BACKGROUND Intraductal papillary neoplasms of the bile duct(IPNBs)are rare and characterized by papillary growth within the bile duct lumen.IPNB is similar to obstructive biliary pathology.In this report,we present an...BACKGROUND Intraductal papillary neoplasms of the bile duct(IPNBs)are rare and characterized by papillary growth within the bile duct lumen.IPNB is similar to obstructive biliary pathology.In this report,we present an unexpected case of asymptomatic IPNB and consolidate our findings with the relevant literature to augment our understanding of this condition.Integrating relevant literature contributes to a more comprehensive understanding of the disease.CASE SUMMARY A 66-year-old Chinese male patient was admitted to our hospital for surgical intervention after gallstones were discovered during a routine physical examination.Preoperative imaging revealed a lesion on the left side of the liver,which raised the suspicion of IPNB.A laparoscopic left hemihepatectomy was performed,and subsequent histopathological examination confirmed the diagnosis of IPNB.At the 3-mo postoperative follow-up,the patient reported good recovery and no metastasis.IPNB can manifest both latently and asymptomatically.Radical surgical resection is the most effective treatment for IPNB.CONCLUSION Hepatic and biliary masses,should be considered to diagnose IPNB.Prompt surgery and vigilant follow-up are crucial in determining prognosis.展开更多
Vanadium-bearing titanomagnetite concentrates were desulfurized with Acidithiobacillus ferrooxidans (A. ferrooxidans). The sulfur content of the concentrates was reduced from 0.69wt% to 0.14wt% after bioleaching for...Vanadium-bearing titanomagnetite concentrates were desulfurized with Acidithiobacillus ferrooxidans (A. ferrooxidans). The sulfur content of the concentrates was reduced from 0.69wt% to 0.14wt% after bioleaching for 15 d with a 10% pulp density at 30℃. Maintaining a stable pH value during biodesulfurization was critical because of high acid consumption, resulting from a combination of nonoxidative and oxidative dissolution of pyrrhotite in acid solution. It is discovered that the citric acid-disodium hydrogen phosphate buffer of pH 2.0 can control the solution pH value smoothly in the optimal range of 2.0-3.0 for A. ferrooxidans growth. Using the buffer in the volume fraction range of 5.0%-15.0% stimulates A. ferooxidans growth and improves the biodesulfurization efficiency. Compared with the buffer-free control case, the maximum increase of biodesulfurization rate is 29.7% using a 10.0vol% buffer. Bioleaching provides an alternative process for desulfurization of vanadium-bearing titanomagnetite ores.展开更多
The Complexation of thiophene with a Lewis acid with moderate acidity as a solvent, such as BF3-ethyl ether (BFEE) remarkedly lowered the electrochemical polymerization potential. The positively charged metal surface ...The Complexation of thiophene with a Lewis acid with moderate acidity as a solvent, such as BF3-ethyl ether (BFEE) remarkedly lowered the electrochemical polymerization potential. The positively charged metal surface of electrode in the process of electrochemical deposition enhanced the coordination interaction between pi-electrons of thiophene unit and the metal, which makes thiophene rings lie parallel to the surface of electrode, resulting in a highly ordered polymeric structure. Because of the large intra-chain transfer integrals, the transport of charge is believed to be principally along the conjugated chains, which is much greater than the inter-chain hopping. The specific electrical resistance across the polythiophene film thickness is more than 10(4) times than that along the surface plane of the film. In this paper we review the recent development of polymerization technique by low potential electrochemical method performed in our lab and several electrical devices in which the compact polythiophene films, such as anionic and cationic sieves, and laminate film junction of undoped polythiophene derivatives were used.展开更多
Traditional triage cannot meet the needs of modern warfare. This paper describes the design of triage and evacuation equipment for casualties at sea that can quickly address mass-casualty triage and store and transmit...Traditional triage cannot meet the needs of modern warfare. This paper describes the design of triage and evacuation equipment for casualties at sea that can quickly address mass-casualty triage and store and transmit information during battlefield treatment and medical evacuation. This equipment consists of a high-capacity medical information card, a simulated patient generator, a triage classifier and a multifunctional airbag triage vest.展开更多
