LIVER DISORDERS, SERIES #7

Long Le, Duminda Suraweera, Gaurav Singhvi

Inherited metabolic liver diseases are a group of disorders caused by the pathologic accumulation of metals or misfolded proteins from disrupted normal metabolic pathways. The common diseases are hemochromatosis, Wilson disease (WD), alpha-1-antitrypsin deficiency (AAT) and glycogen storage diseases (GSD). New pathophysiologic understanding at the molecular level has changed clinical practice and research in recent years. This review article focuses on pathophysiology, clinical presentations, current management strategies and future directions.

NUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #167

Andrew P. Copland, Brian Behm, Carol Rees Parrish

Patients with short bowel syndrome (SBS) often struggle to maintain nutrition and hydration status. Using a combination of diet and pharmacotherapies, partial or full enteral autonomy may be achievable over time as the bowel adapts. There are select groups that cannot achieve autonomy with conventional therapy, but with augmentation of the absorptive capacity of their existing small bowel, autonomy may be feasible and quality of life significantly improved. With the approval of the GLP-2 analog intestinotrophic agent teduglutide, adaptation and intestinal absorption enhancement is possible. This article describes the course of 7 patients on maximal conventional SBS therapy who achieved either enteral autonomy or significant improvements in nutritional status, stool and urine output, and quality of life after initiation of teduglutide.

FRONTIERS IN ENDOSCOPY, SERIES #38

Judith Staub, Douglas G. Adler

Esophagorespiratory fistulas (ERFs) are pathologic communications between the esophagus and any portion of the respiratory tract. ERFs lead to recurrent aspiration that can cause lethal pulmonary infections and significantly decrease quality of life for patients. Treatment of ERFs has been shown to not only improve dysphagia and aspiration, but also lead to increased survival times. While there is limited outcome data to guide clinical decision-making, the purpose of this review is to describe the current literature that supports the various endoscopic techniques utilized to manage ERFs.

INFLAMMATORY BOWEL DISEASE: A PRACTICAL APPROACH, SERIES #103

Adarsh K. Varma, Nirmal Kaur, Seymour Katz

Tumor necrosis factor – alpha (TNF-α) antagonist therapy is highly effective for the treatment of Crohn's disease and ulcerative colitis, broadly termed inflammatory bowel disease (IBD). While this class of medication has revolutionized the field of IBD therapy, 40% of patients lose response within one year of treatment. Therapeutic drug monitoring has emerged as a method to optimize treatment with TNF-α antagonists by guiding treatment decisions, increasing the long term durability of the medications, and maximize the likelihood of a sustained clinical benefit when performed appropriately. Therapeutic drug monitoring with TNF-α antagonists involves measuring serum drug levels and anti-drug antibodies, and maintaining drug levels within a specific therapeutic window. The aim of this review is to provide a comprehensive and practical approach for performing therapeutic drug monitoring for infliximab and adalimumab in patients with IBD.

DISPATCHES FROM THE GUILD CONFERENCE, SERIES #8

Michael P. Curry, Sentia Iriani

Hepatocellular carcinoma continues to be a signi cant cause of morbidity and mortality in patients with chronic liver disease. Among both men and women in the United States, death due to liver cancer has increased at the highest rate of all cancers in the past decade. Despite the improvements in imaging and therapeutics, only tumors diagnosed in early stages effectively respond to treatment. Loco-regional therapies, surgical resection and transplantation allow for improved survival. For patients with advanced stage disease, there is a need for novel and effective therapies. Here we discuss prevention, diagnosis and treatment of HCC.

A SPECIAL ARTICLE

Rupert K. Hung, Jamie Muhly, Mamie Gao, Gary Gong, Martin Auster

Contrast agents of varying consistency, ranging from thin liquids to solids, are utilized in modified barium swallow (MBS) studies, typically without real-time radiographic labeling. We describe a method for identifying the contrast consistency using radiographic labels during each fluoroscopy sequence. Two cases demonstrating the utility of the radiographic labels in clinical practice are described in detail. Our labeling method is an easily implemented and cost-effective technique to promote increased accuracy. Real-time labeling of sequential MBS studies facilitate assessment on sequential studies and for different RIS/PAC systems and will reduce ambiguities and misinterpretations.

A CASE REPORT

Michael Root, Marianna Papademetriou, David M. Poppers

Departments Section