Summary:A 20-month-old boy with infantile leukemia was treated with total body irradiation, etoposide, cyclophosphamide and unrelated cord blood transplantation with a one-antigen mismatch. He relapsed on day 100 and achieved remission after ubenimex administration, and also developed chronic graft-versus-host disease of the skin. He remained in remission for 22 months with repeated courses of ubenimex. Ubenimex may be an alternative to donor lymphocyte transfusion and may be useful for the treatment of a patient who has relapsed after cord blood transplantation.
The purpose of this study was to increase the amount of copper excreted resulting from the administration of Dpenicillamine(DP) in pediatric Wilson's disease(WD) patients. By measuring the urinary copper excretion after adjusting the administration schedules, the appropriate timing for DP administration was investigated. The subjects were three brothers with pediatric WD. The initial daily dose of DP was 5 mg/kg/day, and gradual1y increased to the maintenance dose of 20 mg/kg/day. Until the maintenance daily dose was reached, DP was administered 2 h after the morning and evening meal. After reaching the maintenance daily dose of DP, the appropriate timing for taking DP was investigated in both the morning and evening. Three schedules of DP administration were compared: 2 h after meals; 30 min before meals (with fasting); and 1 h before the morning and l.5 before the evening meal (direction 1). The resulting urinary copper excretion on each dosing schedule was compared. Little diŠerence was found in urinary copper excretion on thê rst two schedules, i. e., 2 h after meals and 30 min before meals. When DP was administered 30 min before meals, urinary copper excretion [ mg/day] was 1173 in theˆrst brother, 918 in the second, and 875 in the third. When DP was administered according to direction 1, however, urinary copper excretion was increased signiˆcantly to 1701 in theˆrst brother, 2701 in the second, and 3808 in the third. It is known that the e‹ciency of urinary copper excretion with DP administration depends on the maintenance of chelating ability after absorption from the gastrointestinal tract. Our results indicate that the excretion was lower when DP was administered 2 h after or 30 min before meals (with fasting), as recommended in the package insert. Thus to achieve better copper excretion e‹ciency, direction 1 is recommended for WD patients.
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