Objective:The objective of this study is to investigate the effect of mild moxibustion on quality of life of patients with type 2 diabetic peripheral neuropathy(DPN).Materials and Methods:Fifty‑five patients with type...Objective:The objective of this study is to investigate the effect of mild moxibustion on quality of life of patients with type 2 diabetic peripheral neuropathy(DPN).Materials and Methods:Fifty‑five patients with type 2 DPN from October 2018 to July 2019 were recruited in the present study and randomly divided into two groups:27 cases in the control group and 28 cases in the intervention group.The control group received routine treatment of hypoglycemic,hypotensive,mecobalamin nutritional nerve,while the intervention group received mild moxibustion on the basis of the control group.The course of treatment for both groups was 10 weeks.The quality of life of the two groups was assessed using short form‑36 health survey and compared with t‑test at 0.05 significance level.Results:The total score of the short form‑36 in the intervention group was higher than the control group(P<0.05);the scores of bodily pain,physical functioning(PF),general health(GH),and mental health dimensions in the intervention group increased significantly compared with the control group(P<0.05);the scores of role physical,social functioning(SF),vitality,and role‑emotional(RE)dimensions showed no between‑group difference(P>0.05).Conclusion:Mild moxibustion can effectively improve the quality of life of patients with type 2 DPN,relieve patient pain symptoms,and improve PF and GH.展开更多
Objective:To assess the efficacy of compound Bai Yu San(CBYS)as a new treatment option,in healing diabetic skin ulcer.Materials and Methods:A total of 64 diabetic patients with skin ulcer were enrolled and randomly as...Objective:To assess the efficacy of compound Bai Yu San(CBYS)as a new treatment option,in healing diabetic skin ulcer.Materials and Methods:A total of 64 diabetic patients with skin ulcer were enrolled and randomly assigned to experimental group(n=33)and control group(n=31).In the control group,normal saline(NS)was used to cleanse the wound.After debridement,the wound was dressed with modern materials.In the experimental group,the NS‑cleansed wound was dressed with CBYS.The infection rate,healing rate,treatment cost,and patient satisfaction between the two groups were compared.Results:On the 35th day after treatment,the infection rate and healing rate showed no between‑group difference(P>0.05);the experimental group showed lower treatment cost and higher satisfaction than the control group(P<0.05).Conclusion:As a new treatment option for diabetes‑induced skin ulcer,CBYS can effectively control the infection,promote the healing,reduce treatment cost,and increase patient satisfaction.Dressing with CBYS can be clinically replicated in the treatment of diabetic skin ulcer.展开更多
Objective:To study the relationship of different respiratory virus infection with pediatric asthma attack as well as cytokine and lymphocyte subset levels.Methods: A total of 85 children who were diagnosed with bronch...Objective:To study the relationship of different respiratory virus infection with pediatric asthma attack as well as cytokine and lymphocyte subset levels.Methods: A total of 85 children who were diagnosed with bronchial asthma in our hospital between May 2013 and March 2016 were selected as asthma group and further divided into asthma-RSV group, asthma-AV group, asthma-PIV group, asthma-IFV group and pure asthma group according to the condition of respiratory virus infection, and 70 healthy children who received physical examination in our hospital during the same period were selected as the control group. Spirometer was used to determine airway function parameters, enzyme-linked immunosorbent assay kits were used to determine serum cytokine contents, and flow cytometry was used to determine peripheral blood lymphocyte subset contents.Results: FEV1/FVC, FEF25, FEF50 and FEF75 levels, serum IL-2, IFN-γ and TGF-β1 contents as well as peripheral blood Th1 and Treg cell contents of asthma groups were significantly lower than those of control group while serum IL-4, IL-5 and IL-17 contents as well as peripheral blood Th2 and Th17 cell contents were significantly higher than those of control group;FEV1/FVC, FEF25, FEF50 and FEF75 levels, serum IL-2, IFN-γ and TGF-β1 contents as well as peripheral blood Th1 and Treg cell contents of asthma-RSV group and asthma-IFV group were significantly lower than those of pure asthma group while serum IL-4, IL-5 and IL-17 contents as well as peripheral blood Th2 and Th17 cell contents were significantly higher than those of pure asthma group;these indexes of asthma-AV group and asthma-PIV group were not significantly different from those of pure asthma group.Conclusion: RSV and IFV infection can affect the airway function and the balance of CD4+T cell subsets to promote the development of asthma.展开更多
