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Clinical and genetic features of Kenny-Caffey syndrome type 2 with multiple electrolyte disturbances:A case report

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World Journal of Clinical Cases 2023年第10期11卷 2290-2300页

作者： Ning Yuan Lin Lu Xiao-Ping Xing Ou Wang Yue Jiang Ji Wu ming-hai he Xiao-Juan Wang Le-Wei Cao Department of Endocrinology Nanchong Central HospitalThe Second Clinical CollegeNorth Sichuan Medical CollegeNanchong 637000Sichuan ProvinceChina Department of Endocrinology Key Laboratory of National health commissionPeking Union Medical College HospitalPeking Union Medical CollegeChinese Academy of Medical ScienceBeijing 100730China Department of Urology Nanchong Central HospitalThe Second Clinical CollegeNorth Sichuan Medical CollegeNanchong 637000Sichuan ProvinceChina

BACKGROUND Hypoparathyroidism,which can be sporadic or a component of an inherited syndrome,is the most common cause of *** hypocalcemia is accompanied by other electrolyte disturbances,such as hypokalemia and hypomagnesemia,then the cause,such as renal tubular disease,should be carefully *** SUMMARY An 18-year-old female visited our clinic because of short stature and facial deformities,including typical phenotypes,such as low ear position,depression of the nasal bridge,small hands and feet,and loss of *** lab results suggested normal parathyroid hormone but *** addition,multiple electrolyte disturbances were found,including hypokalemia,hypocalcemia and *** physical signs showed a short fourth metatarsal bone of both *** X-ray images showed cortical thickening of long bones and narrowing of the medulla of the *** computed tomography indicated calcification in the bilateral basal ***,the genetic investigation showed a de novo heterogenous mutation of“FAM111A”(c.G1706A:p.R569H).Through a review of previously reported cases,the mutation was found to be the most common mutation site in Kenny-Caffey syndrome type 2(KCS2)cases reported thus far(16/23,69.6%).The mutation was slightly more prevalent in females than in males(11/16,68.8%).Except for hypocalcemia,other clinical manifestations are *** As a rare autosomal dominant genetic disease of hypoparathyroidism,the clinical manifestations of KCS2 are atypical and *** girl presented with short stature,facial deformities and skeletal *** laboratory results revealed hypocalcemia as the main electrolyte *** though her family members showed normal phenotypes,gene detection was performed to find the mutation of the FAM111A gene and confirmed the diagnosis of KCS2.

关键词： Hypocalcemia Hypomagnesemia Hypoparathyroidism Kenny-Caffey syndrome type 2 FAM111A gene Case report

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果蔗品种比较试验初报

福建农业科技 2017年第2期48卷 3-4页

作者：李海明李和平张树河福建省农业科学院亚热带农业研究所 363005

以闽引黄皮果蔗、龙黑果蔗为试验材料,黑皮果蔗和大田雪蔗为对照品种,在三明市大田县进行品种比较试验,结果表明:闽引黄皮果蔗、龙黑果蔗均表现较好的综合性状,适应性强;闽引黄皮果蔗每667m2产量9715kg,与对照大田雪蔗相当,比对照黑皮... 详细信息

以闽引黄皮果蔗、龙黑果蔗为试验材料,黑皮果蔗和大田雪蔗为对照品种,在三明市大田县进行品种比较试验,结果表明:闽引黄皮果蔗、龙黑果蔗均表现较好的综合性状,适应性强;闽引黄皮果蔗每667m2产量9715kg,与对照大田雪蔗相当,比对照黑皮果蔗增产达极显著水平;龙黑果蔗蔗糖分含量15.90%,比两对照增糖效果达极显著水平;试验期间,闽引黄皮果蔗、龙黑果蔗均未发现病虫害发生。闽引黄皮果蔗综合表现更好,可进一步推广种植;龙黑果蔗可进一步试验。

关键词：果蔗新品种品比试验

Theoretical Study on the Intermolecular Interactions of Tetrazole Dimers

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Chinese Journal of Structural Chemistry 2005年第10期24卷 1203-1210页

