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本文引用的文献

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Pulmonary arterial hypertension: an update on diagnosis and treatment.肺动脉高压：诊断与治疗的最新进展。
Am Fam Physician. 2010 Aug 15;82(4):370-7.
2
Pharmacotherapeutic management of pulmonary arterial hypertension.肺动脉高压的药物治疗管理。
Cardiol Rev. 2010 May-Jun;18(3):148-62. doi: 10.1097/CRD.0b013e3181d4e921.
3
Clinical and genetic aspects of neurofibromatosis 1.神经纤维瘤病 1 的临床和遗传方面。
Genet Med. 2010 Jan;12(1):1-11. doi: 10.1097/GIM.0b013e3181bf15e3.
4
Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT).肺动脉高压诊断和治疗指南：欧洲心脏病学会（ESC）和欧洲呼吸学会（ERS）肺动脉高压诊断和治疗工作组，得到国际心肺移植学会（ISHLT）认可。
Eur Heart J. 2009 Oct;30(20):2493-537. doi: 10.1093/eurheartj/ehp297. Epub 2009 Aug 27.
5
Updated clinical classification of pulmonary hypertension.肺动脉高压的更新临床分类。
J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S43-S54. doi: 10.1016/j.jacc.2009.04.012.
6
Genetics and genomics of pulmonary arterial hypertension.肺动脉高压的遗传学与基因组学
J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S32-S42. doi: 10.1016/j.jacc.2009.04.015.
7
Type 1 neurofibromatosis complicated by pulmonary artery hypertension: a case report.1型神经纤维瘤病合并肺动脉高压：1例报告
J Med Invest. 2007 Aug;54(3-4):354-8. doi: 10.2152/jmi.54.354.
8
Is pulmonary arterial hypertension in neurofibromatosis type 1 secondary to a plexogenic arteriopathy?1型神经纤维瘤病中的肺动脉高压是继发于丛状动脉病吗？
Chest. 2007 Sep;132(3):798-808. doi: 10.1378/chest.06-3017. Epub 2007 Jun 15.
9
Pulmonary hypertension in neurofibromatosis.
Am J Cardiol. 2007 Apr 15;99(8):1177-8. doi: 10.1016/j.amjcard.2006.11.072. Epub 2007 Mar 8.
10
Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective.神经纤维瘤病1型和神经纤维瘤病2型：21世纪的视角
Lancet Neurol. 2007 Apr;6(4):340-51. doi: 10.1016/S1474-4422(07)70075-3.

与1型神经纤维瘤病相关的肺动脉高压

Pulmonary arterial hypertension associated with neurofibromatosis type 1.

作者信息

Carrascosa Miguel F, Larroque Isabel Celemín, Rivero Juan-Luis García, García José-Antonio Saiz-Quevedo, Hoz Marta Cano, Ares Miguel Ares, López Xabier Arrastio, Caviedes José-Ramón Salcines

机构信息

Department of Internal Medicine, Hospital of Laredo, Laredo, Spain.

出版信息

BMJ Case Rep. 2010 Nov 29;2010:bcr0520102961. doi: 10.1136/bcr.05.2010.2961.

DOI:10.1136/bcr.05.2010.2961
PMID:22798089
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3029927/
Abstract

The authors report a case of severe pulmonary arterial hypertension (PAH) in a 75-year-old woman who had received a diagnosis of neurofibromatosis type 1 (NF1) 23 years before. She presented with progressive dyspnoea and recurrent syncope. Even though the patient initially improved after starting supportive and specific treatment for PAH, she then deteriorated and died from respiratory failure 11 months after the diagnosis of PAH. Prompt recognition of such an unusual association between PAH and NF1 and appropriate therapeutic intervention could ameliorate quality of life and prolong survival in this patient population.

摘要

作者报告了一例75岁女性的严重肺动脉高压（PAH）病例，该患者23年前被诊断为1型神经纤维瘤病（NF1）。她出现进行性呼吸困难和反复晕厥。尽管患者在开始针对PAH的支持性和特异性治疗后最初有所改善，但随后病情恶化，在诊断为PAH后11个月死于呼吸衰竭。及时认识到PAH与NF1之间的这种不寻常关联并进行适当的治疗干预，可以改善该患者群体的生活质量并延长生存期。