Wolfe D M, Gatfield P D
Pediatr Res. 1975 Jun;9(6):531-5. doi: 10.1203/00006450-197506000-00004.
All enzymes of the urea cycle are demonstrable in circulating leukocytes. They show the same relative activities as those in liver except for argininosuccinate synthetase + lyase (combined) which seems to be disproportionately active. To see whether leukocytes reflect liver activity, blood from patients with three hepatic urea cycle disorders was tested. In each case, the leukocytes showed the same enzyme deficiency as was apparent from a liver biopsy (Table 4). Leukocyte assays appear to be reliable indicators of the enzyme lesions in inherited urea cycle enzyme defects and therefore may obviate the need for liver biopsy.
尿素循环的所有酶在循环白细胞中均可检测到。除精氨琥珀酸合成酶+裂解酶（合并）似乎活性不成比例地高外，它们表现出与肝脏中相同的相对活性。为了观察白细胞是否反映肝脏活性，对患有三种肝脏尿素循环障碍的患者的血液进行了检测。在每种情况下，白细胞显示出与肝脏活检中明显相同的酶缺乏（表4）。白细胞检测似乎是遗传性尿素循环酶缺陷中酶损伤的可靠指标，因此可能无需进行肝脏活检。