Objective:To study the effect of pediatric asthma complicated by respiratory virus infection on airway remodeling and inflammation.Methods:A total of 41 children with asthma complicated by respiratory virus infection ...Objective:To study the effect of pediatric asthma complicated by respiratory virus infection on airway remodeling and inflammation.Methods:A total of 41 children with asthma complicated by respiratory virus infection who were treated in our hospital between May 2012 and March 2016 were collected as observation group, and 50 children with asthma alone who were treated in our hospital during the same period were selected as control group. High-resolution CT was used to determine the right upper lobe apical segment (RB1) and the left lower lobe posterior basal segment (LB10) airway remodeling parameters of two groups of patients, and serum airway remodeling index and inflammatory factor contents were detected. Results: LA, WA and TA levels of RB1 and LB10 of observation group were significantly lower than those of control group, serum airway remodeling indexes TGF-β1, Smad3, PⅠNP and PⅢNP contents were higher than those of control group, serum inflammation indexes IL-4, IL-5 and TNF-α contents were higher than those of control group.Conclusion:Complication of respiratory virus infection can aggravate the airway remodeling and systemic inflammation in children with asthma.展开更多
Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vasculitis featured by liability to renal involvement.However,there are few studies on the risk factors...Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vasculitis featured by liability to renal involvement.However,there are few studies on the risk factors and predictive models for renal outcomes of AAV in children.Methods Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected retrospectively.The risk factors and predictive model of end-stage renal disease(ESRD)in AAV were explored.Results Renal involvement was the most typical manifestation(95.5%),and the crescent was the predominant pathological lesion(84.9%).The estimated glomerular filtration rate(eGFR)was evaluated in 114 patients,of whom 59.6%developed ESRD,and the median time to ESRD was 3.20 months.The eGFR[P=0.006,odds ratio(OR)=0.955,95%confidence interval(CI)=0.924–0.987]and the percentages of global glomerulosclerosis(pGGS;P=0.018,OR=1.060,95%CI=1.010–1.112)were independent risk factors for ESRD of renal biopsy.Based on the pGGS and eGFR at renal biopsy,we developed three risk grades of ESRD and one predictive model.The Kaplan‒Meier curve indicated that renal outcomes were significantly different in different risk grades(P<0.001).Compared with serum creatinine at baseline,the predictive model had higher accuracy(0.86 versus 0.58,P<0.001)and a lower coefficient of variation(0.07 versus 0.92)in external validation.Conclusions Renal involvement is the most common manifestation of pediatric AAV in China,of which more than half deteriorates into ESRD.The predictive model based on eGFR at renal biopsy and the pGGS may be stable and accurate in speculating the risk of ESRD in AAV children.展开更多
N-methyl-D-aspartate receptors(NMDARs), a subtype of glutamate-gated ion channels, play a central role in epileptogenesis. Recent studies have identified an increasing number of GRIN2 A(a gene encoding the NMDAR Gl...N-methyl-D-aspartate receptors(NMDARs), a subtype of glutamate-gated ion channels, play a central role in epileptogenesis. Recent studies have identified an increasing number of GRIN2 A(a gene encoding the NMDAR GluN2A subunit) mutations in patients with epilepsy. Phenotypes of GRIN2 A mutations include epilepsy-aphasia disorders and other epileptic encephalopathies, which pose challenges in clinical treatment. Here we identified a heterozygous GRIN2 A mutation(c.1341 T[A, p.N447 K) from a boy with Rolandic epilepsy by whole-exome sequencing. The patient became seizurefree with a combination of valproate and lamotrigine.Functional investigation was carried out using recombinant NMDARs containing a GluN2A-N447 K mutant that is located in the ligand-binding domain of the GluN2A subunit. Whole-cell current recordings in HEK 293 T cells revealed that the N447 K mutation increased the NMDAR current density by;.2-fold, enhanced the glutamate potency by 2-fold, and reduced the sensitivity to Mg;inhibition. These results indicated that N447 K is a gain-offunction mutation. Interestingly, alternative substitutions by alanine and glutamic acid at the same residue(N447 A and N447 E) did not change NMDAR function, suggesting a residual dependence of this mutation in altering NMDAR function. Taken together, this study identified human GluN2A N447 K as a novel mutation associated with epilepsy and validated its functional consequences in vitro.Identification of this mutation is also helpful for advancing our understanding of the role of NMDARs in epilepsy and provides new insights for precision therapeutics in epilepsy.展开更多
