Manco-Johnson M J, Marlar R A, Jacobson L J, Hays T, Warady B A
Department of Pediatrics, University of Colorado School of Medicine, Denver 80262.
J Pediatr. 1988 Aug;113(2):359-63. doi: 10.1016/s0022-3476(88)80284-1.
Eleven infants initially seen in the neonatal period had levels of protein C suggestive of homozygous protein C deficiency but as an apparently acquired condition. Family studies failed to document parental carrier status, the clinical course was not typical of that reported with homozygous protein C deficiency, and protein C levels increased in all restudied infants, six of whom received heparin anticoagulation. No infant had evidence of vitamin K deficiency. Care is advised in the evaluation of infants with low levels of protein C. Parental blood studies, delayed testing, and serial assays can help to establish the correct diagnosis.
11名在新生儿期初诊的婴儿蛋白C水平提示纯合子蛋白C缺乏，但表现为明显的后天性疾病。家族研究未能证实父母为携带者状态，临床病程并非纯合子蛋白C缺乏所报道的典型病程，且所有再次检测的婴儿蛋白C水平均升高，其中6名婴儿接受了肝素抗凝治疗。没有婴儿有维生素K缺乏的证据。对蛋白C水平低的婴儿进行评估时需谨慎。父母血液检查、延迟检测和系列检测有助于确立正确诊断。
