本文選題：肌萎縮側(cè)索硬化 + 胱抑素C��； 參考：《中國人民解放軍醫(yī)學(xué)院》2014年碩士論文

【摘要】：目的：第一部分：應(yīng)用免疫比濁法測定實驗組和對照組腦脊液（CSF）胱抑素C（Cys-C）水平。對實驗組CSF Cys-C水平與發(fā)病年齡、性別、起病部位、進(jìn)展速率和修訂版肌萎縮側(cè)索硬化功能評分（revised amyotrophic lateral sclerosisfunctional rating scale, ALSFRS-R）等臨床指標(biāo)進(jìn)行相關(guān)性分析；探討體質(zhì)指數(shù)（BMI）與CSF和血清Cys-C水平及散發(fā)性肌萎縮側(cè)索硬化（sALS）患者各臨床指標(biāo)的相關(guān)性。第二部分：探討sALS患者血清肌酐（SCr）水平及其與臨床指標(biāo)的關(guān)系。 方法:第一部分：納入121例確診或很可能sALS患者，同時納入22例多系統(tǒng)萎縮（MSA）患者和18例年齡匹配健康對照者（HC），測定CSF Cys-C水平并進(jìn)行分析；對Cys-C水平與起病年齡、起病部位、BMI、病程、ALSFRS-R、用力肺活量（FVC）和進(jìn)展速率之間的關(guān)系進(jìn)行分析。第二部分：測定sALS、MSA和緊張型頭痛（TTH）各80例患者的SCr水平，探討sALS患者SCr水平與ALSFRS-R、FVC、起病部位、病程和疾病進(jìn)展速率之間的關(guān)系。 結(jié)果:第一部分：（1）CSF Cys-C測定發(fā)現(xiàn)sALS (4.17±1.32) mg/L、HC (4.58±1.97) mg/L和MSA (4.05±1.18) mg/L之間差異無統(tǒng)計學(xué)意義。sALS患者CSFCys-C水平與起病年齡、起病部位、BMI、病程、ALSFRS-R、FVC和疾病進(jìn)展速率間無明顯相關(guān)性。（2）血清Cys-C測定發(fā)現(xiàn)sALS (1.03±0.18) mg/L、HC (1.10±0.54) mg/L和MSA (1.10±0.21) mg/L之間差異無統(tǒng)計學(xué)意義。相關(guān)分析發(fā)現(xiàn)sALS患者血清Cys-C水平與ALSFRS-R成負(fù)相關(guān)(r=-0.355, P=0.007)，而與起病部位成正相關(guān)(r=0.274, P=0.041)。（3）sALS患者CSF和血清Cys-C水平差異有統(tǒng)計學(xué)意義(P0.001)，分析發(fā)現(xiàn)CSF和血清Cys-C之間無線性相關(guān)(r=0.071,P=0.605)。（4）相關(guān)性分析發(fā)現(xiàn)sALS患者BMI與FVC有相關(guān)性(Pearson r=0.321,P=0.003)，與CSF和血清Cys-C水平及其他臨床指標(biāo)則無相關(guān)性。第二部分：（1）sALS患者SCr水平明顯低于MSA和TTH組，差異有統(tǒng)計學(xué)意義[（60.86±16.80）μmol/L vs.（70.05±12.79）μmol/L vs.（66.97±14.14）μmol/L, P0.01]。（2）球部起病與肢體起病的sALS患者間SCr水平差異無統(tǒng)計學(xué)意義[（62.82±10.62）μmol/L vs.（60.64±13.26）μmol/L, P=0.544]；病程較短與病程較長的sALS患者間SCr水平差異無統(tǒng)計學(xué)意義[（62.38±13.57）μmol/L vs（.58.24±11.09）μmol/L,P=0.168]。（3）sALS患者SCr水平與ALSFRS-R成正相關(guān)（r=0.315, P=0.005），而與FVC、起病部位、病程和疾病進(jìn)展速率之間無明顯相關(guān)性。 結(jié)論：第一部分：CSF Cys-C尚不足作為ALS生物學(xué)標(biāo)志物。在相關(guān)指標(biāo)測定過程中需積極排除相關(guān)影響因素并結(jié)合適當(dāng)?shù)慕y(tǒng)計學(xué)方法進(jìn)行分析。第二部分：SCr是sALS的一項重要生化指標(biāo)，，對監(jiān)測疾病進(jìn)展可能具有重要的作用。
[Abstract]:Objective: to determine the level of cystatin Cy Cys-Cin in cerebrospinal fluid (CSF) of experimental group and control group by immunoturbidimetric method. The correlation between CSF Cys-C level and onset age, sex, location of onset, progression rate and revised amyotrophic lateral sclerosis score were analyzed in the experimental group, such as the age of onset, sex, location of onset, rate of progression and the revised score of amyotrophic lateral sclerosis (ALSFRS-R). To investigate the correlation between BMI and the levels of CSF, serum Cys-C and sporadic amyotrophic lateral sclerosis (ALS). Part two: to investigate the relationship between serum creatinine (creatinine) and clinical indexes in patients with sALS. Methods: in the first part, CSF Cys-C levels were measured and analyzed in 121 confirmed or probable sALS patients, 22 patients with multiple system atrophy and 18 age-matched healthy controls. The relationship between BMI, ALSFRS-R, forced vital capacity (RV) and progression rate was analyzed. The second part: the SCr levels of 80 patients with sALS and 80 patients with sALS were measured, and the relationship between SCr level and ALSFRS-RV FVC, the location of onset, the course of disease and the rate of disease progression were investigated. Results: in the first part, there was no significant difference between sALS 4.17 鹵1.32 mg / L sALS 4.58 鹵1.97 mg/L and MSA 4.05 鹵1.18 mg/L. There was no significant correlation between the onset site of BMI, the course of ALSFRS-RV and the disease progression rate. The serum Cys-C analysis showed that there was no significant difference between sALS 1.03 鹵0.18) mg / L HC 1.10 鹵0.54 mg/L and MSA 1.10 鹵0.21) mg/L. Correlation analysis showed that there was a negative correlation between serum Cys-C level and ALSFRS-R in patients with sALS, but there was a positive correlation between serum Cys-C level and onset site (P = 0.274). There was a significant difference in CSF and Cys-C levels between P=0.041).(3)sALS patients and patients with P=0.041).(3)sALS (P 0.001). The results showed that there was a wireless correlation between CSF and serum Cys-C. Correlation analysis showed that there was a correlation between BMI and FVC in sALS patients, but no correlation with CSF, serum Cys-C level and other clinical indexes. In the second part, the level of SCr in the patients with SALS was significantly lower than that in the MSA and TTH groups. 宸紓鏈夌粺璁″鎰忎箟[(60.86鹵16.80)渭mol/L vs.(70.05鹵12.79)渭mol/L vs.(66.97鹵14.14)渭mol/L, P0.01].(2)鐞冮儴璧風(fēng)梾涓庤偄浣撹搗鐥呯殑sALS鎮(zhèn)ｈ

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