Class 1. Pulmonary Hypertension Associated with Liver Disease Archives

Non-Extremity Thrombosis Sequelae in Children: Screening, Diagnosis, and Management

Riten Kumar, Vilmarie Rodriguez, Clay Cohen, Madhvi Rajpurkar, Ayesha ZiaBoston Children’s Hospital and Harvard Medical School. Nationwide Children’s Hospital and Ohio State University. Texas Children’s Hospital and Baylor College of Medicine. Central Michigan University, Children’s Hospital of Michigan and Wayne State University. University of Texas Southwestern.United States Journal od Thrombosis and HemostasisJ Thronb Hemost 2026; […]

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Fibrotic liver injury in biliary atresia: long-term implications

Maria Hukkinen, Mikko P PakarinenNew Children’s Hospital, University of Helsinki and Helsinki University Hospital.Finland World Journal of Pediatric SurgeryWorld J Pediatr Surg 2025; 8: DOI: 10.1136/wjps-2025-001098 AbstractNormalization of serum bilirubin after Kasai portoenterostomy (KPE) is a prerequisite for long-term native liver (NL) survival. Following a successful KPE, fibrotic liver injury progresses variably, although a significant proportion

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Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome

Jennifer Merk, Delphine Yung, Jason N. Wright, Raj P. KapurUniversity of Washington and Seattle Children’s Hospital.United States PediatricsPediatrics 2025;DOI: 10.1542/peds.2025-073243 AbstractA 13-year-old girl presented for evaluation of pulmonary hypertension after symptoms of dyspnea and exercise intolerance. Full evaluation was negative except for abdominal ultrasonography with splenomegaly and esophageal varices suggestive of portal hypertension. Cardiac and hepatic

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Pulmonary vascular complications in children who survive long-term after Kasai portoenterostomy for biliary atresia: a narrative review

Jie Zhang, Gong ChenChildren’s Hospital of Fudan University.China World Journal of Pediatric SurgeryWorld J Pediatr Surg 2025; 8: DOI: 10.1136/wjps-2025-001067 AbstractFollowing Kasai portoenterostomy for biliary atresia (BA), long-term survival in children is associated with chronic liver cirrhosis leading to hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). This further causes a diverse array of respiratory and circulatory

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Early portopulmonary hypertension predicts mortality in patients with cirrhosis: insights from the PORTO-DETECT cohort

Luis Téllez, Jesús Donate, Antonio Guerrero, Idoia Agulleiro Beraza, Diego San Martín, Edilmar Alvarado, Victor Echevarría, Ángela Puente, Javier Zamora, Susana Del Prado, Andrés Tenes, Guillem Balagué, Barbara Vidal, Cristina Geli, Miguel Ángel Rodríguez-Gandía, Anna Baiges, Anna Brujats, Daniel Lopéz-Herrera Rodríguez, Santiago Fernández-Gordón Sánchez, José Ignacio Fortea, Maria Jesús Del Cerro, Candid Villanueva, Juan Carlos

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Porto-Pulmonary Hypertension in Children: Insights From a National Registry

Sadia Quyam, Alastair Baker, Alistair Calder, Shahin MoledinaGreat Ormond Street Hospital for Children. University College London. King’s College Hospital .Inited Kingdom Pulmonary CirculationPulm Circ 2025; 15: DOI: 10.1002/pul2.70133 AbstractPorto-pulmonary hypertension (PoPH) represents a rare but significant form of pulmonary arterial hypertension (PAH) in children. Despite its clinical importance, systematic analyses of paediatric presentations and outcomes remain limited.

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Liver and systemic hemodynamics in cirrhotic children.

Roberto Tambucci, Xavier Stephenne, Aniss Channaoui, Catherine de MagnéeCliniques Universitaires Saint-Luc and UCLouvain. Belgium World Journal of HepatologyWorld J Hepatol 2025; 17: DOI: 10.4254/wjh.v17.i7.103179 AbstractPortal hypertension and cirrhosis are associated with severe hemodynamic changes in hepatic and systemic circulation in the adult population. During cirrhosis progression, circulation becomes hyperdynamic, with cardiac, pulmonary and renal consequences. Cirrhotic

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Lethal Neonatal Pulmonary Hypertension in Trisomy 21 (T21) Likely Due to Congenital Portosystemic Shunts

Rachel Guest, Dilshad Dhaliwal, Darryl Kinnear, Debra Kearney, Nahir Cortes-Santiago, Nidhy Varghese, Kalyani R. PatelBaylor College of Medicine and Texas Children’s Hospital.United States Pediatric and Developmental PathologyPediatr Devel Pathol 2025; DOI: 10.1177/10935266251343287 AbstractChildren with Trisomy 21 (T21) have an increased incidence of pulmonary hypertension (PHTN); most commonly due to congenital heart/lung diseases but also in conjunction

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Congenital portosystemic shunts: diagnosis and treatment

Stéphanie Franchi-Abella, Emmanuel Gonzales, Oanez Ackermann, Sophie Banchereau, Daniéle Pariente, Florent Guérin, International Registry of Congenital Poertosystemic Shunt MembersHôpital Bicêtre, Hôpitaux Universitaire Paris-Sud and Assistance Publique Hôpitaux de Paris. National Reference Centre for Rare Pediatric Liver Diseases and Filfoie. University Paris -Sud. France Abdominal RadiologyAbd Radiol 2018; 43: 2023-2036DOI: 10.1007/s00261-018-1619-8 AbstractCongenital portosystemic shunts (CPSS) are rare

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Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single-Center, Retrospective Study in Japan

Athaya Vorasittha, Seisuke Sakamoto, Yusuke Yanagi, Kazuki Degawa, Hirotaka Kato, Tasuku Kodama, Ryuji Komine, Masaki Yamada, Hajime Uchida, Akinari Fukuda, Chiduko Haga, Takako Yoshioka, Mureo KasaharaNational Center for Child Health and Development. Japan Pediatric TransplantationPediatr Transplant 2025; 29: DOI: 10.1111/petr.70078 AbstractAim: Liver transplantation (LT) is a well-accepted treatment for primary sclerosing cholangitis (PSC) with generally good outcomes,

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