一例胰腺纤维钙化性糖尿病的诊疗和基因检测分析

胰腺纤维钙化性糖尿病(fibrocalculous pancreatic diabetes,FCPD)是一类罕见的、胰腺内、外分泌功能同时发生障碍的特殊类型糖尿病。本文报道了1例FCPD患者的临床资料,该患者自幼年起反复出现腹痛,25岁时诊断为糖尿病,腹部CT见胰腺钙化,基因检测发现SPINK1基因有2个纯合突变(c.194+2T>C和-191-24G>A)。基因检测分析发现其父母为杂合变异,其弟弟为纯合变异,但尚未观察到FCPD的典型表现。结合该病例及国内90例FCPD患者的临床特点汇总分析,建议对体型消瘦、无酮症倾向但胰岛功能较差的分型不明确糖尿病患者,需要考虑FCPD可能性,重视胰腺影像学及基因检测,尽早进行鉴别诊断。本文提高了对FCPD的认识和管理,为该疾病后续致病机制的深入研究与药物干预靶点的筛选充实了临床资料。

Corrosion risk analysis of tube-and-shell heat exchangers and design of outlet temperature control system

This study deals with the high-risk shell-and-tube heat exchangers in the effluent system of hydrogenation reaction of the petrochemical industry.The process of hydroprocessing reactor effluent system is simulated in Aspen Plus to study the distribution of corrosive medium in the three phases of oil,gas and water.The least-squares method is utilized to calculate the ammonium salt crystallization temperature.Then,the heat exchanger with risk of ammonium salt crystal corrosion is identified.Dynamic mathematical modeling of the heat exchanger is established to determine the transfer function.A temperature control system with proportional integral derivative(PID)control of the heat exchanger outlet is designed,and fuzzy logic is used to implement self-tuning of PID parameters.After MATLAB simulation,the results show the control system can achieve rapid control of the heat exchanger outlet temperature.

Single-balloon and spiral enteroscopy may have similar diagnostic and therapeutic yields to double-balloon enteroscopy:Results from a meta-analysis of randomized controlled trials and prospective studies

BACKGROUND Double-balloon,single-balloon,and spiral enteroscopy(DBE,SBE,and SE)have revolutionized the management of intestinal diseases.However,evidence about efficacies of these methods is lacking.We aimed to conduct a meta-analysis comparing the clinical outcomes among DBE,SBE,and SE.METHODS We searched randomized controlled trials and prospective studies in MEDLINE,PubMed,EMBASE,Cochrane Library,and Chinese CQVIP database.Studies referencing the comparison of at least two of these three methods were included.Primary outcome was diagnostic yield.Other outcomes were therapeutic yield,total enteroscopy,examination time,time to maximum insertion,and depth of maximal insertion(DMI).RESULTS Eleven studies including 727 patients were identified:DBE vs SE(n=6),DBE vs SBE(n=4),and SBE vs SE(n=1).The diagnostic and therapeutic yields did not differ significantly when comparing DBE with SE[odds ratio(OR)=1.19,95%confidence interval(CI):0.68-2.08;OR=1.17,95%CI:0.61-2.23]and DBE with SBE(OR=0.85,95%CI:0.55-1.33;OR=1.71,95%CI:0.64-4.60).Total enteroscopy,examination time,time to maximum insertion,and DMI were similar between SBE and DBE.DBE was superior to SE with regard to DMI[mean difference(MD)=36.76,95%CI:5.09-68.43],with longer time to maximum insertion(MD=15.14,95%CI:12-18.27)and examination time(MD=12.98,95%CI:9.57-16.38).CONCLUSION DBE and SBE have similar clinical outcomes.Compared with DBE,SE seems to have similar diagnostic and therapeutic yields,but shorter procedural time in cost of less depth of insertion.SE needs further evaluation vs SBE.DBE is recommended for complete enteroscopy.

Local bone metabolism balance regulation via double-adhesive hydrogel for fixing orthopedic implants

The effective osteointegration of orthopedic implants is a key factor for the success of orthopedic surgery.However,local metabolic imbalance around implants under osteoporosis condition could jeopardize the fixation effect.Inspired by the bone structure and the composition around implants under osteoporosis condition,alendronate(A)was grafted onto methacryloyl hyaluronic acid(H)by activating the carboxyl group of methacryloyl hyaluronic acid to be bonded to inorganic calcium phosphate on trabecular bone,which is then integrated with aminated bioactive glass(AB)modified by oxidized dextran(O)for further adhesion to organic collagen on the trabecular bone.The hybrid hydrogel could be solidified on cancellous bone in situ under UV irradiation and exhibits dual adhesion to organic collagen and inorganic apatite,promoting osteointegration of orthopedic implants,resulting in firm stabilization of the implants in cancellous bone areas.In vitro,the hydrogel was evidenced to promote osteogenic differentiation of embryonic mouse osteoblast precursor cells(MC3T3-E1)as well as inhibit the receptor activator of nuclear factor-κB ligand(RANKL)-induced osteoclast differentiation of macrophages,leading to the upregulation of osteogenic-related gene and protein expression.In a rat osteoporosis model,the bone-implant contact(BIC)of the hybrid hydrogel group increased by 2.77,which is directly linked to improved mechanical stability of the orthopedic implants.Overall,this organic-inorganic,dual-adhesive hydrogel could be a promising candidate for enhancing the stability of orthopedic implants under osteoporotic conditions.

