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. 1971 Nov;9(5):625–635.

Alpha-chain disease with involvement of the respiratory tract in a Dutch child

J W Stoop, R E Ballieux, W Hijmans, B J W Zegers
PMCID: PMC1713058  PMID: 4111693

Abstract

A description is given of an 8-year-old girl of pure Dutch extraction who, since age 4, has shown unclassifiable skin changes, marked eosinophilia and diffuse infiltrative pulmonary changes with enlarged mediastinal lymph glands, dyspnoea and impaired diffusion.

The patient's serum contained a large amount of proteins related to the Fc-fragment of IgA. She developed a pharyngeal tumour with the histological characteristics of a paragranuloma. The mucosa of the lower air passages is regarded as a possible site of origin of the abnormal serum protein. The disease was therefore interpreted as a disorder of the secretory IgA system, and this patient could well represent the respiratory form of the alpha-chain disease, described so far.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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