A study on the subclinical hepatic dysfunction as a causative factor of infantile vitamin K deficiency hemorrhage

婴儿维生素K缺乏性出血是一种严重的疾病，主要发生在婴儿早期母乳喂养的婴儿，通常没有任何明显的原因。我们怀疑肝功能在某种程度上与这种疾病的发生有关，并研究了它作为这种疾病的致病因素的作用。9个婴儿中有8个在3周大时表现出较低的正常值，后来发展为肝功能障碍。血清快速转换蛋白水平，如铜蓝蛋白，在患者和正常值较低的婴儿中均升高。提示维生素K缺乏症患儿胆汁淤积，但对蛋白质的产生无明显影响。用气相色谱法测定了8例维生素K缺乏症患儿的血清胆汁酸。所有患儿血清总胆汁酸均明显升高，C/CDC比值升高，提示胆汁淤积症的存在。口服维生素K。对194名5日龄正常新生儿进行了血清维生素K和PIVKA-II水平的测定。其中7名婴儿服用维生素K后3小时血清维生素K水平未见升高，其中6名婴儿在3周龄时PIVKA-II呈阳性，提示婴儿存在维生素K缺乏。本研究结果清楚地表明，婴幼儿早期“生理性”胆汁淤积导致的维生素K吸收不良在特发性婴儿维生素K缺乏性出血的发生中起重要作用。

Infantile vitamin K deficiency hemmorrhage is a serious disease, mainly seen in breast-fed infants during early infancy, usually without any apparent causes. We suspected the liver function somehow related to the development of this desease, and studied to elucidate its role as a causative factor of this disease.1. Eight out of nine infants, who showed low normotest at the age of three weeks, developed hepatic dysfunction later.2. Serum levels of rapid turnover proteins, such as ceruloplasmin, were elevated in the patients, as well as in the infants who showed low normotest. These results suggested that there was no impairment in the production of the proteins, but there existed bile stasis in the infants with vitamin K deficiency.3. Serum bile acids were determined in eight infants with vitamin K deficiency with gas-chromatography. All the infants showed markedly elevated serum total bile acid and high C/CDC ratio, which strongly suggested the existense of bile stasis.4. Vitamin K was administered p.o. to 194 normal newborn infants at the age of 5 days, and serum level of vitamin K and serum PIVKA-II were determined. Among them, seven infants showed no elevation of serum vitamin K after 3 hours post vitamin K adminstration, of whom six became positive for PIVKA- II at the age of 3 weeks, suggesting the presence of vitamin K deficiency.The results of the present study clearly indicate that malabsorption of vitamin K due to "physiologic" cholestasis in early infancy plays an important role in the development of idiopathic infantile vitamin K deficiency hemorrhage.