Resolution of protein-losing enteropathy with low-molecular weight heparin in an adult patient with Fontan palliation
Resolution of protein-losing enteropathy with low-molecular weight heparin in an adult patient with Fontan palliation
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DOI：
10.1016/j.athoracsur.2007.06.064
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发表时间：
2007-12-01
影响因子：
4.6
通讯作者：
Tam, James W.
中科院分区：
文献类型：
--
作者：
Bhagirath, Kapil M.;Tam, James W.
蛋白缺失性肠病是Fontan手术后众所周知的并发症，普通肝素疗法已被尝试作为一种潜在的治疗方法，并取得了一些成功。低分子肝素被认为是无效的。我们现在描述一个病例，在Fontan姑息治疗后，一名患有蛋白缺失性肠病的成人患者成功地接受了全身剂量的低分子肝素治疗，24小时粪便α-1抗胰蛋白酶水平和血清白蛋白完全消失。患者继续接受这种治疗后仍处于缓解期。总而言之，这些发现是新的，迫使我们重新评估我们对这种困难情况的病理生理学理解。
Protein-losing enteropathy is a well-known complication after the Fontan procedure, and unfractionated heparin therapy has been tried with some success as a potential therapy. Low-molecular weight heparin is believed to be ineffective. We now describe a case in which an adult patient with protein-losing enteropathy after Fontan palliation was successfully treated with systemic doses of low-molecular weight heparin, with complete resolution in the 24-hour fecal alpha-1 anti-trypsin level and serum albumin. The patient continues to remain in remission with this therapy. In conclusion, these findings are novel and compel us to re-evaluate our pathophysiologic understanding of this difficult condition.