西地那非成功治疗弥漫性淋巴管瘤病的胸部受累情况
Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil.
作者信息
Maurac Arnaud, Debray Marie-Pierre, Crestani Bruno, Taillé Camille
机构信息
Centre Hospitalier Universitaire de Nancy, Département de pneumologie, CHRU de Nancy, Vandoeuvre-lès-Nancy, France.
Service de Radiologie, Hôpital Bichat, Assistance Publique - Hôpitaux de Paris, Paris, France.
出版信息
BMJ Case Rep. 2019 Apr 23;12(4):e228523. doi: 10.1136/bcr-2018-228523.
General lymphatic anomaly (GLA) is a very rare disorder, characterised by multifocal lymphatic malformations into various tissues that is due to congenital abnormalities of lymphatic development. No treatment has ever proved its efficiency.We report a 22-year-old man with recurrent bronchial casts due to thoracic involvement of GLA. After a 6-month treatment with sildenafil (20 mg three times a day), a phosphodiesterase 5 inhibitor, chest CT scan showed a complete regression of ground-glass opacities and lung function test results improved substantially and remained stable for 1 year. The treatment was well tolerated.This observation suggests that sildenafil may be a therapeutic approach to be tested in thoracic involvement of GLA.
全身性淋巴管异常（GLA）是一种非常罕见的疾病，其特征是由于淋巴管发育的先天性异常，在各种组织中出现多灶性淋巴管畸形。尚无治疗方法证明其有效性。我们报告一名22岁男性，因GLA累及胸部出现复发性支气管铸型。在用磷酸二酯酶5抑制剂西地那非（每日三次，每次20毫克）治疗6个月后，胸部CT扫描显示磨玻璃影完全消退，肺功能测试结果显著改善并在1年内保持稳定。该治疗耐受性良好。这一观察结果表明，西地那非可能是一种有待在GLA累及胸部时进行测试的治疗方法。
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