作者： ZHOU Su-Qin JU Xue-hai XIAO he-ming Department of Chemistry Nanjing University of Science and Technology Nanjing 210094 China

Tetrazole monomers （Ⅰ, Ⅱ） and all of their possible stable dimers （1, 2, 3, 4, 5, 6, 7 and 8） were fully optimized by DFT method at the B3LYP/6-311＋＋G^＊＊ level. Among the eight dimers, there were two 1H-tetrazole dimers, three 2H-tetrazole dimers and three hetero dimers of 1H-tetrazole and 2H-tetrazole. Vibrational frequencies were calculated to ascertain that each structure was stable （no imaginary frequencies）. The basis set superposition errors （BSSE） are 2.78, 2.28, 2.97, 2.75, 2.74, 2.18, 1.23 and 3.10 kJ/mol, and the zero point energy （ZPE） corrections for the interaction energies are 4.88, 4.18, 3.87, 3.65, 3.54, 3.22, 2.87 and 4.34 kJ/mol for 1, 2, 3, 4, 5, 6, 7 and 8, respectively. After BSSE and ZPE corrections, the greatest corrected intermolecular interaction energy of the dimers is -43.71 kJ/mol. The charge redistribution mainly occurs on the adjacent N-H…N atoms between submolecules. The charge transfer between two subsystems is very small. Natural bond orbital （NBO） analysis was performed to reveal the origin of the interaction. Based on the statistical thermodynamic method, the standard thermodynamic functions, heat capacities （C^0P）, entropies （S^0T） and thermal corrections to enthalpy （H^0T）, and the changes of thermodynamic properties from monomer to dimer in the temperature range of 200.00 K to 700 K have been obtained. 1H-tetrazole monomer can spontaneously turn into two stable dimers at 298.15 K.

关键词： tetrazole dimer intermolecular interaction DFT natural bond orbital,thermodynamic properties

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眼斑猛虾蛄幼体的发育

水产学报 2006年第1期30卷 42-49页

作者：郑雅友李正良杨章武郑养福蔡英明福建省水产研究所福建省水产研究所福建厦门361012

通过对眼斑猛虾蛄(Harpiosquillaannandalei)幼体各发育期的连续取样,在显微镜或解剖镜下系统地观察各发育期的形态变化及生长特征并描图。眼斑猛虾蛄假蚤状幼体经11次蜕皮,在水温24～27℃,盐度25.4～29.0的条件下,历时49d,发育变态成... 详细信息

通过对眼斑猛虾蛄(Harpiosquillaannandalei)幼体各发育期的连续取样,在显微镜或解剖镜下系统地观察各发育期的形态变化及生长特征并描图。眼斑猛虾蛄假蚤状幼体经11次蜕皮,在水温24～27℃,盐度25.4～29.0的条件下,历时49d,发育变态成仔虾蛄;第Ⅰ期假蚤状幼体(Z1)体长1.75～1.83mm,头部有一长额角,头胸甲形状似龟壳,有1对腹侧齿,四角有刺,后部中间有一较长棘刺;第Ⅵ期头胸甲形状渐变成梯形,腹侧齿5对,第一触角中鞭萌芽,第二触角内肢发育呈短芽状,第Ⅷ期头胸甲左右两侧边缘开始渐向下弯卷;第Ⅺ期(Z11)体长15.10～17.20mm,第二触角内肢发育成鞭状且分为14～16节,尾肢外肢2节,内肢1节;Ⅰ期仔虾蛄体长14.50～16.10mm,长额角消失,头胸头形状仍为梯形,但光滑无腹侧齿,前侧角圆形,后侧角有二个缺角,这是该种的特征,后部中间棘刺消失,头胸甲占体长的比例明显缩小,形似成体虾蛄。眼斑猛虾蛄的幼体与黑斑口虾蛄的幼体的形态存在一定差异,主要表现在头胸甲与体长的比例,头胸甲的长宽比及第二触鳞片刚毛数和尾节的侧小齿、中间小齿和亚中间小齿的齿数。

