Evaluating the non-invasive tools for assessment of liver fibrosis in children with intrahepatic cholestasis prior to partial biliary diversion: tertiary-center experience

نویسندگان

چکیده

Abstract Background The liver biopsy is an essential element of evaluating progression disease in children with Progressive Familial Intrahepatic Cholestasis (PFIC) and Allagille Syndrome (AGS). Several noninvasive techniques, including radiological imaging blood biomarkers assay, can be used to evaluate stiffness. Objectives To identify whether Transient elastography (FibroScan) AST/PLT Ratio Index (APRI) could reliable tools assess the degree fibrosis prior partial biliary diversion (PBD). Methods A prospective cohort which all patients PFIC AGS who underwent PBD from July 2019 2021 were included. Preoperative functions, pelvic-abdominal ultrasonography FibroScan assessments performed while intraoperative was obtained. Results Eight chronic cholestatic candidates for due intractable pruritus enrolled, ( n = 6; 75%), 2; 25%). results similar histopathological assessment 87.5% cases. APRI ranged 0.1 3.2 (median 1.2). In four cases (50%), consistent histological evaluation samples. concordance three (37.5%). Conclusion current demonstrated that fibroScan histopathology patients, highlighting its value determining surgery, whereas only half

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

partial internal biliary diversion in progressive familial intrahepatic cholestasis: introduction of a new approach

discussion the cholecystojejunocolic anastomosis with intussuscepted valve surgery could be considered as a forthcoming approach in the treatment of intrahepatic cholestasis. case presentation an 11-year-old girl with the diagnosis of pfic underwent cholecystoappendicostomy with myotomy operation. because of anastomosis stricture, she was reoperated with cholecystojejunocolic anastomosis and in...

متن کامل

Partial Internal Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: Introduction of a New Approach

INTRODUCTION Facilitation of biliary salts secretion represents the mainstay of treatment for progressive familial intrahepatic cholestasis (PFIC). The purpose of this study was to introduce a new approach for the treatment of progressive familial intrahepatic cholestasis (PFIC) to avoid ostoma. CASE PRESENTATION An 11-year-old girl with the diagnosis of PFIC underwent cholecystoappendicostom...

متن کامل

Clinical Variability After Partial External Biliary Diversion in Familial Intrahepatic Cholestasis 1 Deficiency.

OBJECTIVES Familial intrahepatic cholestasis 1 (FIC1) deficiency is caused by a mutation in the ATP8B1 gene. Partial external biliary diversion (PEBD) is pursued to improve pruritus and arrest disease progression. Our aim is to describe clinical variability after PEBD in FIC1 disease. METHODS We performed a single-center, retrospective review of genetically confirmed FIC1 deficient patients w...

متن کامل

Non-invasive assessment of liver fibrosis

Liver fibrosis is the final common pathway of most chronic liver diseases of various etiologies.The development of fibrosis determines the management policy as well as the prognosis. In recent years it became clear that the fibrotic process is reversible. Therefore early detection of liver fibrosis is critical. At present, liver biopsy is considered the gold standard for the detection of fibros...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

ژورنال

عنوان ژورنال: Egyptian Pediatric Association Gazette

سال: 2023

ISSN: ['2090-9942', '1110-6638']

DOI: https://doi.org/10.1186/s43054-023-00159-w