National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma.

A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26, 2023, at the Pakistan Kidney and Liver Institute & Research Centre (PKLI & RC) after initial consultations with the experts. The Pakistan Society for the Study of Liver Diseases (PSSLD) and PKLI & RC jointly organised this meeting. This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma (hCCA). The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients. This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation. The diagnostic and staging workup includes high-quality computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography. Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis. However, histopathologic confirmation is not always required before resection. Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging. The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification. Selected patients with unresectable hCCA can be considered for liver transplantation. Adjuvant chemotherapy should be offered to patients with a high risk of recurrence. The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions. Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage. Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

Management of Tacrolimus-Induced Toxicity With Normal Serum Levels After Liver Transplant.

Drug-induced liver injury after liver transplant occurs in 1.7% of patients. Tacrolimus is an effective immunosuppressant that is used to treat acute rejection. Although rare, it can cause toxicity, which is demonstrated by cholestatic liver injury. Here, we present a case of a young male patient who was diagnosed with Wilson disease, had penicillaminechelating therapy, and underwent living related liver transplant. Within 1 month posttransplant, he developed deranged, predominantly cholestatic pattern liver function tests. Laboratory parameters showed total bilirubin of 1.12 mg/ dL, alanine aminotransferase of 553 IU/L, gammaglutamyltransferase of 624 IU/L, and tacrolimus level of 10.2 ng/mL. After thorough evaluation, a liver biopsy was performed. Liver biopsy showed hepatocellular necrosis with centrilobular cholestasis without any evidence of graft rejection. However, with normal level of tacrolimus, the biopsy was suggestive of drug-induced liver injury. Thus, tacrolimus dose was reduced, resulting in improved liver function tests and patient discharge from the hospital. Tacrolimus is an effective immunosuppressant after liver transplant and has the ability to treat early acute rejection. The patient's liver biopsy showed hepatocellular necrosis with centrilobular cholestasis without any evidence of graft rejection. Cholestatic liver injury after tacrolimus usually resolves after dose reduction or by switching to another agent. With demonstrated tacrolimus-induced toxicity in liver transplant recipients, despite normal serum levels, transplant physicians should keep high index of suspicion regarding toxicity in the posttransplant setting.

Pancreatic Tuberculosis mimicking as a Pancreatic Mass Lesion.

Tuberculosis is a global health problem and commonly affects the respiratory system. The involvement of the pancreas in this disease is a rare event. We hereby report a case of a young male who presented with right hypochondrial pain along with significant weight loss. Further workup revealed a raised erythrocyte sedimentation rate along with a pancreatic mass lesion on the CT scan. Endoscopic ultrasound-guided biopsy of the pancreatic lesion revealed evidence of caseation necrosis along with epithelioid granuloma, findings suggestive of tuberculosis. He was started on anti-tuberculous therapy for 6 months and a repeat CT scan showed complete disappearance of the mass lesion and resolution of symptoms. Key Words: Tuberculosis, Pancreatic mass, Endoscopic ultrasound, Biopsy.

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.

Revolution in the diagnosis and management of hepatitis C virus infection in current era.

Chronic hepatitis C virus (HCV) infection is a major global public health problem, particularly in developing part of the world. Significant advances have been made in the early diagnosis and treatment of the disease. Its management has been particularly revolutionized during the past two decades. In this review, we summarize the major advances in the diagnostic and management armamentarium for chronic HCV infection. The focus of the present review is on the newer directly acting anti-viral agents, which have revolutionized the management of chronic HCV infection. Management of uncomplicated chronic HCV infection and of specific complications and special at-risk populations of patients will be covered in detail. Despite the advent and approval of highly effective and well tolerable oral agents, still many challenges remain, particularly the affordability, the equitable distribution and access to later drugs. The World Health Organization aims to eliminate viral hepatitis including HCV by 2030 since its poses a major public health threat. There is an urgent need to ensure uniform and early access to diagnostic and therapeutic facilities throughout the world if the later goal has to be realized.

DAA treatment failures in a low-resource setting with a high burden of hepatitis C infections: a case series.

