Whipple's disease is a rare chronic multi-systemic infection, caused by Gram-positive bacillus Tr... more Whipple's disease is a rare chronic multi-systemic infection, caused by Gram-positive bacillus Tropheryma whipplei. The infection usually involves the small bowel, but other organs may also be involved. The diagnosis is often challenging and can only be made on histopathological examination. This report describes 2 patients presenting with abdominal pain and weight loss who finally were diagnosed to have Whipple's disease. One of the patients was a renal transplant recipient. To the best of authors' knowledge, no case of Whipple's disease has yet been reported in Pakistan. The diagnosis were made on the basis of histopathological evaluation of duodenal biopsies. The cases underscore the need for diligent histopathological evaluation of the upper gastrointestinal biopsies and a high index of suspicion for an accurate diagnosis of the condition. The approach to the diagnosis and management of the condition is discussed.
Journal of Advances in Medicine and Medical Research
Introduction: Endoscopy for the screening of esophageal varices (EVs) is costly and cannot be per... more Introduction: Endoscopy for the screening of esophageal varices (EVs) is costly and cannot be performed in remote areas with limited resources. Recently, certain non-invasive cost effective models have been proposed for the prediction of EVs but have failed recommendation on a larger scale. EVendo score is a recently developed bedside score for the detection of EVs. Therefore, our aim was to determine the utility of EVendo score as a screening tool for the detection of high risk esophageal varices in Pakistani population. Methods: It was a cross sectional study which was conducted in the department of Hepatogastroenterology from January 2021-June 2022.All the patients of either gender aged greater than 18 years with newly diagnosed cirrhosis were included in the study while those patients with prior history of esophageal varices and variceal bleeding as well as those with acute liver failure, renal impairment, non-cirrhotic portal hypertension and those on anticoagulants were exclud...
Journal of Advances in Medicine and Medical Research
The liver biopsy is the gold standard for the diagnosis of liver fibrosis, because of its invasiv... more The liver biopsy is the gold standard for the diagnosis of liver fibrosis, because of its invasiveness, high cost and lack of repeatability its use is limited. A new parameter widely used these days for evaluating the grade of hepatic fibrosis is the gamma-glutamyl transpeptidase (GGT)-to-Platelet ratio (GPR) and has shown great benefit in this regard. The aim of our study was to evaluate the role of GPR as a noninvasive predictor of liver fibrosis in patients with chronic hepatitis C in the study population. Methods: All patients with chronic hepatitis C and compensated liver disease were included in the study after informed consent. Patient’s baseline characteristics were recorded. Patient’s baseline Complete blood count (CBC) and Liver function tests were also recorded. Patients then underwent shear wave elastography (SWE) to stratify the degree of fibrosis. These indices were used to calculate Gamma glutamyl transpeptidase (GGT) / platelet ratio. Results were presented as means ...
Euroasian Journal of Hepato-Gastroenterology, Aug 3, 2023
Background: Despite advancement in imaging techniques, the diagnosis of solid pancreatic lesions ... more Background: Despite advancement in imaging techniques, the diagnosis of solid pancreatic lesions (SPLs) remains challenging. The latest advancement in elastography permits the quantitative measurements of the average elasticity of a lesion. Therefore, our main aim of this study was to determine the utility of endoscopic ultrasound-guided elastography (EUS-EG) and strain ratio (EUS-SR) in predicting SPLs.
Journal of Translational Internal Medicine, Sep 1, 2020
Background and Objective: Extracorporeal shock wave lithotripsy (ESWL) for common bile duct (CBD)... more 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.
