{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T13:10:09Z","timestamp":1788786609338,"version":"build-2803163510"},"reference-count":107,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T00:00:00Z","timestamp":1788652800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Clin. Med.","JCM"],"abstract":"<jats:p>Non-cirrhotic portal hypertension (NCPHT) is defined as portal hypertension (PHT) occurring in the absence of cirrhosis. Major etiological causes of NCPHT include immunological disorders, chronic infections, exposure to medications or toxins, prothrombotic conditions, and several genetic syndromes, highlighting that NCPHT is not a single disease but a shared phenotype arising from diverse underlying pathways. NCPHT is frequently misdiagnosed, largely due to inconsistent nomenclature and limited scientific literature. The broader term non-cirrhotic portal fibrosis (NCPF) or porto-sinusoidal vascular disease (PSVD) includes patients in a preclinical stage who demonstrate histological features similar to NCPHT but lack clinical evidence of PHT. Early detection is linked to a favorable prognosis and improved clinical outcomes. Management strategies in pediatrics continue to rely on extrapolations from adult practice, with sparse evidence to guide pediatric care. Liver biopsy remains the cornerstone of diagnosis, demonstrating nodular regenerative hyperplasia, obliterative portal venopathy, or incomplete septal fibrosis. Management focuses on prophylactic and symptomatic care, with endoscopic therapy for controlling variceal bleeding. Porto-systemic shunts and, ultimately, liver transplantation therapies may be needed for advanced stages. A pressing need exists for standardized diagnostic criteria and multicenter studies to define natural history, refine risk stratification, and evaluate therapeutic approaches in the pediatric population. This review provides an overview of the pediatric causes of NCPHT, outlines the current understanding of pathophysiology, and discusses the clinical presentations and management strategies, while highlighting existing research gaps.<\/jats:p>","DOI":"10.3390\/jcm15176901","type":"journal-article","created":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T12:12:25Z","timestamp":1788783145000},"page":"6901","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Overview of Non-Cirrhotic Portal Hypertension in Pediatric Patients"],"prefix":"10.3390","volume":"15","author":[{"given":"Ambika","family":"Walecha","sequence":"first","affiliation":[{"name":"Lady Hardinge Medical College, New Delhi 110001, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3094-9703","authenticated-orcid":false,"given":"Senthilkumar","family":"Sankararaman","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Children\u2019s Hospital, Cleveland, OH 44122, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kadakkal","family":"Radhakrishnan","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Children\u2019s Hospital, Cleveland, OH 44122, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,9,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1180","DOI":"10.1097\/HEP.0000000000000647","article-title":"AASLD Practice Guidance on risk stratification and management of portal hypertension and varices in cirrhosis","volume":"79","author":"Kaplan","year":"2024","journal-title":"Hepatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"101180","DOI":"10.1016\/j.aohep.2023.101180","article-title":"Evolving portal hypertension through Baveno VII recommendations","volume":"29","author":"Mendizabal","year":"2024","journal-title":"Ann. Hepatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1715","DOI":"10.1053\/j.gastro.2008.03.007","article-title":"Portal hypertension and its complications","volume":"134","author":"Sanyal","year":"2008","journal-title":"Gastroenterology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1111\/imj.12590","article-title":"Portal hypertension: Pathophysiology, diagnosis and management","volume":"45","author":"Bloom","year":"2015","journal-title":"Intern. Med. J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1016\/j.jhep.2021.12.022","article-title":"Baveno VII\u2013renewing consensus in portal hypertension","volume":"76","author":"Bosch","year":"2022","journal-title":"J. Hepatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1111\/1751-2980.13343","article-title":"Etiology Control for Reducing Hepatic Vein Pressure Gradient in Patients With Cirrhosis and Portal Hypertension: A Systematic Review and Meta-Analysis","volume":"26","author":"Xia","year":"2025","journal-title":"J. Dig. Dis."