Globally, 58 million people are living with hepatitis C virus (HCV) infection and 1.5 million new patients are infected every year. The advent of direct acting antivirals (DAAs) has revolutionized the treatment of HCV, opening the door to the ambitious World Health Organization HCV infection elimination strategy by 2030. However, emerging resistance to DAAs could jeopardize any hope of achieving these targets. We discuss a series of 18 patients within a resource-limited setting, who after failing standard sofosbuvir-daclatasvir-based regimen also failed to respond to advanced pan-genotypic treatment regimens, i.e. sofosbuvir-velpatasvir, sofosbuvir-velpatasvir-ribavirin and sofosbuvir-velpatasvir-voxilaprevir. To avoid the spread of refractory HCV strains within the existing epidemic, we call for increased attention and research regarding patients failing treatment on standard pan-genotypic regimens and the spread of HCV-resistant strains within the communities.

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.

Digestive tract neoplasms in young individuals: Demographics, staging and risk factors.

BACKGROUND: Digestive tract neoplasms (DTN) have become increasingly common worldwide among young individuals (YIs) over the last few decades. AIM: Aim of this research was to study the types, demographics, stage at presentation and risk factors of digestive tract neoplasms in young individuals. METHODS AND RESULTS: In this cross-sectional study, YI (ie, ≤40 years) presenting with any DTN including gastrointestinal neoplasms (GIN), hepatobiliary neoplasms (HBN), periampullary neoplasms (PAN) and others from June 2016 to May 2020 were included. Baseline laboratory tests, tissue diagnosis and staging were performed while risk factors were documented. A total of 163 patients were included in the study, of whom 82 (50.3%) were males. Mean age was 29.9 (±9.57) (range: 8 months-40 years). Most DTN (93.3%; n = 152) were malignant. The commonest neoplasms were lower GIN (LGIN) 52 (31.9%), followed by HBN 46 (28.2%), upper GIN (UGIN) 44 (27%) and PAN 18 (11%). Commonest among LGIN were rectal 37; among HBN: hepatocellular cancer (HCC) 9, cholangiocarcinoma (CC) 9; and among UGIN: esophageal 25 and stomach 14. Rectal cancers were mostly sporadic (82.7%) with frequent signet ring cell histology (40.5%), and affected relatively younger ages compared to upper GIN and PAN. GIN were mostly locally advanced with higher resectability (LGIN 90.4%; UGIN 79.5%) while HBN were more advanced with lower resectability (HCC [44.4%]; CC [33.3%]). Poor dietary habits and poor socioeconomic status were common with UGIN (63.6%, 50%) and HBN (56.5%, 54.3%), respectively. CONCLUSION: The commonest DTN among YI were LGIN followed by HBN, UGIN and PAN. Rectal cancers affected relatively younger ages and were mostly sporadic. HBN were more advanced in stage and unresectable compared to GIN. Poor dietary habits and poor socioeconomic status may be important contributors in carcinogenesis.

Autoimmune Hepatitis: Clinical Characteristics and Predictors of Biochemical Response to Treatment.

BACKGROUND AND OBJECTIVES: Autoimmune hepatitis (AIH) is an important cause of chronic liver disease. Aim of this study was to evaluate the clinical characteristics and factors predicting response to treatment in patients with AIH. METHODS: In this prospective observational study, all patients diagnosed with AIH from 2017 to 2019 were included. Biochemical response to the treatment was checked three months after the start of the treatment. Response was considered good if transaminases normalized, or poor if either remained persistently elevated or improved partially. RESULTS: Of the total 56 patients, 41 (73.2%) were females. Mean age was 29.5 (±16.9) years. About half (53.6%; n = 30) the patients were aged < 25 years and majority [47 (83.9%)] were cirrhotic. Autoimmune serology was negative in 20 (35.7%). Seronegativity was associated with severe necroinflammation (P = 0.015) and esophageal varices (P = 0.021). Response to treatment was good in 34 (60.7%). Bivariate analysis showed that good response to treatment was associated with pre-treatment serum IgG level > 20 g/L (P = 0.024), presence of pseudorosettes on histopathology (P = 0.029) and three months post-immunosuppression serum total bilirubin < 2mg/dL (P < 0.001). Multivariate logistic regression analysis showed that only pre-treatment serum IgG >20 g/L (P = 0.038) and post-treatment serum total bilirubin <2 mg/dL (P = 0.004) were independent predictors of good response to treatment. CONCLUSION: Majority of AIH patients in our study were young and cirrhotic. A negative autoimmune serology does not rule out AIH and liver biopsy may be required to confirm the diagnosis. Seronegative AIH rapidly progresses to advanced liver disease. Response to treatment is good with pre-treatment IgG > 20g/L and post-treatment total bilirubin < 2 mg/dL.

