Simultaneous Pancreas and Kidney Transplantation in a Patient With Congenital Hemophilia A: A rare Case Report and Management Insights

IF 0.8 4区医学 Q4 IMMUNOLOGY

Transplantation proceedings Pub Date : 2026-06-01 Epub Date: 2026-05-18 DOI:10.1016/j.transproceed.2026.04.024

Takahiro Shimaoka , Kazuki Sasaki , Hirofumi Akita , Yosuke Mukai , Shinichiro Hasegawa , Daisaku Yamada , Yoshito Tomimaru , Takehiro Noda , Hidenori Takahashi , Shogo Kobayashi , Hirokazu Kashiwagi , Hiroaki Yonishi , Kazuyuki Miyashita , Kazuaki Yamanaka , Shigeaki Nakazawa , Yoichi Kakuta , Norio Nonomura , Yuichiro Doki , Hidetoshi Eguchi

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引用次数: 0

Abstract

Background

Simultaneous pancreas-kidney transplantation (SPK) is a definitive treatment for end-stage kidney disease (ESKD) and type 1 diabetes mellitus. However, even with careful perioperative control of hemostasis, SPK carries a high risk of postoperative bleeding, which is exacerbated in patients with hemophilia A. Here, we report the first known successful SPK in a patient with congenital hemophilia A.

Case report

A 52-year-old man was diagnosed with hemophilia A at 2 years of age. He developed type 1 diabetes mellitus at 25 years of age, which progressed to ESKD at 43 years of age. A comprehensive preoperative FVIII replacement protocol was applied during the perioperative period, using continuous infusion with bolus supplementation to maintain target VIII activity levels (100% intraoperatively, 80% during the first postoperative week, and ≥30% thereafter).

Conclusion

This case demonstrates that SPK transplantation can be safely performed in patients with congenital hemophilia A, by implementing appropriate FVIII replacement strategies and close multidisciplinary collaboration.

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先天性血友病a患者同时胰肾移植一例：罕见病例报告及治疗见解。

背景：同步胰肾移植（SPK）是终末期肾病（ESKD）和1型糖尿病的决定性治疗方法。然而，即使在围手术期小心控制止血，SPK也会带来术后出血的高风险，这在a型血友病患者中会加剧。在这里，我们报告了已知的第一例先天性a型血友病患者成功的SPK。病例报告：一名52岁的男性在2岁时被诊断为a型血友病。25岁时患1型糖尿病，43岁时发展为ESKD。围手术期采用全面的术前FVIII替代方案，持续输注并补充小丸以维持靶VIII活性水平（术中100%，术后第一周80%，术后≥30%）。结论：该病例表明，通过实施适当的FVIII替代策略和密切的多学科合作，SPK移植可以安全地用于先天性A型血友病患者。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Transplantation proceedings 医学-免疫学

CiteScore

1.70

自引率

0.00%

发文量

502

审稿时长

60 days

期刊介绍： Transplantation Proceedings publishes several different categories of manuscripts, all of which undergo extensive peer review by recognized authorities in the field prior to their acceptance for publication. The first type of manuscripts consists of sets of papers providing an in-depth expression of the current state of the art in various rapidly developing components of world transplantation biology and medicine. These manuscripts emanate from congresses of the affiliated transplantation societies, from Symposia sponsored by the Societies, as well as special Conferences and Workshops covering related topics. Transplantation Proceedings also publishes several special sections including publication of Clinical Transplantation Proceedings, being rapid original contributions of preclinical and clinical experiences. These manuscripts undergo review by members of the Editorial Board. Original basic or clinical science articles, clinical trials and case studies can be submitted to the journal?s open access companion title Transplantation Reports.