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"664","DOI":"10.4240\/wjgs.v15.i4.664","article-title":"Computed tomography perfusion in differentiating portal hypertension: A correlation study with hepatic venous pressure gradient","volume":"15","author":"Dong","year":"2023","journal-title":"World J. Gastrointest. Surg."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1016\/j.jhep.2015.05.022","article-title":"Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension","volume":"63","author":"Faculty","year":"2015","journal-title":"J. Hepatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1056\/NEJMra0901512","article-title":"Management of varices and variceal hemorrhage in cirrhosis","volume":"362","author":"Bosch","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"16996","DOI":"10.3748\/wjg.v20.i45.16996","article-title":"Transjugular intrahepatic portosystemic shunts and portal hypertension-related complications","volume":"20","author":"Siramolpiwat","year":"2014","journal-title":"World J. Gastroenterol. WJG"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1527","DOI":"10.1111\/apt.17987","article-title":"Prospective evaluation of patients with non-cirrhotic portal hypertension: A single centre study","volume":"59","author":"Mironova","year":"2024","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/j.jhep.2013.08.013","article-title":"Non-cirrhotic portal hypertension\u2013diagnosis and management","volume":"60","author":"Khanna","year":"2014","journal-title":"J. Hepatol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1346","DOI":"10.1002\/hep4.1560","article-title":"Natural course of pediatric portal hypertension","volume":"4","author":"Kassam","year":"2020","journal-title":"Hepatol. Commun."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1016\/j.dld.2018.01.132","article-title":"Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis","volume":"50","author":"Gioia","year":"2018","journal-title":"Dig. Liver Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e240210","DOI":"10.1148\/rg.240210","article-title":"Noncirrhotic portal hypertension: Current concepts and overview of causes, pathophysiology, and imaging findings","volume":"45","author":"Borhani","year":"2025","journal-title":"Radiographics"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1002\/jpn3.70318","article-title":"Clinical presentation; management, and outcomes for noncirrhotic portal hypertension in children","volume":"82","author":"Jarasvaraparn","year":"2026","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1523","DOI":"10.1111\/liv.15603","article-title":"Paediatric porto-sinusoidal vascular disease: Two different clinical phenotypes with subtle histological differences","volume":"43","author":"Matarazzo","year":"2023","journal-title":"Liver Int."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.4254\/wjh.v13.i10.1269","article-title":"Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations","volume":"13","author":"Sarma","year":"2021","journal-title":"World J. Hepatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1186\/s13023-015-0288-8","article-title":"Idiopathic non-cirrhotic portal hypertension: A review","volume":"10","author":"Schouten","year":"2015","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"81","DOI":"10.2147\/HMER.S85544","article-title":"Idiopathic noncirrhotic portal hypertension: Current perspectives","volume":"8","author":"Riggio","year":"2016","journal-title":"Hepatic Med. Evid. Res."},{"key":"ref_21","first-page":"e25934","article-title":"Frequency of causes of portal hypertension in children","volume":"14","author":"Sooraj","year":"2022","journal-title":"Cureus"},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Grama, A., P\u00eervan, A., S\u00eerbe, C., Burac, L., \u015etef\u0103nescu, H., Fufezan, O., Bordea, M.A., and Pop, T.L. (2021). Extrahepatic portal vein thrombosis, an important cause of portal hypertension in children. J. Clin. Med., 10.","DOI":"10.3390\/jcm10122703"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1016\/j.jhep.2016.09.006","article-title":"Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding","volume":"66","author":"Ducot","year":"2017","journal-title":"J. Hepatol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"301","DOI":"10.5223\/pghn.2023.26.6.301","article-title":"Portal hypertension in children: A tertiary center experience in Turkey","volume":"26","author":"Yayla","year":"2023","journal-title":"Pediatr. Gastroenterol. Hepatol. Nutr."