Angelo Di Georgio, 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
Hospital Papa Giovanni XXIII. IRCCS Bambino Gesù, Pediatric Hospital. University Hospital of Padova. Istituto Mediterraneo per i Trapianti e Terapie.
IRCCS Burlo Garofolo. University of Naples Federico II. Fondazione IRCCS Casa Sollievo della Sofferenza.
Italy
Liver International
Liver Int 2023;
DOI: 10.1111/liv.15603
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/mm3 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 phenotypes, one characterised by PH and one by chronic elevation of transaminases without PH. PSVD should be included among the conditions causing isolated hypertransaminasaemia. On histology, the differences between the two groups are subtle. Medium-term outcome is favourable in patients without PH; progression of the disease is observed in those with PH.
Category
Class I. Pulmonary Hypertension Associated with Liver Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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