Abstract
IFALD is a common and potentially life-threatening condition for patients with SBS requiring long-term PN. There exists the potential for decreasing its incidence by optimizing the composition and the rate of infusion of parenteral solutions, by advocating a multidisciplinary approach, and by early referral for intestinal-liver transplantation to ensure long-term survival of patients with SBS.
Highlights
Intestinal failure (IF) refers to any clinical condition that alters the physiological absorption of nutrients
Symptoms and signs of advanced intestinal failure associated liver disease (IFALD) are similar to those seen in other liver diseases and include jaundice, hepatomegaly, splenomegaly, ascites, edema, spider nevi, palmar erythema, and caput medusae
Clinicians taking care of these patients should have a low threshold to order radiological investigations to rule out the presence of biliary obstruction and serological tests to rule out the presence of hepatitis and other types of infections that can induce cholestasis
Summary
Intestinal failure (IF) refers to any clinical condition that alters the physiological absorption of nutrients. PN is necessary to sustain patients with IF, it is associated with several side effects and liver injury is one of the most prevalent and severe complications. Massive intestinal resection continues to be associated with significant morbidity and mortality [6, 7] and only 70% of patients are able to leave the hospital [7] and their expected 5-year survival is 75% [8]. Superior mesenteric artery insufficiency, superior mesenteric vein thrombosis, small bowel volvulus, strangulation, congenital malrotation, and recurrent strictures or fistulas due to Crohn’s disease and radiation enteritis are the most common causes [10,11,12,13,14,15,16] (Table 1). In infants, necrotizing enterocolitis is the most common etiology of SBS, followed by intestinal atresia, gastroschisis, and midgut volvulus [17]
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