Background:The present study was undertaken to investigate the clinical characteristics of hemolytic uremic syndrome(HUS)secondary to cobalamin C disorder(cbl-C disorder).Methods:We reviewed retrospectively the medica...Background:The present study was undertaken to investigate the clinical characteristics of hemolytic uremic syndrome(HUS)secondary to cobalamin C disorder(cbl-C disorder).Methods:We reviewed retrospectively the medical records of 3 children with HUS secondary to cbl-C disorder who had been treated between April 1,2009 and October 31,2013.Results:The 3 patients with HUS secondary to cbl-C disorder presented with progressive hemolytic anemia,acute renal failure,thrombocytopenia,poor feeding,and failure to thrive.Two of the 3 patients once had high blood pressure.The mutations of c.609G>A(P.W203X),c.217C>T(p.R73X)and c.365A>T(p.H122L)in the methylmalonic aciduria(cobalamin deficiency)cbl-C type,with homocystinuria gene were detected in the 3 patients.In these patients the levels of lactate dehydrogenase and homocysteine in serum were elevated and the level of methylmalonic acid(MMA)in urine was also elevated.After treatment with hydroxocobalamin,2 patients were discharged with no obvious abnormal growth and neurological development and 1 patient died of multiple organ failure.Conclusions:The results of this study demonstrated that cbl-C disorder should be investigated in any child presenting with HUS.The high concentrations of homocysteine and MMA could be used for timely recognization of the disease.Once the high levels of plasma homocystein and/or plasma or urine MMA are detected,the treatment with parenteral hydroxocobalamin should be prescribed immediately.The early diagnosis and treatment would contribute to the good prognosis of the disease.展开更多
CoCrFeNiNb_(0.25)hypoeutectic high-entropy alloy(HEA)has shown good mechanical properties.However,due to interdendritic eutectics,it is challenging to further optimize the microstructure through cold rolling and annea...CoCrFeNiNb_(0.25)hypoeutectic high-entropy alloy(HEA)has shown good mechanical properties.However,due to interdendritic eutectics,it is challenging to further optimize the microstructure through cold rolling and annealing.The current study showed that hot defor-mation effectively tailored the hypoeutectic microstructure.We investigated the hot deformation behavior and microstructure evolution of the dual-phase CoCrFe-NiNb_(0.25) HEA at different temperatures from 800 to 1000℃and different strain rates from 0.01 to 10.00 s^(-1).The systematic investigation showed the deformation activation energy around 500 kJ·mol^(-1).Microstructure with refined grains and uniformly distributed Laves phases can be obtained after 70%compression at 900℃with a strain rate of 0.01 s^(-1).展开更多
基金Zhejiang Provincial Natural Science Foundation of China Under Grant,No.LY21H160046.
文摘BACKGROUND Intraductal papillary neoplasms of the bile duct(IPNBs)are rare and characterized by papillary growth within the bile duct lumen.IPNB is similar to obstructive biliary pathology.In this report,we present an unexpected case of asymptomatic IPNB and consolidate our findings with the relevant literature to augment our understanding of this condition.Integrating relevant literature contributes to a more comprehensive understanding of the disease.CASE SUMMARY A 66-year-old Chinese male patient was admitted to our hospital for surgical intervention after gallstones were discovered during a routine physical examination.Preoperative imaging revealed a lesion on the left side of the liver,which raised the suspicion of IPNB.A laparoscopic left hemihepatectomy was performed,and subsequent histopathological examination confirmed the diagnosis of IPNB.At the 3-mo postoperative follow-up,the patient reported good recovery and no metastasis.IPNB can manifest both latently and asymptomatically.Radical surgical resection is the most effective treatment for IPNB.CONCLUSION Hepatic and biliary masses,should be considered to diagnose IPNB.Prompt surgery and vigilant follow-up are crucial in determining prognosis.