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1016\/S2468-1253(19)30047-0","article-title":"Porto-sinusoidal vascular disease: Proposal and description of a novel entity","volume":"4","author":"Rautou","year":"2019","journal-title":"Lancet Gastroenterol. Hepatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1007\/s12072-026-11105-4","article-title":"A multisociety consensus statement on a new common definition and diagnostic criteria for PSVD or NCPF","volume":"20","author":"Paradis","year":"2026","journal-title":"Hepatol. Int."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1016\/j.humpath.2004.06.016","article-title":"Intrasinusoidal cytotoxic CD8+ T cells in nodular regenerative hyperplasia of the liver","volume":"35","author":"Ziol","year":"2004","journal-title":"Hum. Pathol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"49","DOI":"10.14740\/gr1376","article-title":"Idiopathic non-cirrhotic portal hypertension and porto-sinusoidal vascular disease: Review of current data","volume":"14","author":"Kmeid","year":"2021","journal-title":"Gastroenterol. Res."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1007\/s00428-018-2331-3","article-title":"Vascular liver diseases on the clinical side: Definitions and diagnosis, new concepts","volume":"473","author":"Valla","year":"2018","journal-title":"Virchows Arch."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1083","DOI":"10.1111\/his.12114","article-title":"Histological features in western patients with idiopathic non-cirrhotic portal hypertension","volume":"62","author":"Verheij","year":"2013","journal-title":"Histopathology"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1684","DOI":"10.1007\/s12072-024-10739-6","article-title":"Non-cirrhotic portal fibrosis\/idiopathic portal hypertension: APASL recommendations for diagnosis and management","volume":"18","author":"Shukla","year":"2024","journal-title":"Hepatol. Int."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1002\/hep.31646","article-title":"Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease: 2020 practice guidance by the American Association for the Study of Liver Diseases","volume":"73","author":"Northup","year":"2021","journal-title":"Hepatology"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1016\/j.jhep.2025.08.001","article-title":"EASL Clinical Practice Guidelines on vascular diseases of the liver","volume":"84","author":"Rautou","year":"2025","journal-title":"J. Hepatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1007\/s12072-007-9010-9","article-title":"Noncirrhotic portal fibrosis\/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment","volume":"1","author":"Sarin","year":"2007","journal-title":"Hepatol. Int."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"468","DOI":"10.4103\/ijmr.IJMR_1405_17","article-title":"What makes non-cirrhotic portal hypertension a common disease in India? Analysis for environmental factors","volume":"149","author":"Goel","year":"2019","journal-title":"Indian J. Med. Res."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1111\/hepr.13343","article-title":"Japanese periodical nationwide epidemiologic survey of aberrant portal hemodynamics","volume":"49","author":"Ohfuji","year":"2019","journal-title":"Hepatol. Res."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"2767","DOI":"10.1007\/s10620-013-2736-4","article-title":"Obliterative portal venopathy: A clinical and histopathological review","volume":"58","author":"Aggarwal","year":"2013","journal-title":"Dig. Dis. Sci."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1016\/j.dld.2025.04.038","article-title":"Prevalence of portal vein thrombosis in pediatric patients with cirrhosis and intrahepatic non-cirrhotic portal hypertension","volume":"57","author":"Ozturk","year":"2025","journal-title":"Dig. Liver Dis."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"e132","DOI":"10.1097\/MPG.0000000000003445","article-title":"Porto-sinusoidal vascular disease: A pediatric study of 30 patients","volume":"74","author":"Girard","year":"2022","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1078\/0344-0338-5710012","article-title":"Pathology and pathogenesis of idiopathic portal hypertension with an emphasis on the liver","volume":"197","author":"Nakanuma","year":"2001","journal-title":"Pathol.-Res. Pract."