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  本文選題：原發(fā)性膽汁性肝硬化 + 臨床表現(xiàn)�。� 參考：《大連醫(yī)科大學(xué)》2014年碩士論文

【摘要】：目的：通過分析原發(fā)性膽汁性肝硬化（Primary Biliary Cirrhosis,PBC）患者的臨床特征，探討在不同臨床分期生化、免疫學(xué)指標的改變及治療轉(zhuǎn)歸，提高對本病的認識及治療水平，減少誤診率、改善患者預(yù)后。 方法：收集我院具有完整病例資料經(jīng)住院確診146例PBC患者的一般資料、臨床表現(xiàn)、血清生化指標、免疫學(xué)指標、影像學(xué)檢查及治療轉(zhuǎn)歸等進行回顧性分析。并進一步探討了肝硬化前期與肝硬化期兩組患者在血清生化、免疫學(xué)指標及治療是否存在統(tǒng)計學(xué)差異。 結(jié)果：146例PBC患者中男性9例，女性137例，男女比為1：15，確診平均年齡52歲，其平均值為（56.90±11.51）。從出現(xiàn)癥狀至臨床確診時間為1-96個月不等，平均48.5個月。最常見臨床癥狀包括：乏力(45.89%）、皮膚瘙癢（37.67%）、黃疸（30.14%）、腹脹（26.71%）。主要體征為：脾腫大（37.67%）、肝腫大（17.81%）、肝掌（10.96%）、蜘蛛痣（8.22%）、腹水（12.33%）及皮膚色素沉著（3.42%）。實驗室檢查中，所有患者均以堿性磷酸酶(alkaline phosphatase,ALP)、γ-谷氨酰轉(zhuǎn)肽酶(Gamma Glutamyl Transpeptidase,GGT)升高為主，分別為（354.88±275.23）U/L和（391.85±400.33）U/L。伴血清總膽紅素(total bilirubin，TBil)不同程度的升高為（47.919±59.98）umol/L，其中以直接膽紅素(direct bilirubin,DBIL)升高為主(26.39±40.14) umol/L。谷丙轉(zhuǎn)氨酶（glutamic-pyruvic transaminase,ALT）、谷草轉(zhuǎn)氨酶（Aspartate transaminase,AST）多表現(xiàn)為輕中度升高，分別為(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）升高為主，其平均值為（401.03±267.13）g/L。血清抗線粒體抗體（antimitochondrial antibody,AMA）尤其是M2亞型（AMA-M2）陽性作為診斷本病最突出的免疫指標[1]，具有較高特異及敏感性，本研究AMA陽性率為82.20%。PBC患者不同臨床分期的血清TBIL、DBIL、TBA、ALB、ALT、IgM值差異經(jīng)比較后有統(tǒng)計學(xué)意義（P�d0.05）。治療上均采用以熊去氧膽酸(ursodeoxycholic acid, UDCA)為主的綜合治療，結(jié)果顯示經(jīng)治療后，肝硬化前期組患者癥狀得到改善，ALP、GGT值均明顯下降，總膽紅素水平亦不同程度降低。對于肝硬化期組患者雖聯(lián)合糖皮質(zhì)激素及免疫抑制劑，但效果欠佳。 結(jié)論：PBC多見于中年女性，以瘙癢、黃疸、乏力和肝脾腫大為主要臨床表現(xiàn)，其自然演變史是一個緩慢漸進性的膽汁淤積過程，在病程的早期可表現(xiàn)為原發(fā)性膽汁性膽管炎，需較長一段時間可發(fā)展為原發(fā)性膽汁性肝硬化。血清生化指標以ALP和GGT水平的明顯升高伴高膽紅素血癥、高球蛋白血癥為特點，而ALT及AST輕中度升高。高滴度的AMA/AMA-M2是診斷PBC的主要指標。PBC的診斷及評估病情輕重應(yīng)當綜合分析其臨床癥狀、生化指標、免疫學(xué)及組織學(xué)等檢查，，必要時行肝穿是診斷本病的最可靠依據(jù)。目前PBC的治療仍比較局限，UDCA為療效肯定的一線治療藥物。患者的病程、血清白蛋白(albumin，ALB）、TBIL水平、組織學(xué)分期及UDCA早期應(yīng)用與否均與疾病進展和預(yù)后密切相關(guān)。PBC晚期預(yù)后差，必須重視其早期診斷、早期治療，才能更有效延緩病情進展、真正改善患者預(yù)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.
【學(xué)位授予單位】：大連醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R575.22

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