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2.
Liang Wang Jinfu Lin Fu Xiong Yingyin Liang Huan Li Ziyu Liao Cheng Zhang 《Neuromuscular disorders : NMD》2021,31(5):456-461
Twins with Duchenne muscular dystrophy (DMD) have been widely studied. We report the first rare case of monozygotic triplets with DMD who shared consistent phenotypes, including delayed motor and language milestones, muscle wasting and weakness, joint contracture, and lumbar lordosis. Muscle magnetic resonance imaging and biopsy revealed the similar muscle injury characteristics and dystrophin absence. Short tandem repeat analysis confirmed monozygosity. A de novo mutation (exon 49–52 deletion) was found in the triplets but not in their mother. Treatment included prednisone, idebenone, and rehabilitation management. At the 2-year follow-up, motor function had deteriorated, and muscle fatty infiltration was more extensive and severe. Our case offers a unique opportunity for genetic and therapeutic research. Furthermore, it highlights the critical role of genetic factors in DMD phenotypes and provides a potential choice for treatment observations. 相似文献
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目的分析乙型肝炎(乙肝)病毒(Hepatitis B virus,HBV)高载量孕妇孕期HBV脱氧核糖核酸(HBV desoxyribonucleic acid,HBV-DNA)水平和HBVe抗原(HBV e antigen,HBeAg)阳性率以及孕期抗病毒治疗结合标准阻断措施对其所生婴儿母婴传播阻断失败率的影响。方法通过医院信息系统收集HBV-DNA高载量(≥2×10^6IU/mL)孕妇血清学检测结果、抗病毒药物使用等信息,描述HBV-DNA载量和HBeAg阳性率;对HBV-DNA高载量孕妇所生婴儿进行乙肝疫苗(Hepatitis B vaccine,HepB)和乙肝免疫球蛋白(Hepatitis B immunoglobulin,HBIG)联合免疫,在完成第3剂HepB后7月龄-2岁对乙肝表面抗原和HBV-DNA进行随访检测,分析母婴传播阻断失败率。结果共纳入1822名HBV-DNA高载量孕妇,接受、未接受抗病毒治疗分别占75.19%、24.81%。孕妇妊娠期、分娩前HBV-DNA≥1.0×10^8IU/mL比例分别为68.10%(933/1370)、0.15%(2/1370)(χ^2=2692.27,P<0.0001)。接受抗病毒治疗组妊娠期、分娩前HBeAg阳性率分别为96.53%(1001/1037)、96.16%(1251/1301)(χ^2=0.23,P=0.635),未接受抗病毒治疗组妊娠期、分娩前HBeAg阳性率分别为97.70%(298/305)、96.98%(417/430)(χ^2=0.36,P=0.550)。两组HepB和HBIG联合免疫后母婴传播阻断失败率分别为0.42%(3/714)、6.67%(14/210)(χ^2=31.69,P<0.0001)。结论孕妇HBV-DNA高载量以≥1.0×10^8IU/mL为主,孕期抗病毒治疗可显著降低孕妇HBV-DNA载量,结合HepB和HBIG联合免疫可显著降低其所生婴儿HBV母婴传播阻断失败率。 相似文献
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肺癌合并慢性阻塞性肺疾病外科治疗的肺功能评价 总被引:2,自引:0,他引:2
目的评价慢性阻塞性肺疾病(COPD)患者合并肺癌行肺叶切除的手术风险和手术对肺功能的影响.方法回顾分析1998年1月至2005年1月我院收治的中度COPD合并周围型非小细胞肺癌32例患者的临床资料.其中,男29例,女3例;平均年龄(65.0±5.4)岁.病理分型:鳞癌2l例,腺癌11例;病理分期:Ⅰ期2例,Ⅱ期5例,Ⅲa期24例,Ⅲb期l例.所有患者均接受肺叶切除术.结果术前患者肺功能第1秒用力呼吸容积(FEV1)和动脉氧分压平均值分别为(64±9)%和(85±12)mmHg,术后分别为(62±10)%和(87±11)mmHg,虽然FEV1略有下降,但均无显著性差异(P>0.05).本组无围手术期死亡者.结论中度COPD患者仍有一定的肺功能储备,可耐受肺叶切除手术. 相似文献
6.
