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[先天性肺囊性腺瘤样畸形。3例迟发表现病例报告]

[Congenital cystic adenomatoid malformation of the lung. Report of 3 cases with late presentation].

作者信息

Ayadi-Kaddour A, Chaabouni S, Meraï S, Ben Mrad S, Djilani H, Tritar F, El Mezni F

机构信息

Service d'Anatomie et de cytologie pathologiques, Hôpital Abderrahman-Mami, Ariana, Tunisie.

出版信息

Rev Mal Respir. 2008 Mar;25(3):338-43. doi: 10.1016/s0761-8425(08)71555-9.

Abstract

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. In many cases, respiratory distress occurs during the neonatal period, and in about 80- 85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. It is very rare that presentation is delayed until adulthood. We report three cases of CCAM presenting in adults. The diagnosis was based on clinical and radiological findings in one case and two patients were not diagnosed until surgery. The lesion was present in the right lung in two and in left lung in one patient. All patients underwent surgical resection. The result of histopathological examination confirmed CCAM Stocker type 1, without malignancy. The post operative follow up showed an excellent recovery. Clinicians and pathologists need to be aware of the fact that CCAM can be present for the first time in adolescents or in adults. The clinical diagnosis is suggested by radiographic findings and is confirmed at pathology as surgery is generally indicated.

摘要

肺先天性囊性腺瘤样畸形(CCAM)是一种罕见的先天性发育异常,约占所有先天性肺部病变的25%。在许多病例中,新生儿期会出现呼吸窘迫,约80 - 85%的患者因呼吸道感染在两岁前被诊断出CCAM。症状延迟至成年期出现的情况非常罕见。我们报告三例成人CCAM病例。其中一例根据临床和影像学检查结果确诊,另外两例患者直到手术时才被诊断出来。两例患者的病变位于右肺,一例位于左肺。所有患者均接受了手术切除。组织病理学检查结果证实为CCAM Stocker 1型,无恶性病变。术后随访显示恢复良好。临床医生和病理学家需要意识到CCAM可能首次出现在青少年或成人中。影像学检查结果提示临床诊断,手术通常是必要的,最终通过病理检查得以确诊。

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