), incarcerated stone (P = 0.020) and pre-endoscopic retrograde cholangiopancreatography cholangitis (P = 0.047). CONCLUSION: ESWL is a noninvasive, safe and effective therapeutic alternative to electrohydraulic lithotripsy and surgical exploration for difficult biliary stones.

Use of sofosbuvir based regimen in patients with end-stage renal disease and chronic hepatitis C; an open label, non-randomized, single arm, single center study from Pakistan.

AIM: we aimed to determine the virological response and safety of Sofosbuvir-based direct-acting antiviral agents (DAAs) in chronic hepatitis C (CHC) patients on long-term hemodialysis (HD). BACKGROUND: With the advent of interferon-free DAAs, the treatment of CHC has been revolutionized. Pakistan is among the countries where novel sofosbuvir (SOF)-free antiviral agents are not available. METHODS: This non-randomized, single-arm, open-label study enrolled all HD patients with chronic HCV infection after informed consent. They were treated with SOF in combination with Ribavirin (RBV) with either interferon (IFN group) or daclatasvir (DAC group), with the virological response assessed according to standard guidelines. Data were analyzed using SPSS version 20.00. RESULTS: Out of 133 patients, the majority (72.9%) were males with the mean age of 31.92 ± 9.88 years. Most patients (50.3%) had HCV genotype (GN) 1, followed by GN 3 in 42.9%, 4 in 1.48% and 2 in 0.7%, while mix GN was documented in 6 (4.4%) patients. Among these, 60 (45.1 %) patients received standard SOF, IFN, and RBV (IFN group) and 73 (54.9 %) received SOF, DAC and RBV (DAC group). End of treatment and sustained virological response at 12 weeks post-treatment were achieved in 133 (100%) and 129 (97 %) patients, respectively. The adverse effects were anemia in 58 (43.6 %) patients and elevated alanine transaminases in 11 (8.1%) patients. CONCLUSION: SOF in combination with either IFN or DAC is an equally efficacious and effective treatment regimen for patients on maintenance HD, especially in resource-poor countries.

Virological Response to Sofosbuvir-Based Treatment in Renal Transplant Recipients With Hepatitis C in Pakistan.

OBJECTIVES: Direct-acting antiviral agents have recently been recommended in renal transplant recipients. Considering our previous encouraging responses with combined sofosbuvir and ribavirin in renal transplant recipients and the availability of daclatasvir, we aimed to evaluate the effectiveness and safety of sofosbuvir-based direct-acting antiviral agents in our population. MATERIALS AND METHODS: All renal transplant recipients who received sofosbuvir-based direct-acting antivirals from August 2015 to March 2018 were included in our study. Patients were treated with sofosbuvir and ribavirin for 24 weeks or with combined sofosuvir, daclatasvir, and ribavirin for 12 weeks. Patient demographics and baseline laboratory parameters were collected. Rapid virologic response, end of treatment response, and sustained virologic response at 12 weeks were analyzed. Statistical analyses were performed with SPSS software (SPSS: An IBM Company, version 20.0, IBM Corporation, Armonk, NY, USA). RESULTS: In our study group of 79 patients, mean age was 36.5 ± 10.2 years and 60 were men (78.5%). Fiftysix patients (70.9%) were treatment naive; of the remaining patients, 20 received interferon before transplant and 3 were treated with sofosbuvir and ribavirin after renal transplant. Genotype 1 was observed in 42 patients (53.2%), whereas mixed genotype (1 and 3) was shown in 10 patients (12.6%). Sixty-two patients (78.5%) received sofosbuvir and ribavirin, and 17 patients (21.5%) received sofosbuvir, daclatasvir, and ribavirin. End of treatment response was achieved in 78 recipients (98.1%). Anemia was observed in 13 patients (16.4%). CONCLUSIONS: Hepatitis C virus was successfully eradicated in renal transplant recipients who received a combination of sofosbuvir plus ribavirin or sofosbuvir, daclatasvir, and ribavirin. These combinations were effective and well tolerated in our study population, even in those with mixed genotype, with no major adverse events.

