Optimal operation of Internet Data Center with PV and energy storage type of UPS clusters

With the development of green data centers,a large number of Uninterruptible Power Supply(UPS)resources in Internet Data Center(IDC)are becoming idle assets owing to their low utilization rate.The revitalization of these idle UPS resources is an urgent problem that must be addressed.Based on the energy storage type of the UPS(EUPS)and using renewable sources,a solution for IDCs is proposed in this study.Subsequently,an EUPS cluster classification method based on the concept of shared mechanism niche(CSMN)was proposed to effectively solve the EUPS control problem.Accordingly,the classified EUPS aggregation unit was used to determine the optimal operation of the IDC.An IDC cost minimization optimization model was established,and the Quantum Particle Swarm Optimization(QPSO)algorithm was adopted.Finally,the economy and effectiveness of the three-tier optimization framework and model were verified through three case studies.

Research of heat treatment of low-Co AB_5 type hydrogen storage alloys for MH-Ni batteries

The effects of low-Co AB_5 type hydrogen storage alloys prepared by quenchingand annealing on the performances of MH-Ni batteries were investigated, and the characteristics ofthe low-Co AB_5 type hydrogen storage alloys were compared with those of the high-Co AB_5 typehydrogen storage alloy as well. The results showed that the faster the cooling of the low-Cohydrogen storage alloy is, the better homogeneity of the chemical composition for the alloy and thelonger cycle life of the battery are, but the electrochemical discharge capacity and high-ratedischarge ability are reduced. The high-rate discharge ability and charge retention of MH-Nibatteries for the conventional as-cast annealed low-Co hydrogen storage alloy were superior to thosefor the rapidly quenched low-Co hydrogen storage alloy and the high-Co hydrogen storage alloy, buta little inferior in the cycle life.

Double filtration plasmapheresis for pregnancy with hyperlipidemia in glycogen storage disease type Ia:A case report

BACKGROUND Glycogen storage disease type Ia(GSDIa) is an autosomal recessive inborn error of carbohydrate metabolism that is caused by deficiency of the enzyme glucose-6-phosphatase(G6Pase),leading to disturbed glycogenolysis and gluconeogenesis.Patients with GSDIa show severe fasting hypoglycemia,hyperlipidemia,hyperlactacidemia,and hyperuricemia,which are associated with fatal outcomes in pregnant women and fetuses.CASE SUMMARY Herein,we report the case of a 24-year-old female who on her first visit to the hospital,presented with pregnancy combined with extremely high hyperlipidemia and hyperlactic acidosis with anemia,and frequent hypoglycemia occurred during the treatment.Genetic tests revealed a mutation in the G6Pase gene(G6PC) at 17q21,the patient was finally diagnosed with glycogen storage disease type Ia for the first time after 22 years of inaccurate treatment.She has been treated with a continuous double filtration plasmapheresis(DFPP) strategy to remove blood lipids,and a cornstarch diet therapy.The patient did not develop pancreatitis during the course of the disease and a healthy baby girl weighing 3 kg was delivered.CONCLUSION Patients with GSDIa may be misdiagnosed as epilepsy.DFPP can be used to control hyperlipidemia in GSDIa patients during pregnancy.

Electrochemical Properties of Strip Casting LPCNi_(3.55) Co_(0.75) Mn_(0.4) Al_(0.3) Hydrogen Storage Alloys

LPCNi 3.55 Co 0.75 Mn 0.4 Al 0.3 hydrogen storage alloy was investigated, and the effects of thickness of its strip casting ingots(as cast) on the electrochemical performances were discussed. It was found that the 0.2 C discharge capacity increased with the increase of the thickness (from 1 mm to 10 mm) of the ingots, mainly due to the enlargement of the unit cell volume; Among the thickness of the ingots in our study, 10 mm sample showed a better activation property; LPCNi 3.55 Co 0.75 Mn 0.4 Al 0.3 alloy with 10mm showed higher comprehensive properties than those with other thickness under 1C rate.