Euroasian Journal of Hepato-Gastroenterology, Jul 13, 2022
Introduction: Post-endoscopic retrograde cholangiopancreatography (ERCP) cholangitis (PEC) is ass... more Introduction: Post-endoscopic retrograde cholangiopancreatography (ERCP) cholangitis (PEC) is associated with increased morbidity and mortality in patients ERCP. The aim of the present study was to analyze the predictors of PEC and to formulate a predictive model for early diagnosis and management. Materials and methods: It was a cross-sectional study that was carried out at the Sindh Institute of Urology and Transplantation from September 2019 to June 2021. All patients aged between 18 and 75 years and undergoing ERCP due to obstructive jaundice were included. Patients with altered biliary anatomy, history of hepatobiliary surgery, and concurrent sepsis were excluded. Endoscopic retrograde cholangiopancreatography intervention was performed by an expert gastroenterologist. Laboratory parameters (total leukocyte count, total bilirubin, alanine transaminase) and patient temperature were checked on admission, at 12 hours, 24 hours, and 36 hours after ERCP to document PEC. Results: A total of 349 patients were included in the study. Among them, 176 (50.4%) patients were males. Common bile duct (CBD) stricture was the most common indication of ERCP seen in 148 (42.4%) patients followed by CBD stone and cholangiocarcinoma in 108 (30.9%) and 48 (13.8%) patients, respectively. The most common presenting complaint was jaundice noted in 300 (86%) patients followed by right hypochondrial pain in 280 (80.2%) and weight loss in 194 (55.6%) patients, respectively. Post-ERCP cholangitis developed in 251 (71.9%) patients. On univariate analysis, age >50 years, female gender, right hypochondrial pain, fever, bilirubin >5 mg/dL on admission, CBD stricture on ERCP, TLC of >10,000 cells/L at 12 hours, 24 hours, and 36 hours post-ERCP and rise in ALT >50 IU 24 and 48 hours post-ERCP were significantly associated with PEC. While on multivariate analysis, female gender, bilirubin >5 mg/dL on admission, CBD stricture on ERCP, post-ERCP fever, and rise in TLC of > 10000 cells/L at 24 hours post-ERCP were independently associated with PEC. HinCh score was formulated and was found to be significantly associated with the presence of cholangitis. Area under the receiver operating characteristics (AUROC) of HinCh score was 0.74 and at cutoff of ≥4, the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of HinCh were 81.67%, 59.18%, 83.67%, and 55.71%, respectively with a diagnostic accuracy of 75.36%. Conclusion: The performance of HinCh score in predicting PEC was accurate in 86% of the patients. However, further studies are needed to validate the score.
Background and aim The role of expensive, risky, and unnecessary endoscopic interventions can be ... more Background and aim The role of expensive, risky, and unnecessary endoscopic interventions can be avoided by the use of noninvasive tests to predict common bile duct (CBD) stones. Therefore, our aim was to identify non-invasive predictors of choledocholithiasis (CL) in patients and further to predict a model and assess its diagnostic accuracy in predicting CL. Methods This cross-sectional study was carried out from June 1, 2020, to December 31, 2021. Patients having gall bladder stones on percutaneous transabdominal sonography and fulfilling intermediate probability criteria of CL were enrolled. These patients then underwent radial endoscopic ultrasound (EUS) followed by endoscopic retrograde cholangiopancreatography (ERCP) for detecting CBD stones. Univariate logistic regression analysis, followed by multivariate logistic regression analysis, was performed to ascertain the independent predictors of CBD stone in patients with intermediate probability. A model was proposed, and the diagnostic accuracy was calculated at an optimal cutoff. The model was then internally validated in the patients with intermediate probability and was also compared with the pre-existing score. Results Out of 131 patients included in the study, CBD stone was noted in 85 (66%) and 88 (67.2%) patients on EUS and ERCP, respectively. On multivariate analysis, high serum bilirubin (>2 mg/dL) and alkaline phosphatase (200 IU) and dilated CBD (>6 mm) on transabdominal sonography at baseline were significant predictors of CBD stone in these patients. Using these variables, a scoring system (BATS score) was developed, which had an area under the receiver operating curve (AUROC) of 0.98 in predicting the presence of CBD stone with a sensitivity of 93.18%, a specificity of 76.74%, and a diagnostic accuracy of 87.79%. In the validation cohort, a BATS score of ≥5 had a diagnostic accuracy of 95.91% in predicting CL. Conclusion The BATS score showed excellent sensitivity and good diagnostic accuracy in predicting the CBD stone with excellent results on internal validation. However, external validation of our results is required to recommend this model on a larger scale.
Aim: Wilson's disease (WD) is a rare autosomal recessive disease, that can involve any organ of t... more 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.
