ABSTRACT
BACKGROUND & AIMS: Diagnostic criteria, progression risk and optimal monitoring for intestinal failure (IF)-associated liver disease (IFALD) remain undefined. We assessed predictors, non-invasive markers and progression of histopathological liver disease in patients with IF. METHODS: In total, 77 children with IF and median age of 1.7 years underwent diagnostic liver biopsy, which was repeated in 48 patients after 2.9 years with simultaneous evaluation of liver biochemistry, liver stiffness, serum citrulline (a surrogate for viable enterocyte mass), spleen size, esophageal varices and clinical data. Patients were staged according to histopathological liver disease activity: active IFALD (cholestasis and/or inflammation), chronic IFALD (significant fibrosis and/or steatosis), or no IFALD (none of these features). RESULTS: Diagnostic liver biopsy revealed active, chronic or no IFALD in 48%, 21% and 31% of patients. Active IFALD was segregated by low serum citrulline, parenteral nutrition (PN) dependency and young age, while weaning off PN and older age predicted chronic IFALD. Although the liver histopathology in most patients either normalized (52%) or transformed to a less reactive (chronic) disease stage (23%), 19% of patients retained and 6.3% progressed to an active cholestatic/inflammatory IFALD phenotype. Decreased serum citrulline and PN-dependency also predicted active IFALD in follow-up biopsies. Increased median liver biochemistry values and liver stiffness only associated with active IFALD, which was accurately identified by gamma-glutamyltransferase (GGT), citrulline and liver stiffness, their combinations reaching diagnostic and follow-up AUROC values above 0.90. CONCLUSIONS: Active IFALD, essentially predicted by intestinal disruption and PN-dependency, was accurately detected by GGT, liver stiffness and citrulline, which together with recent advances in clinical management options, provides new avenues for monitoring and targeted liver protection in patients with IF. LAY SUMMARY: Liver disease is a common and critical complication in patients with intestinal failure, who require intravenous nutrition for survival due to severe intestinal dysfunction. We showed that both intravenous nutrition dependency and intestinal disruption essentially predicted development of active histopathological liver disease, which persisted in 25% of patients during long-term follow-up and could be accurately detected without the need for liver biopsy. Identification of the active and potentially progressive histopathology offers new possibilities for monitoring and targeted liver protection in patients with intestinal failure.
Subject(s)
Disease Progression , Intestinal Failure/complications , Intestinal Failure/diagnosis , Liver Diseases/complications , Liver Diseases/diagnosis , Adolescent , Biomarkers/blood , Child , Child, Preschool , Cholestasis/pathology , Citrulline/blood , Female , Follow-Up Studies , Humans , Infant , Intestinal Failure/blood , Liver/pathology , Liver Diseases/blood , Male , Parenteral Nutrition , Prognosis , Prospective Studies , Retrospective Studies , gamma-Glutamyltransferase/bloodABSTRACT
En el síndrome de intestino corto-insuficiencia intestinal, uno de los principales factores pronóstico, es la longitud intestinal residual, por lo que estrategias quirúrgicas que permitan aumentar la longitud cobran relevar importancia en el proceso de adaptación y eventual autonomía intestinal.Caso clínico: Paciente femenina, portadora de enfermedad de Crohn, con antecedentes de resección masiva de intestino por cuadro hemorrágico, se evidencia en el estudio radiológico moderada dilatación de asas delgadas, por lo que se practica cirugía de elongación intestinal tipo STEP, pudiendo aumentar la longitud intestinal de 32cm a 54cm. Resultados:Tiempo operatorio 270 min, período de seguimiento 10 meses, recuperación nutricional de 14,48 IMC a 22,5 IMC,con un esquema nutricional actual de 3 veces a la semana de 12 horas de administración(AU)
In the short bowel syndrome-intestinal failure, one of the most important key factors is the intestinal measure, so the surgical strategy searching to improve intestinal length is very important in order to facilitate the intestinal adaptation process. Clinic presentation: Female patient, with Crohn disease, previou surgery: massive intestinal resection due to hemorrhage. On intestinal X ray was observed mild dilated jejunum. It was performed intestinal lengthening surgery (STEP procedure). Intestinal length previous surgery 32cm, after procedure 54cm. Results: Operative time 27 min, follow up 10 months, nutritional recover IMC 14,48 to 22,5 Kg/m2 , nutritional therapy: 3 days/week TPN(AU)