Structure and electrochemical performance of hydrogen storage alloy La_(0.7)Mg_(0.3)Ni_(2.875)Co_(0.525)Mn_(0.1)-boron composite

The structure and electrochemical characteristics of La_(0.7)Mg_(0.3)Ni_(2.875)Co_(0.525)Mn_(0.1)-boron composite was studied systematically.The AB_(3) type hydrogen storage alloys La_(0.7)Mg_(0.3)Ni_(2.875)Co_(0.525)Mn_(0.1) were successfully synthesized by means of inter-media alloy La_(2)Mg_(17).The alloys were composited with boron at different weight rate.From the XRD analyses,each alloy of this series is mainly composed of(La,Mg)Ni_(3) phase and the LaNi_(5) phase,and the phase abundance of each phase varies with the boron weight rate,moreover,after composition,the c and cell volumes of(La,Mg)Ni_(3) phase increase,and the LaNi_(5) phase keep the same,which indicate that the boron may enter(La,Mg)Ni_(3) phase.The electrochemical studies show that the maximum discharge capacity of the composites decreases,but the cycling life improved.And the high rate discharge ability and exchange impendence spectroscopy(EIS)of the AB_(3) alloys and its composite were also studied.

Infliximab treatment of glycogenosis Ib with Crohn's-like enterocolitis: A case report

BACKGROUND Glycogen storage disease type Ib(GSD-Ib)is a glycogen metabolism disorder that leads to the manifestations of inflammatory bowel disease(IBD),especially Crohn’s disease(CD)-like colitis.Although biological agents are effective for treating CD,their application in the treatment of GSD-Ib with CD-like colitis has been rarely reported.CASE SUMMARY A 13-year-old Han male was diagnosed with GSD-Ib with CD.The patient was treated with granulocyte colony-stimulating factor.When he had symptoms of CD-like colitis,he was continuously pumped with enteral nutrition and administered oral mesalazine for 2 wk;however,the symptoms did not improve significantly.Hence,infliximab(IFX)was administered.Hitherto,the patient has been followed up for 1 year,and no clinical manifestations have been observed.After 6 mo of treatment(fifth IFX treatment),the disease activity index and all inflammatory indexes decreased,and a review of the colonoscopy data showed that the ulcers appeared smooth.CONCLUSION In this study,the patient was successfully treated with IFX.In cases of GSD-Ib,IBD should be highly considered.

Recent development and gene therapy for glycogen storage disease type Ia

Glycogen storage disease type Ia(GSD-Ia)is an autosomal recessive metabolic disorder caused by a deficiency in glucose-6-phosphatase-α(G6Pase-αor G6PC)that is expressed primarily in the liver,kidney,and intestine.G6Pase-αcatalyzes the hydrolysis of glucose-6-phosphate(G6P)to glucose and phosphate in the terminal step of gluconeogenesis and glycogenolysis,and is a key enzyme for endogenous glucose production.The active site of G6Pase-αis inside the endoplasmic reticulum(ER)lumen.For catalysis,the substrate G6P must be translocated from the cytoplasm into the ER lumen by a G6P transporter(G6PT).The functional coupling of G6Pase-αand G6PT maintains interprandial glucose homeostasis.Dietary therapies for GSD-Ia are available,but cannot prevent the long-term complication of hepatocellular adenoma that may undergo malignant transformation to hepatocellular carcinoma.Animal models of GSD-Ia are now available and are being exploited to both delineate the disease more precisely and develop new treatment approaches,including gene therapy.

Clinical and Technological Dependence Characteristics on a Series of Brazilian Cases with Infantile Onset Pompe Disease in Enzyme Replacement Therapy

Pompe disease (PD) is a rare inborn error of metabolism due to an abnormal acid alpha-glucosidase (GAA) activity that comprises glycogen breakdown mainly in the lysosomes. Since the introduction of enzyme replacement therapy (ERT), with recombinant human GAA for the early onset PD patient, a relevant field of clinical research due to the benefits regarding survival rate has been widely documented worldwide. Objective: To describe the clinical characteristics and the ERT effects in a series of Brazilian patients with infantile onset PD (IOPD) under ERT. Methods: Brazilian patients diagnosed with IOPD under ERT were recruited through their physicians participating in the International Pompe Disease Registry from 2009 to 2017. Data were collected by an online survey. Results: 10 IOPD patients were identified through the survey with a death rate of 30% and technology dependency rate reported as 80% (motor, respiratory or nutritional fields) of the patients. After the third year of ERT, motor disabilities were lost in 50% of ambulated patients. The overall characteristics were similar to international studies. Conclusion: Despite ERT benefits in cardiac involvement, motor disabilities seem to be much more compromised in IOPD patients, with high technology dependence, especially after three years of age.

