Proteus Syndrome High-resolution CT and CT Pulmonary Densitovolumetry Findings

被引:14
|
作者
Irion, Klaus L. [1 ,2 ,3 ]
Hocchegger, Bruno [4 ,5 ]
Marchiori, Edson [4 ,5 ]
Holemans, John A. [1 ,2 ,3 ]
Smith, Rupert A. [6 ]
Raja, Ramesh C. [6 ]
Singanayagam, Selvadurai [6 ]
机构
[1] Liverpool Heart & Chest Hosp NHS Trust, Liverpool, Merseyside, England
[2] Royal Liverpool Univ Hosp, Liverpool, Merseyside, England
[3] Postgrad Program Resp Sci UFRGS, Porto Alegre, RS, Brazil
[4] UFRJ, Postgrad Program Radiol, Rio De Janeiro, Brazil
[5] Univ Fed Fluminense, Niteroi, RJ, Brazil
[6] Pennine Acute Hosp NHS Trust, Great Manchester, Brazil
关键词
Proteus syndrome; high-resolution computed tomography; chest; quantitative computed tomography; DIAGNOSTIC-CRITERIA; COMPUTED DENSITY; MANIFESTATIONS; MORPHOMETRY; EMPHYSEMA; DEATH;
D O I
10.1097/RTI.0b013e31818b20cd
中图分类号
R8 [特种医学]; R445 [影像诊断学];
学科分类号
1002 ; 100207 ; 1009 ;
摘要
Cystic transformation of the lungs in Proteus syndrome is considered an important manifestation of this disease. We describe a case of an 11-year-old girl with a diagnosis of Proteus syndrome with lung involvement. Low-dose multidetector computed tomography (CT) revealed extensive diffuse cystic lung disease wit 1 left long predominance. affecting mostly the lower lung zones. The cystic lesions had various sizes and variable wall thickness. Postprocessing using CT histogram densitometric volumetry software (CT densitovolumetry), using the threshold -950 Houndsfield units (HU) for quantifying emphysema, revelled that 31%. of her total lung volume was composed of areas with CT. attenuation values below -950 HU.
引用
收藏
页码:45 / 48
页数:4
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