Pegado C S, Garcia A G
Departamento de Anatomia Patologica, Instituto Fernandes Figueira, FIOCRUZ, Rio de Janeiro, Brazil.
Pediatr Pathol. 1994 Sep-Oct;14(5):781-6. doi: 10.3109/15513819409037675.
We present an unusual case of extralobar pulmonary sequestration associated with hyaline membrane disease (HMD) that caused the death of a premature baby in the first day of life. The sequestered parenchyma was nourished by an aberrant aortic vessel. Notable was the presence of typical HMD in all the lung parenchyma perfused by the pulmonary artery; the sequestered lung tissue presented a dysplastic structure compatible with CCAM. A few similar cases have been found in the literature. In all of the reported cases there are morphologic aspects characteristic of HMD in the portions normally receiving blood from the pulmonary artery. These findings suggest the importance of the blood pulmonary circulation in the pathogenesis of HMD, whose exact causes are not fully known.
我们报告了一例罕见的肺叶外型肺隔离症合并透明膜病(HMD)的病例,该病例导致一名早产儿在出生第一天死亡。隔离的实质组织由一支异常的主动脉血管供血。值得注意的是,在所有由肺动脉灌注的肺实质中均存在典型的HMD;隔离的肺组织呈现出与先天性肺气道畸形(CCAM)相符的发育异常结构。文献中已发现一些类似病例。在所有报道的病例中,正常由肺动脉供血的部分均存在HMD的形态学特征。这些发现提示肺血液循环在HMD发病机制中的重要性,而HMD的确切病因尚未完全明确。