{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T13:27:09Z","timestamp":1774531629360,"version":"3.50.1"},"reference-count":8,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,6,19]],"date-time":"2025-06-19T00:00:00Z","timestamp":1750291200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JPGN rep."],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Prolidase deficiency (PD) is a rare autosomal recessive disorder affecting collagen turnover, leading to diverse clinical manifestations including dermatologic lesions, hepatosplenomegaly, and vascular anomalies. Liver involvement in PD is poorly understood, with few reported cases. We present a child with early\u2010onset non\u2010cirrhotic portal hypertension and PD. The patient initially presented with neonatal hemolytic anemia and hepatosplenomegaly. At age 9, recurrent epistaxis and splenomegaly led to splenectomy. Liver biopsy revealed sinusoidal dilation and parenchymal nodularity, later progressing to esophageal varices. Genetic testing identified pathogenic variants in peptidase\u2010D gene, suggestive of PD, and biochemical testing confirmed the diagnosis. Given suspected vasculopathy, tocilizumab was initiated with clinical improvement. This case suggests a potential link between PD and porto\u2010sinusoidal vascular disorder (PSVD), particularly nodular regenerative hyperplasia. Further research is needed to explore prolidase's role in vascular remodeling and its contribution to PSVD\u2010related liver pathology. Early recognition may improve management and outcomes.<\/jats:p>","DOI":"10.1002\/jpr3.70055","type":"journal-article","created":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T00:50:29Z","timestamp":1750380629000},"page":"519-523","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Porto\u2010sinusoidal vascular disorder in a pediatric patient with prolidase deficiency: A case report"],"prefix":"10.1002","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-4120-1478","authenticated-orcid":false,"given":"Melissa","family":"Castro","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition Vanderbilt University Medical Center Nashville Tennessee USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Martinez","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nicklaus Children's Hospital Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carole","family":"Brathwaite","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Nicklaus Children's Hospital Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reuven","family":"Bromberg","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology Nicklaus Children's Hospital Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana M.","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Division of Clinical Genetics &amp; Metabolism Nicklaus Children's Hospital Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erick","family":"Hernandez","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition Nicklaus Children's Hospital Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,6,19]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.3390\/biology9050108"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fmolb.2021.723003"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2022.05.033"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11901-023-00613-8"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.3389\/frtra.2023.1221765"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/jvh.13501"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1159\/000536117"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2019.07.001"}],"container-title":["JPGN Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jpr3.70055","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T04:02:19Z","timestamp":1763006539000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jpr3.70055"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,19]]},"references-count":8,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2025,11]]}},"alternative-id":["10.1002\/jpr3.70055"],"URL":"https:\/\/doi.org\/10.1002\/jpr3.70055","archive":["Portico"],"relation":{},"ISSN":["2691-171X","2691-171X"],"issn-type":[{"value":"2691-171X","type":"print"},{"value":"2691-171X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,19]]},"assertion":[{"value":"2024-12-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-05-16","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-06-19","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}