Keyur A. Sheth

Outcome and determinants of mortality in 269 patients with combination anti-tuberculosis drug-induced liver injury

Worldwide anti‐tuberculosis (TB) drug‐induced liver disease (DILI) is an important cause of hepatotoxicity, and drug‐induced acute liver failure (ALF). Reported series on anti‐TB DILI are limited by a mix of cases with mild transaminase elevation or adaptation. Our aim was to analyze the clinical features, laboratory characteristics, outcome, and determine predictors of 90‐day mortality.

Factors That Predict 1-Month Mortality in Patients With Pregnancy-Specific Liver Disease

BACKGROUND & AIMS Pregnancy-specific liver diseases such as acute fatty liver of pregnancy; hemolysis, elevated liver enzymes and low platelet syndrome; and preeclampsia-associated liver disease are associated with considerable morbidity and mortality. We investigated the ability of the model for end-stage liver disease (MELD) to predict 1-month mortality among patients with pregnancy-specific liver diseases. We also developed and tested a model to predict mortality based on features of pregnancy-specific liver diseases. METHODS We performed a retrospective study, analyzing hospital admission, clinical, hematologic, and biochemical data collected from 130 patients with pregnancy-specific liver diseases admitted to the St. Johns Medical College Hospital (Bangalore, India) from January 2000 through April 2011. Patients were followed up until 3 months after delivery or death. Logistic regression models were fitted using the MELD score and other variables identified as clinically or statistically significant. The predictive accuracy and calibration of the models were assessed by receiver operating characteristic curves and the Hosmer-Lemeshow goodness-of-fit test. RESULTS Thirty-two patients (24.6%) died. Mortalities from pregnancy-specific liver diseases within 1 month of admission among patients with MELD scores of 20 to 29, 30 to 39, or 40 or greater were 24.2%, 45.45%, and 90.9%, respectively. Univariate analysis identified encephalopathy, ascites, serum total protein, bilirubin, platelet count, alkaline phosphatase, serum creatinine, and international normalized ratio (INR) as significant variables. Multivariate analysis identified total bilirubin (P < .001) and INR (P < .003) as significant predictors of mortality. MELD score and a model based on only 2 variables (bilirubin level and INR) accurately predicted mortality (C statistics, 0.83 and 0.86, respectively) and were well calibrated (Hosmer-Lemeshow χ(2) = 9.7 [P = .28] and 1.9 [P = .98], respectively). CONCLUSIONS A new logistic model based on only 2 variables (INR and total bilirubin) was comparable with the MELD model in predicting mortality among women with pregnancy-specific liver diseases.

Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices

Wilson disease (WD) associated acute liver failure (ALF) affects children more than adults. The predictors of mortality and outcome in patients without encephalopathy are not clear. We investigated the ability of prognostic factors and various models including model for end‐stage liver disease (MELD) to predict mortality among children with WD and ALF.

An unusual experience with endoscopic retrograde cholangiopancreatography

The endoscopic retrograde cholangiopancreatography (ERCP) is known for its varied diagnostic and therapeutic utility for a variety of disorders. However it has greater likelihood of procedure related complications among the endoscopic procedures of gastrointestinal tract. The extraluminal hemorrhagic complications following ERCP are potentially life threatening though relatively rare. We present a 50 year patient with choledocholithiasis and cholelithiasis developing rare complication of subcapsular hepatic hematoma, following ERCP due to guide wire injury.

Duodenal gastrointestinal stromal tumor presenting as massive gastrointestinal bleed