Correlation Between MELD and UNa/K Ratio in Predicting Renal Dysfunction in Cirrhotic Patients.

BACKGROUND: Renal dysfunction is one of the dreaded complications of cirrhosis. MELD is a validated chronic liver disease (CLD) severity scoring system. Urinary (U) Na/K ratio closely correlates with renal dysfunction in terms of low GFR in cirrhotic patients. PATIENTS AND METHODS: All consecutive patients with decompensated cirrhosis between the age of 18 to 70 years, of either gender, presenting in the outpatients' department of Sindh Institute of Urology and Transplantation, Karachi, from June 2015 to June 2017 were included. The MELD score was calculated and the UNa/K ratio less than 1 was taken as surrogate marker of renal dysfunction. Statistical analysis was performed by SPSS (version 20.0). RESULTS: A total of 71 patients were enrolled. The mean age was 43.79 years and majority were male (67.6%). The most common cause of liver cirrhosis was HCV, found in 42 (59.2%) patients. The mean CTP score was 10.48 ± 2.069 (range: 6-14) with majority of the patients following in class C, that is, 48 (67.6%). Mean MELD score was 21.75 ± 8.96 (range: 8-43). In 57 patients (80.3%), MELD score was > 15.The mean serum creatinine and mean serum sodium were 1.5 ± 1.1 mg/dl (range: 0.37-5.3) and 133.79 ± 6.9 mmol/L (range: 112-152), respectively. Mean urinary sodium and urinary potassium were 38.60 ± 46.64 mmol/L (range: 5-181) and 38.15 ± 23.9 mmol/L (range: 4.3-112), respectively. In majority of study population, UNa/K ratio was below 1, that is, in 52 patients (73.2%). Statistically significant correlation was documented between MELD score and UNa/K ratio (ɤ = 0.34, P = 0.004). CONCLUSION: The inverse correlation between MELD scores and UNa/K ratio indicates that patients with CLD and higher MELD scores might have renal dysfunction. This finding however should be corroborated by large scale studies.

Effectiveness of Sofosbuvir and Ribavirin for Eradicating Hepatitis C Virus in Renal Transplant Recipients in Pakistan: Where Resources Are Scarce.

OBJECTIVES: Although direct-acting antiviral agents have revolutionized hepatitis C virus treatment, these novel agents are not widely available in the developing world. Further, no treatment recommendation for renal transplant recipients includes these agents. We aimed to evaluate the effectiveness of sofosbuvir and ribavirin, the only direct-acting antiviral agents available in Pakistan, in renal transplant recipients. MATERIALS AND METHODS: All renal transplant recipients receiving sofosbuvir and ribavirin from August 2015 to March 2016 were enrolled in the study. Patients' demographics and baseline laboratory parameters were collected. Rapid virologic response, early virologic response, end-of-treatment response, and sustained virologic response at 12 and 24 weeks were analyzed. Statistical analyses were performed using IBM SPSS Statistics software, version 20.0. RESULTS: Of the 37 renal transplant recipients, the mean age was 37.2 ± 10.7 years and the majority (33 [89.2% ]) were men. Twenty-five patients were treatment naive; of the remaining 12 patients, 10 were responders, 2 were nonresponders, and 5 were relapsers to pretransplant hepatitis C treatment. The genotype most commonly seen posttransplant was genotype 1 (56.8%). Rapid virologic response was achieved in 33 patients (89.2%). Early virologic response, end-oftreatment response, and sustained virologic response at 12 weeks were achieved in all 37 patients (100%). Until the time of data collection, 14 patients had achieved a sustained virologic response at 24 weeks. No complications were noted during therapy. In 2 of 4 patients who developed decompensated cirrhosis, treatment led to the resolution of ascites. CONCLUSIONS: Sofosbuvir and ribavirin are well tolerated and effective in renal transplant recipients for eradicating hepatitis C virus. Their effectiveness is not limited to renal transplant recipients with genotypes 1, 2, 3, and 4 but also extends to those with mixed genotype (in this study, genotypes 1 and 3).