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1002\/hep.24422","article-title":"Idiopathic noncirrhotic portal hypertension","volume":"54","author":"Schouten","year":"2011","journal-title":"Hepatology"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1007\/s12016-017-8638-z","article-title":"Common variable immunodeficiency and liver involvement","volume":"55","author":"Song","year":"2018","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"Szczawi\u0144ska-Pop\u0142onyk, A., Bekalarska, J., J\u0119ch, K., Knobloch, N., \u0141ukasik, O., Ossowska, A., Ruducha, J., and Wysocka, Z. (2025). The burden of non-infectious organ-specific immunopathology in pediatric common variable immunodeficiency. Int. J. Mol. Sci., 26.","DOI":"10.20944\/preprints202501.1309.v1"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"672458","DOI":"10.1155\/2014\/672458","article-title":"Idiopathic non cirrhotic portal hypertension and spleno-portal axis abnormalities in patients with severe primary antibody deficiencies","volume":"2014","author":"Pulvirenti","year":"2014","journal-title":"J. Immunol. Res."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1007\/s10875-016-0334-x","article-title":"Ataxia telangiectasia associated with nodular regenerative hyperplasia","volume":"36","author":"Milligan","year":"2016","journal-title":"J. Clin. Immunol."},{"key":"ref_46","unstructured":"Aksentijevich, I., Sampaio Moura, N., and Barron, K. (1993). Adenosine Deaminase 2 Deficiency. GeneReviews\u00ae, University of Washington."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1917","DOI":"10.1053\/j.gastro.2008.02.081","article-title":"Hepatic involvement and portal hypertension predict mortality in chronic granulomatous disease","volume":"134","author":"Feld","year":"2008","journal-title":"Gastroenterology"},{"key":"ref_48","doi-asserted-by":"crossref","unstructured":"Sparchez, M., Damian, L., Socaciu, M.A., Fufezan, O., and Sparchez, Z. (2026). Hepatic Vascular Involvement in Adenosine Deaminase 2 Deficiency (DADA2): Case Reports and Literature Review. Diagnostics, 16.","DOI":"10.3390\/diagnostics16020189"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1016\/j.cld.2006.08.021","article-title":"Noncirrhotic portal hypertension","volume":"10","author":"Sarin","year":"2006","journal-title":"Clin. Liver Dis."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1136\/gut.51.2.275","article-title":"Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: A re-evaluation in 28 patients","volume":"51","author":"Hillaire","year":"2002","journal-title":"Gut"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1309","DOI":"10.1111\/liv.15840","article-title":"Prevalence of hepatic steatosis and fibrosis in Turner syndrome: A prospective case\u2013control study","volume":"44","author":"Twohig","year":"2024","journal-title":"Liver Int."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1111\/cen.13811","article-title":"Liver biochemical abnormalities in Turner syndrome: A comprehensive characterization of an adult population","volume":"89","author":"Calanchini","year":"2018","journal-title":"Clin. Endocrinol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1002\/hep.20026","article-title":"Vascular involvement of the liver in Turner\u2019s syndrome","volume":"39","author":"Roulot","year":"2004","journal-title":"Hepatology"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1002\/ajmg.a.30724","article-title":"Adams\u2013Oliver syndrome and hepatoportal sclerosis: Occasional association or common mechanism?","volume":"135","author":"Girard","year":"2005","journal-title":"Am. J. Med. Genet. Part A"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1002\/ajmg.a.31192","article-title":"Association of Adams\u2013Oliver syndrome and hepatoportal sclerosis: An additional case","volume":"140","author":"Pouessel","year":"2006","journal-title":"Am. J. Med. Genet. Part A"},{"key":"ref_56","unstructured":"Adam, M.P., Feldman, J., Mirzaa, G.M., Pagon, R.A., Wallace, S.E., and Amemiya, A. (2026, August 25). Prolidase Deficiency, GeneReviews\u00ae, Available online: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK299584\/."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1159\/000536117","article-title":"Chronic liver disease in patients with prolidase deficiency: A case series","volume":"18","author":"Gopalakrishna","year":"2024","journal-title":"Case Rep. Gastroenterol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1097\/HEP.0000000000000461","article-title":"Progression of liver disease and portal hypertension in dyskeratosis congenita and related telomere biology disorders","volume":"78","author":"Vittal","year":"2023","journal-title":"Hepatology"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.1002\/lt.24842","article-title":"Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy","volume":"23","author":"Hillaire","year":"2017","journal-title":"Liver Transplant."