Pleuropulmonary pathology of vascular Ehlers-Danlos syndrome: spontaneous laceration, haematoma and fibrous nodules
Pleuropulmonary pathology of vascular Ehlers-Danlos syndrome: spontaneous laceration, haematoma and fibrous nodules
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DOI：
10.1111/j.1365-2559.2010.03574.x
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发表时间：
2010-06-01
期刊：
影响因子：
6.4
通讯作者：
Fukuda, Yuh
中科院分区：
文献类型：
--
作者：
Kawabata, Yoshinori;Watanabe, Akira;Fukuda, Yuh
目的：探讨血管性Ehler-Danlos综合征(VEDS)的胸膜肺病理表现。方法和结果：9例经细胞培养和/或分子生物学检查确诊的vEDS患者，均经手术肺活检、肺叶切除或尸检证实。男6例，女3例，平均年龄23.2岁。组织学特征如下：(1)与脆性和自发性撕裂有关的主要肺部病变：血肿7例，急性出血9例，纤维结节8例，骨化或骨髓形成6例，血管破裂5例，管腔内含铁血黄素沉着症9例，间质性含铁血黄素沉着症7例，肺泡壁和/或血管壁铁沉积5例，异物反应2例，肺气肿改变8例，滤泡形成2例；(Ii)SLB或肺叶切除术中继发性医源性胸膜肺损伤包括胸膜撕裂伤7例和肺撕裂伤8例。结论：肺组织自发性撕裂伤是其基本特征，继发于血肿和纤维结节形成。
Aims:The aim was to clarify the pleuropulmonary pathological findings of vascular Ehlers-Danlos syndrome (vEDS).Methods and results:Nine patients with confirmed vEDS by means of cell culture and/or molecular biological studies who had undergone surgical lung biopsy (SLB), lobectomy or autopsy were studied. Six patients were male and three were female with a mean age of 23.2 years. Histological features were as follows: (i) the main pulmonary lesions related to fragility and spontaneous laceration, these being haematomas in seven, acute haemorrhage in nine, fibrous nodule in eight, with ossification or bone marrow formation in six; vascular disruption in five; intraluminal haemosiderosis in nine; interstitial haemosiderosis in seven, with iron deposition in the alveolar wall and/or vessel wall in five and foreign body reaction in two; emphysematous changes in eight; and bleb formation in two; (ii) secondary iatrogenic pleuropulmonary injuries during SLB or lobectomy comprised pleural laceration in seven of 10 and lung laceration in eight of 10 specimens.Conclusions:Spontaneous laceration of lung tissue is an essential feature and is followed by haematoma and possible fibrous nodule formation.