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Clinical Oral Investigations - The aim of this study was the analysis of WNT10A variants in seven families of probands with various forms of tooth agenesis and self-reported family history of...  相似文献   
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BACKGROUND Degree of portal hypertension(PH) is the most important prognostic factor for the decompensation of liver cirrhosis and death, therefore adequate care for patients with liver cirrhosis requires timely detection and evaluation of the presence of clinically significant PH(CSPH) and severe PH(SPH). As the most accurate method for the assessment of PH is an invasive direct measurement of hepatic venous pressure gradient(HVPG), the search for non-invasive methods to diagnose these conditions is actively ongoing.AIM To evaluate the feasibility of parameters of endogenously induced displacements and strain of liver to assess degree of PH.METHODS Of 36 patients with liver cirrhosis and measured HVPG were included in the casecontrol study. Endogenous motion of the liver was characterized by derived parameters of region average tissue displacement signal(dantero, dretro, d RMS) and results of endogenous tissue strain imaging using specific radiofrequency signal processing algorithm. Average endogenous strain μ and standard deviation σ of strain were assessed in the regions of interest(ROI)(1 cm × 1 cm and 2 cm × 2 cm in size) and different frequency subbands of endogenous motion(0-10 Hz and 10-20 Hz).RESULTS Four parameters showed statistically significant(P 0.05) correlation with HVPG measurement. The strongest correlation was obtained for the standard deviation of strain(estimated at 0-10 Hz and 2 cm × 2 cm ROI size). Three parameters showed statistically significant differences between patient groups with CSPH, but only dretro showed significant results in SPH analysis. According to ROC analysis area under the curve(AUC) of the σROI[0…10 Hz, 2 cm × 2 cm] parameter reached 0.71(P = 0.036) for the diagnosis of CSPH; with a cut-off value of 1.28 μm/cm providing 73% sensitivity and 70% specificity. AUC for the diagnosis of CSPH for μROI[0…10 Hz, 1 cm × 1 cm] was 0.78(P = 0.0024); with a cut-off value of 3.92 μm/cm providing 73% sensitivity and 80% specificity. Dretro parameter had an AUC of 0.86(P = 0.0001) for the diagnosis of CSPH and 0.84(P = 0.0001) for the diagnosis of SPH. A cut-off value of-132.34 μm yielded 100% sensitivity for both conditions, whereas specificity was 80% and 72% for CSPH and SPH respectively.CONCLUSION The parameters of endogenously induced displacements and strain of the liver correlated with HVPG and might be used for non-invasive diagnosis of PH.  相似文献   
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Background

We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.

Methods

In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor. Plasma HE4 was measured by immunoassay before treatment (baseline) and 1–6?months after commencement of ivacaftor, and were correlated with FEV1 (% predicted), sweat chloride, C-reactive protein (CRP) and body mass index (BMI).

Results

After 1?month of therapy, HE4 levels were significantly lower than at baseline and remained decreased up to 6?months. A significant inverse correlation between absolute and delta values of HE4 and FEV1 (r?=??0.5376; P?<?.001 and r?=??0.3285; P?<?.001), was retrospectively observed in pooled groups, including an independent association of HE4 with FEV1 by multiple regression analysis (β?=??0.57, P?=?.019). Substantial area under the receiver operating characteristic curve (ROC-AUC) value was determined for HE4 when 7% mean change of FEV1 (0.722 [95% CI 0.581–0.863]; P?=?.029) were used as classifier, especially in the first 2?months of treatment (0.806 [95% CI 0.665–0.947]; P?<?.001).

Conclusions

This study shows that plasma HE4 levels inversely correlate with lung function improvement in CF patients receiving ivacaftor. Overall, this potential biomarker may be of value for routine clinical and laboratory follow-up of CFTR modulating therapy.  相似文献   
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Heike  A.  Bischoff-Ferrari  Waiter  C.  Willett  John  B.  Wong  苗峥 《美国医学会杂志》2006,25(4):248-248
背景:对于脊椎以外的骨折而言,补充口服维生素D的预防作用和用量仍无定论。 目的:评估补充维生素D在预防老年髋骨骨折和非脊椎骨折方面的效果。 数据来源:使用MEDLINE、Cochrance对照试验记录(1960~2005年)以及EMBASE(1991-2005年),对英文和非英文文章进行系统回顾。通过与临床专家接触,通过检索美国社会骨和骨矿研究协会提供的参考文献和摘要(1995~2004年).进一步寻找更多的研究。检索词包括随机对照试验(randomized controlled trial,RCT)、临床对照试验、随机分配、双盲法、维生素D3、维生素D2;25-羟基维生素D、骨折、人类、老年、摔倒和骨密度。 研究选取:纳入的研究仅限于口服补充维生素D(维生素D3、维生素D2、补钙或不补钙)与补钙或安慰剂比较的双盲RCTs。试验于检查髋部骨折或非脊椎骨折的老年人(年龄≥60岁)中进行。数据提取:两位作者根据预先规定独立提取相关数据,其中包括研究质量指标。 数据综合:所有的汇总分析均以随机效应模型为基础。5项有关髋部骨折的RCTs(n=9294)和7项有关非脊椎骨折危险的RCTs(n=9820)符合我们的纳入标准。所有试验均使用了维生素D3。对髋部和非脊椎骨折预防研究的异质性亦进行观察,用低剂量(400IU/d)和高剂量(700~800IU/d)分别合并RCTs后异质性消失。与补钙或安慰剂相比,每天服700~800IU的维生素D可使髋部骨折的相对危险(relative risk,aa)下降26%(3项RCTs共计5572人;RR,0.74;95%可信区间[confidence interval,CI],0.61~0.88),使非脊椎骨折的相对危险下降23%(5项RCTs共计6098人;RR,0.77;95%CI,0.68~0.87)。每天服400IU的维生素D(2项RCTs共计3722人;髋部骨折RR,1.15;95%CI,0.88~1.50;非脊椎骨折RR,1.03;95%CI,0.86—1.24)未见明显获益。 结论:口服补充维生素D(700~800IU/d)可以降低尚能活动的老人或慈善机构收容的老年人发生髋部骨折和脊椎以外骨折的危险,每天口服400IU维生素D并不足以预防骨折。  相似文献   
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