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Pulmonary manifestations in Proteus syndrome: pulmonary varicosities and bullous lung disease

 Gye-Yeon Lim  ;  Ok-Hwa Kim  ;  Tae-Joon Cho  ;  Hae-Ryong Song  ;  Kyung Hee Kang  ;  Ki Seok Lee  ;  Hyun Woo Kim 
 American Journal of Medical Genetics Part B - Neuropsychiatric Genetics, Vol.155A(4) : 865-869, 2011 
Journal Title
 American Journal of Medical Genetics Part B - Neuropsychiatric Genetics 
Issue Date
We report on two patients with Proteus syndrome (PS), with emphasis on its pulmonary manifestations. The first patient was a 6-year-old girl diagnosed with PS at 5 years of age. The pulmonary abnormalities first observed at age 3 years and included streaky densities with accentuated vascular markings detected by chest radiography. The patient had persistent abnormalities on follow-up chest radiographs. Chest computed tomography (CT) scans showed diffuse pulmonary venous dilatations. The second patient was a 10-year-old boy diagnosed with PS at age 4 years. Chest radiography and CT scans showed patchy and streaky densities intermixed with small bullae, which were interpreted as pneumonia with post-inflammatory pneumatoceles. The patient developed diffuse enlargement of air spaces of the lungs at age 10 years with severe respiratory compromise. Although pulmonary manifestations in PS are uncommon, recognition of pulmonary vein malformation and the presentation of enlarged air spaces in the lungs at an earlier age are important for accurate diagnosis. The plain radiograph findings of accentuated vascular markings seen in patients with PS may appear similar to interstitial or chronic pneumonia. This report emphasizes the features of lung involvement in children with PS and suggests that specific attention be paid to pulmonary manifestations using chest CT scans
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1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Orthopedic Surgery (정형외과학교실)
Yonsei Authors
김현우(Kim, Hyun Woo) ; 이기석(Lee, Ki Seok)
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