基金financially supported by the National Natural Science Foundation of China(No.51074107)Shanghai Municipal Education Commission(Shanghai Leading Academic Discipline Project J51504)
文摘Vanadium-bearing titanomagnetite concentrates were desulfurized with Acidithiobacillus ferrooxidans (A. ferrooxidans). The sulfur content of the concentrates was reduced from 0.69wt% to 0.14wt% after bioleaching for 15 d with a 10% pulp density at 30℃. Maintaining a stable pH value during biodesulfurization was critical because of high acid consumption, resulting from a combination of nonoxidative and oxidative dissolution of pyrrhotite in acid solution. It is discovered that the citric acid-disodium hydrogen phosphate buffer of pH 2.0 can control the solution pH value smoothly in the optimal range of 2.0-3.0 for A. ferrooxidans growth. Using the buffer in the volume fraction range of 5.0%-15.0% stimulates A. ferooxidans growth and improves the biodesulfurization efficiency. Compared with the buffer-free control case, the maximum increase of biodesulfurization rate is 29.7% using a 10.0vol% buffer. Bioleaching provides an alternative process for desulfurization of vanadium-bearing titanomagnetite ores.
文摘The Complexation of thiophene with a Lewis acid with moderate acidity as a solvent, such as BF3-ethyl ether (BFEE) remarkedly lowered the electrochemical polymerization potential. The positively charged metal surface of electrode in the process of electrochemical deposition enhanced the coordination interaction between pi-electrons of thiophene unit and the metal, which makes thiophene rings lie parallel to the surface of electrode, resulting in a highly ordered polymeric structure. Because of the large intra-chain transfer integrals, the transport of charge is believed to be principally along the conjugated chains, which is much greater than the inter-chain hopping. The specific electrical resistance across the polythiophene film thickness is more than 10(4) times than that along the surface plane of the film. In this paper we review the recent development of polymerization technique by low potential electrochemical method performed in our lab and several electrical devices in which the compact polythiophene films, such as anionic and cationic sieves, and laminate film junction of undoped polythiophene derivatives were used.
基金the funding from the Military Program during the 12th Five-year Plan Period,Proposal No.2011YY027
文摘Traditional triage cannot meet the needs of modern warfare. This paper describes the design of triage and evacuation equipment for casualties at sea that can quickly address mass-casualty triage and store and transmit information during battlefield treatment and medical evacuation. This equipment consists of a high-capacity medical information card, a simulated patient generator, a triage classifier and a multifunctional airbag triage vest.
基金This study was support by Jiangsu Administration of Traditional Chinese Medicine:National TCM clinical research base open topic(JD:201711)。
文摘Objective:The objective of this study is to investigate the effect of mild moxibustion on quality of life of patients with type 2 diabetic peripheral neuropathy(DPN).Materials and Methods:Fifty‑five patients with type 2 DPN from October 2018 to July 2019 were recruited in the present study and randomly divided into two groups:27 cases in the control group and 28 cases in the intervention group.The control group received routine treatment of hypoglycemic,hypotensive,mecobalamin nutritional nerve,while the intervention group received mild moxibustion on the basis of the control group.The course of treatment for both groups was 10 weeks.The quality of life of the two groups was assessed using short form‑36 health survey and compared with t‑test at 0.05 significance level.Results:The total score of the short form‑36 in the intervention group was higher than the control group(P<0.05);the scores of bodily pain,physical functioning(PF),general health(GH),and mental health dimensions in the intervention group increased significantly compared with the control group(P<0.05);the scores of role physical,social functioning(SF),vitality,and role‑emotional(RE)dimensions showed no between‑group difference(P>0.05).Conclusion:Mild moxibustion can effectively improve the quality of life of patients with type 2 DPN,relieve patient pain symptoms,and improve PF and GH.
基金Jiangsu TCM Bureau Project(YB2015043)Jiangsu Science and Technology Department Project(BK20161606).
文摘Objective:To assess the efficacy of compound Bai Yu San(CBYS)as a new treatment option,in healing diabetic skin ulcer.Materials and Methods:A total of 64 diabetic patients with skin ulcer were enrolled and randomly assigned to experimental group(n=33)and control group(n=31).In the control group,normal saline(NS)was used to cleanse the wound.After debridement,the wound was dressed with modern materials.In the experimental group,the NS‑cleansed wound was dressed with CBYS.The infection rate,healing rate,treatment cost,and patient satisfaction between the two groups were compared.Results:On the 35th day after treatment,the infection rate and healing rate showed no between‑group difference(P>0.05);the experimental group showed lower treatment cost and higher satisfaction than the control group(P<0.05).Conclusion:As a new treatment option for diabetes‑induced skin ulcer,CBYS can effectively control the infection,promote the healing,reduce treatment cost,and increase patient satisfaction.Dressing with CBYS can be clinically replicated in the treatment of diabetic skin ulcer.
