Recurrent pleural effusion, protein-losing enteropathy, malabsorption, and mosaic warts associated with generalized lymphatic hypoplasia

A patient is described who presented with pleural effusions, asymmetrical oedema of the face and limbs, and numerous warts of the hands and feet. He was shown to have vitamin B12 malabsorption, steatorrhoea, and a protein-losing enteropathy. Lymphography showed asymmetrical lymphatic hypoplasia. Jej...

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Veröffentlicht in:Thorax 1971-01, Vol.26 (1), p.119-124
Hauptverfasser: Ross, J. D., Reid, K. D. G., Ambujakshan, V. P., Kinloch, J. D., Sircus, W.
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Sprache:eng
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Zusammenfassung:A patient is described who presented with pleural effusions, asymmetrical oedema of the face and limbs, and numerous warts of the hands and feet. He was shown to have vitamin B12 malabsorption, steatorrhoea, and a protein-losing enteropathy. Lymphography showed asymmetrical lymphatic hypoplasia. Jejunal biopsy showed intestinal lymphangiectasia. The hypoproteinaemia responded well to a low-fat, high-protein diet. Corticosteroids at first appeared to speed resolution of the pleural effusion but had only a limited effect. The effusion was attributed to infections injuring a lymphatic system already impaired.
ISSN:0040-6376
1468-3296
DOI:10.1136/thx.26.1.119