CAJ | 학술논문

目的：探讨肝豆状核变性（HLD）患者同型半胱氨酸与血浆脂蛋白（a）、维生素B12、叶酸相关性及与驱铜治疗的关系。方法将63例HLD住院患者分为33例HLD规范治疗组（规范组）和30例HLD非规范治疗组（非规范组），测定二巯基丙磺酸钠（DMPS）驱铜疗程前血浆同型半胱氨酸（Hcy）、脂蛋白（a）、维生素B12及叶酸的水平，并留取患者疗前和第一个DMPS驱铜疗程中的24 h尿铜，以35例正常人为健康对照组。结果非规范组患者血浆Hcy异常率显著高于规范组及对照组（P＜0.0167），而规范组与对照组Hcy异常率无统计学差异（P＞0.05）。三组患者脂蛋白（a）、叶酸均有统计学差异（P＜0.05），而维生素 B12水平无统计学差异（P＞0.05）。非规范组疗前、第一疗程尿铜显著高于规范组（P＜0.05），且非规范组患者血浆Hcy与第一疗程尿铜呈线性正相关。对照组血浆维生素B12、叶酸对Hcy具有显著负相关，而脂蛋白（a）对Hcy具有不显著正相关。结论未经规范化驱铜治疗的HLD患者更易合并高Hcy血症；HLD患者存在脂蛋白（a）的代谢异常及叶酸缺乏情况；正常人的血浆脂蛋白（a）、叶酸、维生素B12水平与血浆Hcy水平存在相关性，但有关HLD的相关性有待进一步研究。
목적：탐토간두상핵변성（HLD）환자동형반광안산여혈장지단백（a）、유생소B12、협산상관성급여구동치료적관계。방법장63례HLD주원환자분위33례HLD규범치료조（규범조）화30례HLD비규범치료조（비규범조），측정이구기병광산납（DMPS）구동료정전혈장동형반광안산（Hcy）、지단백（a）、유생소B12급협산적수평，병류취환자료전화제일개DMPS구동료정중적24 h뇨동，이35례정상인위건강대조조。결과비규범조환자혈장Hcy이상솔현저고우규범조급대조조（P＜0.0167），이규범조여대조조Hcy이상솔무통계학차이（P＞0.05）。삼조환자지단백（a）、협산균유통계학차이（P＜0.05），이유생소 B12수평무통계학차이（P＞0.05）。비규범조료전、제일료정뇨동현저고우규범조（P＜0.05），차비규범조환자혈장Hcy여제일료정뇨동정선성정상관。대조조혈장유생소B12、협산대Hcy구유현저부상관，이지단백（a）대Hcy구유불현저정상관。결론미경규범화구동치료적HLD환자경역합병고Hcy혈증；HLD환자존재지단백（a）적대사이상급협산결핍정황；정상인적혈장지단백（a）、협산、유생소B12수평여혈장Hcy수평존재상관성，단유관HLD적상관성유대진일보연구。
Objective To explore the correlation between plasma homocysteine and lipoprotein (a), folate, Vitamin B12 in hepatolenticular degeneration(HLD) and the relationship with decoppering therapy. Methods 63 inpatients of HLD were divided into 33 cases of standardized treatment group(specification group) and 30 cases of non-standardized treatment group(non-specification group). The serum concentration of homocysteine(Hcy), lipoprotein (a), folate and vitamin B12 were measured before decoppering therapy with sodium dimercaptosulphonate(DMPS), 24-hour urine copper were remained before drug treatment and after first period treatment with DMPS. 35 cases of normal served as controls(control group). Results The abnormal rate of serum Hcy in non-specification group was higher than that in specification group and control group(P<0.05), but there was no statistical significance between specification group and control group(P>0.05). The concentration of lipoprotein (a) and folate between three groups had statistical significance(P<0.05), but vitamin B12 among the three groups present no statistical significance(P>0.05). 24-hour urine before drug treatment and after first period treatment with DMPS in non-specification group were higher than that in specification group(P<0.05), and 24-hour urine after first period treatment with DMPS had positively linearly related to serum Hcy in non-specification group. The serum concentration of vitamin B12 and folate had a significant negatively related to Hcy in control group, but lipoprotein(a) had no significant positively related to Hcy. Conclusions HLD patients without standardized decoppering therapy are more easily complicated with hyperhomocysteine. HLD patients have metabolic abnormalities of lipoprotein(a) and folate deficiency. The correlation between hyperhomocycteinemia and lipoprotein(a), folate, vitamin B12 is existed in normal human, but this correlation in HLD patients remains to be further exploration.