Acquired alpha 1-antitrypsin deficiency in tropical pulmonary eosinophilia

被引:0
|
作者
Ray, Debidas [2 ]
Harikrishna, S. [2 ]
Immanuel, Chandra [3 ]
Victor, Lalitha [3 ]
Subramanyam, Sudha [4 ]
Kumaraswami, V. [1 ]
机构
[1] TB Res Ctr ICMR, Dept Immunol, Madras 600031, Tamil Nadu, India
[2] TB Res Ctr ICMR, Dept Clin Res, Madras 600031, Tamil Nadu, India
[3] TB Res Ctr ICMR, Dept Biochem, Madras 600031, Tamil Nadu, India
[4] TB Res Ctr ICMR, Dept Pathol, Madras 600031, Tamil Nadu, India
关键词
alpha; 1-antitrypsin; acquired deficiency; tropical pulmonary eosinophilia; phenotyping; ALPHA1-ANTITRYPSIN; LUNG;
D O I
暂无
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Background & objectives: Observation of an increased frequency of an intermediate deficiency of serum alphal-antitrypsin (alpha 1-AT) in patients with Tropical Pulmonary Eosinophilia (TPE) was earlier reported. Though the possibility of existence of an acquired deficiency was suggested, without phenotyping a hereditary alpha 1-AT deficiency in TPE could not totally be ruled out. In this study, we have done Pi (Protease inhibitor) phenotyping to investigate the possibility of association of any heterozygous (or homozygous) alpha 1-AT deficiency in patients with TPE. Methods: Serum a1antitrypsin (alpha 1-AT) was measured in 103 patients (Group A) with TPE, 99 patients with pulmonary eosinophilia who had associated intestinal worm infestation (Group B) and 43 healthy volunteers who served as controls. In 19 alpha 1-AT deficient patients (9 of Group A and 10 of Group B), alpha 1-AT level was measured before and after treatment. In 58 patients with TPE and in 5 controls, phenotyping was done. Results: Fifteen patients of Group A and 16 from Group B showed intermediate alpha 1-AT deficiency (150 mg % or less. None of the control subjects had alpha 1-AT deficiency (<200 mg%). After treatment with DEC and/or deworming, in 19 patients there was a significant (P<0.001) rise in alpha 1-AT levels. Results of phenotyping showed that all had M-1 or M-2 allele and none had S or Z variant (either homozygous or heterozygous) thus ruling out any underlying genetic cause for the observed alpha 1-AT deficiency. Interpretation & conclusions: The observed alpha 1-AT deficiency may be due to the chronic inflammation in TPE and associated oxidative stress. However, in such alpha 1-AT deficient patients with TPE and those with worm infested pulmonary eosinophilia, faecal alpha 1-AT concentration and faecal alpha 1-AT clearance should be routinely estimated to rule out the possibility of any intestinal protein loss.
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页码:79 / 82
页数:4
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