Paediatric porto‐sinusoidal vascular disease: Two different clinical phenotypes with subtle histological differences

作者：Angelo Di Giorgio, Lorenza Matarazzo, Aurelio Sonzogni, Emanuele Nicastro, Andrea Pietrobattista, Mara Cananzi, Paola Gaio, Marco Sciveres, Grazia Di Leo, Raffaele Iorio, Antonio Marseglia, Greta Carioli, Giuseppe Maggiore, Maria Guido, Lorenzo D’Antiga · 发表于：Liver International · 年份：2023 · DOI：10.1111/liv.15603 · 被引用次数：19 · 研究领域：Liver Disease and Transplantation、Liver Diseases and Immunity、Pulmonary Hypertension Research and Treatments

Abstract Background and Aims In paediatrics, porto‐sinusoidal vascular disease (PSVD) is relatively unknown and probably underdiagnosed. We aimed to describe clinical phenotypes, histology and outcome of children diagnosed with PSVD. Methods Retrospective multicentre study of children diagnosed with PSVD. Diagnosis of PSVD was based on histopathology reports; liver specimens were re‐evaluated by two expert liver pathologists. Results Sixty two children diagnosed with PSVD (M/F = 36/26, median age 6.6 years, range 3.3–10.6), from 7 centres, were included. Thirty‐six presented with non‐cirrhotic portal hypertension, PH, (PH‐PSVD Group = 58%) while 26 had a liver biopsy because of chronic elevation of transaminases without PH (noPH‐PSVD Group = 42%). On histology review, the two groups differed for the prevalence of obliterative portal venopathy (more prevalent in PH‐PSVD, p = 0.005), and hypervascularised portal tracts (more common in noPH‐PSVD, p = 0.039), the other histological changes were equally distributed. At multivariate analysis, platelet count ≤185 000/mm 3 was the only independent determinant of PH ( p < 0.001). After a median follow‐up of 7 years (range 3.0–11.2), in PH‐PSVD group 3/36 (8%) required TIPS placement, 5/36 (14%) developed pulmonary vascular complications of PH, and 7/36 (19%) required liver transplantation. In noPH‐PSVD none progressed to PH nor had complications. Conclusions Paediatric patients with PSVD present with two different clinical phenotyp...