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| 1 | Δ4-3-oxosteroid-5β-reductase deficiency: Responses to oral bile acid therapy and long-term outcomes显示文摘BACKGROUND Disorders of primary bile acid synthesis may be life-threatening if undiagnosed,or not treated with primary bile acid replacement therapy. To date, there are few reports on the management and follow-up of patients with Δ4-3-oxosteroid 5β-reductase(AKR1 D1) deficiency. We hypothesized that a retrospective analysis of the responses to oral bile acid replacement therapy with chenodeoxycholic acid(CDCA) in patients with this bile acid synthesis disorder will increase our understanding of the disease progression and permit evaluation of this treatment regimen as an alternative to the Food and Drug Administration(FDA) approved drug cholic acid, which is currently unavailable in China.AIM To evaluate the therapeutic responses of patients with AKR1 D1 deficiency to oral bile acid therapy, specifically CDCA.METHODS Twelve patients with AKR1 D1 deficiency, confirmed by fast atom bombardment ionization-mass spectrometry analysis of urine and by gene sequencing for mutations in AKR1 D1, were treated with differing doses of CDCA or ursodeoxycholic acid(UDCA). The clinical and biochemical responses to therapy were monitored over a period ranging 0.5-6.4 years. Dose adjustment, to optimize the therapeutic dose, was based on changes in serum biochemistry parameters,notably liver function tests, and suppression of the urinary levels of atypical hepatotoxic 3-oxo-Δ4-bile acids measured by mass spectrometry.RESULTS Physical examination, serum biochemistry parameters, and sonographic findings improved in all 12 patients during bile acid therapy, except one who underwent liver transplantation. Urine bile acid analysis confirmed a significant reduction in atypical hepatotoxic 3-oxo-Δ4 bile acids concomitant with clinical and biochemical improvements in those patients treated with CDCA. UDCA was ineffective in down-regulating endogenous bile acid synthesis as evidenced from the inability to suppress the urinary excretion of atypical 3-oxo-Δ4-bile acids. The dose of CDCA required for optimal clinical and biochemical responses varied from 5.5-10 mg/kg per day among patients based on maximum suppression of the atypical bile acids and improvement in serum biochemistry parameters, and careful titration of the dose was necessary to avoid side effects from CDCA.CONCLUSION The primary bile acid CDCA is effective in treating AKR1 D1 deficiency but the therapeutic dose requires individualized optimization. UDCA is not recommended for long-term management. | Mei-Hong Zhang Kenneth DR Setchell Jing Zhao Jing-Yu Gong Yi Lu Jian-She Wang | 2019 | World Journal of Gastroenterology2019，25，7： | 7 |
| 2 | Silvicultural alternatives to conventional even-aged forest man-agement-what limits global adoption?显示文摘Background: The development of forestry as a scientific and management discipline over the last two centuries has mainly emphasized intensive management operations focused on increased commodity production, mostly wood. This 'conventional' forest management approach has typically favored production of even-aged, single-species stands. While alternative management regimes have generally received less attention, this has been changing over the last three decades, especially in countries with developed economies. Reasons for this change include a combination of new information and concerns about the ecological consequences of intensive forestry practices and a willingness on the part of many forest owners and society to embrace a wider set of management objectives. Alternative silvicultural approaches are characterized by a set of fundamental principles, including avoidance of clearcutting, an emphasis on structural diversity and small-scale variability, deployment of mixed species with natural regeneration,and avoidance of intensive site-preparation methods.Methods: Our compilation of the authors' experiences and perspectives from various parts of the world aims to initiate a larger discussion concerning the constraints to and the potential of adopting alternative silvicultural practices.Results: The results suggest that a wider adoption of alternative silvicultural practices is currently hindered by a suite of ecological, economic, logistical, informational, cultural, and historical constraints. Individual contexts display their own unique combinations and relative significance of these constraints, and accordingly, targeted efforts, such as regulations and incentives, may help to overcome specific