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. 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.
D000328:Adult; D001609:Beta-Globulins / Q000032:analysis; D001706:Biopsy; D002446:Celiac Disease / Q000276:immunology / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004035:Diet Therapy; D005455:Fluorescent Antibody Technique; D005951:Glucose Tolerance Test; D005983:Glutens; D006362:Heavy Chain Disease / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006801:Humans; D007122:Immunoelectrophoresis; D007070:Immunoglobulin A / Q000032:analysis*; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007408:Intestinal Absorption; D007413:Intestinal Mucosa / Q000473:pathology; D007583:Jejunum / Q000473:pathology; D008297:Male; D011241:Prednisone / Q000627:therapeutic use*; D013601:T-Lymphocytes / Q000276:immunology; D013752:Tetracycline / Q000627:therapeutic use*; D014994:Xylose
D001609:Beta-Globulins; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D005983:Glutens; D003520:Cyclophosphamide; D014994:Xylose; D013752:Tetracycline; D011241:Prednisone