Oxygen metabolism-balanced engineered hydrogel microspheres promote the regeneration of the nucleus pulposus by inhibiting acid-sensitive complexes

Intervertebral disc degeneration(IVDD)is commonly caused by imbalanced oxygen metabolism-triggered inflammation.Overcoming the shortcomings of antioxidants in IVDD treatment,including instability and the lack of targeting,remains challenging.Microfluidic and surface modification technologies were combined to graft chitosan nanoparticles encapsulated with strong reductive black phosphorus quantum dots(BPQDs)onto GelMA microspheres via amide bonds to construct oxygen metabolism-balanced engineered hydrogel microspheres(GM@CS-BP),which attenuate extracellular acidosis in nucleus pulposus(NP),block the inflammatory cascade,reduce matrix metalloproteinase expression(MMP),and remodel the extracellular matrix(ECM)in intervertebral discs(IVDs).The GM@CS-BP microspheres reduce H_(2)O_(2) intensity by 229%.Chemical grafting and electrostatic attraction increase the encapsulation rate of BPQDs by 167%and maintain stable release for 21 days,demonstrating the antioxidant properties and sustained modulation of the BPQDs.After the GM@CS-BP treatment,western blotting revealed decreased acid-sensitive ion channel-3 and inflammatory factors.Histological staining in an 8-week IVDD model confirmed the regeneration of NP.GM@CS-BP microspheres therefore maintain a balance between ECM synthesis and degradation by regulating the positive feedback between imbalanced oxygen metabolism in IVDs and inflammation.This study provides an in-depth interpretation of the mechanisms underlying the antioxidation of BPQDs and a new approach for IVDD treatment.

Cortical Mechanisms of Multisensory Linear Self-motion Perception

Accurate self-motion perception,which is critical for organisms to survive,is a process involving multiple sensory cues.The two most powerful cues are visual(optic flow)and vestibular(inertial motion).Psychophysical studies have indicated that humans and nonhuman primates integrate the two cues to improve the estimation of self-motion direction,often in a statistically Bayesian-optimal way.In the last decade,single-unit recordings in awake,behaving animals have provided valuable neurophysiological data with a high spatial and temporal resolution,giving insight into possible neural mechanisms underlying multisensory self-motion perception.Here,we review these findings,along with new evidence from the most recent studies focusing on the temporal dynamics of signals in different modalities.We show that,in light of new data,conventional thoughts about the cortical mechanisms underlying visuo-vestibular integration for linear self-motion are challenged.We propose that different temporal component signals may mediate different functions,a possibility that requires future studies.

Unveiling Dynamic System Strategies for Multisensory Processing: From Neuronal Fixed-Criterion Integration to Population Bayesian Inference

Multisensory processing is of vital importance for survival in the external world.Brain circuits can both integrate and separate visual and vestibular senses to infer self-motion and the motion of other objects.However,it is largely debated how multisensory brain regions process such multisensory information and whether they follow the Bayesian strategy in this process.

Cross-cultural adaption and psychometric properties of the Chinese version of the Diabetes Behavior Rating Scale: a pilot study

Self-care behavior plays a major role in diabetic management. However, in China, a satisfactory instrument has not yet been developed to evaluate the compliance of self-care behavior for young patients with type 1 diabetes mellitus （T 1DM）. The Diabetes Behavior Rating Scale （DBRS） has a potential to be the first mature instrument. The purpose of this study is to cross-culturally adapt the DBRS, and preliminarily evaluate its psychometric properties. The instrument translation included translation, back translation and culture adaptation. Psychometric properties were assessed in a sample of 116 young patients with T1DM adapting insulin injection therapy. The Chinese version of the DBRS was divided to four subscales. Cronbaeh＇s α for the total scale was 0.92. The mean inter-item and item-total correlations were 0.35 and 0.54 respectively. Test-retest reliability showed good temporal stability （r=0.81, P=0.001）. Negative correlations were found between DBRS scores with the Diabetes Distress Scale scores （r=-0.32, P=-0.003） and hemoglobin Ale （HbAlc） levels （r=-0.36, P=0.002）. Higher DBRS scores correlated with better glycemic control. The Chinese insulin injection therapy version of the DBRS is well translated and culturally adapted. It shows good overall reliability and validity and appears to be a valuable tool for assessing the diabetic self-care behaviors for young patients with T 1DM.

