青霉胺激发试验致肝豆状核变性患儿粒细胞减少

Granulocytopenia in a child with hepatolenticular degeneration induced by a penicillamine provocation test

1例8岁疑似肝豆状核变性男性患儿为确诊行青霉胺激发试验.试验前外周血白细胞计数8.3×10^9/L,中性粒细胞计数6.0×10^9/L,血红蛋白101 g/L,血小板计数120×10^9/L.顿服青霉胺250 mg后24h,患儿白细胞计数1.6×10^9/L,中性粒细胞计数0.6×10^9/L,血红蛋白105 g/L,血小板计数92×10^9/L.给予患儿口服泼尼松（10 mg、2次/d）和地榆升白片（2片、2次/d）.5d后复查,白细胞计数3.5×10^9/L,中性粒细胞计数1.9×10^9/L,血红蛋白108 g/L,血小板计数88×10^9/L.

An 8-year-old boy underwent a penicillamine provocation test to confirm the diagnosis of hepatolenticular degeneration.Before the test,the laboratory examination showed the following levels：white blood cell count 8.3 ×10^9/L,neutrophil 6.0 ×10^9/L,hemoglobin 101 g/L,and platelet count 120 ×10^9/L.And 24 hours after oral penicillamine 250 mg once,the levels of his white blood cell count, neutrophil, hemoglobin, and platelet count were 1.6 ×10^9/L,0.6 ×10^9/L,105 g/L,and 92 ×10^9/L,respectively.The child was given oral prednisone 10 mg twice daily and two tablets of Diyu Shengbai （地榆升白片） twice daily.Five days later,the re-examination showed his white blood cell count of 3.5 ×10^9/L with neutrophil of 1.9 ×10^9/L,the hemoglobin level of 108 g/L,and the platelet count of 88 ×10^9/L.