Autoimmune pancreatitis with extraocular muscles involvement.

Autoimmune pancreatitis is characterised by diffuse enlargement of pancreas, narrowing of pancreatic duct, lymphoplasmacytic infiltrations and fibrosis. The disease is responsive to corticosteroid. We report the case of a 32-year-old male who presented with unilateral exophthalmos and obstructive jaundice secondary to pancreatic head mass and biliary tract stricture. Serum immunoglobulin G level was raised with a very high immunoglobulin G4 subclass. Ophthalmological imaging revealed unilateral thickening of extraocular muscles. The patient responded well to corticosteroid with resolution of biliary strictures, pancreatic head mass and exophthalmos.

Clinical presentation and predictors of survival in patients with Budd Chiari syndrome: experience from a tertiary care hospital in Pakistan.

OBJECTIVE: To determine aetiology, clinical presentation and predictors of survival in Budd Chiari Syndrome patients. METHODS: The prospective observational study based on non-probability convenient sampling was conducted at the Sindh Institute of Urology and Transplantation (SIUT), Karachi, and comprised Budd Chiari Syndrome patients between January 2004 and December 2013. The patients were evaluated for onset of symptoms, causes, mode of presentation and predictors of survival. SPSS 20 was used for statistical analysis. RESULTS: Of the 25 patients, 16(64%) were males, and 16(64%) belonged to the paediatric age group. Overall age range was 2-50 years with a mean of 14.7 ± 12.41 years. Presentation was chronic in 14(56%) patients, acute in 10(40%) and acute on chronic in 1(4%). Commonest morphological abnormality involved was hepatic veins alone in 14(56%). Probable aetiologies were hypercoagulable states in 21(84%) patients, infections in 2(8%) and malignancy in 1(4%). Among hypercoagulable states, protein C deficiency was the commonest, affecting 9 (36%) patients. Seven (28%) patients died; acute 4 (16%) and chronic 3 (12%). Causes of death included sepsis 4 (16%), fulminant hepatic failure 1 (4%), gastrointestinal bleeding 1 (4%), and bleeding from liver biopsy site 1 (4%). Poor survival was associated with bilirubin > 5 mg/dl (p < 0.031), serum alanine transaminase > 40 U/L (p < 0.005), serum albumin < 2.8 g/dl (p < 0.008), Child-Turcotte-Pugh score > 10 (p < 0.001) and absence of varices (p < 0.025). Cox regression analysis failed to show any significant independent predictors of survival. CONCLUSION: Budd Chiari Syndrome affected young patients more frequently and was associated with high mortality. The commonest aetiology was hypercoagulable state. Survival was poor in patients with decompensated liver disease and those with an acute clinical presentation.

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.

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.

Non-alcoholic fatty liver disease among visitors to a hepatitis awareness programme.

BACKGROUND AND AIMS: Non-alcoholic fatty liver disease (NAFLD) is rapidly becoming the most common liver disease worldwide. The present study aimed to evaluate the prevalence of NAFLD among the visitors screened on World Hepatitis Day 2010 and 2011. METHODS: Attendees of the hepatitis awareness programme (n = 928) were offered screening to rule out liver ailments. The participants were evaluated on history, body mass index (BMI) measurement, blood tests for hepatitis B surface antigen (HBsAg), anti-hepatitis C virus (anti-HCV), alanine aminotransferase (ALT) levels and abdominal ultrasound (US). RESULTS: On ultrasonography, fatty liver was present in 142 of 806 subjects who were found negative for viral serology and had no history of regular alcohol intake. This accounted for 15.3% of all the visitors. Comparing them with the rest of the serology-negative alcohol-negative subjects, there were more housewives (p = 0.005) with fatty liver. They were older with mean age 43.3 +/- 12.1 years compared to 33.1 +/- 16.1 years (p = 0.000) in patients without fatty liver and had a higher BMI 28.6 +/- 6.0 kg/m2 vs. 22.2 +/- 5.3 kg/m2 (p = 0.000). Regression analysis suggested BMI as the only significant independent risk factor (p = 0.000). However, 32 subjects with fatty liver had BMI < 25 kg/m2 and 6 of them had diabetes mellitus. Thirty-one subjects (21.8%) with fatty liver had raised ALT level (p = 0.000) and possible non-alcoholic steatohepatitis (NASH) which accounts for 3.34% (31/928) of the total visitors. CONCLUSIONS: This analysis reveals that our general population is at risk of having NAFLD and NASH and these should be looked into even in non-obese persons.

Adverse events following transarterial chemoembolization for hepatocellular carcinoma and factors predicting such events.