Journal of Advances in Medicine and Medical Research
Niemann pick disease refers to a group of autosomal recessive disorder lysosomal lipid storage di... more Niemann pick disease refers to a group of autosomal recessive disorder lysosomal lipid storage disorders with a multisystem involvement with a wide variety of clinical manifestations. The pathogenesis of disease is yet to be defined clearly. Here, we report to you a series of two cases including that of a three-year old girl and a four-year old boy.
Coeliac disease or gluten intolerance is a frequent cause of chronic diarrhoea leading to malabso... more Coeliac disease or gluten intolerance is a frequent cause of chronic diarrhoea leading to malabsorptive symptoms. Refractory coeliac disease is a rare entity, which is not only harder to diagnose but managing it can be challenging. We hereby present three such cases.
Introduction: Gallbladder cancer (GBC) is the most common malignant biliary tract tumor with the ... more Introduction: Gallbladder cancer (GBC) is the most common malignant biliary tract tumor with the shortest survival from the time of diagnosis. This poor prognosis is due to the destructive biologic behavior of GBC, lack of sensitive screening tests for early detection, and vague nature of first presentation. Here in this study, we will evaluate the baseline characteristics of the patients presenting with gallbladder carcinoma in our population.
Journal of Advances in Medicine and Medical Research
Melanosis coli is a condition in which there is blackish to brown discoloration of colon and is c... more Melanosis coli is a condition in which there is blackish to brown discoloration of colon and is commonly associated with chronic anthroquinolone laxative usage. It is frequently diagnosed as an incidental finding on colonoscopy or histopathological evaluation. Here, we present to you a case of a sixty-six years old man with history of constipation and laxative use.
A middle aged male presented with abdominal distension since one month. Further workup showed pla... more A middle aged male presented with abdominal distension since one month. Further workup showed plasma hemoglobin of 18.1 g/dL with a high pack cell volume (PCV), raised urea, creatinine and disturbed liver function tests. Abdominal ultrasonography showed an enlarged caudate lobe with thrombi in the inferior vena cava while CT scan of abdomen confirmed the same findings and was suggestive of Budd-Chiari syndrome. Further workup was conducted to rule out other causes and to find out the possible cause of Budd-Chiari syndrome. A peripheral film was requested, which showed hyper-segmented neutrophils. Later on JAK2 mutation and thrombophilia profile was ordered, which was positive for JAK2 mutation. Even though the patient was started on low molecular weight heparin but he eventually passed away. Abstract Citation: Majid Z, Tasneem AA, Luck NH, Ul Haque MM, Mandhwani RK, Laeeq SM, et al. Budd-Chiari syndrome secondary to polycythemia vera with inferior vena cava thrombosis Immunopathol P...
Background and Objectives Autoimmune hepatitis (AIH) is an important cause of chronic liver disea... more 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...
Journal of the College of Physicians and Surgeons--Pakistan : JCPSP, 2017
Acute liver failure (ALF) is an acute medical emergency which carries high mortality without live... more Acute liver failure (ALF) is an acute medical emergency which carries high mortality without liver transplantation. Various hepatotropic viruses, drug induced liver injury, auto immune hepatitis, and metabolic liver diseases are the commonly implicated etiologic agents. Liver involvement in dengue hemorrhagic fever (DHF) is quite common, but acute liver failure is its rare complication. Neurological complications are also commonly seen in DHF. Ateenage girl presented with high grade fever and subconjunctival hemorrhage, and later developed jaundice due to acute liver failure. Liver transplantation could not be offered due to fungemia. During hospital stay, she had seizures and intracranial hemorrhage culminating in brain death. ALF with neurological involvement is a rare but very important and fatal complication of DHF; and it should be considered as a cause of acute liver failure, especially in endemic areas.
