2 61

Cited 6 times in

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

Authors
 Gye-Yeon Lim ; Ok-Hwa Kim ; Tae-Joon Cho ; Hae-Ryong Song ; Kyung Hee Kang ; Ki Seok Lee ; Hyun Woo Kim 
Citation
 American Journal of Medical Genetics part A, Vol.155A(4) : 865~869, 2011 
Journal Title
 American Journal of Medical Genetics part A 
ISSN
 1552-4825 
Issue Date
2011
Abstract
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
URI
http://ir.ymlib.yonsei.ac.kr/handle/22282913/93345
DOI
10.1002/ajmg.a.33926
Appears in Collections:
1. 연구논문 > 1. College of Medicine > Dept. of Orthopedic Surgery
Yonsei Authors
사서에게 알리기
  feedback
Link
 http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.33926/abstract;jsessionid=73EE1C11D0F1CAFC5F5FC09ECD95B828.f04t01
Export
RIS (EndNote)
XLS (Excel)
XML

qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

Browse