OBJECTIVE: To document the adverse events after transarterial chemoembolisation and factors predicting such events. METHODS: The prospective observational study was conducted at the Sindh Institute of Urology and Transplantation, Karachi, from November 2009 to November 2011. All patients diagnosed as hepatocellular carcinoma were included in this study. Complications developing within the first 6 weeks of the procedure were recorded. SPSS version 16 was used for statistical analysis. RESULTS: Of the total 80 patients, 59 (73.8%) were male. The overall mean age was 52.25 +/- 9.24 (range: 28-76 years). Most common etiology was hepatitis C related cirrhosis in 55 (68.8%). Adverse events developed in 46 (57.5%) patients. Post transarterial chemoembolisation syndrome was seen in 37 (46.3%). Of those with the syndrome, 24 (64.8%) patients had no additional complications, while 3 (8%) had renal dysfunction, 2 (5%) hypertensive crisis, and 1 (2.7%) patient each had urinary tract infection, pneumonia and sepsis. Decompensation of cirrhosis occurred in 6 (7.5%) patients of whom 3 (50%) developed sepsis and died. The syndrome was associated with tumour size > 5cm (p = 0.001) and higher dose of lipoidol (p = 0.0001). Decompensation of cirrhosis was associated with low basal albumin (p = 0.002), advanced basal child turcotte pugh (p = 0.005) and model for end-stage liver disease (p = 0.006) scores. CONCLUSION: Transarterial chemoembolisation, though generally safe, may lead to serious complications in patients with advanced liver disease. Post-procedure syndrome was associated with increased tumour size and lipoidol dose.

Three years old child with juvenile hyaline fibromatosis presenting with rectal bleeding.

Juvenile hyaline fibromatosis is a rare inherited autosomal recessive disorder which is caused by mutation of CMG2 gene on chromosome 4q21. Mutation of this gene protein can disrupt the formation of basement membranes. Hyalinization of various body tissues like skin, joints, and bones leads to development of skin papules, gingival hyperplasia, osteolytic lesions in bones, and joint contractures. We had a case of a 3 years old female child with Juvenile Hyaline Fibromatosis who presented with rectal bleeding. She had a bleeding mucocutaneous lesion in anal canal along with papullonodular lesions on the face, gingival hypertrophy and flexion contractures of small joints of hands and feet. Excision of the anal lesion revealed histopathological features of Juvenile Hyaline Fibromatosis.

Job's syndrome with an atypical presentation.

Hyper Immunoglobulin E syndrome also called Job's or Buckley Syndrome is a rare primary immunodeficiency disease characterized by elevated serum IgE levels (> 2000 IU/ml), recurrent infections and eosinophilia. Other features include coarse facies and non-immunologic abnormalities of the dentition, bones, vasculature and connective tissues. We are reporting a case of a twenty four years old male with coarse facies who presented with severe pallor and upper gastrointestinal bleeding. Investigations revealed markedly elevated serum IgE levels (11,800 IU/ml), severe anaemia, esophageoduodenal erosions, Helicobacter pylori (H. pylori) gastritis and oro-esophageal candidiasis.

Coeliac disease and multiple immunodeficiencies: case report of a diagnostic dilemma.

Coeliac disease (CD) often coexists with other autoimmune and primary immunodeficiency diseases (PID), creating a problem in timely diagnosis and management. An unusual case of coeliac disease that was difficult to diagnose and manage because of its unusual clinical presentation. Initially diagnosed as celiac disease but showed poor response to standard therapy is reported. Frequent attacks of opportunistic infections led to immunodeficiency work-up that revealed natural killer cell (NK) deficiency with low serum IgA and IgG2 levels. The patient eventually succumbed to recurrent infections. The co-existence of PID is unusual in a patient with CD. This case report highlights the importance of investigating PID in patients with autoimmunity.

Thalamic and midbrain infarct during transarterial chemoembolization of hepatocellular carcinoma.

Transarterial chemoembolization (TACE) is a well accepted and a relatively safe procedure, however certain rare and serious complications may occur during or after the procedure with profound morbidity and mortality. Only a few cases have been reported describing cerebral embolization during the procedure. We are reporting a case in which cerebral lipiodal embolization occurred in the second session of TACE, during the procedure and without any evidence of pulmonary embolism causing midbrain and thalamic infarct. The possible explanation could be an aberrant connection between the hepatic and pulmonary vessels which might have developed due to the involvement of the diaphragm, pleura and pulmonary vessels after the first session of TACE.

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.

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.

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.

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.