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"S98","DOI":"10.1002\/ppul.26869","article-title":"Pathophysiology of cystic fibrosis liver disease","volume":"59","author":"Kasper","year":"2024","journal-title":"Pediatr. Pulmonol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"2134","DOI":"10.1016\/j.cgh.2018.10.046","article-title":"Obliterative portal venopathy without cirrhosis is prevalent in pediatric cystic fibrosis liver disease with portal hypertension","volume":"17","author":"Wu","year":"2019","journal-title":"Clin. Gastroenterol. Hepatol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1007\/s11845-022-03153-0","article-title":"Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis","volume":"192","author":"ONeill","year":"2023","journal-title":"Ir. J. Med. Sci."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"e11","DOI":"10.1016\/j.jcf.2017.03.006","article-title":"Liver disease in cystic fibrosis presents as non-cirrhotic portal hypertension","volume":"16","author":"Witters","year":"2017","journal-title":"J. Cyst. Fibros."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.iliver.2022.06.001","article-title":"A HRG novel mutation associated with idiopathic portal hypertension: Case report and literature review","volume":"1","author":"Tang","year":"2022","journal-title":"Iliver"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1016\/j.jhep.2015.11.027","article-title":"A de novo mutation in KCNN3 associated with autosomal dominant idiopathic non-cirrhotic portal hypertension","volume":"64","author":"Koot","year":"2016","journal-title":"J. Hepatol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1977","DOI":"10.1002\/hep.28499","article-title":"Recurrent recessive mutation in deoxyguanosine kinase causes idiopathic noncirrhotic portal hypertension","volume":"63","author":"Vilarinho","year":"2016","journal-title":"Hepatology"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1093\/cid\/cit001","article-title":"Genetic determinants of idiopathic noncirrhotic portal hypertension in HIV-infected patients","volume":"56","author":"Vispo","year":"2013","journal-title":"Clin. Infect. Dis."},{"key":"ref_68","doi-asserted-by":"crossref","unstructured":"Ye, X., Quan, X., Guo, X., Wang, Z., and Wu, H. (2023). Idiopathic non-cirrhotic portal hypertension in a patient with Talaromyces marneffei infection: A case report. BMC Infect. Dis., 23.","DOI":"10.1186\/s12879-023-08090-6"},{"key":"ref_69","doi-asserted-by":"crossref","unstructured":"Chen, X.-L., Serrano, D., Ghobadi, F., Mayhue, M., Hoebe, K., Ilangumaran, S., and Ramanathan, S. (2016). TCR and IL-7 signaling are altered in the absence of functional GTPase of the immune associated nucleotide binding protein 5 (GIMAP5). PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0151837"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"e20201745","DOI":"10.1084\/jem.20201745","article-title":"GIMAP5 maintains liver endothelial cell homeostasis and prevents portal hypertension","volume":"218","author":"Drzewiecki","year":"2021","journal-title":"J. Exp. Med."},{"key":"ref_71","first-page":"4905","article-title":"Impaired survival of peripheral T cells, disrupted NK\/NKT cell development, and liver failure in mice lacking Gimap5","volume":"112","author":"Schulteis","year":"2008","journal-title":"Blood J. Am. Soc. Hematol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1097\/MPG.0b013e31822960e9","article-title":"Long-term follow-up of children with 6-thioguanine-related chronic hepatoxicity following treatment for acute lymphoblastic leukaemia","volume":"53","author":"Rawat","year":"2011","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_73","doi-asserted-by":"crossref","unstructured":"Moulik, N.R., and Taj, M.M. (2017). Long-term risk of portal hypertension and related complications in children treated with 6-thioguanine for acute lymphoblastic leukemia: A single-center experience. Pediatr. Blood Cancer, 64.","DOI":"10.1002\/pbc.26495"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/0168-8278(90)90276-W","article-title":"Arsenic and non-cirrhotic portal hypertension: A report of eight cases","volume":"11","author":"Nevens","year":"1990","journal-title":"J. Hepatol."