Present paper reports our experiences on the second case of alpha-chain disease in Japan.
Alpha-chain disease was reported for the first time by Seligmann in 1968 as the disorder of the Fc segment of secretory Ig-A system. Shortly after that, there were successive reports of 65 cases mainly from the near-and middle east areas, and this was thought to indicate the racial relationship to the etiology of this disease. Later on, there were sporadic cases here and there in the world, and now the etiology of the disease is thought also to have something to do with the factors of circumstances. In Japan, the first case of this disease was reported by Kitani in 1973, and our case is the second.
The patient (S. H.) is 54-years-old pure Japanese male. He was admitted to the Second Tokyo National Hospital with the complaints of abdominal pain, watery diarrhea and loss of body weight. His past, and family history was nothing contributory. The physical examination revealed emaciation, pretibial and dorsal pedic edema, dabbed finger, epigastric tenderness and weakness of the extremities. The laboratory data was followings: C. B. C. showed slight anemia. Urinalysis was normal and negative of Bence-Jones protein. Blood chemistry revealed hypoproteinemia of high grade (3.7g/dl). Serum iron, Ca and total cholesterol was low, but alkaline phosphatase was high.
Electrophoretic pattern of serum protein showed low albumin, increase of alpha-1, -2, and β-γ linking. M-protein was always negative. Ig-A was extremely high (1030.4mg/dl) and showed double precipitation ring by SRID method. On immunoelectrophoresis by anti-Ig-A serum, there was a broad precipitation line at the area of alpha-2 and gamma area, and aside from this, slight residual normal Ig-A precipitation line was recognized. The extract from the anodic reacted part to anti-Ig-A on the agar gel plate was examined by Ouchterlony method. This extract reacted to only anti-Ig-A, and did not to anti-K, and -γ serum: this is the evidence of absence of light-chain in the patient's serum. On the examination by immunoselection plate method by Rüdl, there was also no reaction to anti-K, and -γ serum, and residual free alpha-chain migrated to the anode.
The fluoloscopic examination of the gastrointestinal tract revealed narrowing of the intestine in distal to the duodenum. Biopsy of the intestinal mucosa showed mononuclear cell infiltration in the proper mucosal layer.
All of the findings of this patient were characteristic of the alpha-chain disease according to the report of Seligmann.
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