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先天性肺气道畸形(CPAM):一例病例报告及文献综述

Congenital pulmonary airway malformation (CPAM): A case report and review of the literature.

作者信息

Aryal Krishnaraj, Regmi Pradeep Raj, Adhikari Gauri, Bhhattarai Ujjwal, Sedhain Sagar Prasad

机构信息

Department of Radiology, Institute of Medicine, Tribhuvan University Teaching Hospital, Maharajgunj, Kathmandu, Nepal.

Department of Medicine, Nepalese Army Student of Health Science- College of Medicine, Kathmandu, Nepal.

出版信息

Radiol Case Rep. 2023 Jul 25;18(10):3483-3486. doi: 10.1016/j.radcr.2023.07.018. eCollection 2023 Oct.

Abstract

Congenital pulmonary airway malformation (CPAM) is a rare congenital dysplastic malformation characterized by failure of bronchial development and localized glandular overgrowth. Previously known as Congenital Cystic Adenoid Malformation (CCAM), CPAM is classified into 5 types, from type 0 to type IV, depending upon the origin of pulmonary areas of the lung, cyst size, and cyst appearance. CPAM is a rare congenital anomaly typically diagnosed prenatally in ultrasound. However, few cases are diagnosed in childhood and even fewer in adulthood. CPAM can be differentiated from pulmonary sequestration based on the origin of the arterial supply; the former has its arterial supply from the pulmonary artery, whereas pulmonary sequestration has its arterial supply from the systemic circulation. Another differential diagnosis of CPAM includes congenital bronchogenic cyst, congenital lobar emphysema, pleuropulmonary blastoma, congenital cystic bronchiectasis, and congenital diaphragmatic hernia. The most common presentation is recurrent chest infection and chest pain, whereas other presentation includes pneumothorax and hemoptysis. Here, we present a case of a 6-year-old child with recurrent episodes of fever and cough diagnosed as a type II CPAM based on computed tomography findings.

摘要

先天性肺气道畸形(CPAM)是一种罕见的先天性发育异常畸形,其特征为支气管发育失败和局部腺性过度生长。CPAM以前被称为先天性囊性腺瘤样畸形(CCAM),根据肺内肺区域的起源、囊肿大小和囊肿外观,可分为0型至IV型5种类型。CPAM是一种罕见的先天性异常,通常在产前超声检查中被诊断出来。然而,很少有病例在儿童期被诊断出来,在成年期被诊断出来的更少。CPAM可根据动脉供应的起源与肺隔离症相鉴别;前者的动脉供应来自肺动脉,而肺隔离症的动脉供应来自体循环。CPAM的另一个鉴别诊断包括先天性支气管囊肿、先天性大叶性肺气肿、胸膜肺母细胞瘤、先天性囊性支气管扩张和先天性膈疝。最常见的表现是反复的胸部感染和胸痛,而其他表现包括气胸和咯血。在此,我们报告一例6岁儿童,根据计算机断层扫描结果,反复出现发热和咳嗽,被诊断为II型CPAM。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/817f/10393603/c26fa5720ea7/gr1.jpg

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