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成人患者先天性肺囊性腺瘤样畸形合并支气管闭锁累及不同肺叶:一例报告

Congenital cystic adenomatoid malformation of the lung associated with bronchial atresia involving a different lobe in an adult patient: a case report.

作者信息

Discioscio Valerio, Feraco Paola, Bazzocchi Alberto, Femia Rayka, Romeo Chiara, Fasano Luca, Pacilli Angela M, Zompatori Maurizio

机构信息

Imaging Section, Department of Radiologic and Histocytopathologic Sciences, University of Bologna, S, Orsola--Malpighi Hospital, Via Massarenti 9, 40100 Bologna, Italy.

出版信息

J Med Case Rep. 2010 May 28;4:164. doi: 10.1186/1752-1947-4-164.

Abstract

INTRODUCTION

Congenital cystic adenomatoid malformation of the lung is an uncommon cause of respiratory distress in neonates and babies. The disorder is usually diagnosed in the neonatal period and the first two years of life. This anomaly has been described in association with bronchopulmonary sequestration, extralobar intra-abdominal sequestration or bronchial atresia in live and stillborn babies. It is rarely encountered in adults, in whom the diagnosis is made incidentally from mass lesion features seen on chest radiographs. The oldest patients recorded with this malformation have been about 35 years old, and only 10% of primary diagnoses are made after the first year of life. Delayed diagnosis can be related to infection or serendipitous discovery.

CASE PRESENTATION

We describe the radiological findings of a 34-year-old Caucasian woman with a clinical history of recurrent pneumonia, intermittent anterior pleuritic chest pain and haemoptysis. Congenital cystic adenomatoid malformation of the lung associated with bronchial atresia involving a different lobe was discovered.

CONCLUSION

Although rare in adults, congenital cystic adenomatoid malformation should be suspected in adult patients who suffer from recurrent or persistent non-productive coughs. The discovery of an association of congenital cystic adenomatoid malformation with bronchial atresia in adulthood is rare but possible, even in different lobes.

摘要

引言

先天性肺囊性腺瘤样畸形是新生儿和婴儿呼吸窘迫的罕见原因。这种疾病通常在新生儿期以及出生后的头两年被诊断出来。在活产和死产婴儿中,这种异常情况已被描述为与支气管肺隔离症、叶外型腹腔内隔离症或支气管闭锁有关。在成年人中很少见,其诊断是通过胸部X光片上发现的肿块特征偶然做出的。记录到的患有这种畸形的最年长患者约35岁,仅10%的初步诊断是在出生后第一年之后做出的。延迟诊断可能与感染或偶然发现有关。

病例报告

我们描述了一名34岁白种女性的放射学检查结果,她有复发性肺炎、间歇性前侧胸膜炎性胸痛和咯血的临床病史。发现了与支气管闭锁相关的先天性肺囊性腺瘤样畸形,且涉及不同肺叶。

结论

尽管先天性肺囊性腺瘤样畸形在成年人中罕见,但对于患有复发性或持续性干咳的成年患者应怀疑此病。成年期发现先天性肺囊性腺瘤样畸形与支气管闭锁相关的情况虽罕见但有可能,甚至在不同肺叶中也可能出现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f44e/2893193/fcd8b48f7f07/1752-1947-4-164-1.jpg

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