Identification of Seven Novel Mutations in the Acid Alpha-glucosidase Gene in Five Chinese Patients with Late-onset Pompe Disease

Background： Pompe disease is a rare lysosomal glycogen storage disorder linked to the acid alpha-glucosidase gene （GAA）. A wide clinical and genetic variability exists between patients from different ethnic populations, and the genotype-phenotype correlations are still not well understood. The aim of this study was to report the clinicopathological and genetic characteristics of five Chinese patients with late-onset Pompe disease （LOPD） who carried novel GAA gene mutations. Methods： Clinical and pathological data of patients diagnosed with glycogen storage disease at our institution from April 1986 to August 2017 were collected, and next-generation sequencing of frozen muscle specimens was conducted. Results： Of the five patients included in the study, the median disease onset age was 13 years, with a median 5 years delay in diagnosis. The patients mainly manifested as progressive weakness in the proximal and axial muscles, while one patient developed respiratory insufficiency that required artificial ventilation. In muscle biopsies, vacuoles with variable sizes and shapes appeared inside muscle fibers, and they stained positive for both periodic acid-Schiff and acid phosphatase staining. Ten GAA gene mutations, including seven novel ones （c.796C〉A, c. 1057C〉T, c. 1201C〉A, c. 1780C〉T, c. 1799G〉C, c.2051C〉A, c.2235dupG）, were identified by genetic tests. Conclusions： The seven novel GAA gene mutations revealed in this study broaden the genetic spectrum of LOPD and highlight the genetic heterogeneity in Chinese LOPD patients.

Reservoir and development characteristics of the Da'anzhai tight oil in Sichuan Basin,SW China

The oil in the Jurassic Da'anzhai reservoirs in the Sichuan Basin is unconventional tight oil,which accumulated in or near source rocks,and did not experience extensive migration in a large-scale long distance.The first submember,second submember and third submember of Da'anzhai Member are dominated by shell limestone which is widely and continuously distributed,and are typical near-source lacustrine shell limestone tight reservoirs.Complex lithology,multiple types of reservoir space and complicated pore structure are developed in these reservoirs.The effective reservoir space mainly includes micro-pores and micro-fractures with strong fabric selectivity.The petrophysics experiment reveals that the average connected matrix porosity of tight oil reservoir in Jurassic Da'anzhai Member is about 2.13%,lower than that of other tight oil reservoirs but higher than the average effective porosity(0.97%)from previous single alcohol-saturated method.According to production performance data,the Da'anzhai shell limestone reservoir is not a simple porous or fractured reservoir,but has complex porethroat-fracture association or storage-seepage mode.Because the development of fossil shells controls the development of micro-fractures,fluids are difficult to enter into but easy to escape from the reservoirs.Although the pore-throat is fine,the sorting is poor and the displacement pressure is high,the movable fluid saturation and mercury ejection efficiency of the reservoir in the Da'anzhai Member is only slightly lower than that of some storage-seepage modes,and higher than that of Oil-bearing Group 7 of Yanchang Formation in the Ordos Basin.The reservoir in the Da'anzhai Member is one of the few tight oil reservoirs with high natural productivity.The tight oil in the shell limestone of the Da'anzhai Member has great development potential,but its extensive and effective development also has some challenges,such as high seepage resistance of matrix and ineffective single development mode.The development mode of the Da'anzhai tight oil should draw lessons from the Bakken Formation in North America and Oil-bearing Gourp 7 of Yanchang Formation in the Ordos Basin,and thus,effective development technologies based on volume fracturing and fine operation for shell limestone tight oil in the Da'anzhai Member in Sichuan Basin are developed to realize the development of profit and scale.