Kartagener＇s syndrome （KS） is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia （PCD）. As it is accompanied by many complications, PCD/KS severely ...Kartagener＇s syndrome （KS） is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia （PCD）. As it is accompanied by many complications, PCD/KS severely affects the patient＇s quality of life. Therapeutic approaches for PCD/KS aim to enhance prevention, facilitate rapid definitive diagnosis, avoid misdiagnosis, maintain active treatment, control infection and postpone the development of lesions. In male patients, sperm flagella may show impairment in or complete absence of the ability to swing, which ultimately results in male infertility. Assisted reproductive technology will certainly benefit such patients. For PCD/KS patients with completely immotile sperm, intracytoplasmic sperm injection may be very important and even indispensable. Considering the number of PCD/KS susceptibility genes and mutations that are being identified, more extensive genetic screening is indispensable in patients with these diseases. Moreover, further studies into the potential molecular mechanisms of these diseases are required. In this review, we summarize the available information on various aspects of this disease in order to delineate the therapeutic objectives more clearly, and clarify the efficacy of assisted reproductive technology as a means of treatment for patients with PCD/KS-associated infertility.展开更多
目的通过内镜下测定 Oddi 括约肌压力研究腹腔镜胆囊切除术(LC)后胆道运动功能,证明腹腔镜胆囊切除术后腹痛、上腹部不适可能与胆道动力障碍有关。同时证明胃泌素的升高与此相关。方法选择腹腔镜胆囊切除术后腹痛组20例,腹腔镜胆囊切除...目的通过内镜下测定 Oddi 括约肌压力研究腹腔镜胆囊切除术(LC)后胆道运动功能,证明腹腔镜胆囊切除术后腹痛、上腹部不适可能与胆道动力障碍有关。同时证明胃泌素的升高与此相关。方法选择腹腔镜胆囊切除术后腹痛组20例,腹腔镜胆囊切除术后上腹部不适组18例;对照组15例进行 B 超及 ERCP 测压。结果第一组病人基础压、波幅及波宽均升高。第二组基础压、波幅及波宽轻度升高。第三组正常。结论胆囊切除术后胆道动力障碍与 Oddi 括约肌痉挛,胆管壁顺应性,胃泌素分泌增多有关。展开更多
Tyrosine hydroxylase is a key enzyme in dopamine biosynthesis. Change in tyrosine hydroxylase expression in the nigrostriatal system is closely related to the occurrence and development of Parkinson＇s disease. Verbas...Tyrosine hydroxylase is a key enzyme in dopamine biosynthesis. Change in tyrosine hydroxylase expression in the nigrostriatal system is closely related to the occurrence and development of Parkinson＇s disease. Verbascoside, an extract from Radix Rehmanniae Praeparata has been shown to be clinically effective in treating Parkinson＇s disease. However, the underlying mechanisms remain unclear. It is hypothesized that the effects of verbascoside on Parkinson＇s disease are related to tyrosine hydroxylase expression change in the nigrostriatal system. Rat models of Parkinson＇s disease were established and verbascoside（60 mg/kg） was administered intraperitoneally once a day. After 6 weeks of verbascoside treatment, rat rotational behavior was alleviated; tyrosine hydroxylase m RNA and protein expression and the number of tyrosine hydroxylase-immunoreactive neurons in the rat right substantia nigra were significantly higher than the Parkinson＇s model group. These findings suggest that the mechanism by which verbascoside treats Parkinson＇s disease is related to the regeneration of tyrosine hydroxylase-immunoreactive neurons in the substantia nigra.展开更多