关键词：眼斑猛虾姑假蚤状幼体形态发育

Theoretical Studies on the Structure and Spectrum of Imidazole-Chloranil Charge Transfer Complex

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Chinese Journal of Chemical Physics 2008年第6期21卷 560-568页

作者： hai-long Wang Tong-tong Lu Tian-jing he Dong-ming Chen Department of Chemical Physics University oF Science and Technology of China Hefei 230026 China

UV-Vis absorption spectra of the molecular complex formed by imidazole （Ira） and chloranil （CA） were measured in chloroform. The stoichiometry of the imidazole-chloranil （hn-CA） complex was determined as 1：1 by applying Benesi-Hildebrand＇s equation and Job＇s continuous variation method. Density function theory （DFT） and MP2 calculations were performed to study the structures and the binding energies of the Im-CA complex. The calculations located four conformations （denoted as S1-S4） for the Ira-CA complex, two edge（Im）-to-face（CA） linked and two edge（Im）-to-edge（CA） linked. It was found that the edge-to-face conformers are more stable than the edge-to-edge ones. The bonding characteristics of these conformers were investigated with natural population analysis （NPA）, topological analysis of electron density, and natural bond orbital （NBO） analysis. It was revealed that the edge-to-face conformers are charge-transfer （CT） complexes whereas the edge-to-edge conformers are the hydrogen bond complexes. For the most stable conformation of the Ira-CA complex （S1）, the charge transfer interaction of the imidazole n（N15） lone pair orbital with the chloranil π＊（C1=O7） orbital plays a crucial role in the Ira-CA binding, and the binding is further strengthened by the O7… H2O hydrogen bond. The electronic excitation energies of the complex （S1） were calculated with time-dependent DFT （TDDFT）, and the observed UV-Visible spectrum of the complex was analyzed based on the computed results.

关键词： Charge-transfer complex Density functional theory MP2 Imidazole-chloranil

Theoretical Study on Intermolecular Interactions and Thermodynamic Properties of Dimethylnitroamine Clusters

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Chinese Journal of Chemistry 2002年第3期20卷 629-637页

作者： JU，Xue－hai XIAO，he－ming 等 DepartmentofChemistry NanjingUniversityofScienceandTechnologyNanjing210094China

Ab initio SCF and Moller-Plesset correlation correction methods incombination with counterpoise procedure for BSSE correction have been applied to the theroeticalstudying of dimethylnitroamine and its dimers and trimers. Three optimized stable dimers and twotrimers have been obtained. The corrected binding energies of the most stable dimer and trimer werepredicted to be - 24.68 kJ/mol and - 47.27 kJ/mol, respectively at the MP2/6-31G ~*//HF/6-31G~*level. The proportion of correlated interaction energies to their total interaction energies for allclusters was at least 29.3 percent, and the BSSE of ΔE(MP2) was at least 10.0 kJ/mol. Dispersionand/ or electrostatic force were dominant in all clusters. There exist cooperative effects in boththe chain and the cyclic trimers. The vibrational frequencies associated with N―O stretches or wagsexhibit slight red shifts, but the modes associated with the motion of hydrogen atoms of the methylgroup show somewhat blue shifts with respect to those of monomer. Thermodynamic properties ofdimethylnitroamine and its clusters at different temperatures have been calculated on the basis ofvibrational analyses. The changes of the Gibbs free energies for the aggregation from monomer to themost stable dimer and trimer were predicted to be 14.37 kJ/mol and 30.40 kJ/mol, respectively, at 1atm and 298.15 K.

关键词： dimethylnitroamine cluster intermolecular interaction ah initio thermodynamic property

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Hierarchical Zn_(3)V_(2)O_(8)microspheres interconnected via conductive carbon nanotubes as promising anode materials for lithium-ion battery applications

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Rare Metals 2023年第8期42卷 2601-2611页

作者： ming-ming Liu hai-Tao Yu Lang Yuan Ting-Feng Yi Fei he Ying Xie Key Laboratory of Functional Inorganic Material Chemistry Ministry of EducationSchool of Chemistry and Materials ScienceHeilongjiang UniversityHarbin 150080China School of Materials Science and Engineering Northeastern UniversityShenyang 110819China Key Laboratory of Superlight Materials and Surface Technology Ministry of EducationCollege of Materials Science and Chemical EngineeringHarbin Engineering UniversityHarbin 150001China