},{"key":"ref_75","doi-asserted-by":"crossref","unstructured":"Ye, J., Xie, Y., Xu, Y., Chen, N., and Tu, Y. (2023). Case report: Oxaliplatin-induced idiopathic non-cirrhotic portal hypertension: A case report and literature review. Front. Med., 10.","DOI":"10.3389\/fmed.2023.1285064"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1111\/apt.12049","article-title":"Risk factors and outcome of HIV-associated idiopathic noncirrhotic portal hypertension","volume":"36","author":"Schouten","year":"2012","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1007\/s12072-019-09979-8","article-title":"Pediatric non-cirrhotic portal fibrosis: Role of endoscopic management in determining long-term outcome","volume":"14","author":"Prasad","year":"2020","journal-title":"Hepatol. Int."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1002\/hep.1840150210","article-title":"Portal-vein obstruction in children leads to growth retardation","volume":"15","author":"Sarin","year":"1992","journal-title":"Hepatology"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1016\/j.cld.2018.06.007","article-title":"Cirrhosis and portal hypertension in the pediatric population","volume":"22","author":"Chapin","year":"2018","journal-title":"Clin. Liver Dis."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1111\/apt.15186","article-title":"TIPSS for variceal bleeding in patients with idiopathic non-cirrhotic portal hypertension: Comparison with patients who have cirrhosis","volume":"49","author":"Lv","year":"2019","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1055\/s-2004-830341","article-title":"Extrahepatic portal hypertension: Quality of life and somatic growth after surgery","volume":"15","author":"Menon","year":"2005","journal-title":"Eur. J. Pediatr. Surg."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1111\/hepr.14118","article-title":"Guidelines for the diagnosis and treatment of idiopathic portal hypertension, extrahepatic portal obstruction, and Budd\u2013Chiari syndrome in Japan","volume":"54","author":"Furuichi","year":"2024","journal-title":"Hepatol. Res."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"3000","DOI":"10.3748\/wjg.v26.i22.3000","article-title":"Diagnostic challenges in non-cirrhotic portal hypertension-porto sinusoidal vascular disease","volume":"26","author":"Rusu","year":"2020","journal-title":"World J. Gastroenterol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1148\/radiol.12111785","article-title":"Obliterative portal venopathy: Findings at CT imaging","volume":"263","author":"Glatard","year":"2012","journal-title":"Radiology"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1097\/MPG.0000000000003674","article-title":"Splenic stiffness is the best predictor of clinically significant varices in children with portal hypertension","volume":"76","author":"Upadhyay","year":"2023","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1097\/MPG.0000000000002327","article-title":"Hepatic venous pressure gradient measurements in children: Correlation with hepatic histology and clinical indicators of portal hypertension","volume":"68","author":"Ebel","year":"2019","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.3748\/wjg.v18.i11.1176","article-title":"Management of portal hypertension in children","volume":"18","author":"Gugig","year":"2012","journal-title":"World J. Gastroenterol. WJG"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1097\/MPG.0b013e318205993a","article-title":"Primary prophylaxis of variceal hemorrhage in children with portal hypertension: A framework for future research","volume":"52","author":"Ling","year":"2011","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1007\/s10620-011-1832-6","article-title":"Hepatopulmonary syndrome in children with cirrhotic and non-cirrhotic portal hypertension: A single-center experience","volume":"57","author":"Sari","year":"2012","journal-title":"Dig. Dis. Sci."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1016\/j.dld.2019.02.014","article-title":"Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study","volume":"51","author":"Cheli","year":"2019","journal-title":"Dig. Liver Dis."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"1368","DOI":"10.1002\/hep.28153","article-title":"Primary prophylaxis of variceal bleeding in children and the role of MesoRex bypass: Summary of the Baveno VI Pediatric Satellite Symposium","volume":"63","author":"Shneider","year":"2016","journal-title":"Hepatology"},{"key":"ref_92","first-page":"617","article-title":"The use of nonselective beta blockers for treatment of portal hypertension","volume":"13","year":"2017","journal-title":"Gastroenterol. Hepatol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1002\/cld.1182","article-title":"Nonselective Beta-Blockers in Portal Hypertension: Why, When, and How?","volume":"19","author":"Rabiee","year":"2022","journal-title":"Clin. Liver Dis."