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Childhood Langerhans cell histiocytosis:a disease with many faces

World Journal of Pediatrics 2019年第6期15卷 536-545页

作者： Alexander K.C.Leung joseph m.lam Kin Fon Leong Department of Pediatrics The University of Calgaryand The Alberta Children's Hospital#20023316th Avenue NWCalgaryAB T2M 0H5Canada Department of Pediatrics University of British ColumbiaVancouverBCCanada Department of Dermatology and Skin Sciences University of British ColumbiaVancouverBCCanada Pediatric Institute Kuala Lumpur General HospitalKuala LumpurMalaysia

Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans *** presentations of LCH vary *** sources A Pubmed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observational studies,and *** paper is based on,but not limited to,the search *** Generally,patients with LCH can be divided into two groups based on the extent of involvement at diagnosis,namely,single-system LCH and multisystem *** involvement may be unifocal or *** with isolated bone lesions typically present between 5 and 15 years of age,whereas those with multisystem LCH tend to present before 5 years of *** clinical spectrum is broad,ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem *** manifestations include,among others,"punched out" lytic bone lesion,seborrheic dermatitis-like erup-tion,erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches,and eczematous lesions,diabetes insipidus,hepatosplenomegaly,cytopenias,lymphadenopathy,and an acute fulminant disseminated multisystem condition presenting with fever,skin rash,anemia,thrombocytopenia,lymphadenopathy,and *** diagnosis is clinicopathologic,based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional *** CD1a,S100,and/or CD207(Langerin)immunohistochemical staining of lesional cells is required for a definitive *** waiting is recommended for patients with skin-only *** with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids,topical nitrogen mustard,oral methotrexate,or oral *** current recommended first-line therapy for patients with multisystem LCH i

关键词： Cytopenia Diabetes insipidus Eczematous lesions Hepatosplenomegaly Lymphadenopathy Seborrheic dermatitis-like eruption

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Erythema nodosum

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World Journal of Pediatrics 2018年第6期14卷 548-554页

作者： Alexander K.C.Leung Kin Fon Leong joseph m.lam Department of Pediatrics The Alberta Children's HospitalThe University of Calgary#200233-16th Avenue NWCalgaryAB T2M 0H5Canada Pediatric Institute Kuala Lumpur General HospitalKuala LumpurMalaysia Department of Pediatrics and Department of Dermatology and Skin Sciences University of British ColumbiaVancouverBCCanada

Background Erythema nodosum can be associated with a number of systemic diseases. There is, however, a paucity of information in the pediatric literature on this condition. The purpose of this article is to familiarize pediatricians with the evaluation, diagnosis, and treatment of erythema nodosum. Data sources A Pubmed search was completed in Clinical Queries using the key terms 'erythema nodosum'. Results Clinically, erythema nodosum presents with a sudden onset of painful, erythematous, subcutaneous nodules mainly localized to the pretibial areas. Lesions are usually bilateral and symmetrical, ranging from 1 to 5 cm in diameter. Erythema nodosum may be associated with a variety of conditions such as infection, medications, sarcoidosis, pregnancy, inflamma-tory bowel disease, vaccination, autoimmune disease, malignancy, and miscellaneous causes. The condition is idiopathic in approximately 50% of cases. The diagnosis is mainly clinical with biopsy reserved for atypical cases. To evaluate for the underlying cause, some basic laboratory screening studies are worthwhile in most cases and include a complete blood cell count, erythrocyte sedimentation rate and/or C-reactive protein, throat swab culture, antistreptococcal O titers, and a chest radiograph. Other tests should be individualized, guided by the history and physical examination results. most cases of ery-thema nodosum are self-limited and require no treatment. Bed rest and leg elevation are generally recommended to reduce the discomfort. Nonsteroidal anti-inflammatory drugs are the first-line treatment for pain management. Conclusions As erythema nodosum is often a cutaneous manifestation of a systemic disease, a thorough search should be performed to reveal the underlying cause.

