血小板衍生生长因子抑制——先天性膈疝肺动脉高压的一种新疗法？

Platelet-derived growth factor inhibition--a new treatment of pulmonary hypertension in congenital diaphragmatic hernia?

作者信息

Frenckner Björn, Broomé Michael, Lindström Maria, Radell Peter

机构信息

Department of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska Institutet, SE-171 76 Stockholm, Sweden.

出版信息

J Pediatr Surg. 2008 Oct;43(10):1928-31. doi: 10.1016/j.jpedsurg.2008.07.022.

DOI:10.1016/j.jpedsurg.2008.07.022

PMID:18926235

Abstract

Increased pulmonary vascular resistance causing pulmonary artery hypertension is a major problem in the treatment of congenital diaphragmatic hernia with a strong association to mortality. We here report a patient with intractable pulmonary hypertension at 4 weeks of age unresponsive to conventional treatment. After administration of the platelet-derived growth factor (PDGF) receptor antagonist imatinib, pulmonary artery pressure gradually decreased to acceptable levels and the patient's clinical condition gradually improved.

摘要

肺血管阻力增加导致肺动脉高压是先天性膈疝治疗中的一个主要问题，与死亡率密切相关。我们在此报告一名4周龄患有顽固性肺动脉高压的患者，对传统治疗无反应。在给予血小板衍生生长因子（PDGF）受体拮抗剂伊马替尼后，肺动脉压力逐渐降至可接受水平，患者的临床状况逐渐改善。