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肝移植后门静脉血栓形成患儿门静脉高压的独特临床特征

Distinctive Clinical Features of Portal Hypertension in Children with Portal Vein Thrombosis Following Liver Transplantation.

作者信息

Sansotta Naire, Di Giorgio Angelo, Colusso Mara Marcella, Salvi Marco, Marra Paolo, Pinelli Domenico, Carobbio Alessandra, D'Antiga Lorenzo

机构信息

Paediatric Hepatology Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Piazza Oms 1, 24127 Bergamo, Italy.

Paediatric Surgery Unit, Hospital Papa Giovanni XXIII, 24127 Bergamo, Italy.

出版信息

Biomedicines. 2025 Aug 24;13(9):2061. doi: 10.3390/biomedicines13092061.

Abstract

: Portal vein thrombosis (PVT) occurs in nearly 8% of pediatric liver transplants (LT), leading to portal hypertension (PH). This study aims to describe the clinical features and management of PVT in children post-transplant (PVTt) compared to those with PVT in native livers (PVTn). : All children diagnosed with PVTt between January 2002 and October 2021 were included. The control group comprised pediatric patients with PVTn diagnosed and managed at our center during the same period. : PVTt was diagnosed in 37 out of 610 children (6%), while 36 children with PVTn were included as controls. At 5-year follow-up, medium-to-large esophageal varices (grade II-III) developed in 15/37 (38%) PVTt patients compared to 23/36 (64%) PVTn patients ( = 0.002). Among 11 patients who bled, upper gastrointestinal bleeding occurred in 2/7 (29%) with PVTt, versus 4/4 (100%) PVTn patients ( = 0.06). Mean spleen length was 9.3 cm in PVTt versus 7.4 cm in PVTn ( = 0.039). Mean platelet count was 76 × 10/L in PVTt versus 93 × 10/L in PVTn ( = 0.16). : Despite more severe PH and marked hypersplenism, children with PVTt have a reduced risk of developing esophageal varices, but an increased risk of bleeding from the lower gastrointestinal tract. This suggests the need for a different surveillance strategy in this patient group. Individualized care is key, mainly in PVTt, where hypersplenism does not correlate with risk of bleeding from esophageal varices.

摘要

门静脉血栓形成(PVT)在近8%的小儿肝移植(LT)中出现,导致门静脉高压(PH)。本研究旨在描述移植后儿童(PVTt)与原发性肝脏PVT(PVTn)患儿相比PVT的临床特征及处理方法。

纳入2002年1月至2021年10月期间所有诊断为PVTt的儿童。对照组包括同期在本中心诊断和处理的患有PVTn的儿科患者。

610名儿童中有37名(6%)被诊断为PVTt,36名患有PVTn的儿童被纳入作为对照。在5年随访中,15/37(38%)的PVTt患者出现中至大的食管静脉曲张(II-III级),而PVTn患者为23/36(64%)(P = 0.002)。在11例出血患者中,2/7(29%)的PVTt患者发生上消化道出血,而PVTn患者为4/4(100%)(P = 0.06)。PVTt患者的平均脾脏长度为9.3 cm,而PVTn患者为7.4 cm(P = 0.039)。PVTt患者的平均血小板计数为76×10⁹/L,而PVTn患者为93×10⁹/L(P = 0.16)。

尽管PVTt患儿存在更严重的PH和明显的脾功能亢进,但其发生食管静脉曲张的风险降低,但下消化道出血的风险增加。这表明该患者群体需要不同的监测策略。个体化护理是关键,主要是在PVTt中,脾功能亢进与食管静脉曲张出血风险无关。

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