Living-Donor Liver Transplant Follow-Up: A SingleCenter Experience.

OBJECTIVES: Liver transplant is a definite treatment of decompensated liver disease. Because of the shortage of livers from deceased donors, living-donor liver transplant is becoming more common. Here, we analyzed our clinical experience in the follow-up care of these patients. MATERIALS AND METHODS: Liver transplant recipients seen at the Sindh Institute of Urology and Transplantation (Karachi, Pakistan) were included in this analysis. Baseline characteristics and follow-up events were recorded. RESULTS: Our study population included 76 liver transplant patients registered at our clinic. Median age was 42 years, with 62 patients (81.6%) being males. The most common indication of transplant was hepatitis C virus-related cirrhosis (42 patients; 55%), followed by hepatitis B-hepatitis D virus coinfection (8 patients; 10.5%). Anastomotic biliary stricture developed in 16 patients (21.1%),which required biliary stenting. Biliary leak developed in 5 patients (6.6%), and renal cell carcinoma developed in 1 patient. Two recipients died due to hepatitis C virus-related fibrosing cholestasis hepatitis and pulmonary com plications. Posttransplant diabetes mellitus developed in 36 (47.1%), hypertension in 17 (38.6%), and dyslipidemia in 19 patients (25%). Of 42 patients with hepatitis C virus infection, 26 were treated with pegylated interferon and ribavirin, of which 65.3% achieved sustained virologic response at 24 weeks. The other 16 patients received sofosbuvir com - bined with ribavirin for 24 weeks. A sustained virologic response at 12 weeks was achieved in 5 patients, with not yet determined results in the remaining patients. Seven patients were lost to follow-up. CONCLUSIONS: Hepatitis C-related cirrhosis was the most common indication for liver transplant, and infection recurrence was observed in our patients. Biliary anastomotic stricture formation was the most prevalent complication after transplant. As liver transplants are becoming more widely available for Pakistani patients at home and abroad, gastroenterologists and trainees in our country should be sensitized, educated, and skilled in the posttransplant care of these patients.

Noninvasive Clinical Predictors of Portal Hypertensive Gastropathy in Patients with Liver Cirrhosis.

BACKGROUND AND OBJECTIVES: Portal hypertensive gastropathy (PHG) is described endoscopically as "mosaic-like appearance" of gastric mucosa with or without the red spots. It can only be diagnosed by upper gastrointestinal (GI) endoscopy. The aim of this study was to determine the diagnostic accuracy of platelet count to spleen diameter ratio (PSR) and right liver lobe diameter to albumin ratio (RLAR) in the detection of PHG using upper GI endoscopy as a gold standard in patients with liver cirrhosis. MATERIAL AND METHODS: This cross-sectional study was conducted in the Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi. All consecutive patients with ages 18-65 years who were screened using esophagogastroduodenoscopy (EGD) to exclude esophageal varices were enrolled. At the same time, findings related to PHG were noted. After informed consent, all the patients had blood tests including platelet count and albumin and abdominal ultrasound determining spleen diameter and right liver lobe diameter. RESULTS: Out of 111 patients, 59 (53.15%) were males with a mean age of 44 ± 12.61 years. Rate of PHG was observed in 84.68% (94/111) cases confirmed by EGD. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of PSR were 87.23%, 5.88%, 83.67%, 7.69%, and 74.7%, respectively, and those of RLAR were 28.72%, 70.59%, 84.38%, 15.19%, and 35.14%, respectively. CONCLUSION: PSR is better predictor of PHG than RLAR but at the expense of relatively lower specificities and NPV likely because of underlying pathophysiology (portal hypertension) which is similar for esophageal varices, PHG, and ascites.

Refractory coeliac disease a reality: views from Pakistan.

Refractory coeliac disease (RCD) is described as persistence or recurrence of signs and symptoms of malabsorption with small-intestinal villous atrophy despite being on a strict gluten-free diet (GFD) for more than 12 months. RCD is a diagnosis of exclusion. There are two types of RCD, based upon the immunohistochemical features (presence of intraepithelial lymphocytes), response to treatment and prognosis. The treatment of RCD includes GFD and immunosuppressive agents. We hereby present a case of refractory celiac disease type II in a young man who later went on to develop Addisonian crisis and did not survive.

