http://chineseinput.net/에서 pinyin(병음)방식으로 중국어를 변환할 수 있습니다.
변환된 중국어를 복사하여 사용하시면 됩니다.
Jakhongir F. Alidjanov,Andre Overesch,Dimitri Abramov-Sommariva,Martina Hoeller,Hubert Steindl,Florian M. Wagenlehner,Kurt G. Naber 대한비뇨의학회 2020 Investigative and Clinical Urology Vol.61 No.5
Purpose: The Acute Cystitis Symptom Score (ACSS) used in a clinical trial comparing the phytodrug Canephron®N (BNO 1045) with an antibacterial agent (fosfomycin trometamol [FT]) in the treatment of acute uncomplicated cystitis (AC) in women was evaluated as a patient-reported outcome measure in a post hoc analysis. Materials and Methods: This double-blind, randomized, multicenter, phase III noninferiority trial was performed in 51 centers in Europe. The ACSS questionnaire was used to assess severity and course of symptoms. Results: The post hoc analysis included 325 patients treated with BNO 1045 and 332 patients treated with FT (total of 657 patients). The mean sum-scores of the ACSS-typical domain were comparable between groups on day 1 (BNO 1045: 10.2; FT: 10.1), and then decreased on day 4 (BNO 1045: 5.1; FT: 4.5), at end of treatment on day 8 (BNO 1045: 2.1; FT: 2.1), and at late follow-up on day 38 (BNO 1045: 0.8; FT: 0.9). Predefined thresholds using the scoring system of the ACSS could be established and validated to define “clinical cure.”Conclusions: Evaluating not only antibacterial but also nonantibacterial agents indicated for the treatment of AC in women, clinical criteria for diagnostics, and measures of patient-reported outcomes are more important as main objectives than microbiological criteria. In this post hoc evaluation, we showed that the ACSS questionnaire, validated in several languages, has the potential to be used as a suitable instrument for diagnostics and patient-reported outcomes in well-designed, international, clinical studies investigating different treatment modalities of uncomplicated urinary tract infections.
<i>ZC4H2</i> , an XLID gene, is required for the generation of a specific subset of CNS interneurons
May, Melanie,Hwang, Kyu-Seok,Miles, Judith,Williams, Charlie,Niranjan, Tejasvi,Kahler, Stephen G.,Chiurazzi, Pietro,Steindl, Katharina,Van Der Spek, Peter J.,Swagemakers, Sigrid,Mueller, Jennifer,Stef Oxford University Press 2015 Human Molecular Genetics Vol.24 No.17
<P>Miles–Carpenter syndrome (MCS) was described in 1991 as an XLID syndrome with fingertip arches and contractures and mapped to proximal Xq. Patients had microcephaly, short stature, mild spasticity, thoracic scoliosis, hyperextendable MCP joints, rocker-bottom feet, hyperextended elbows and knees. A mutation, p.L66H, in <I>ZC4H2</I>, was identified in a XLID re-sequencing project. Additional screening of linked families and next generation sequencing of XLID families identified three <I>ZC4H2</I> mutations: p.R18K, p.R213W and p.V75in15aa. The families shared some relevant clinical features. <I>In silico</I> modeling of the mutant proteins indicated all alterations would destabilize the protein. Knockout mutations in <I>zc4h2</I> were created in zebrafish and homozygous mutant larvae exhibited abnormal swimming, increased twitching, defective eye movement and pectoral fin contractures. Because several of the behavioral defects were consistent with hyperactivity, we examined the underlying neuronal defects and found that sensory neurons and motoneurons appeared normal. However, we observed a striking reduction in GABAergic interneurons. Analysis of cell-type-specific markers showed a specific loss of V2 interneurons in the brain and spinal cord, likely arising from mis-specification of neural progenitors. Injected human wt <I>ZC4H2</I> rescued the mutant phenotype. Mutant zebrafish injected with human p.L66H or p.R213W mRNA failed to be rescued, while the p.R18K mRNA was able to rescue the interneuron defect. Our findings clearly support <I>ZC4H2</I> as a novel XLID gene with a required function in interneuron development. Loss of function of <I>ZC4H2</I> thus likely results in altered connectivity of many brain and spinal circuits.</P>