Zn_(3)V_(2)O_(8) was considered as a promising anode material for lithium-ion battery(LIB),because of its high theoretical specific capacity,environmental friendliness,and ease of ***,the large volume change and low electronic conductivity of Zn_(3)V_(2)O_(8)in repeated charge/discharge cycles have severely limited its *** solve the above issues,hierarchical Zn_(3)V_(2)O_(8) microspheres assembled by two-dimensional(2D)nanosheets were successfully synthesized,and carbon nanotubes(CNTs)were further introduced to cross-link the Zn_(3)V_(2)O_(8) *** interconnected nature of the three-dimensional(3D)conducting network and the special hierarchical morphology were beneficial for improving the stability and conductivity of the composite,leading to the reduction of the impedance and a significant improvement of the electrochemical *** reversible capacity of the as-prepared composite can achieve 1049.5mAh·g^(-1)at a current density of 0.2 A·g^(-1)with a capacity retention of~81%after 100 *** is suggested that morphology modulation coupled with interconnecting CNT network is an effective method to boost the electrochemical performance of the anode materials for lithium-ion batteries.

关键词： Anode material Zn_(3)V_(2)O_(8) Carbon nanotube(CNT) Electrochemical performance Lithium-ion battery(LIB)

Theoretical Studies on the Thermodynamic Properties and Detonation Performances of Bicyclic Nitramines:TNAD Isomers

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Chinese Journal of Structural Chemistry 2006年第11期25卷 1309-1320页

作者： QIU Ling XIAO he-ming GONG Xue-Dong JU Xue-hai Institute for Computation in Molecular and Material Science School of Chemical Engineering Nanjing University of Science and Technology Nanjing 210094 China

Ab initio molecular orbital calculations have been performed at Hartree-Fock （HF）, second-order Mrller-Plesset （MP2）, and hybrid density functional theory （DFT） B3LYP levels with 6-31G^＊＊ basis set to investigate the low sensitive explosive trans-1,4,5,8-tetranitro-1,4,5,8- tetraazadecalin （TNAD） and its seven bicyclic isomers. Their molecular geometries, electronic structures, thermodynamic properties, and detonation performances were predicted and compared. The relationships between structures and various properties were discussed in detail. The calculated results agree well with the available experimental data, and suggest that some compounds may be novel potential candidates of high energy density materials （heDMs） with performances better than TNT （2,4,6-trinitrotoluene） and similar to RDX （1,3,5-trinitro-1,3,5-triazacyclohexane）.

关键词： bicyclic nitramine Hartree-Fock method MP2 method density functional theory thermodynamic properties detonation performances

Analytical model for straight hemming based on minimum energy method

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Journal of Zhejiang University-Science A(Applied Physics & Engineering) 2011年第7期12卷 532-542页

作者： Qian WANG Xiang-huai DONG he-zong LI hai-ming ZHANG National Engineering Research Center of Die & Mold CAD Shanghai Jiao Tong University Shanghai 200030 China

An analytical model for straight hemming was developed based on minimum energy method to study the effect of flanging die corner radius on hemming *** order to calculate plastic strain and strain energy more exactly,the neutral layer of specimen corner after hemming is assumed to be a half ellipse with its major semi-axis *** hardening rule is adopted to describe bending and reverse bending processes neglecting Bauschinger *** model takes into account the material property parameters in order to satisfy a wide application range of different *** profile,creepage/growing(roll-in/roll-out) and maximum equivalent strain are predicted,which are greatly influenced by the flanging die corner *** facilities were designed and hemming experiments were *** predicted results of the present analytical model were compared to experimental data as well as finite element(FE) simulation *** was confirmed that they are in good agreement,and the model can be used to evaluate whether the material used as an outer panel for hemming is appropriate and to optimize process parameters when the material used for hemming is changed.

关键词： Analytical model Minimum energy method Defects Large deformation hemming