PE-21: Assessment of Moderate and Severe Intestinal Failure Associated Liver Disease. Clinical, Analytical and Histological Correlation

Introduction: To assess clinical, analytical and pathological correlation of intestinal failure associated liver disease (IFALD) in pediatric patients assessed in an intestinal rehabilitation unit of a tertiary hospital between 2008-2018. Methods: Patients with intestinal failure with moderate (F2) and severe liver disease (F3 and F4), in liver biopsy performed at our center at the time of assessment, are retrospectively evaluated. Results: During the study period, 26 patients with IFALD were analyzed with a median age of 6 months at the time of the assessment (range 2-86 months). The etiology of intestinal failure was, in 80% of patients, short bowel syndrome, followed by motility disorders (7.7%), congenital disorders of intestinal epithelium (3.8%) and other genetic-based disorders such as Martínez Frías Syndrome (3.8%) and Tricohepatoenteric Syndrome (3.8%). 96% received parenteral nutrition at the time of assessment, with duration of 145 days on average (range 65-713 days), with complete macronutrients support, 48% had it cycled 6 hours on average (range 4–12 hours). 76% received concomitantly support with enteral nutrition. At the time of the assessment, 35% of the patients did not show hypertransaminasemia (considering as such values greater than 1.5 times the normal value) and 15.4% did not present GGT elevation. 42% did not developed data of cholestasis with normal bilirubin levels (<2 mg/dl) and in 58% of patients a prothrombin activity showed a normal range (> 70%). 38% of the patients had splenomegaly and 26% thrombopenia. 65% of the studied patients had liver disease F2 in the liver biopsy; 27% an F3 with bridging fibrosis and 7.7% an F4 (cirrhosis). Conclusions: IFALD is a very frequent complication in pediatric patients with intestinal failure. There are cases of advanced liver disease in histological samples with absence of cholestasis or relevant clinical-analytical data in those patients. It is important to refer pediatric patients with intestinal failure to an intestinal rehabilitation unit as soon as possible, in order to optimize medical, surgical and nutritional management and thus prevent or slow down the progression of IFALD.