A clinical review of congenital hepatic fibrosis diagnosed in adulthood: presentation, complications, and outcomes.

INTRODUCTION AND OBJECTIVES: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described. We sought to define the presentation and outcomes of adults with biopsy-proven CHF. MATERIALS AND METHODS: A retrospective chart review was conducted of patients diagnosed with CHF by liver biopsy. Continuous variables were summarized with the sample median and range. Categorical variables were summarized with number and percentage of patients. RESULTS: We identified 24 patients evaluated over a 20-year period, with a median age of 51 years (range 22-72 years) at initial presentation; 14 were male. The most common imaging findings were renal cysts (91.3%), splenomegaly (69.6%), and a cirrhotic-appearing liver (60.9%). The most commonly treated liver-related complications were cholangitis (45.8%), varices (45.8%), and hepatic encephalopathy (25%). Two patients died with a median length of follow-up of 2.9 years (range: 0.0-20.0 years). Two patients underwent transjugular intrahepatic portosystemic shunt (TIPS) placement to manage bleeding esophageal varices. Eight patients underwent liver transplantation (LT), the most common indication being decompensated disease (50%). CONCLUSIONS: CHF should be considered when patients present with cholangitis and/or complications of portal hypertension and have a cirrhotic appearing liver and renal cysts on imaging. Depending upon the disease severity, interventions such as TIPS or LT may be required.

Clarifying the Hazy Concepts of Food Allergies and Sensitivities.

Food allergies, food sensitivities, and adverse reactions to food represent common reasons for gastroenterology and allergy referral. The epidemiology of these disorders is changing; food allergies are more common than previously thought, and there is a heightened sense of awareness about food sensitivities. Symptoms do not always accurately predict the underlying pathophysiology nor distinguish the underlying etiology. This may lead to unnecessary testing, which is often unrewarding and expensive. Myths and misconceptions about food allergies and sensitivities are common and may lead to unwarranted treatment using untested therapies. Importantly, a missed diagnosis of a true food allergy can have serious consequences. This article discusses the differences between food allergies and sensitivities, including their epidemiology, underlying pathophysiology, key symptoms, and diagnostic criteria, as well as their treatment options.