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患有心脏病儿童的支气管铸型:肺淋巴异常的作用

Bronchial casts in children with cardiopathies: the role of pulmonary lymphatic abnormalities.

作者信息

Languepin J, Scheinmann P, Mahut B, Le Bourgeois M, Jaubert F, Brunelle F, Sidi D, de Blic J

机构信息

Department of Pediatric Pulmonology, Hôpital Necker Enfants Malades, Paris, France.

出版信息

Pediatr Pulmonol. 1999 Nov;28(5):329-36. doi: 10.1002/(sici)1099-0496(199911)28:5<329::aid-ppul4>3.0.co;2-k.

DOI:10.1002/(sici)1099-0496(199911)28:5<329::aid-ppul4>3.0.co;2-k
PMID:10536063
Abstract

Expectoration of bronchial casts, a condition also called plastic bronchitis, is very rare in children. Bronchial casts may be associated with bronchopulmonary diseases associated with mucus hypersecretion, bronchopulmonary bacterial infections, congenital and acquired cardiopathies, or pulmonary lymphatic abnormalities. A classification based on anatomy and pathology has been proposed which identifies an "acellular" group associated with congenital cardiopathies and palliative surgery. We report on 3 cases with bronchial casts associated with cardiopathy. Observations suggest that the formation of bronchial casts may result from lymphatic leakage into the bronchi. The 3 cases on which we report were immunodeficient and had pulmonary lymphatic abnormalities. The bronchial casts contained lymphocytes and lipids, as determined by histologic examination. In the absence of congenital pulmonary or diffuse lymphatic dysplasia associated with cardiopathy, the principal factors resulting in the formation of bronchial casts appear to be surgical trauma to the lymphatic channels surrounding the bronchi, pleural adhesions, and high systemic venous blood pressure. The prognosis for these patients is poor, and possibilities for treatment are limited.

摘要

咳出支气管铸型(一种也被称为塑形支气管炎的病症)在儿童中非常罕见。支气管铸型可能与伴有黏液分泌过多的支气管肺部疾病、支气管肺部细菌感染、先天性和后天性心脏病或肺淋巴管异常有关。已经提出了一种基于解剖学和病理学的分类方法,该方法确定了一个与先天性心脏病和姑息性手术相关的“无细胞”组。我们报告了3例与心脏病相关的支气管铸型病例。观察结果表明,支气管铸型的形成可能是由于淋巴管漏入支气管所致。我们报告的这3例病例均存在免疫缺陷且有肺淋巴管异常。经组织学检查确定,支气管铸型含有淋巴细胞和脂质。在不存在与心脏病相关的先天性肺或弥漫性淋巴管发育异常的情况下,导致支气管铸型形成的主要因素似乎是围绕支气管的淋巴管的手术创伤、胸膜粘连以及高体循环静脉血压。这些患者的预后很差,治疗选择有限。

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