关键词： Drug-induced Group A β-hemolytic streptococcal infection Sarcoidosis Subcutaneous pretibial nodules Tuberculosis Yersinia infection

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Gianotti-Crosti syndrome(papular acrodermatitis of childhood)in the era of a viral recrudescence and vaccine opposition

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World Journal of Pediatrics 2019年第6期15卷 521-527页

作者： Alexander K.C.Leung Consolato maria Sergi joseph m.lam Kin Fon Leong Department of Pediatrics The University of CalgaryCalgaryCanada The Alberta Children's Hospital #200233-16th Avenue NWCalgaryAB T2M OH5Canada The University of Alberta EdmontonABCanada Departments of Pediatrics Dermatology and Skin SciencesUniversity of British ColumbiaVancouverBCCanada Pediatric Institute Kuala Lumpur General HospitalKuala LumpurMalaysia

Background Gianotti-Crosti syndrome is characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical *** sources A Pubmed search was conducted using Clinical Queries with the key terms"Gianotti-Crosti syndrome"OR"papular acrodermatitis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observa-tional studies,and *** paper is based on,but not limited to,the search *** The eruption of Gianotti-Crosti syndrome is found predominantly on the cheeks,extensor surfaces of the extremities,and *** is a sparing of antecubital and popliteal fossae as well as palms,soles,and mucosal *** often asymptomatic,the lesions may be mildly to moderately ***-Crosti syndrome is most common in children between 1 and 6 years of *** Epstein-Barr virus and the hepatitis B virus are the most common pathogens associated with Gianotti-Crosti *** treatment for Gianotti-Crosti syndrome is necessary because it is *** an era of vaccine hesitancy and refusal,Gianotti-Crosti syndrome may be important to mention to parents,because it can occur and trigger *** Gianotti-Crosti syndrome is mainly a disease of early childhood,characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical *** the advent of more universal vaccination against hepatitis B virus,Epstein-Barr virus has become the most common etiologic agent of Gianotti-Crosti *** cases of post-vaccination Gianotti-Crosti syndrome have been ***,the emphasis should be placed on its self-limiting attribution.

关键词： Epstein-Barr virus Hepatitis B virus Papular acrodermatitis Papular symmetrical eruption

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Advances in discovery of novel investigational agents for functional cure of chronic hepatitis B:A comprehensive review of phases II and III therapeutic agents

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World Journal of Hepatology 2024年第3期16卷 331-343页

作者： Robert lam joseph K Lim Section of Digestive Diseases Yale University School of MedicineNew HavenCT 06520United States

Chronic hepatitis B virus(HBV)infection affects over 295 million people globally and an estimated 1.6 million people in the United *** is associated with significant morbidity and mortality due to cirrhosis,liver failure,and liver *** therapy with oral nucleos(t)ide analogues is associated with high rates of virologic suppression,which in turn has been associated with a decreased risk of liver ***,current antiviral regimens are limited by concerns with adverse effects,adherence,resistance,long-term treatment,and ongoing risk for liver *** investigational agents are currently in development and are targeted at achieving functional cure with sustained hepatitis B surface antigen(HBsAg)loss and suppression of HBV *** we review key evidence from phases II and III trials defining the efficacy and safety profiles for key investigational agents for functional cure of chronic hepatitis B,including core/capsid inhibitors,entry inhibitors,RNA interference(siRNA/ASO),HBsAg inhibitors,Toll-like receptor agonists,checkpoint inhibitors,and therapeutic vaccines.

