Saaybi Stephanie R, Shiau Henry, Lee Goo, Orandi Babak John, Gutierrez Sanchez Luz Helena
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.
Am J Transplant. 2025 May;25(5):1123-1126. doi: 10.1016/j.ajt.2025.01.002. Epub 2025 Jan 8.
The association between hypopituitarism and metabolic dysfunction-associated steatotic liver disease is increasingly recognized, although data about therapies targeting recurrence posttransplant is limited. An 8-year-old with hypopituitarism-associated metabolic dysfunction-associated steatotic liver disease underwent a liver transplant due to rapid progression of metabolic dysfunction-associated steatohepatitis. Hepatosteatosis recurred within weeks. Her therapeutic plan included a glucagon-like peptide-1 agonist and growth hormone replacement. Her transaminases normalized in 2.5 months, and her macrosteatosis significantly improved on the 1-year surveillance biopsy. This case highlights one of the youngest reported children with hypopituitarism to have undergone transplantation for rapidly progressing metabolic dysfunction-associated steatohepatitis and its recurrence post-operatively. We observed that steatosis improved with growth hormone replacement and glucagon-like peptide-1 agonist therapy. If started early, this combination could help delay recurrence of steatosis post-transplantation. Further research is needed to determine long-term effects and establish protocols.
垂体功能减退与代谢功能障碍相关脂肪性肝病之间的关联日益受到认可，尽管针对移植后复发的治疗数据有限。一名8岁患有垂体功能减退相关代谢功能障碍相关脂肪性肝病的患儿因代谢功能障碍相关脂肪性肝炎快速进展而接受了肝移植。肝脂肪变性在数周内复发。她的治疗方案包括使用胰高血糖素样肽-1激动剂和生长激素替代治疗。她的转氨酶在2.5个月内恢复正常，在1年的监测活检中，她的大泡性脂肪变性显著改善。该病例突出了报道中最年轻的因快速进展的代谢功能障碍相关脂肪性肝炎接受移植且术后复发的垂体功能减退患儿之一。我们观察到，生长激素替代治疗和胰高血糖素样肽-1激动剂治疗可改善脂肪变性。如果早期开始使用，这种联合治疗可能有助于延迟移植后脂肪变性的复发。需要进一步研究以确定长期影响并制定方案。