Editor Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal (GI) tract, derived from the malignant transformation of the interstitial cells of Cajal or their precursors. GISTs are more common in the 50– 60 years age group and occur commonly in the stomach (60% to 70 %) and small intestine (25 % to 35 %) [1]. Duodenal GISTs are rare, comprising about 12 % to 18 % of small intestine GIST and less than 4 % of all GISTs. They usually present with anemia due to chronic GI blood loss, but duodenal GIST presenting as massive GI bleed with shock is rare. Duodenal GISTs most frequently involve the second portion of the duodenum in close relationship to the ampulla of Vater, followed by the third, fourth, and first portions. The relationship to the ampulla determines surgical treatment strategies. We have treated four patients of GIST presenting with massive upper GI bleed and shock in the last 18 months. Their clinical, laboratory, operative, and histopathology details are outlined in Table 1. All four were between 40–60 years of age. Three out of four presented with hematemesis, and all four were critically ill with hemodynamic instability. Laboratory evaluations revealed severe microcytic hypochromic anemia in all four patients with hemoglobin ranging from 5.6 to 6.8 g/ dL. Coagulation parameters were normal. Gastroduodenoscopy showed submucosal nodule with surface ulceration and active ooze in the second part of the duodenum in three patients and submucosal nodule with active spurt of blood in the third part of the duodenum in one patient (Fig. 1). Hemostasis was achieved with injection of the diluted adrenaline. Contrast-enhanced computed tomography of abdomen confirmed intramural mass in duodenum with distinct fat planes and no lymph node enlargement. All four were operated with limited resection and primary anastomosis. All patients had small size tumors which presented early due to endoenteric growth and had mitosis less than 5 per 50 high-power fields. Their small size, endoenteric growth, complete resection, and lower mitosis were good prognostic signs in our patients. Immunohistochemical analysis revealed a positive CD117 in all four patients, positive CD34 in three patients, and positive smooth muscle actin (SMA) in one. All are alive with no recurrence, with a mean follow up of 10 months ranging from 5 to 18 months. Massive GI bleeding, as in our patients, is unusual in duodenal GIST. In a large study of 156 duodenal GIST patients, 75 cases presented with anemia due to chronic GI loss, but massive GI bleed with hemodynamic instability was only occasional [2]. In the same study, occasional massive GI bleed presented with melena, but none had hematemesis [2]. The typical features of GISTs on duodenoscopy include gross ulceration in the mucosa or an intramural mass with central ulceration. The most specific diagnostic criterion is strong and diffuse positive staining for CD117. GISTs harbor positivity for vimentin in nearly all cases, CD34 in 50 % to 70 %, smooth muscle actin in 30 % to 40 %, and platelet-derived growth factor receptor alpha (PDGFR-α) in about 5 %, while desmin (intermediate filament typical for muscle) and S-100 (a neural cell marker) are usually negative [3]. Poor prognostic parameters of GISTs include extragastric location, size greater than 5 cm, central necrosis, extension into adjacent organs, M. Patil (*) :K. A. Sheth :C. K. Adarsh :H. Devarbhavi Department of Gastroenterology, St. John’s Medical College, Bangalore 560 034, India e-mail: drmalli_arjun@yahoo.co.in

Endoscopy in a rare presentation of worms

Upper gastrointestinal bleeding can be a life threatening complication. Hookworms infestation usually causes chronic blood loss resulting in anemia. A 52 year old male presented to us with massive gastrointestinal blood loss and hemodynamic instability. On examination he had anemia, which on evaluation was iron deficient microcytic hypochromic anemia with eosinophilia. On evaluation of small bowel mucosa showed multiple hookworms moving on the surface. He improved with anthelmintics, blood transfusion and supportive management. The repeat evaluation showed eradication of worms. Hookworms presenting as a massive gastrointestinal bleeding is a rare entity. Enteroscopy plays an important role in diagnosing hookworm infestation.

An uncommon evaluation in recurrent acute pancreatitis

Recurrent acute pancreatitis can be caused due to mechanical, metabolic, anatomical abnormalities and other miscellaneous causes. Among the anatomical causes, choledochoceles are rare entities that result in recurrent acute pancreatitis. Choledochoceles are different in morphological and clinical features as compared to other choledochal cysts. We present a case of 21-year-old female with recurrent episodes of acute pancreatitis due to choledochocele as etiology. She was managed with endoscopic sphincterotomy and was asymptomatic for last 24 months.

Sudden Onset Abdominal Wall Swellings in Patient of Liver Cirrhosis

Spontaneous hematomas in cirrhotic patients are uncommon. Severe coagulopathy in advanced hepatic disease is characterized by both coagulation factor deficiencies and accelerated fibrinolysis. Hyperfibrinolysis in cirrhosis is a result of excess fibrin breakdown leading to defective hemostasis. We present a case of spontaneous hematomas with an acute drop in hemoglobin level, in a patient with cirrhosis which was due to primary hyperfibrinolysis.

Portosystemic Myelopathy: A Rare Neurological Presentation of Portosystemic Shunts

Portosystemic myelopathy is an unusual complication in patients with chronic liver disease with hepatic encephalopathy and portosystemic shunts. Here we present a case of 35-year-old male patient who presented to us with difficulty in walking and progressive stiffness in both lower limbs for two months. He had undergone splenectomy with distal splenorenal shunt 20 years back. On physical examination, he had spasticity in both lower limbs of grade 3, with minimal pyramidal weakness in lower limbs, brisk knee and ankle jerks. The plantar response was extensor. Upper limb examination was normal. On investigations, he had hypoalbuminemia, hyperbilirubinemia, increased plasma ammonia levels. Contrast enhanced CT scan abdomen revealed dilated splenorenal shunt and MRI spine showed no spinal cord compression. Electromyoneurogram was also normal. Spastic paraparesis due to portosystemic shunts was diagnosed. Liver transplantation can reverse the myelopathy only in earlier stages, hence early and accurate diagnosis is important.