Endoscopic ultrasonography-guided transmural drainage of walled-off pancreatic necrosis: Comparison between a specially designed fully covered bi-flanged metal stent and multiple plastic stents.

BACKGROUND AND AIM: Endoscopic ultrasonography (EUS)-guided drainage of walled-off necrosis (WON) may be carried out by placement of multiple plastic stents (MPS) or specially designed fully covered bi-flanged metal stents (BFMS). Comparative data on efficacy of these two stent types for WON drainage are limited. This retrospective study compares outcomes of WON drainage using BFMS and MPS. METHODS: During a 10-year period, 133 patients underwent EUS-guided WON drainage. MPS or BFMS were placed in a WON cavity through a single puncture, and direct endoscopic necrosectomy (DEN) was carried out whenever clinically necessary. Data in the two cohorts were retrospectively compared for primary outcomes - clinical success, adverse events and mortality; and secondary outcomes - DEN requirement, mean DEN sessions, need for salvage surgery and hospital stay. RESULTS: MPS were placed in 61 and BFMS in 72 patients. Patients undergoing BFMS drainage required fewer DEN sessions (mean 1.46 vs 2.74, P < 0.05), had fewer adverse events (5.6% vs 36.1%, P < 0.05), needed salvage surgery less often (2.7% vs 26.2%, P < 0.05), and had significantly shorter hospital stay (4.1 vs 8 days, P < 0.05) compared to those undergoing MPS drainage. There was no difference in DEN requirement (P = 0.217) and mortality (P = 0.5) in both groups. Overall clinical success with BFMS was superior to MPS (94% vs 73.7%, P < 0.05). CONCLUSION: BFMS appear to be superior to MPS for EUS-guided WON drainage in terms of clinical success, number of DEN sessions, adverse events, need for salvage surgery and hospital stay.

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. John's 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.

Elevated alpha fetoprotein, no hepatocellular carcinoma.

Alpha fetoprotein is a fetal specific glycoprotein which falls rapidly after birth. High level of alpha fetoprotein is suspicious of hepatocellular carcinoma but may be elevated in chronic viral hepatitis. A 35-year-old presented to us with jaundice for 7 days. He had chronic hepatitis B infection for last 12 months and was taking medicines irregularly for same. He had high alpha fetoprotein levels (740.9 ng/ml) without evidence of hepatocellular carcinoma which reduced with antiviral therapy. Such elevation can be explained due to hepatic inflammation and viral replication.

A review and current perspective on Wilson disease.

Wilson disease is a rare, inherited autosomal recessive disease of copper metabolism and may be more common where consanguinity is prevalent. Much has been known about the disease after it was first described by Kinnier Wilson as 'progressive lenticular degeneration in 1912. Over 500 mutations of the ATP7B gene has been identified with no clear genotype to phenotype correlation. Loss of ATP7B function leads various grades of reduced biliary excretion of copper and reduced incorporation of copper into ceruloplasmin; accumulation and toxicity of copper in the liver, brain and other tissues results in liver toxicity and other myriad manifestations of the disease. The clinical features may vary from asymptomatic state to chronic liver disease, acute liver failure, neuropsychiatric manifestations and hemolytic anemia. Diagnosis is based on the combination of clinical sign's, biochemical features, histologic findings and mutation analysis of ATP7B gene. Subtle geographical differences exist with a disproportionate proportion of children presenting with acute liver failure. A high index of suspicion is needed for an early diagnosis. Ratios of biochemical indices for early diagnosis need validation across geographical regions and may not be particularly applicable in children. Better biomarkers or the need for tests for early detection of ALF persists. Drugs used in the treatment of Wilson disease include copper chelating agents such as d-Penicillamine, trientine and zinc salt. Untreated Wilson disease uniformly leads to death from liver disease or severe neurological disability. Early recognition and treatment has excellent prognosis. Liver transplantation is indicated in acute liver failure and end stage liver disease. Family screening in order to detect the disorder in the first-degree relatives is warranted. This review provides an overview of different aspects of Wilson disease including geographical differences in presentations and clinical management and the limitations of currently available tests.

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.