1.Screening analysis of 4 710 cases of inherited metabolic diseases in high - risk infants using urease pretreat-ment-gas chromatography-mass spectrometry
Hu HAO ; Sitao LI ; Xuejun OUYANG ; Suzhen FANG ; Hekui LAN ; Chunhua ZHANG ; Congcong SHI ; Xin XIAO
Chinese Journal of Applied Clinical Pediatrics 2015;(21):1660-1662
Objective To detect the incidence of inherited metabolic diseases(IMD)and disorders of metabo-lism in 4 710 high - risk infants,as well as providing basis of clinical diagnosis and treatment by using urease pretreat-ment - gas chromatography - mass spectrometry(UP - GC - MS). Methods Samples were collected from high - risk infants with IMD,after removing urea,putting in internal standard,removing protein,vacuum drying and bis (trimethyl - silyl)trifluoroacetamide / trimethyl - chlorosilane derivativing,UP - GC - MS was used to analyze compo-sitions such as organic acids,amino acids,carbohydrates,pyridoxines,purines and pyrimidines,then metabolic analysis was proceeded to refer to the normal detection value of the healthy children,finally a metabolic diagnosis was made ba-sing on the clinical data such as the high - risk clinical manifestations and general biochemical tests and other special examinations. Results In the 4 710 cases,there were 98 cases of IMD(2. 1% ),326 cases of suspected IMD(6. 9% ), 2 610 cases of metabolic disorders(55. 4% ). There were 98 cases of IMD,including 57 cases of methylmalonic aciduria,12 cases of propionic acidemia,7 cases of glutaric aciduria,5 cases of hyperphenylalaninemia,maple syrup u-rine disease and multiple carboxylase defects each,4 cases of isovaleric acidemia and 3 cases of 4 - hydroxy butyric acid urine disease. Conclusions UP - GC - MS is a effective way to diagnose IMD and metabolic disorders of infants. Common IMD in Guangdong Province include methylmalonic aciduria,propionic academia,glutaric aciduria,hyperphe-nylalaninemia,maple syrup urine disease and multiple carboxylase defects. The results of the tests can provide effective guidance for diagnosis and treatment of suspected infants.
2.Three Cases of Drug-drug Interaction Intervened by Therapeutic Drug Monitoring for Voriconazole
Jingjie YU ; Sitao YANH ; Xing ZHOU ; Guiying CHEN ; Guang DU ; Dong LIU ; Yang ZHANG
Herald of Medicine 2017;36(8):879-883
Objective To discuss the role of therapeutic drug monitoring (TDM) in pharmaceutical care by successful intervention of severe drug-drug interaction in 3 patients with hematological disease treated with voriconazole and rifampin.Methods Three patients with hematological disease were monitored for the plasma concentration of voriconazole before,during,and after the concomitant use of rifampin.The severity of this drug interaction was revealed,risks for developing invasive fungal infection and tuberculosis dissemination after chemotherapy were evaluated based on the TDM results,and alternative regimens were recommended.Results Voriconazole plasma concentration was normal at baseline but significantly depressed after combination with rifampin in all 3 cases.Concomnitant use of rifampin leads to a rapid decline in plasma concentration of voriconazole in 2-3 days,and withdraw of this enzyme induction effect takes 8-10 days after discontinuation of rifampin.Conclusion TDM is a helpful tool for providing pharmaceutical care,it helps to objectively visualize the degree of clinically important drug-drug interactions.Clinical evidence together with TDM results suggests high risk for developing invasive fungal infection and tuberculosis dissemination in hematology patients while using this combination therapy.Discontinuation of rifampin was suggested and accepted.For these patients,combination of voriconazole and rifampin should be avoided.
3. ASIC2 Synergizes with TRPV1 in the Mechano-Electrical Transduction of Arterial Baroreceptors
Xiaodong YAN ; Ping LIU ; Haixia HUANG ; Weizhen NIU ; Wei WANG ; Sitao ZHANG ; Haiyan ZHAO ; Wei WANG ; Chen ZHANG
Neuroscience Bulletin 2021;37(10):1381-1396
Mechanosensitive ion channels (MSCs) are key molecules in the mechano-electrical transduction of arterial baroreceptors. Among them, acid-sensing ion channel 2 (ASIC2) and transient receptor potential vanilloid subfamily member 1 (TRPV1) have been studied extensively and documented to play important roles. In this study, experiments using aortic arch–aortic nerve preparations isolated from rats revealed that both ASIC2 and TRPV1 are functionally necessary, as blocking either abrogated nearly all pressure-dependent neural discharge. However, whether ASIC2 and TRPV1 work in coordination remained unclear. So we carried out cell-attached patch-clamp recordings in HEK293T cells co-expressing ASIC2 and TRPV1 and found that inhibition of ASIC2 completely blocked stretch-activated currents while inhibition of TRPV1 only partially blocked these currents. Immunofluorescence staining of aortic arch–aortic adventitia from rats showed that ASIC2 and TRPV1 are co-localized in the aortic nerve endings, and co-immunoprecipitation assays confirmed that the two proteins form a compact complex in HEK293T cells and in baroreceptors. Moreover, protein modeling analysis, exogenous co-immunoprecipitation assays, and biotin pull-down assays indicated that ASIC2 and TRPV1 interact directly. In summary, our research suggests that ASIC2 and TRPV1 form a compact complex and function synergistically in the mechano-electrical transduction of arterial baroreceptors. The model of synergism between MSCs may have important biological significance beyond ASIC2 and TRPV1.
4.ASIC2 Synergizes with TRPV1 in the Mechano-Electrical Transduction of Arterial Baroreceptors.
Xiaodong YAN ; Sitao ZHANG ; Haiyan ZHAO ; Ping LIU ; Haixia HUANG ; Weizhen NIU ; Wei WANG ; Chen ZHANG
Neuroscience Bulletin 2021;37(10):1381-1396
Mechanosensitive ion channels (MSCs) are key molecules in the mechano-electrical transduction of arterial baroreceptors. Among them, acid-sensing ion channel 2 (ASIC2) and transient receptor potential vanilloid subfamily member 1 (TRPV1) have been studied extensively and documented to play important roles. In this study, experiments using aortic arch-aortic nerve preparations isolated from rats revealed that both ASIC2 and TRPV1 are functionally necessary, as blocking either abrogated nearly all pressure-dependent neural discharge. However, whether ASIC2 and TRPV1 work in coordination remained unclear. So we carried out cell-attached patch-clamp recordings in HEK293T cells co-expressing ASIC2 and TRPV1 and found that inhibition of ASIC2 completely blocked stretch-activated currents while inhibition of TRPV1 only partially blocked these currents. Immunofluorescence staining of aortic arch-aortic adventitia from rats showed that ASIC2 and TRPV1 are co-localized in the aortic nerve endings, and co-immunoprecipitation assays confirmed that the two proteins form a compact complex in HEK293T cells and in baroreceptors. Moreover, protein modeling analysis, exogenous co-immunoprecipitation assays, and biotin pull-down assays indicated that ASIC2 and TRPV1 interact directly. In summary, our research suggests that ASIC2 and TRPV1 form a compact complex and function synergistically in the mechano-electrical transduction of arterial baroreceptors. The model of synergism between MSCs may have important biological significance beyond ASIC2 and TRPV1.
Acid Sensing Ion Channels/physiology*
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Animals
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HEK293 Cells
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Humans
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Pressoreceptors/physiology*
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Rats
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TRPV Cation Channels/physiology*