Alpha-chain Disease with Clinical, Immunological, and Histological Recovery

Clinical, immunological, and histological recovery in a patient with alpha-chain disease is described. The patient, a 27-year-old Greek man, presented with severe steatorrhoea, abdominal pain, oedema, and hypogammaglobulinaemia. Treatment with tetracycline produced only temporary remission. Intermit...

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Bibliographic Details
Published in:BMJ 1974-05, Vol.2 (5916), p.409-412
Main Authors: Manousos, O. N., Economidou, J. C., Georgiadou, D. E., Pratsika-Ougourloglou, K. G., Hadziyannis, S. J., Merikas, G. E., Henry, K., Doe, W. F.
Format: Article
Language:English
Subjects:
Adult
Antibiotics
Beta-Globulins - analysis
Biopsies
Biopsy
Celiac Disease - immunology
Celiac Disease - pathology
Cyclophosphamide - therapeutic use
Diarrhea
Diet Therapy
Disease remission
Fluorescent Antibody Technique
Glucose Tolerance Test
Glutens
Heavy Chain Disease - drug therapy
Heavy Chain Disease - immunology
Heavy Chain Disease - pathology
Histology
Humans
Immunoelectrophoresis
Immunoglobulin A - analysis
Immunoglobulin G - analysis
Immunoglobulin M - analysis
Immunoproliferative small intestinal disease
Intestinal Absorption
Intestinal Mucosa - pathology
Jejunum - pathology
Male
Mucosa
Papers and Originals
Plasma cells
Prednisone - therapeutic use
Small intestine
T-Lymphocytes - immunology
Tetracycline - therapeutic use
Urine
Xylose
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Description
Summary:Clinical, immunological, and histological recovery in a patient with alpha-chain disease is described. The patient, a 27-year-old Greek man, presented with severe steatorrhoea, abdominal pain, oedema, and hypogammaglobulinaemia. Treatment with tetracycline produced only temporary remission. Intermittent therapy with prednisone and cyclophosphamide together with antibiotics was followed by clinical recovery, return of histological appearances of the small intestine to normal, and disappearance of free alpha-chain protein from the serum. The patient remained well one year later without treatment.
ISSN:0007-1447
0959-8138
1468-5833
DOI:10.1136/bmj.2.5916.409