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.

Intrahepatic Portosystemic Shunt in a Young Female: Views from a Developing Country.

A young female presented to us with abdominal distension along with pedal edema. She had no prior medical or surgical history apart from a caesarean section done a few years prior. Initial workup showed low hemoglobin, low serum albumin and slight raised ESR. Her LFTs were slightly deranged. Ultrasound abdomen had evidence of portal hypertension along with splenomegaly. While ultrasound hepatic Doppler revealed a portosystemic shunt between the portal vein and the left hepatic vein, with a shunt ratio of 7.1%. CT scan abdomen confirmed these findings and a diagnosis of Type III intrahepatic portosystemic shunt and spleno-renal shunt was made. Since the patient was currently asymptomatic, she was advised regular follow-ups and was managed conservatively.

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.

Primary hepaticobiliary tuberculosis mimicking gall bladder carcinoma with liver invasion: a case report.

Primary hepatic tuberculosis is a rare presentation and sporadically reported in the literature, mostly from our part of the world. Sometimes the presentation can be atypical and mimics hepatic tumor and poses diagnostic challenge. We, herein, present a case of a 58-year-old man who presented to us with abdominal pain and weight loss. Raised serum alkaline phosphatase (ALP) and imaging raised a suspicion of gall bladder carcinoma with hepatic invasion. Peroperative frozen section revealed hepatic chronic granulomatous inflammation with caseous necrosis consistent with the diagnosis of hepatic tuberculosis. Surgery was postponed and he was put on antituberculous treatment. It is important to consider tuberculosis in the differential diagnosis of the space occupying lesion of liver in a patient with vague symptoms and abnormal findings on imaging.

To determine the prognostic value of the albumin-bilirubin grade (ALBI) in patients underwent transarterial chemoembolization for unresectable hepatocellular carcinoma.

AIM: We aimed at determining the prognostic value of the albumin-bilirubin grade (ALBI) in patients undergoing transarterial Chemoembolization for unresectable Hepatocellular carcinoma. BACKGROUND: Various noninvasive liver reserve markers are used to predict the severity of liver injury. The role and probability of these markers in predicting the prognosis of patients with hepatocellular carcinoma (HCC) is still unknown. METHODS: Patients who underwent TACE from 2013 to 2017 were included. Patient's age, gender, cause of cirrhosis, ALBI Grade along with the site, size and number of tumors were recorded. Radiological response to TACE was assessed by CT scan at 1 and 3 months after the procedure, respectively. Survival assessment was performed and all patients were assessed for survival until the last follow-up. RESULTS: A total of 71 patients were included. Majority of them were male (80.3 %). The mean tumor size of 6 ± 3.9 cm. Majority of patients (54.9 %) had a single lesion and it was mostly localized to the right lobe (60.5 %). The most common cause of chronic liver disease was HCV (65.3%). Median Child class score (CTP) and MELD score were 7 and 10, respectively. Ascites was treated prior to TACE in 12 patients (16.9 %).Mean ALBI score in the study population was -1.59 ± 0.69, with the majority (49. 2 %) falling in grade 2. The mean duration of survival at the last follow up was of 12.1 ± 12.14 months (1- 49).Univariate analysis showed serum albumin (p = 0.003), serum bilirubin (p = 0.018), CTP score (p = 0.019), ALBI grade (p = 0.001) and presence of varices (p = 0.04) to be the main predictors of 6 months survival after TACE. On Cox analysis, only ALBI score (p = 0.038) showed statistical significant association. CONCLUSION: ALBI grade may serve as a surrogate marker in predicting the prognosis of HCC patients undergoing Transarterial Chemoembolization.

Non alcoholic fatty liver disease in a young male with celiac disease.