},{"key":"ref_94","doi-asserted-by":"crossref","unstructured":"Cifuentes, L.I., Gattini, D., Torres-Robles, R., and Gana, J.C. (2021). Beta-blockers versus placebo or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Cochrane Database Syst. Rev., CD011973.","DOI":"10.1002\/14651858.CD011973.pub2"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"2413","DOI":"10.1002\/hep.30132","article-title":"Idiopathic portal hypertension","volume":"68","author":"Baiges","year":"2018","journal-title":"Hepatology"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"751","DOI":"10.4254\/wjh.v16.i5.751","article-title":"Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis","volume":"16","author":"Willington","year":"2024","journal-title":"World J. Hepatol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"2276","DOI":"10.1002\/hep.26904","article-title":"Idiopathic portal hypertension: Natural history and long-term outcome","volume":"59","author":"Siramolpiwat","year":"2014","journal-title":"Hepatology"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"483","DOI":"10.4254\/wjh.v11.i5.483","article-title":"Successful treatment of noncirrhotic portal hypertension with eculizumab in paroxysmal nocturnal hemoglobinuria: A case report","volume":"11","author":"Alexopoulou","year":"2019","journal-title":"World J. Hepatol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/S0973-6883(11)60234-X","article-title":"Progression of noncirrhotic portal hypertension in a pediatric population","volume":"1","author":"Wojcik","year":"2011","journal-title":"J. Clin. Exp. Hepatol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1097\/MEG.0000000000001067","article-title":"Ten-year experience of transjugular intrahepatic portosystemic shunt for noncirrhotic portal hypertension","volume":"30","author":"Regnault","year":"2018","journal-title":"Eur. J. Gastroenterol. Hepatol."},{"key":"ref_101","doi-asserted-by":"crossref","unstructured":"AlRamahi, R., Marra, P., Chewning, R.H., Shin, D.S., Chick, J.F., and Monroe, E.J. (2025). Pediatric Portal Hypertension Interventions: Ectopic Varices, Portal Vein Recanalization, Portosystemic Shunt Creation, and Maintenance of Surgical Shunts. Semin Intervent Radiol.","DOI":"10.1055\/s-0045-1810053"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"18","DOI":"10.25259\/JCIS_36_2023","article-title":"Transjugular intrahepatic portosystemic shunt for pediatric portal hypertension: A meta-analysis","volume":"13","author":"Raissi","year":"2023","journal-title":"J. Clin. Imaging Sci."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1002\/lt.20431","article-title":"Liver transplantation for severe intrahepatic noncirrhotic portal hypertension","volume":"11","author":"Krasinskas","year":"2005","journal-title":"Liver Transplant."},{"key":"ref_104","doi-asserted-by":"crossref","unstructured":"Kawachi, S., Chiba, N., Nakagawa, M., Kobayashi, T., Hikita, K., Sano, T., Tomita, K., Hirano, H., Abe, Y., and Obara, H. (2020). Living donor liver transplantation for idiopathic portal hypertension with extrahepatic portal vein stenosis and splenic artery aneurysms: A case report and review of the literature. BMC Surg., 20.","DOI":"10.1186\/s12893-020-00921-6"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1330","DOI":"10.1097\/TP.0000000000004444","article-title":"Liver transplantation for porto-sinusoidal vascular liver disorder: Long-term outcome","volume":"107","author":"Magaz","year":"2023","journal-title":"Transplantation"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1016\/j.bbmt.2016.03.001","article-title":"Survival after hematopoietic stem cell transplant in patients with dyskeratosis congenita: Systematic review of the literature","volume":"22","author":"Barbaro","year":"2016","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"S183","DOI":"10.1016\/j.gim.2022.01.324","article-title":"eP289: Non-cirrhotic portal hypertension: Expanding the phenotype of CBL syndrome-related vasculopathy","volume":"24","author":"Yadav","year":"2022","journal-title":"Genet. Med."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/17\/6901\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T12:39:39Z","timestamp":1788784779000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/17\/6901"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,6]]},"references-count":107,"journal-issue":{"issue":"17","published-online":{"date-parts":[[2026,9]]}},"alternative-id":["jcm15176901"],"URL":"https:\/\/doi.org\/10.3390\/jcm15176901","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,6]]}}}