原发性胆汁性肝硬化146例临床分析

发布时间：2018-05-23 14:14

本文选题：原发性胆汁性肝硬化 + 临床表现　；参考：《大连医科大学》2014年硕士论文

【摘要】：目的：通过分析原发性胆汁性肝硬化（Primary Biliary Cirrhosis,PBC）患者的临床特征，探讨在不同临床分期生化、免疫学指标的改变及治疗转归，提高对本病的认识及治疗水平，减少误诊率、改善患者预后。方法：收集我院具有完整病例资料经住院确诊146例PBC患者的一般资料、临床表现、血清生化指标、免疫学指标、影像学检查及治疗转归等进行回顾性分析。并进一步探讨了肝硬化前期与肝硬化期两组患者在血清生化、免疫学指标及治疗是否存在统计学差异。结果：146例PBC患者中男性9例，女性137例，男女比为1：15，确诊平均年龄52岁，其平均值为（56.90±11.51）。从出现症状至临床确诊时间为1-96个月不等，平均48.5个月。最常见临床症状包括：乏力(45.89%）、皮肤瘙痒（37.67%）、黄疸（30.14%）、腹胀（26.71%）。主要体征为：脾肿大（37.67%）、肝肿大（17.81%）、肝掌（10.96%）、蜘蛛痣（8.22%）、腹水（12.33%）及皮肤色素沉着（3.42%）。实验室检查中，所有患者均以碱性磷酸酶(alkaline phosphatase,ALP)、γ-谷氨酰转肽酶(Gamma Glutamyl Transpeptidase,GGT)升高为主，分别为（354.88±275.23）U/L和（391.85±400.33）U/L。伴血清总胆红素(total bilirubin，TBil)不同程度的升高为（47.919±59.98）umol/L，其中以直接胆红素(direct bilirubin,DBIL)升高为主(26.39±40.14) umol/L。谷丙转氨酶（glutamic-pyruvic transaminase,ALT）、谷草转氨酶（Aspartate transaminase,AST）多表现为轻中度升高，分别为(101.43±123.14)U/L、(93.52±83.76)U/L。血清免疫球蛋白中以免疫球蛋白M（immunoglobin M，IgM）升高为主，其平均值为（401.03±267.13）g/L。血清抗线粒体抗体（antimitochondrial antibody,AMA）尤其是M2亚型（AMA-M2）阳性作为诊断本病最突出的免疫指标[1]，具有较高特异及敏感性，本研究AMA阳性率为82.20%。PBC患者不同临床分期的血清TBIL、DBIL、TBA、ALB、ALT、IgM值差异经比较后有统计学意义（P㩳0.05）。治疗上均采用以熊去氧胆酸(ursodeoxycholic acid, UDCA)为主的综合治疗，结果显示经治疗后，肝硬化前期组患者症状得到改善，ALP、GGT值均明显下降，总胆红素水平亦不同程度降低。对于肝硬化期组患者虽联合糖皮质激素及免疫抑制剂，但效果欠佳。结论：PBC多见于中年女性，以瘙痒、黄疸、乏力和肝脾肿大为主要临床表现，其自然演变史是一个缓慢渐进性的胆汁淤积过程，在病程的早期可表现为原发性胆汁性胆管炎，需较长一段时间可发展为原发性胆汁性肝硬化。血清生化指标以ALP和GGT水平的明显升高伴高胆红素血症、高球蛋白血症为特点，而ALT及AST轻中度升高。高滴度的AMA/AMA-M2是诊断PBC的主要指标。PBC的诊断及评估病情轻重应当综合分析其临床症状、生化指标、免疫学及组织学等检查，，必要时行肝穿是诊断本病的最可靠依据。目前PBC的治疗仍比较局限，UDCA为疗效肯定的一线治疗药物。患者的病程、血清白蛋白(albumin，ALB）、TBIL水平、组织学分期及UDCA早期应用与否均与疾病进展和预后密切相关。PBC晚期预后差，必须重视其早期诊断、早期治疗，才能更有效延缓病情进展、真正改善患者预后。
[Abstract]:Objective: to analyze the clinical characteristics of Primary Biliary Cirrhosis (PBC) and explore the changes of biochemical and immunological indexes in different clinical stages and the treatment outcome, improve the understanding and treatment level of the disease, reduce the rate of misdiagnosis and improve the prognosis of the patients.
Methods: the general data, clinical manifestation, serum biochemical index, immunological index, imaging examination and treatment of 146 cases of PBC patients with complete case data in our hospital were collected and analyzed. The serum biochemical, immunological indexes and treatment of two groups of patients in pre cirrhosis and liver cirrhosis were further discussed. Whether there is a statistical difference.
Results: there were 9 men and 137 women in 146 cases of PBC. The average age of men and women was 1:15 and the average age was 52 years old. The average value was (56.90 + 11.51). The average time was 1-96 months from the onset of symptoms to 1-96 months, averaging 48.5 months. The most common clinical symptoms included fatigue (45.89%), skin itching (37.67%), jaundice (30.14%), abdominal distension (26.71%). The signs were: splenomegaly (37.67%), hepatomegaly (17.81%), liver palmar (10.96%), spider nevus (8.22%), ascites (12.33%) and skin pigmentation (3.42%). In laboratory examination, all patients were raised mainly by alkaline