Introduction: Intestinal Failure Associated Liver Disease (IFALD) is a multifactorial hepatobiliary disfunction resulting from medical and surgical management of intestinal failure (IF). Recent data suggested a 20% prevalence of IFALD in patients with IF, of which Short Bowel Syndrome (SBS) seems to be the one of the most frequent mechanism. The aim of this study is to evaluate the risk of IFALD by performing liver biopsies in children with on PN and undergoing intestinal lengthening surgery. Methods: A retrospective study of SBS children undergone intestinal lengthening surgery between January 2019 and July 2020 was performed. Patients treated with long-term PN were selected and those who received liver biopsies at the time of surgery were analysed. Local ethical was approved. Results: Twelve SBS patients (M:F=6:6) were studied. Five of them were less than one year old, ten were preterm, including two with a gestational age of less than 28 weeks and two with extremely low body weight <1000g. All patients received SMOF lipid formula and cycling PN for more than 43 days, for an average time of 26 months. Ten started PN at birth. Seven patients had at least one sepsis. Nine were treated with bacterial overgrowth prophylaxis. Liver biopsies were performed: eight showed fibrosis and ductal proliferation, one presented biliary duct hypoplasia without fibrosis, one biopsy was unremarkable and two were not available. Conclusion: According to IFALD diagnostic criteria reported, 8/10 (80%) liver biopsies performed allowed IFALD diagnosis in the analysed sample. The study confirmed the already known IFALD risk factors and showed that patients with intestinal failure due to SBS with PN treatment and undergoing intestinal lengthening surgery presented higher risk of IFALD. In SBS children with greater risk of IFALD, surgery can improve intestinal function and consequently liver function.
Intestinal Failure Associated Liver Disease in Paediatric Patients Underwent Intestinal Lengthening Surgery for Short Bowel Syndrome / antonino morabito, fabio dell'Otto, maria chiara cianci, elisa negri, zulli andrea. - In: TRANSPLANTATION. - ISSN 0041-1337. - STAMPA. - 105:(2021), pp. 58-58.
Intestinal Failure Associated Liver Disease in Paediatric Patients Underwent Intestinal Lengthening Surgery for Short Bowel Syndrome
antonino morabitoWriting – Review & Editing
;fabio dell'OttoMembro del Collaboration Group
;maria chiara cianci
Conceptualization
;elisa negriMethodology
;zulli andreaValidation
2021
Abstract
Introduction: Intestinal Failure Associated Liver Disease (IFALD) is a multifactorial hepatobiliary disfunction resulting from medical and surgical management of intestinal failure (IF). Recent data suggested a 20% prevalence of IFALD in patients with IF, of which Short Bowel Syndrome (SBS) seems to be the one of the most frequent mechanism. The aim of this study is to evaluate the risk of IFALD by performing liver biopsies in children with on PN and undergoing intestinal lengthening surgery. Methods: A retrospective study of SBS children undergone intestinal lengthening surgery between January 2019 and July 2020 was performed. Patients treated with long-term PN were selected and those who received liver biopsies at the time of surgery were analysed. Local ethical was approved. Results: Twelve SBS patients (M:F=6:6) were studied. Five of them were less than one year old, ten were preterm, including two with a gestational age of less than 28 weeks and two with extremely low body weight <1000g. All patients received SMOF lipid formula and cycling PN for more than 43 days, for an average time of 26 months. Ten started PN at birth. Seven patients had at least one sepsis. Nine were treated with bacterial overgrowth prophylaxis. Liver biopsies were performed: eight showed fibrosis and ductal proliferation, one presented biliary duct hypoplasia without fibrosis, one biopsy was unremarkable and two were not available. Conclusion: According to IFALD diagnostic criteria reported, 8/10 (80%) liver biopsies performed allowed IFALD diagnosis in the analysed sample. The study confirmed the already known IFALD risk factors and showed that patients with intestinal failure due to SBS with PN treatment and undergoing intestinal lengthening surgery presented higher risk of IFALD. In SBS children with greater risk of IFALD, surgery can improve intestinal function and consequently liver function.File | Dimensione | Formato | |
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