Pulmonary function in thalassaemia major and its correlation with body iron stores

被引:9
|
作者
Sohn, Eugene Y. [2 ]
Noetzli, Leila J. [1 ]
Gera, Aakanksha [1 ]
Kato, Roberta [2 ]
Coates, Thomas D. [3 ]
Harmatz, Paul [4 ]
Keens, Thomas G. [2 ]
Wood, John C. [1 ]
机构
[1] Childrens Hosp Los Angeles, USC Keck Sch Med, Div Pediat Cardiol, Los Angeles, CA 90027 USA
[2] Childrens Hosp Los Angeles, USC Keck Sch Med, Div Pediat Pulmonol, Los Angeles, CA 90027 USA
[3] Childrens Hosp Los Angeles, USC Keck Sch Med, Div Hematol, Los Angeles, CA 90027 USA
[4] Childrens Hosp Oakland, Div Gastroenterol, Oakland, CA USA
关键词
iron overload; pulmonary function; thalassaemia; magnetic resonance imaging; lung disease; BETA-THALASSEMIA; FUNCTION ABNORMALITIES; TRANSFUSION; CHILDREN;
D O I
10.1111/j.1365-2141.2011.08808.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for airways obstruction, there were indicators of small airways disease and air trapping. PFTs did not correlate with somatic iron burden, blood counts or haemolysis. Restrictive lung disease was associated with inflammation. We conclude that TM patients have pulmonary abnormalities consistent with small airways obstruction. Restrictive disease and impaired diffusion are less common.
引用
收藏
页码:102 / 105
页数:4
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