{"title":"单侧多囊性肺疾病在一个年轻人:一个不寻常的诊断","authors":"I. Goel, P. Kaushik, Vassilis Xydis","doi":"10.1097/CPM.0000000000000297","DOIUrl":null,"url":null,"abstract":"Congenital pulmonary airway malformation (CPAM) is an uncommon developmental hamartomatous anomaly caused by abnormal branching of immature bronchioles with replacement of the lung by cysts/disorganized tissue. CPAMs are usually lobar or sublobar in extent, and ∼90% of lesions are identified within the first 2 years of life. CPAM occupying an entire lung, with a delayed adulthood presentation, is extremely rare; only 4 such occurrences have been previously described. We present a case of a 20-year-old woman diagnosed with unilateral entire lung CPAM on the basis of radiologic findings that was surgically resected via video-assisted thoracoscopic surgery.","PeriodicalId":10393,"journal":{"name":"Clinical Pulmonary Medicine","volume":"26 1","pages":"101 - 98"},"PeriodicalIF":0.0000,"publicationDate":"2019-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1097/CPM.0000000000000297","citationCount":"0","resultStr":"{\"title\":\"Unilateral Multicystic Pulmonary Disease in a Young Adult: An Unusual Diagnosis\",\"authors\":\"I. Goel, P. Kaushik, Vassilis Xydis\",\"doi\":\"10.1097/CPM.0000000000000297\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Congenital pulmonary airway malformation (CPAM) is an uncommon developmental hamartomatous anomaly caused by abnormal branching of immature bronchioles with replacement of the lung by cysts/disorganized tissue. CPAMs are usually lobar or sublobar in extent, and ∼90% of lesions are identified within the first 2 years of life. CPAM occupying an entire lung, with a delayed adulthood presentation, is extremely rare; only 4 such occurrences have been previously described. We present a case of a 20-year-old woman diagnosed with unilateral entire lung CPAM on the basis of radiologic findings that was surgically resected via video-assisted thoracoscopic surgery.\",\"PeriodicalId\":10393,\"journal\":{\"name\":\"Clinical Pulmonary Medicine\",\"volume\":\"26 1\",\"pages\":\"101 - 98\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-05-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.1097/CPM.0000000000000297\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical Pulmonary Medicine\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1097/CPM.0000000000000297\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Pulmonary Medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1097/CPM.0000000000000297","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
Unilateral Multicystic Pulmonary Disease in a Young Adult: An Unusual Diagnosis
Congenital pulmonary airway malformation (CPAM) is an uncommon developmental hamartomatous anomaly caused by abnormal branching of immature bronchioles with replacement of the lung by cysts/disorganized tissue. CPAMs are usually lobar or sublobar in extent, and ∼90% of lesions are identified within the first 2 years of life. CPAM occupying an entire lung, with a delayed adulthood presentation, is extremely rare; only 4 such occurrences have been previously described. We present a case of a 20-year-old woman diagnosed with unilateral entire lung CPAM on the basis of radiologic findings that was surgically resected via video-assisted thoracoscopic surgery.
期刊介绍:
Clinical Pulmonary Medicine provides a forum for the discussion of important new knowledge in the field of pulmonary medicine that is of interest and relevance to the practitioner. This goal is achieved through mini-reviews on focused sub-specialty topics in areas covered within the journal. These areas include: Obstructive Airways Disease; Respiratory Infections; Interstitial, Inflammatory, and Occupational Diseases; Clinical Practice Management; Critical Care/Respiratory Care; Colleagues in Respiratory Medicine; and Topics in Respiratory Medicine.