CAJ | 학술논문

目的：探讨骨髓间充质干细胞（BMSCs）对肝豆状核变性肝纤维化患者的治疗作用。方法：60例确诊肝豆状核变性肝纤维化患者，随机分为3组：青霉胺组、BMSCs 组和BMSCs＋青霉胺组，口服青霉胺剂量为每日40 mg／kg ，将采集的BMSCs液2 mL＋生理盐水100 mL经静脉输注入人体。分别于治疗前后做肝组织病理活检，检测0、4、8、12周血清透明质酸（HA）、Ⅲ型前胶原（PCⅢ）、层黏蛋白（LN）、Ⅳ型胶原（CⅣ）、基质金属蛋白酶-1（MMP-1）、基质金属蛋白酶抑制剂-1（TIMP-1）的变化。结果：BMSCs＋青霉胺组肝组织活检肝纤维化明显改善；各组血清中HA、PCⅢ、LN、CⅣ、TIMP-1、MMP-1均持续降低，BMSCs＋青霉胺组血清中各细胞因子的改善比其他组明显，差异具有统计学意义（P ＜0．05）。结论：BMSCs联合青霉胺治疗肝豆状核变性合并肝纤维化有明显改善作用。
목적：탐토골수간충질간세포（BMSCs）대간두상핵변성간섬유화환자적치료작용。방법：60례학진간두상핵변성간섬유화환자，수궤분위3조：청매알조、BMSCs 조화BMSCs＋청매알조，구복청매알제량위매일40 mg／kg ，장채집적BMSCs액2 mL＋생리염수100 mL경정맥수주입인체。분별우치료전후주간조직병리활검，검측0、4、8、12주혈청투명질산（HA）、Ⅲ형전효원（PCⅢ）、층점단백（LN）、Ⅳ형효원（CⅣ）、기질금속단백매-1（MMP-1）、기질금속단백매억제제-1（TIMP-1）적변화。결과：BMSCs＋청매알조간조직활검간섬유화명현개선；각조혈청중HA、PCⅢ、LN、CⅣ、TIMP-1、MMP-1균지속강저，BMSCs＋청매알조혈청중각세포인자적개선비기타조명현，차이구유통계학의의（P ＜0．05）。결론：BMSCs연합청매알치료간두상핵변성합병간섬유화유명현개선작용。
Objective To evaluate the effect of bone marrow stem cells (BMSCs) transplantation in the treatment of hepatolenticular degeneration of liver fibrosis. Methods Sixty cases with confirmed hepatolenticular degeneration of liver fibrosis were randomly divided into 3 groups: penicillamine group [40 mg/(kg·d)], BMSCs group and BMSCs + penicillamine group. Autologous BMSCs (2 mL) were injected into vein with normal saline (100 mL). Liver tissue pathology biopsy was inspected and the changes in HA, PCⅢ, LN, CⅣ, TIMP-1 and MMP-1 were observed at 0, 4th, 8th and 12th week during the therapeutic process. Results The level of serum fibrotic markers were reduced in every group , while the changes in BMSCs + penicillamine group were especially obvious (P < 0.05). Conclusion Penicillamine combined with BMSCs was effective in the therapy of hepatolenticular degeneration of liver fibrosis.