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Biliary Atresia Patients With Successful Kasai Portoenterostomy Can Present With Features of Obliterative Portal Venopathy
- Source :
- Journal of Pediatric Gastroenterology & Nutrition. 71:91-98
- Publication Year :
- 2020
- Publisher :
- Ovid Technologies (Wolters Kluwer Health), 2020.
-
Abstract
- OBJECTIVE Study of liver explants of biliary atresia (BA) patients with successful Kasai portoenterostomy (KP). METHODS Pathology and medical records of BA liver explants from January 2009 to June 2018 with successful KP were reviewed along with appropriate controls. RESULTS Fourteen out of 68 (20.6%) BA patients with LT had a successful KP. Median age at BA diagnosis, KP and LT was 60.5 days, 61 days, and 10 years, respectively, with conjugated bilirubin (c-bil) normalizing at 12.5 weeks after KP. Advanced fibrosis was diffuse in 2/14 (14.3%) explants, limited to periphery in 11/14 (78.6%) and absent in 1. Hilar partial nodular transformation (PNT) was seen in 11 explants (78.6%) and diffuse nodular regenerative hyperplasia (NRH) in 2 (14.3%). Areas of PNT and NRH showed diffuse portal sclerosis (100%), complete and incomplete portal vein (PV) stenosis (100%), PV herniation (100%), hypervascular portal tracts (20%), periportal abnormal vessels (100%), abundant lymphatic collaterals (100%), mild medial hepatic arterial hypertrophy (100%), and delicate fibrous septae (100%). Extrahepatic PVs showed variable luminal occlusion with mean PV intima to full thickness ratio of 0.6 +/- 0.11; significantly higher than age-matched noncirrhotic (n = 27, 0.08 +/- 0.09; P
- Subjects :
- medicine.medical_specialty
medicine.medical_treatment
Portoenterostomy, Hepatic
Context (language use)
Liver transplantation
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Biliary Atresia
Biliary atresia
Fibrosis
030225 pediatrics
Internal medicine
Hypertension, Portal
Humans
Medicine
business.industry
Infant
medicine.disease
Liver Transplantation
Stenosis
Lymphatic system
Pediatrics, Perinatology and Child Health
Portal hypertension
030211 gastroenterology & hepatology
business
Nodular regenerative hyperplasia
Subjects
Details
- ISSN :
- 15364801 and 02772116
- Volume :
- 71
- Database :
- OpenAIRE
- Journal :
- Journal of Pediatric Gastroenterology & Nutrition
- Accession number :
- edsair.doi.dedup.....ded32bbec55f11aa1fc62159f48b5e9f