phosphatase (alkaline phosphatase, ALP), gamma glutamyl transaminopeptidase (Gamma Glutamyl Transpeptidase, GGT), respectively (354.88 + 275.23) U/L, respectively. And (391.85 + 400.33) U/L. with serum total bilirubin (total bilirubin, TBil) in varying degrees (47.919 + 59.98) umol/L, in which direct bilirubin (direct bilirubin, DBIL) increased mainly (26.39 + 40.14) umol/L. glutamic pyruvic transaminase (glutamic-pyruvic transaminase, ALT). Light and moderate increase, respectively (101.43 + 123.14) U/L, (93.52 + 83.76) U/L. serum immunoglobulin M (immunoglobin M, IgM) increased mainly, the average value of (401.03 + 267.13) g/L. serum anti mitochondrial antibody (antimitochondrial antibody, AMA), especially M2 subtype (AMA-M2) positive as the diagnosis of the most prominent immunity of the disease immunity Index [1], with high specificity and sensitivity. The AMA positive rate in this study was the serum TBIL, DBIL, TBA, ALB, ALT, IgM values in 82.20%.PBC patients with different clinical stages. The difference was statistically significant (P? 0.05). The treatment was treated with ursodeoxycholic acid (ursodeoxycholic acid), and the results showed that after treatment, liver hard. The symptoms of the patients in the prophase group were improved, the value of ALP and GGT decreased significantly, and the total bilirubin level was also reduced in varying degrees. The effect of the patients with liver cirrhosis was not good, although it combined with glucocorticoid and immunosuppressant.
Conclusion: PBC is mostly seen in middle-aged women with pruritus, jaundice, fatigue and hepatomegaly as the main clinical manifestation. Its natural history is a slow progressive cholestasis process. It can be manifested as primary biliary cholangitis at the early stage of the disease. It takes a long time to develop into primary biliary cirrhosis. The serum biochemical index is A The significant elevation of LP and GGT levels was associated with hyperbilirubinemia, hypergloidemia, and hyperglobulin, while ALT and AST were mild and moderate. The AMA/AMA-M2 of the high titer was the main indicator of PBC for the diagnosis and evaluation of the severity of the disease. The clinical symptoms, biochemical indexes, immunology and histology should be analyzed synthetically, and the diagnosis of the liver was necessary when the liver was necessary. The most reliable basis of the disease is that the treatment of PBC is still limited and UDCA is a first-line therapeutic drug. The course of the disease, the serum albumin (albumin, ALB), the level of TBIL, the histology staging and the early application of UDCA are all closely related to the progression and prognosis of the disease, and the prognosis is poor in the late stage of.PBC. It is necessary to pay attention to the early diagnosis and early treatment. It is more effective in delaying the progression of the disease and improving the prognosis of the patients.
【学位授予单位】：大连医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R575.22

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