Background: Gastro-esophageal reflux disease (GERD) has a prevalence of 10-20% in the Western cou... more Background: Gastro-esophageal reflux disease (GERD) has a prevalence of 10-20% in the Western countries while its prevalence amongst the Pakistani population is between 22 to 24%. Esophageal manometry is currently the gold standard for diagnosing esophageal motility disorders. Aims and Objectives: To determine the frequency of esophageal motility disorder in patients with GERD. Materials and Methods: This cross-sectional study was conducted at the
Whipple's disease is a rare chronic multi-systemic infection, caused by Gram-positive bacillus Tr... more Whipple's disease is a rare chronic multi-systemic infection, caused by Gram-positive bacillus Tropheryma whipplei. The infection usually involves the small bowel, but other organs may also be involved. The diagnosis is often challenging and can only be made on histopathological examination. This report describes 2 patients presenting with abdominal pain and weight loss who finally were diagnosed to have Whipple's disease. One of the patients was a renal transplant recipient. To the best of authors' knowledge, no case of Whipple's disease has yet been reported in Pakistan. The diagnosis were made on the basis of histopathological evaluation of duodenal biopsies. The cases underscore the need for diligent histopathological evaluation of the upper gastrointestinal biopsies and a high index of suspicion for an accurate diagnosis of the condition. The approach to the diagnosis and management of the condition is discussed.
Journal of Advances in Medicine and Medical Research
Introduction: Endoscopy for the screening of esophageal varices (EVs) is costly and cannot be per... more Introduction: Endoscopy for the screening of esophageal varices (EVs) is costly and cannot be performed in remote areas with limited resources. Recently, certain non-invasive cost effective models have been proposed for the prediction of EVs but have failed recommendation on a larger scale. EVendo score is a recently developed bedside score for the detection of EVs. Therefore, our aim was to determine the utility of EVendo score as a screening tool for the detection of high risk esophageal varices in Pakistani population. Methods: It was a cross sectional study which was conducted in the department of Hepatogastroenterology from January 2021-June 2022.All the patients of either gender aged greater than 18 years with newly diagnosed cirrhosis were included in the study while those patients with prior history of esophageal varices and variceal bleeding as well as those with acute liver failure, renal impairment, non-cirrhotic portal hypertension and those on anticoagulants were exclud...
Journal of Advances in Medicine and Medical Research
The liver biopsy is the gold standard for the diagnosis of liver fibrosis, because of its invasiv... more The liver biopsy is the gold standard for the diagnosis of liver fibrosis, because of its invasiveness, high cost and lack of repeatability its use is limited. A new parameter widely used these days for evaluating the grade of hepatic fibrosis is the gamma-glutamyl transpeptidase (GGT)-to-Platelet ratio (GPR) and has shown great benefit in this regard. The aim of our study was to evaluate the role of GPR as a noninvasive predictor of liver fibrosis in patients with chronic hepatitis C in the study population. Methods: All patients with chronic hepatitis C and compensated liver disease were included in the study after informed consent. Patient’s baseline characteristics were recorded. Patient’s baseline Complete blood count (CBC) and Liver function tests were also recorded. Patients then underwent shear wave elastography (SWE) to stratify the degree of fibrosis. These indices were used to calculate Gamma glutamyl transpeptidase (GGT) / platelet ratio. Results were presented as means ...
Euroasian Journal of Hepato-Gastroenterology, Aug 3, 2023
Background: Despite advancement in imaging techniques, the diagnosis of solid pancreatic lesions ... more Background: Despite advancement in imaging techniques, the diagnosis of solid pancreatic lesions (SPLs) remains challenging. The latest advancement in elastography permits the quantitative measurements of the average elasticity of a lesion. Therefore, our main aim of this study was to determine the utility of endoscopic ultrasound-guided elastography (EUS-EG) and strain ratio (EUS-SR) in predicting SPLs.
Journal of Translational Internal Medicine, Sep 1, 2020
Background and Objective: Extracorporeal shock wave lithotripsy (ESWL) for common bile duct (CBD)... more 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.
