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.

The Use of Albumin-to-bilirubin Score in Predicting Variceal Bleed: A Pilot Study from Pakistan.

Variceal hemorrhage is a serious consequence of patients having chronic liver disease (CLD). Various scores exist that predict the outcome for non-variceal bleed. However, only a few scores evaluate patients with variceal bleed. We, in our study, evaluated 48 cirrhotics who presented with variceal gastrointestinal (GI) bleed over a period of 3 months. Majority of these were males and the most common etiology was hepatitis C infection. The main presenting complaints were hematemesis seen in 39.6% followed by hematemesis and melena in 31.25%. Most bleeding episodes were secured via banding in 62.5% followed by injection of histoacryl in 12.5%. Finally, Child-Turcotte-Pugh (CTP), model for end-stage liver disease (MELD), albumin-to-bilirubin (ALBI), and the ABC score were applied and none correlated with the presence of esophageal varices. However, the ALBI score did correlate with the presence of tachycardia in our study, a pertinent sign of upper GI bleed. How to cite this article: Majid Z, Khan SA, Akbar N, et al. The Use of Albumin-to-bilirubin Score in Predicting Variceal Bleed: A Pilot Study from Pakistan. Euroasian J Hepato-Gastroenterol 2022;12(2):77-80.

Factors Influencing Quality of Life in Patients with Gastroesophageal Reflux Disease in a Tertiary Care Hospital in Pakistan.

OBJECTIVE: To assess the impact of gastroesophageal reflux disease (GERD) on quality of life (QOL) in local population. STUDY DESIGN: Cross-sectional, descriptive study. PLACE AND DURATION OF STUDY: Hepatogastroenterology Department, Sindh Institute of Urology and Transplantation, Karachi, from June 2016 to December 2017. METHODOLOGY: Patients with GERD visiting the outpatients' clinics were enrolled. Those who had history of dysphagia, malignant disease, anemia, weight loss, cardiovascular diseases, renal failure, cirrhosis, rheumatologic diseases and patients taking non-steroidal anti-inflammatory drugs (NSAIDS) or steroids during the last two weeks were exluded. A predesigned questionnaire was used to calculate GERD impact scale (GIS) score, with a value of >18 being considered as impaired QOL. Chi-square test was used to identify factors associated with impaired QOL. RESULTS: A total of 782 patients with mean age of 37.3 ±8.99 years (range: 18-65 years) were studied. Majority (505, 64.5%) were younger than 40 years of age. Predominant study population were females, i.e. 413 (52.8%). Among these, 127 (16.2%) patients had symptoms lasting more than a year, 132 (16.9%) were smokers, 82 (10.5%) had diabetes mellitus (DM), and 63 (8.1%) had hypertension. A total of 297 (38%) patients had impaired QOL. On logistic regression analysis, the impaired QOL was associated with age greater than 40 years (p=0.001), body mass index (BMI) >25 Kg/m2 (p= 0.001), smoking (p=0.001), hypertension (p=0.001) and diabetes mellitus (p=0.001). CONCLUSION: A significant proportion of patients had impaired QOL due to GERD. Factors affecting QOL in such patients were higher age, increased BMI, history of smoking, hypertension and DM.

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.

Frequency of Hepatotropic Viruses Leading To Deranged Liver Function Tests in Renal Transplant Recipients.

OBJECTIVES: The preferred modality for renal replacement therapy is renal transplantation. Marked improvements in early graft survival and long-term graft function have made renal transplantation a more cost-effective alternative to dialysis. The presence of liver disease in the posttransplant period adversely affects graft function and survival. Determining the cause of deranged liver function tests can be helpful in treating the underlying cause, leading to improved graft survival and overall quality of life in patients after renal transplant. Here, we determined the frequency of hepatotropic viral infections leading to deranged liver function tests in renal transplant recipients. MATERIALS AND METHODS: Our study included 132 patients with deranged liver function tests who had undergone renal transplant within the past 6 months. Reactivity and nonreactivity of hepatotropic viruses leading to deranged liver function tests were recorded. RESULTS: Average age of patients was 37.17 ± 8.75 years. There were 84 male (63.64%) and 48 female (36.36%) patients. Rates of hepatitis C virus antibodies and hepatitis B surface antigen were 62.88% (83/132) and 37.12% (49/132), respectively, whereas no patients had hepatitis E virus immunoglobulin M antibodies or hepatitis A virus immunoglobulin M antibodies. CONCLUSIONS: Among the hepatotropic viral infections leading to deranged liver function tests in renal transplant recipients, hepatitis B virus accounted for a small fraction. In contrast, hepatitis C virus was highly prevalent in transplant recipients who developed deranged liver function tests. Renal transplant recipients with hepatic viral infections have worse patient and allograft survival after transplant compared with noninfected renal transplant recipients. We recommend that transplant candidates be screened for hepatitis B and C virus infection, thus allowing increased graft survival and improved quality of life in renal transplant recipients.

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).