A young emaciated male, known case of celiac disease came with complaints of diarrhea along with 5kgs of weight loss in 3 months' time. He had severe electrolyte abnormalities along with low albumin, low calcium and a high phosphate with deranged liver function test. Ultrasound abdomen had shown fatty liver. Nutrition consult was sought and he was found to have a BMI of 6.8kg/m2. He was started on nutrition support along with supportive therapy, which resulted in weight gain and improvement in his condition.

Intestinal Spirocheteosis in a Patient with Celiac Disease.

A young girl presented to us with recurrent diarrhea along with a history of 5 kg weight loss in one year. On examination, she appeared pale, while her laboratory reports showed a low hemoglobin, mean corpuscular volume (MCV) and serum albumin. Her erythrocyte sedimentation rate (ESR) was slightly raised with her iron profile suggestive of iron deficiency anemia. Viral markers, human immunodeficiency virus (HIV) serology along with thyroid profile were all unremarkable. There was no history of tuberculosis, and purified protein derivative (PPD) skin test was also negative. Computed tomography (CT) abdomen showed thickening of the terminal ileum with multiple enlarged lymph nodes. An esophagogastroduodenoscopy (EGD) along with colonoscopy was done. Multiple biopsies were taken, which were suggestive of sprue along with intestinal spirochetosis. Her tissue transglutaminase (TTG) was negative while deamidated gliadin peptide (DGP) was positive. She was kept on gluten-free diet and started on tablet metronidazole. This case shows that intestinal spirochetosis should be kept in mind in patients belonging to lower socio-economic status, who present with chronic diarrhea symptoms.

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.

The use of Karnofsky Performance Status (KPS) as a predictor of 3 month post discharge mortality in cirrhotic patients.

AIM: Is Karnofsky Performance Status (KPS) a predictor of 3 month post discharge mortality in cirrhotic patients? BACKGROUND: Cirrhotic patients often experience an abrupt decline in their health, which often leads to frequent hospitalization and can cause morbidity and mortality. Various models are currently used to predict mortality in cirrhotics however these have their limitations. The Karnofsky Performance Status (KPS) being one of the oldest performance status scales, is a health care provider-administered assessment that has been validated to predict mortality across the elderly and in the chronic disease populations. METHODS: We used the KPS performance status scale to envisage short-term mortality in cirrhotic and HCC patients who survive to be discharged from hospital. RESULTS: Our study showed that KPS one week post-discharge, child pugh score, hospital stay, international normalized ratio, serum albumin, total bilirubin and serum creatinine showed statistical significance on univariate analysis. On multivariate analysis, KPS was found to be statistical significant predictor of 3-month mortality. CONCLUSION: Hence KPS can be utilized to identify cirrhotic patients at risk of 3-month post discharge mortality.

Clinical presentation of autoimmune hepatitis in Pakistani children.

INTRODUCTION: Autoimmune hepatitis (AIH) is one of the most common disorder resulting in end stage liver disease (ESLD) among children. Scarce data is available in this regard from Pakistan. In this study we have analyzed clinical and biochemical parameters of children suffering from this disorder. METHODS: It was a cross sectional study conducted in the Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation (SIUT) Karachi from January 2005 to June 2016. Patients aged up-to 18 years were included. AIH was diagnosed by using International Autoimmune hepatitis group (IAIHG) pre-treatment and simplified AIH score. Patients with both probable and definite score were included. Biochemical, serological, sonographic and demographics were recorded at the time of diagnosis, liver biopsy was also performed in most of the cases. Data was analyzed by using SPSS ver.20 and p-value of < 0.05 was considered significant. RESULTS: Total 51 patients were enrolled most of them were females (68.6%). Mean age of presentation was around 10 years. Males had statistically significant earlier age of presentation, p-value = 0.007. The most common presenting complain was jaundice. Hypergammaglobulinemia is seen in almost all patients. Type I AIH was the most common entity while Type II AIH was statistically more significant in males p-value = 0.019. Raised GGT was also seen in male patient specifically in Type II AIH, p-value = 0.001. CONCLUSION: Autoimmune hepatitis predominantly affects female children who have late age of presentation as compare to the males. Type I AIH was the most common while Type II AIH was more common in males and they also had raised GGT.