Euroasian Journal of Hepato-Gastroenterology, Jul 13, 2022
Introduction: Post-endoscopic retrograde cholangiopancreatography (ERCP) cholangitis (PEC) is ass... more Introduction: Post-endoscopic retrograde cholangiopancreatography (ERCP) cholangitis (PEC) is associated with increased morbidity and mortality in patients ERCP. The aim of the present study was to analyze the predictors of PEC and to formulate a predictive model for early diagnosis and management. Materials and methods: It was a cross-sectional study that was carried out at the Sindh Institute of Urology and Transplantation from September 2019 to June 2021. All patients aged between 18 and 75 years and undergoing ERCP due to obstructive jaundice were included. Patients with altered biliary anatomy, history of hepatobiliary surgery, and concurrent sepsis were excluded. Endoscopic retrograde cholangiopancreatography intervention was performed by an expert gastroenterologist. Laboratory parameters (total leukocyte count, total bilirubin, alanine transaminase) and patient temperature were checked on admission, at 12 hours, 24 hours, and 36 hours after ERCP to document PEC. Results: A total of 349 patients were included in the study. Among them, 176 (50.4%) patients were males. Common bile duct (CBD) stricture was the most common indication of ERCP seen in 148 (42.4%) patients followed by CBD stone and cholangiocarcinoma in 108 (30.9%) and 48 (13.8%) patients, respectively. The most common presenting complaint was jaundice noted in 300 (86%) patients followed by right hypochondrial pain in 280 (80.2%) and weight loss in 194 (55.6%) patients, respectively. Post-ERCP cholangitis developed in 251 (71.9%) patients. On univariate analysis, age >50 years, female gender, right hypochondrial pain, fever, bilirubin >5 mg/dL on admission, CBD stricture on ERCP, TLC of >10,000 cells/L at 12 hours, 24 hours, and 36 hours post-ERCP and rise in ALT >50 IU 24 and 48 hours post-ERCP were significantly associated with PEC. While on multivariate analysis, female gender, bilirubin >5 mg/dL on admission, CBD stricture on ERCP, post-ERCP fever, and rise in TLC of > 10000 cells/L at 24 hours post-ERCP were independently associated with PEC. HinCh score was formulated and was found to be significantly associated with the presence of cholangitis. Area under the receiver operating characteristics (AUROC) of HinCh score was 0.74 and at cutoff of ≥4, the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of HinCh were 81.67%, 59.18%, 83.67%, and 55.71%, respectively with a diagnostic accuracy of 75.36%. Conclusion: The performance of HinCh score in predicting PEC was accurate in 86% of the patients. However, further studies are needed to validate the score.
Background and aim The role of expensive, risky, and unnecessary endoscopic interventions can be ... more Background and aim The role of expensive, risky, and unnecessary endoscopic interventions can be avoided by the use of noninvasive tests to predict common bile duct (CBD) stones. Therefore, our aim was to identify non-invasive predictors of choledocholithiasis (CL) in patients and further to predict a model and assess its diagnostic accuracy in predicting CL. Methods This cross-sectional study was carried out from June 1, 2020, to December 31, 2021. Patients having gall bladder stones on percutaneous transabdominal sonography and fulfilling intermediate probability criteria of CL were enrolled. These patients then underwent radial endoscopic ultrasound (EUS) followed by endoscopic retrograde cholangiopancreatography (ERCP) for detecting CBD stones. Univariate logistic regression analysis, followed by multivariate logistic regression analysis, was performed to ascertain the independent predictors of CBD stone in patients with intermediate probability. A model was proposed, and the diagnostic accuracy was calculated at an optimal cutoff. The model was then internally validated in the patients with intermediate probability and was also compared with the pre-existing score. Results Out of 131 patients included in the study, CBD stone was noted in 85 (66%) and 88 (67.2%) patients on EUS and ERCP, respectively. On multivariate analysis, high serum bilirubin (>2 mg/dL) and alkaline phosphatase (200 IU) and dilated CBD (>6 mm) on transabdominal sonography at baseline were significant predictors of CBD stone in these patients. Using these variables, a scoring system (BATS score) was developed, which had an area under the receiver operating curve (AUROC) of 0.98 in predicting the presence of CBD stone with a sensitivity of 93.18%, a specificity of 76.74%, and a diagnostic accuracy of 87.79%. In the validation cohort, a BATS score of ≥5 had a diagnostic accuracy of 95.91% in predicting CL. Conclusion The BATS score showed excellent sensitivity and good diagnostic accuracy in predicting the CBD stone with excellent results on internal validation. However, external validation of our results is required to recommend this model on a larger scale.
Aim: Wilson's disease (WD) is a rare autosomal recessive disease, that can involve any organ of t... more 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.