关键词： Hepatitis B virus Treatment Clinical trials RNA interference Entry inhibitors Core inhibitors Immunomodulators

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Event-triggered sliding mode control of linear repetitive processes and its application in metal rolling process

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Science China(Information Sciences) 2024年第3期67卷 167-181页

作者： Xinyu LV Yugang NIU James lam Key Laboratory of Smart manufacturing in Energy Chemical Process (East China University of Science and Technology) Ministry of Education Department of mechanical Engineering The University of Hong Kong

This paper investigates the design problem of a sliding mode controller for linear repetitive processes(LRPs) with a finite pass length on each pass. Under limited communication resources, an eventtriggered mechanism is implemented from the sensors to the controller, whose triggered sequence is consistent with the evolution direction of LRPs. To periodically orchestrate communication between the controller and actuator, a round-robin scheduling mechanism is established based on the pass length. A sliding function involving the state and pass profile is constructed, and the sliding mode controller is designed using triggered states and pass profile signals. Feasible controller gains are attained to ensure the stability of the resultant closed-loop system. Finally, the proposed strategy is implemented for a multi-roll metal rolling process.

关键词： event-triggered mechanism linear repetitive processes metal rolling process round-robin scheduling sliding mode control

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Emerging roles and therapeutic potentials of sphingolipids in pathophysiology:emphasis on fatty acyl heterogeneity

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Journal of Genetics and Genomics 2024年第3期51卷 268-278页

作者： Jinming mu Sin man lam Guanghou Shui State Key Laboratory of molecular Developmental Biology Institute of Genetics and Developmental BiologyChinese Academy of SciencesBeijing 100101China University of Chinese Academy of Sciences Beijing 100101China Lipidall Technologies Company Limited ChangzhouJiangsu 213000China

Sphingolipids not only exert structural roles in cellular membranes,but also act as signaling molecules in various physiological and pathological processes.A myriad of studies have shown that abnormal levels of sphingolipids and their metabolic enzymes are associated with a variety of human ***,blood sphingolipids can also be used as biomarkers for disease *** review summarizes the biosynthesis,metabolism,and pathological roles of sphingolipids,with emphasis on the biosynthesis of ceramide,the precursor for the biosynthesis of complex sphingolipids with different fatty acyl *** possibility of using sphingolipids for disease prediction,diagnosis,and treatment is also *** endogenous ceramides and complex sphingolipids along with their specific fatty acyl chain to promote future drug development will also be discussed.

关键词： Sphingolipids Ceramide Therapeutic targets microdomain Biomarker

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monogenic diabetes in children:An underdiagnosed and poorly managed clinical dilemma

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World Journal of Diabetes 2024年第6期15卷 1051-1059页

作者： Saptarshi Bhattacharya joseph m Pappachan Department of Endocrinology Indraprastha Apollo HospitalsDelhi 110076India Department of Endocrinology and metabolism Lancashire Teaching Hospitals NHS TrustPreston PR29HTUnited Kingdom Faculty of Science Manchester Metropolitan UniversityManchester M156BHUnited Kingdom Faculty of Biology Medicine and HealthThe University of ManchesterManchester M139PLUnited Kingdom

monogenic diabetes,constituting 1%-2%of global diabetes cases,arises from single gene defects with distinctive inheritance *** over 50 associated genetic disorders,accurate diagnoses and management of monogenic diabetes remain inadequate,underscoring insufficient clinician *** disease spectrum encompasses maturity-onset diabetes of the young(mODY),characterized by distinct genetic mutations affecting insulin secretion,and neonatal diabetes mellitus(NDm)-a heterogeneous group of severe hyperglycemic disorders in *** diabetes,autoimmune monogenic diabetes,genetic insulin resistance and lipodystrophy syndromes further diversify the monogenic diabetes landscape.A tailored approach based on phenotypic and biochemical factors to identify candidates for genetic screening is recommended for suspected cases of *** diagnosis warrants immediate molecular genetic testing for infants under six *** these genetic defects presents a unique opportunity for precision *** research aimed to develop cost-effective genetic testing methods and gene-based therapy can facilitate appropriate identification and optimize clinical *** and study of new genes offer a valuable opportunity to gain deeper insights into pancreatic cell biology and the pathogenic mechanisms underlying common forms of *** clinical review published in the recent issue of World Journal of Diabetes is such an attempt to fill-in our knowledge gap about this enigmatic disease.