challenges.Conclusions: In a broader context, we propose that less emphases on strict applications of principles and on stand structures might provide additional flexibility and facilitate the adoption of alternative silvicultural regimes in a broader set of circumstances. At the same time, the acceptance of alternative silvicultural systems as the 'preferred or default mode of management' wil necessitate and benefit from the continued development of the scientific basis and valuation of a variety of ecosystem goods and services. This publication is aimed to further the discussion in this context. | Klaus J Puettmann Scott McG Wilson Susan C Baker Pablo J Donoso Lars Dr?ssler Girma Amente Brian D Harvey Thomas Knoke Yuanchang Lu Susanna Nocentini Francis E Putz Toshiya Yoshida Jürgen Bauhus | 2015 | Forest Ecosystems2015，2，2： | 4 |
| 3 | Preparation and evaluation of sustained release AZT-loaded microspheres: Optimization of the release characteristics using response surface methodology显示文摘 | Abu-Izza KA Garcia-Contreras L Lu DR | 1996 | J Pharm Sci1996，85，： | 2 |
| 4 | WONCA研究论文摘要汇编——有流产史女性患心血管疾病的风险:女性健康行动显示文摘目的与流产/死产相关的新陈代谢、激素和凝血机制的变化可导致心血管疾病(CVD)。本研究评价了有流产史女性与CVD的相关性。方法 1993—1998年,将77 701名绝经后妇女纳入研究。收集基线信息,包括既往史、社会人口学和CVD风险因素。对有1次或≥2次流产、≥1次死胎与发生CVD的关系行多元Logistic回归分析。结果 77 701名妇女中有23 538名(30.3%)报告有流产史,1 670名(2.2%)有死胎史,1 673名(2.2%)既有流产史又有死胎史。与无死胎史者相比,≥1次死胎史者患冠心病(CHD)的风险升高〔OR=1.27,95%CI(1.07,1.51)〕;与无流产史者相比,有1次流产史、≥2次流产史者患CHD的风险升高〔OR=1.19,95%CI(1.08,1.32);OR=1.18,95%CI(1.04,1.34)〕。缺血性卒中者与死产或流产史者患CHD的风险比间无显著差异。结论流产史与CHD相关,但与缺血性卒中无关。有≥1次死产或有≥1次流产史妇女患CVD的风险增加,应该列为密切观察或行早期干预的对象;流产导致CVD风险增加的病理生理学机制还需行进一步研究。 | Parker DR Lu B Sands-Lincoln M 周淑新 | 2014 | 中国全科医学2014，17，32： | 2 |
| 5 | The mechanism ofincreasing outflow facility by rho-kinase inhibition with Y-27632 in bovine eyes显示文摘 | Lu Z Overby DR Scott PA | 2008 | Exp Eye Res2008，86，2： | 1 |
| 6 | Preparation and evaluation of sustained release AZT loaded micro- spheres: optimizgion of the release characteristerics using response sur face methodology显示文摘 | Abu-lzza KA Garcia-Contreras L Lu DR | 1996 | J Pharm Sci1996，85，2： | 1 |
| 7 | An automated approach to salt selection fornew unique trazodone salts显示文摘 | Ware EC Lu DR | 2004 | Pharm Res2004，21，1： | 1 |
| 8 | Schizophrenia,Ⅲ:Brain-Derived Neurotrophic Factor and Genetic Risk显示文摘 | Egan MF Weinberger DR Lu B | 2003 | Am J Psychiatry2003，160，： | 1 |
| 9 | Hypertension as a biomarker of efficacy in patients with metastatic renal cell carcinoma treated with sunitinib显示文摘 | RINI BI COHEN DP LU DR | 2011 | J Natl Cancer Inst2011，103，9： | 1 |
| 10 | Combined colonoscopy and endometrial biopsy cancer screening results in women with Lynch syndrome显示文摘 | Nebgen DR Lu KH Rimes S | 2014 | Gynecol Oncol2014，135，1： | 1 |
| 11 | Improved cathode materials for microbial electrosynthesis 显示文摘 | Zhang T Nie H Bain TS Lu H Cui M Snoeyenbos-West OL Franks AE Nevin KP Russell TP Lovley DR | 2012 | Energy Environ Sci2012，6，1： | 1 |
| 12 | CT diagnosis of the small - bowel obstruction: efficacy in 60 patients 显示文摘 | Fukuya T Hawes DR Lu CC | 1992 | AJR1992，，： | 1 |
| 13 | Preparation and evaluation of sustained release AZT-1oaded microspheres:optimization of the release characteristics using response surface methodology显示文摘 | Abu-Izza KA Garcia-Contreras L Lu DR | 1996 | J Pharm Sci1996，85，2： | 1 |
| 14 | Improved cathode materials for microbial electrosynthesis 显示文摘 | Zhang T Nie H Bain TS Lu H Cui M Snoeyenbos-West OL Franks AE Nevin KP Russell TP Lovley DR | 2013 | Energy Environ Sci2013，6，1： | 1 |
| 15 | CT diagnosis of small bowel obstruction efficacy in 60 patients显示文摘 | Fukuya T Hawes DR Lu CC | 1992 | A JR Am J Roentgenol1992，158，4： | 1 |
| 16 | CT diagnosis of small bowelobstruction:efficacyin60patients显示文摘 | Fukuya T Hawes DR Lu CC | | 0，，04： | 1 |
| 17 | Cell culture and animal studies for intracerebral delivery of borocaptate in liposomal formulation显示文摘 | Ji B Chen W Lu DR | 2001 | Drug Deliv2001，8，1： | 1 |
| 18 | Anaplastic lyrnphoma ki- nase gene rearrangements in non-small cell lung cancer are asso- ciated with prolonged progression-free survival on pemetrexed 显示文摘 | Carnidge DR Kono SA Lu X | 2011 | J Thorac Oncol2011，6，4： | 1 |
| 19 | Acrylic acid containing copolymera as reactive compatibilizers for toughening nylon 6显示文摘 | Lu M Keskkula H Paul DR | 1994 | Polym Eng Sci1994，34，1： | 1 |
| 20 | Concurrent trastuzumab and HER-2/neu-specific vaccination in patients with metastatic breast cancer显示文摘 | Disis ML Wallace DR Gooley TA Dang Y Slota M Lu H | 2009 | J Clin Oncol2009，27，28： | 1 |
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