文摘Objective:To study the relationship of different respiratory virus infection with pediatric asthma attack as well as cytokine and lymphocyte subset levels.Methods: A total of 85 children who were diagnosed with bronchial asthma in our hospital between May 2013 and March 2016 were selected as asthma group and further divided into asthma-RSV group, asthma-AV group, asthma-PIV group, asthma-IFV group and pure asthma group according to the condition of respiratory virus infection, and 70 healthy children who received physical examination in our hospital during the same period were selected as the control group. Spirometer was used to determine airway function parameters, enzyme-linked immunosorbent assay kits were used to determine serum cytokine contents, and flow cytometry was used to determine peripheral blood lymphocyte subset contents.Results: FEV1/FVC, FEF25, FEF50 and FEF75 levels, serum IL-2, IFN-γ and TGF-β1 contents as well as peripheral blood Th1 and Treg cell contents of asthma groups were significantly lower than those of control group while serum IL-4, IL-5 and IL-17 contents as well as peripheral blood Th2 and Th17 cell contents were significantly higher than those of control group;FEV1/FVC, FEF25, FEF50 and FEF75 levels, serum IL-2, IFN-γ and TGF-β1 contents as well as peripheral blood Th1 and Treg cell contents of asthma-RSV group and asthma-IFV group were significantly lower than those of pure asthma group while serum IL-4, IL-5 and IL-17 contents as well as peripheral blood Th2 and Th17 cell contents were significantly higher than those of pure asthma group;these indexes of asthma-AV group and asthma-PIV group were not significantly different from those of pure asthma group.Conclusion: RSV and IFV infection can affect the airway function and the balance of CD4+T cell subsets to promote the development of asthma.
文摘Objective:To study the effect of pediatric asthma complicated by respiratory virus infection on airway remodeling and inflammation.Methods:A total of 41 children with asthma complicated by respiratory virus infection who were treated in our hospital between May 2012 and March 2016 were collected as observation group, and 50 children with asthma alone who were treated in our hospital during the same period were selected as control group. High-resolution CT was used to determine the right upper lobe apical segment (RB1) and the left lower lobe posterior basal segment (LB10) airway remodeling parameters of two groups of patients, and serum airway remodeling index and inflammatory factor contents were detected. Results: LA, WA and TA levels of RB1 and LB10 of observation group were significantly lower than those of control group, serum airway remodeling indexes TGF-β1, Smad3, PⅠNP and PⅢNP contents were higher than those of control group, serum inflammation indexes IL-4, IL-5 and TNF-α contents were higher than those of control group.Conclusion:Complication of respiratory virus infection can aggravate the airway remodeling and systemic inflammation in children with asthma.
基金approved by the Ethics Committee of the Children’s Hospital of Chongqing Medical University(approval Number:149/2022)other enrolled centers.This study was registered at the Chinese Clinical Trial Registry(registered number:ChiCTR2000034203).
文摘Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vasculitis featured by liability to renal involvement.However,there are few studies on the risk factors and predictive models for renal outcomes of AAV in children.Methods Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected retrospectively.The risk factors and predictive model of end-stage renal disease(ESRD)in AAV were explored.Results Renal involvement was the most typical manifestation(95.5%),and the crescent was the predominant pathological lesion(84.9%).The estimated glomerular filtration rate(eGFR)was evaluated in 114 patients,of whom 59.6%developed ESRD,and the median time to ESRD was 3.20 months.The eGFR[P=0.006,odds ratio(OR)=0.955,95%confidence interval(CI)=0.924–0.987]and the percentages of global glomerulosclerosis(pGGS;P=0.018,OR=1.060,95%CI=1.010–1.112)were independent risk factors for ESRD of renal biopsy.Based on the pGGS and eGFR at renal biopsy,we developed three risk grades of ESRD and one predictive model.The Kaplan‒Meier curve indicated that renal outcomes were significantly different in different risk grades(P<0.001).Compared with serum creatinine at baseline,the predictive model had higher accuracy(0.86 versus 0.58,P<0.001)and a lower coefficient of variation(0.07 versus 0.92)in external validation.Conclusions Renal involvement is the most common manifestation of pediatric AAV in China,of which more than half deteriorates into ESRD.The predictive model based on eGFR at renal biopsy and the pGGS may be stable and accurate in speculating the risk of ESRD in AAV children.