文摘目的探讨帕金森病(Parkinson's disease,PD)患者既往服用左旋多巴情况与异动症风险的相关性,并基于数据驱动方法分析PD患者左旋多巴每日最高安全剂量以及累积安全剂量。方法选择于2017年3～12月于我院住院的PD患者142例,将伴发异动症患者41例作为异动组,不伴异动症患者101例作为非异动组。logistic回归分析PD患者服用左旋多巴情况与异动症风险的相关性;利用分类决策树模型计算左旋多巴安全剂量。结果异动组病程、服用药物时间明显高于非异动组,发病年龄和体质量明显低于非异动组。异动组左旋多巴累积剂量和峰日剂量明显高于非异动组[(949.10±750.39)g vs (510.77±428.36)g,P<0.01;(639.81±363.87)mg/d vs(381.93±169.89)mg/d,P<0.01]。logistic回归分析显示,左旋多巴累积剂量和峰日剂量与PD异动症风险均具有相关性(OR=1.002,P=0.002;OR=1.006,P=0.000)。分类决策树模型预测提示,左旋多巴峰日剂量400.00mg/d、左旋多巴累积剂量609.75g为区分异动症与非异动症的安全剂量。结论左旋多巴峰日剂量和累积剂量与PD异动风险可能具有相关性。
文摘Kartagener＇s syndrome （KS） is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia （PCD）. As it is accompanied by many complications, PCD/KS severely affects the patient＇s quality of life. Therapeutic approaches for PCD/KS aim to enhance prevention, facilitate rapid definitive diagnosis, avoid misdiagnosis, maintain active treatment, control infection and postpone the development of lesions. In male patients, sperm flagella may show impairment in or complete absence of the ability to swing, which ultimately results in male infertility. Assisted reproductive technology will certainly benefit such patients. For PCD/KS patients with completely immotile sperm, intracytoplasmic sperm injection may be very important and even indispensable. Considering the number of PCD/KS susceptibility genes and mutations that are being identified, more extensive genetic screening is indispensable in patients with these diseases. Moreover, further studies into the potential molecular mechanisms of these diseases are required. In this review, we summarize the available information on various aspects of this disease in order to delineate the therapeutic objectives more clearly, and clarify the efficacy of assisted reproductive technology as a means of treatment for patients with PCD/KS-associated infertility.
文摘目的通过内镜下测定 Oddi 括约肌压力研究腹腔镜胆囊切除术(LC)后胆道运动功能,证明腹腔镜胆囊切除术后腹痛、上腹部不适可能与胆道动力障碍有关。同时证明胃泌素的升高与此相关。方法选择腹腔镜胆囊切除术后腹痛组20例,腹腔镜胆囊切除术后上腹部不适组18例;对照组15例进行 B 超及 ERCP 测压。结果第一组病人基础压、波幅及波宽均升高。第二组基础压、波幅及波宽轻度升高。第三组正常。结论胆囊切除术后胆道动力障碍与 Oddi 括约肌痉挛,胆管壁顺应性,胃泌素分泌增多有关。
基金supported by a grant from Science and Technology Support Traditional Chinese Drug Research and Development Project of Shanghai of China,No.12401900302Traditional Chinese Medicine Research Foundation of Shanghai Municipal Health Bureau of China,No.2012J009A+2 种基金a grant from Annual Research Budget of Shanghai University of Traditional Chinese Medicine of China in 2013,No.2013JW25the National Natural Science Foundation of China,No.3067268430973722
文摘Tyrosine hydroxylase is a key enzyme in dopamine biosynthesis. Change in tyrosine hydroxylase expression in the nigrostriatal system is closely related to the occurrence and development of Parkinson＇s disease. Verbascoside, an extract from Radix Rehmanniae Praeparata has been shown to be clinically effective in treating Parkinson＇s disease. However, the underlying mechanisms remain unclear. It is hypothesized that the effects of verbascoside on Parkinson＇s disease are related to tyrosine hydroxylase expression change in the nigrostriatal system. Rat models of Parkinson＇s disease were established and verbascoside（60 mg/kg） was administered intraperitoneally once a day. After 6 weeks of verbascoside treatment, rat rotational behavior was alleviated; tyrosine hydroxylase m RNA and protein expression and the number of tyrosine hydroxylase-immunoreactive neurons in the rat right substantia nigra were significantly higher than the Parkinson＇s model group. These findings suggest that the mechanism by which verbascoside treats Parkinson＇s disease is related to the regeneration of tyrosine hydroxylase-immunoreactive neurons in the substantia nigra.