The heterogeneity of islet autoantibodies and the progression of islet failure in type 1 diabetic patients

Type 1 diabetes mellitus is heterogeneous in many facets. The patients suffered from type 1 diabetes present several levels of islet function as well as variable number and type of islet-specific autoantibodies. This study was to investigate prevalence and heterogeneity of the islet autoantibodies and clinical phenotypes of type 1 diabetes mellitus; and also discussed the process of islet failure and its risk factors in Chinese type 1 diabetic patients. A total of 1,291 type 1 diabetic patients were enrolled in this study. Demographic information was collected. Laboratory tests including mixed-meal tolerance test, human leukocyte antigen alleles, hemoglobin A1 c, lipids, thyroid function and islet autoantibodies were conducted. The frequency of islet-specific autoantibody in newly diagnosed T1 DM patients(duration shorter than half year) was 73% in East China. According to binary logistic regressions, autoantibody positivity, longer duration and lower Body Mass Index were the risk factors of islet failure. As the disease developed, autoantibodies against glutamic acid decarboxylase declined as well as the other two autoantibodies against zinc transporter 8 and islet antigen 2. The decrease of autoantibodies was positively correlated with aggressive beta cell destruction. Autoantibodies can facilitate the identification of classic T1 DM from other subtypes and predict the progression of islet failure. As there were obvious heterogeneity in autoantibodies and clinical manifestation in different phenotypes of the disease, we should take more factors into consideration when identifying type 1 diabetes mellitus.

A systematic survey on the diagnosis strategy and patient management of type 1 diabetes by Chinese physicians

The qualification of physicians is a key factor in controlling type 1 diabetes(T1 D) since they provide crucial information to their patients about self-management. To investigate whether Chinese physicians' medical strategies influence the control of T1 D in their patients, we designed a questionnaire to survey Chinese physicians, which covered their diagnosis and patient-management strategies for T1 D. A total of 442 completed questionnaires were received from 35 public hospitals in 12 cities. The results showed Chinese physicians mainly diagnosed T1 D based on the clinical features and islet dysfunction. One-third of physicians in this study still prescribed non-basal-bolus insulin regimens to their T1 D patients. More than 80% of the doctors prescribed alpha-glucosidase inhibitors as adjunctive therapy, in addition to insulin therapy. Moreover, most of the physicians in China did not pay attention to identify coexistent autoimmune diseases. T1 D patients in China were not armed with self-management knowledge and skills, which should be provided by their doctors. One of the circumstances leading to insufficient disease control in Chinese T1 D patients is the ineffective therapeutic strategy prescribed by their physicians. We need to promote knowledge of efficient strategies among physicians in China to achieve better disease control in Chinese T1 D patients in the future.

Screening and identification of human Zn T8-specific single-chain variable fragment (scFv) from type 1 diabetes phage display library

Zinc transporter 8 （ZnT8） is a major autoantigen and a predictive marker in type 1 diabetes （T1D）. To investigate ZnT8-specific antibodies, a phage display library from TID was constructed and single-chain antibodies against ZnT8 were screened and identified. Human T1D single-chain variable fragment （scFv） phage display library consists of approximately 1 ~ l0s clones. After four rounds of bio-panning, seven unique clones were positive by phage ELISA. Among them, C27 and C22, which demonstrated the highest affinity to ZnT8, were expressed in Escherichia coli Topl0F＇ and then purified by affin- ity chromatography. C27 and C22 specifically bound ZnT8 N/C fusion protein and ZnT8 C terminal dimer with one Arg325Trp mutation. The specificity to human islet cells of these scFvs were further confirmed by immunohistochemistry. In conclusion, we have successfully constructed a T1D phage display antibody library and identified two ZnT8-specific scFv clones, C27 and C22. These ZnT8-specific scFvs are potential agents in immunodiagnostic and immunotherapy of T1D.

Magnetic properties and microstructure of Fe69.5-xNd7B21Nb2.5Gax（x=0-1）alloys

The Fe69.5-xNd7 B21Nb2.5Gax(x = 0-1)permanent magnets in the form of rods were prepared by annealing the bulk amorphous alloys.The magnetic properties,phase evolution and microstructure of the alloys were investigated systematically.It is found that the glass forming ability(GFA), microstructure and magnetic properties are sensitive to Ga content for Fe69.5-xNd7 B21Nb2.5Gax(x = 0-1)bulk alloys.The annealed alloys are mainly composed of soft α-Fe,hard Nd2 Fe14B and nonmagnetic Nd1.1Fe4 B4 phases.When x = 0.3,the optimally annealed magnets exhibit magnetic properties of the remanence Br = 0.63 T,intrinsic coercivity Hcj = 368.68 kA/m and maximum energy product(BH)max = 33.73 kJ/m3.Furthermore,magnetic field heat treatment at the temperature close to Curie temperature of Nd2 Fe14B phase was applied to the annealed Fe69.2Nd7 B21Nb2.5Ga0.3 magnet.The results of X-ray diffraction(XRD)and transmission electron microscopy(TEM)indicate that the magnetic field heat treatment can be beneficial for the precipitation of α-Fe.Thus,the Br,Hcj and(BH)max are enhanced by 8.7%,6.3% and 16.3%,respectively.