基金supported by the grants of the National Natural Science Foundation of China(81671162,81521062,81561168,and 81571273)the National Basic Research Development Program of China(2014CB910300 and 2013CB530904)Key Research Project of the Ministry of Science and Technology of China(2016YFC0904400)
文摘N-methyl-D-aspartate receptors(NMDARs), a subtype of glutamate-gated ion channels, play a central role in epileptogenesis. Recent studies have identified an increasing number of GRIN2 A(a gene encoding the NMDAR GluN2A subunit) mutations in patients with epilepsy. Phenotypes of GRIN2 A mutations include epilepsy-aphasia disorders and other epileptic encephalopathies, which pose challenges in clinical treatment. Here we identified a heterozygous GRIN2 A mutation(c.1341 T[A, p.N447 K) from a boy with Rolandic epilepsy by whole-exome sequencing. The patient became seizurefree with a combination of valproate and lamotrigine.Functional investigation was carried out using recombinant NMDARs containing a GluN2A-N447 K mutant that is located in the ligand-binding domain of the GluN2A subunit. Whole-cell current recordings in HEK 293 T cells revealed that the N447 K mutation increased the NMDAR current density by;.2-fold, enhanced the glutamate potency by 2-fold, and reduced the sensitivity to Mg;inhibition. These results indicated that N447 K is a gain-offunction mutation. Interestingly, alternative substitutions by alanine and glutamic acid at the same residue(N447 A and N447 E) did not change NMDAR function, suggesting a residual dependence of this mutation in altering NMDAR function. Taken together, this study identified human GluN2A N447 K as a novel mutation associated with epilepsy and validated its functional consequences in vitro.Identification of this mutation is also helpful for advancing our understanding of the role of NMDARs in epilepsy and provides new insights for precision therapeutics in epilepsy.
基金supported by a grant from the Special Program for Clinical Research of the Beijing Municipal Commission of Science and Technology and WU JIE PING Medical Foundation,China(Grant Z121107005112008)
文摘Background:The present study was undertaken to investigate the clinical characteristics of hemolytic uremic syndrome(HUS)secondary to cobalamin C disorder(cbl-C disorder).Methods:We reviewed retrospectively the medical records of 3 children with HUS secondary to cbl-C disorder who had been treated between April 1,2009 and October 31,2013.Results:The 3 patients with HUS secondary to cbl-C disorder presented with progressive hemolytic anemia,acute renal failure,thrombocytopenia,poor feeding,and failure to thrive.Two of the 3 patients once had high blood pressure.The mutations of c.609G>A(P.W203X),c.217C>T(p.R73X)and c.365A>T(p.H122L)in the methylmalonic aciduria(cobalamin deficiency)cbl-C type,with homocystinuria gene were detected in the 3 patients.In these patients the levels of lactate dehydrogenase and homocysteine in serum were elevated and the level of methylmalonic acid(MMA)in urine was also elevated.After treatment with hydroxocobalamin,2 patients were discharged with no obvious abnormal growth and neurological development and 1 patient died of multiple organ failure.Conclusions:The results of this study demonstrated that cbl-C disorder should be investigated in any child presenting with HUS.The high concentrations of homocysteine and MMA could be used for timely recognization of the disease.Once the high levels of plasma homocystein and/or plasma or urine MMA are detected,the treatment with parenteral hydroxocobalamin should be prescribed immediately.The early diagnosis and treatment would contribute to the good prognosis of the disease.
基金financially supported by the National Key R&D Program of China (No.2018YFC0310400)the Research Fund of the State Key Laboratory of Solidification Processing,China (Nos.2020-TS-06 and 2021-TS-02)
文摘CoCrFeNiNb_(0.25)hypoeutectic high-entropy alloy(HEA)has shown good mechanical properties.However,due to interdendritic eutectics,it is challenging to further optimize the microstructure through cold rolling and annealing.The current study showed that hot defor-mation effectively tailored the hypoeutectic microstructure.We investigated the hot deformation behavior and microstructure evolution of the dual-phase CoCrFe-NiNb_(0.25) HEA at different temperatures from 800 to 1000℃and different strain rates from 0.01 to 10.00 s^(-1).The systematic investigation showed the deformation activation energy around 500 kJ·mol^(-1).Microstructure with refined grains and uniformly distributed Laves phases can be obtained after 70%compression at 900℃with a strain rate of 0.01 s^(-1).