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Objective To investigate the value of MRI in diagnosis of congenital cystic lung disease.Methods The MRI was per-formed in 105 fetuses with congenital cystic lung disease.Subjects were classified into two groups including group A (the gestational age ranged from 12-20 w)and group B (the gestational age>20 w).The MRI and ultrasonography characteristics,as well as path-ologic diagnosis were compared.Results Among 105 cases,there were 80 cases of congenital cystic adenomatoid malformation (CCAM)(including 23 cases of typeⅠ,38 cases of typeⅡ and 1 9 cases of typeⅢ),18 cases of bronchopulmonary sequestration (BPS),5 cases of congenital lobar emphysema,1 case of bronchogenic cyst,and 1 case was BPS with CCAM.In group A,MRI can diagnose the majority of the anomalies (41/45),ultrasonography can diagnose anomalies of 39 cases.There was no significant differ-ence of diagnostic accuracy between the two methods (P >0.05).In group B,compared with ultrasonography,MRI could diagnose more cases accurately (58 vs 52),with statistical significance (P 20 w compared with ultra-sonography.
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PURPOSE: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. METHODS: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. RESULTS: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. CONCLUSION: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.