Honghong Wang Xingang Li Shusen Sun Guifu Mao Ping Xiao Chan Fu Zhuoxin Liang Min Zheng Yuling Huang Haihong Tang Renhao Ou Ni Yang Xi Ling Zhigang Zhao 《Journal of pharmaceutical sciences》2018,107(5):1416-1422
An accurate dosage determination is required in neonates when antibiotics are used. The adult data cannot be simply extrapolated to the pediatric population due to significant individual differences. We aimed to identify factors impacting ceftazidime exposure in neonates and to provide drug dosing guidance to clinicians. Forty-three neonates aged less than 60 days with proven or suspected infections were enrolled in this study. After intravenous administration, blood samples were collected, and plasma ceftazidime concentration was determined using a HPLC method. Pharmacokinetic data were fitted using a nonlinear mixed-effects model approach. One-compartmental model could nicely characterize the ceftazidime in vivo behavior. The covariate test found that the postmenstrual age (day) was strongly associated with systemic drug clearance (L/h), and the effect of body weight (kg) was identified as the covariate on distribution volume (L). Compared with the base model, the addition of covariates improved the goodness-of-fit of the final model. Model validation (bootstrap, visual predictive check, and prediction-corrected visual predictive check) suggested a robust and reliable pharmacokinetic model was developed. Personalized dosage regimens were provided based on model simulations. The intravenous dose should be adjusted according to postmenstrual age, body weight, and minimum inhibitory concentration. 相似文献
7.
Linfeng Wu Wu Chen Feng Li Brian R. Morrow Franklin Garcia-Godoy Liang Hong 《Journal of pharmaceutical sciences》2018,107(12):3134-3142
It is important to address the periodontitis-associated bacteria in the residual subgingival plaque after scaling and root planing to successfully treat periodontitis. In this study, we explored the possibility of exploiting the ion pairing/complexation of minocycline, Ca2+, and sulfate/sulfonate-bearing biopolymers to develop an intrapocket delivery system of minocycline as an adjunct to scaling and root planing. Minocycline-calcium-dextran sulfate complex microparticles were synthesized from minocycline, CaCl2, and dextran sulfate. They were characterized using Fourier-transform infrared spectroscopy, scanning electron microscopy, and energy-dispersive X-ray spectroscopy. An in vitro release study was conducted to evaluate the release kinetics of minocycline from these microparticles. Agar disk diffusion assays and biofilm-grown bacteria assays were used to assess antibacterial capability. High loading efficiency (96.98% ± 0.12%) and high loading content (44.69% ± 0.03%) for minocycline were observed for these complex microparticles. Mino-Ca-DS microparticles achieved sustained release of minocycline for at least 9 days at pH 7.4 and 18 days at pH 6.4 in phosphate-buffered saline, respectively. They also demonstrated potent antimicrobial effects against Streptococcus mutans and Aggregatibacter actinomycetemcomitans in agar disk diffusion and biofilm assays. These results suggested that the ion pairing/complexation of minocycline, Ca2+, and sulfonate/sulfate-bearing biopolymers can be exploited to develop complex microparticles as local delivery systems for periodontitis treatment. 相似文献
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摘 要 目的:建立苍耳子药材的HPLC特征图谱,为评价不同产地苍耳子的质量提供参考。方法: 采用Alltima C18(250 mm×4.6 mm,5 μm),流动相乙腈 0.1%磷酸溶液梯度洗脱,流速:0.8 ml·min-1,检测波长:278 nm,柱温:25 ℃,对来自河南、吉林、安徽等地共47批苍耳子样品运用“中药指纹图谱相似度评价系统”进行分析。 结果:以绿原酸为参照峰,初步构建了由5个特征峰组成的苍耳子HPLC 特征图谱。结论:该方法简便、可靠,为苍耳子药材的质量控制提供了有效的评价方法。 相似文献
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