Abou Hamdan K, Chabot F, Capron F, Debelle L, Fery M, Polu J M
Service des Maladies Respiratoires et Réanimation Respiratoire, CHU de Brabois, rue du Morvan, 54511 Vandoeuvre-les-Nancy, France.
Rev Mal Respir. 2001 Sep;18(4 Pt 1):436-9.
We report a new case of diffuse pulmonary lymphangiomatosis in a 22 year-old man with a dysmorphic syndrome. The disease started with dyspnea which became rapidly disabling. The diagnosis was established from a pulmonary biopsy. Our patient developed severe chronic respiratory failure. Diffuse pulmonary lymphangiomatosis is a very uncommon disease. It is seen predominantly in children, exceptionally in adults, and affects both sexes equally. Symptoms like dyspnea and cough, pulmonary function with restrictive pattern, and interstitial syndrome, are not specific. Only pathology is evocative, characterized primarily by multifocal proliferation of pulmonary lymphatic vessels and increased number of complex anastomosing channels. These channels tend to dilate with time. The prognosis is poor and the treatment essentially palliative.
我们报告一例22岁患有畸形综合征男性的弥漫性肺淋巴管瘤病新病例。该病始于呼吸困难，且迅速发展至功能丧失。诊断通过肺活检得以确立。我们的患者出现了严重的慢性呼吸衰竭。弥漫性肺淋巴管瘤病是一种非常罕见的疾病。它主要见于儿童，成人中极为罕见，且男女发病率相同。诸如呼吸困难和咳嗽等症状、具有限制性模式的肺功能以及间质性综合征并不具有特异性。只有病理学检查具有提示意义，其主要特征为肺淋巴管的多灶性增生以及复杂吻合通道数量增加。这些通道往往会随着时间推移而扩张。预后较差，治疗基本以姑息治疗为主。