Journal of Advances in Medicine and Medical Research
Niemann pick disease refers to a group of autosomal recessive disorder lysosomal lipid storage di... more Niemann pick disease refers to a group of autosomal recessive disorder lysosomal lipid storage disorders with a multisystem involvement with a wide variety of clinical manifestations. The pathogenesis of disease is yet to be defined clearly. Here, we report to you a series of two cases including that of a three-year old girl and a four-year old boy.
Coeliac disease or gluten intolerance is a frequent cause of chronic diarrhoea leading to malabso... more Coeliac disease or gluten intolerance is a frequent cause of chronic diarrhoea leading to malabsorptive symptoms. Refractory coeliac disease is a rare entity, which is not only harder to diagnose but managing it can be challenging. We hereby present three such cases.
Introduction: Gallbladder cancer (GBC) is the most common malignant biliary tract tumor with the ... more Introduction: Gallbladder cancer (GBC) is the most common malignant biliary tract tumor with the shortest survival from the time of diagnosis. This poor prognosis is due to the destructive biologic behavior of GBC, lack of sensitive screening tests for early detection, and vague nature of first presentation. Here in this study, we will evaluate the baseline characteristics of the patients presenting with gallbladder carcinoma in our population.
Journal of Advances in Medicine and Medical Research
Melanosis coli is a condition in which there is blackish to brown discoloration of colon and is c... more Melanosis coli is a condition in which there is blackish to brown discoloration of colon and is commonly associated with chronic anthroquinolone laxative usage. It is frequently diagnosed as an incidental finding on colonoscopy or histopathological evaluation. Here, we present to you a case of a sixty-six years old man with history of constipation and laxative use.
A middle aged male presented with abdominal distension since one month. Further workup showed pla... more A middle aged male presented with abdominal distension since one month. Further workup showed plasma hemoglobin of 18.1 g/dL with a high pack cell volume (PCV), raised urea, creatinine and disturbed liver function tests. Abdominal ultrasonography showed an enlarged caudate lobe with thrombi in the inferior vena cava while CT scan of abdomen confirmed the same findings and was suggestive of Budd-Chiari syndrome. Further workup was conducted to rule out other causes and to find out the possible cause of Budd-Chiari syndrome. A peripheral film was requested, which showed hyper-segmented neutrophils. Later on JAK2 mutation and thrombophilia profile was ordered, which was positive for JAK2 mutation. Even though the patient was started on low molecular weight heparin but he eventually passed away. Abstract Citation: Majid Z, Tasneem AA, Luck NH, Ul Haque MM, Mandhwani RK, Laeeq SM, et al. Budd-Chiari syndrome secondary to polycythemia vera with inferior vena cava thrombosis Immunopathol P...
Background and Objectives Autoimmune hepatitis (AIH) is an important cause of chronic liver disea... more 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...
Journal of the College of Physicians and Surgeons--Pakistan : JCPSP, 2017
Acute liver failure (ALF) is an acute medical emergency which carries high mortality without live... more Acute liver failure (ALF) is an acute medical emergency which carries high mortality without liver transplantation. Various hepatotropic viruses, drug induced liver injury, auto immune hepatitis, and metabolic liver diseases are the commonly implicated etiologic agents. Liver involvement in dengue hemorrhagic fever (DHF) is quite common, but acute liver failure is its rare complication. Neurological complications are also commonly seen in DHF. Ateenage girl presented with high grade fever and subconjunctival hemorrhage, and later developed jaundice due to acute liver failure. Liver transplantation could not be offered due to fungemia. During hospital stay, she had seizures and intracranial hemorrhage culminating in brain death. ALF with neurological involvement is a rare but very important and fatal complication of DHF; and it should be considered as a cause of acute liver failure, especially in endemic areas.
Background: Gastro-esophageal reflux disease (GERD) has a prevalence of 10-20% in the Western cou... more Background: Gastro-esophageal reflux disease (GERD) has a prevalence of 10-20% in the Western countries while its prevalence amongst the Pakistani population is between 22 to 24%. Esophageal manometry is currently the gold standard for diagnosing esophageal motility disorders. Aims and Objectives: To determine the frequency of esophageal motility disorder in patients with GERD. Materials and Methods: This cross-sectional study was conducted at the
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