Cornelissen E A, van Lieburg A F, Motohara K, van Oostrom C G
Department of Paediatrics, University Hospital Nijmegen, The Netherlands.
Acta Paediatr. 1992 Sep;81(9):658-61. doi: 10.1111/j.1651-2227.1992.tb12327.x.
Appearance of PIVKA-II (protein induced by vitamin K absence-II) in serum is a biochemical sign of insufficient vitamin K-dependent carboxylation of prothrombin. Plasma concentrations of PIVKA-II and vitamin K1 were determined in 24 children with cystic fibrosis. Eight were supplemented with vitamin K1. The purpose of the study was to determine the occurrence of vitamin K deficiency in cystic fibrosis and to evaluate the effect of vitamin K supplementation. PIVKA-II was detectable in only one unsupplemented child. In this patient, the concentration of vitamin K1 was below the limit of detection of 60 ng/l. Vitamin K1 levels in the other unsupplemented children were normal (mean 476 ng/l = 1 mmol/l). The supplemented patients showed extremely high levels of vitamin K1 (mean 22445 ng/l = 50 nmol/l). In conclusion, vitamin K deficiency occurs infrequently in cystic fibrosis. Checking the coagulation system is advised, but routine vitamin K supplementation is not recommended. If additional vitamin K is needed, the starting dose should not exceed 1 mg daily.
血清中异常凝血酶原（维生素K缺乏诱导蛋白-II）的出现是凝血酶原维生素K依赖性羧化不足的生化标志。对24名囊性纤维化患儿测定了血浆中异常凝血酶原和维生素K1的浓度。其中8名患儿补充了维生素K1。本研究的目的是确定囊性纤维化患儿维生素K缺乏的发生率，并评估补充维生素K的效果。仅在一名未补充维生素K的患儿中检测到异常凝血酶原。该患儿的维生素K1浓度低于60 ng/l的检测下限。其他未补充维生素K的患儿维生素K1水平正常（平均476 ng/l = 1 mmol/l）。补充维生素K的患儿维生素K1水平极高（平均22445 ng/l = 50 nmol/l）。总之，囊性纤维化患儿维生素K缺乏很少见。建议检查凝血系统，但不建议常规补充维生素K。如需额外补充维生素K，起始剂量不应超过每日1 mg。