Upper Gastrointestinal Bleeding in Patients with End Stage Renal Disease: Causes, Characteristics and Factors Associated with Need for Endoscopic Therapeutic Intervention.

BACKGROUND AND OBJECTIVES: The risk of upper gastrointestinal bleeding (UGIB) is increased among the end-stage renal disease (ESRD) patients. The aim of the current study was to describe the causes and characteristics of UGIB in ESRD patients at our center and to assess the need for endoscopic therapeutic intervention (ETI) using Rockall (RS) and Glasgow Blatchford scores (GBS). MATERIAL AND METHODS: All patients with ESRD and UGIB with age ≥14 years were included. Frequencies and percentages were computed for categorical variables. Chi square test or Fischer's exact test was used for statistical analysis. RESULTS: A total of 59 subjects had a mean age of 47.25 ± 15 years.The most common endoscopic findings seen were erosions in 33 (55.9%) patients, followed by ulcers in 18 (30.3%) patients. ETI was required in 33 (55.9%) patients, which included adrenaline injection in 19 (32.3%), hemoclip in 9 (15.2%) and argon plasma coagulation in 5 (8.4%) patients. Factors associated with the need of ETI were identified as: a combined presentation of hematemesis and melena (P=0.033), ulcer (P=0.002) and associated chronic liver disease (P=0.015). Six (10.1%) patients died. Death was more common if ETI was not performed (P=0.018). CONCLUSION: ETI was more commonly required in patients on maintenance hemodialysis with UGIB, who had presence of combined hematemesis and melena, ulcers and associated chronic liver disease. A Glasgow Blatchford score of >14 was helpful in assessing the need for ETI in these patients.

Clinicopathological Study of Seronegative Celiac Disease in Adults in Pakistan: A Pilot Study.

BACKGROUND Celiac disease (CD) is usually missed, if the serology is negative. We aimed to evaluate the clinicopathological characteristics of seronegative CD (SNCD) and its response to gluten-free diet (GFD) in adult patients. METHODS This observational study was carried out at the Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan from 2009 to 2015. All consecutive adult patients (≥17 years) with features of marked villous atrophy (Marsh class≥III) on duodenal biopsy, negative tissue transglutaminase IgA and IgG antibodies (anti-tTg IgA and IgG) and human leukocyte antigen (HLA) DQ2 or DQ8 serotypes were studied. Clinical characteristics, laboratory parameters, and response to GFD were analyzed by SPSS software version 20. Median and interquartile range (IQR) were used for summarizing quantitative data. Frequency (percentages) was used for qualitative data. RESULTS A total of 12 patients with median age of 31.5 years (IQR: 19.75-46.75 years), of whom five (41.6%) were men were studied. The presenting complaints were: weight loss in 11 (91.6%) and abdominal pain in 9 (75%) patients. Anemia was observed in 10 (83.3%) patients with median hemoglobin of 9.5 g/dL (IQR: 6.3-13.25 g/dL). Median alanine transaminase (ALT) was 21 U/L (IQR: 13-27 U/L) and median albumin was 3 g/dL (IQR: 2.4-3.6 g/dL). Anti-tTg IgA and IgG were negative in all patients. HLA DQ serotyping showed homozygous DQ2 and DQ8 in four and one patients, respectively; while heterozygous DQ2 and DQ8 in five and two patients, respectively. All patients were advised to receive GFD. Nine (75%) patients showed complete clinical response. Two patients were non-compliant and one with non-alcoholic fatty liver disease (NAFLD)-related cirrhosis had partial clinical response. Out of the nine responders, two patients showed response within 6 months while the remaining showed improvement over a year period. CONCLUSION The diagnosis of SNCD is rewarding as it responds favorably to GFD in most patients. HLA serology provides an important tool for diagnosis of this entity.