关键词： monogenic diabetes maturity-onset diabetes of the young Neonatal diabetes mitochondrial diabetes Insulin resistance syndromes Genetic testing Next generation sequencing

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Population Dynamics in an Advective Environment

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Communications on Applied mathematics and Computation 2024年第1期6卷 399-430页

作者： King-Yeung lam Ray Lee Yuan Lou Department of mathematics The Ohio State UniversityColumbusOH43210USA School of mathematical Sciences CMA-Shanghai and MOE-LSCShanghai Jiao Tong UniversityShanghai200240China

We consider a one-dimensional reaction-diffusion equation describing single-and two-species population dynamics in an advective environment,based on the modeling frameworks proposed by Lutscher et *** *** analyze the effect of rate of loss of individuals at both the upstream and downstream *** the single-species case,we prove the existence of the critical domain size and provide explicit formulas in terms of model *** further derive qualitative properties of the critical domain size and show that,in some cases,the critical domain size is either strictly decreasing over all diffusion rates,or monotonically increasing after first decreasing to a *** also consider competition between species differing only in their diffusion *** two species having large diffusion rates,we give a sufficient condition to determine whether the faster or slower diffuser wins the *** also briefly discuss applications of these results to competition in species whose spatial niche is affected by shifting isotherms caused by climate change.

关键词： Reaction-diffusion-advection Critical domain size Competition Climate change

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Periodontitis:An often-neglected complication of diabetes

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World Journal of Diabetes 2024年第3期15卷 318-325页

作者： marina G Kudiyirickal joseph m Pappachan Primary Care Dental Practice The Smile ClinicPreston PR16PZUnited Kingdom Department of Endocrinology and metabolism Lancashire Teaching Hospitals NHS TrustPreston PR29HTUnited Kingdom Faculty of Science Manchester Metropolitan UniversityManchester M156BHUnited Kingdom Faculty of Biology Medicine and HealthThe University of ManchesterManchester M139PLUnited Kingdom

The bidirectional association between type 2 diabetes mellitus(T2Dm)and periodontitis is now well established,resulting in periodontal disease being considered as the 6th major complication of diabetes mellitus(Dm)after cardiovascular disease,eye disease,neuropathy,nephropathy,and peripheral vascular *** can worsen the virulence and invasiveness of pathogenic oral microbial flora aggravating the local inflammation and infection in those with periodontal *** the other hand,the chemical and immunological mediators released into the circulation as part of periodontal inflammation worsen the systemic insulin resistance with worsening of *** if undiagnosed or left untreated can also result in eventual tooth loss.A study by Xu et al in the World Journal of Diabetes examined the predictive factors associated with periodontitis in Chinese patients with *** prevalence of periodontitis was found to be 75.7%in this *** on logistic regression analysis,the predictive factors for higher risk were low tooth brushing frequency[odds ratio(OR)=4.3],high triglycerides(TG;OR=3.31),high total cholesterol(TC;OR=2.87),higher glycated hemoglobin(HbA1c;OR=2.55),and higher age(OR=1.05)while higher education level was protective(OR=0.53).However,the most influential variables were HbA1c followed by age,TC,TG,low education level,brushing frequency,and sex on the random forest model(this model showed higher sensitivity for predicting the risk).A good understanding of the predictors for periodontitis in T2Dm patients is important in prevention,early detection of susceptible patients,and intervention to improve periodontal health and enable long-term glycaemic control as observed by Xu et al.

关键词： Diabetes mellitus Periodontitis Predictive factors Cardiovascular disease Glycaemic control

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Interfacial friction induced capillary flow within nanofiber-supported ionic liquid droplets

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Green Energy & Environment 2024年第5期9卷 789-791页

作者： Yuanyuan Zhao Gang Xia Yintung lam John Haozhong Xin School of Fashion and Textiles The Hong Kong Polytechnic UniversityHong KongChina Shenzhen Research Institute The Hong Kong Polytechnic UniversityShenzhenChina

As global economic growth increases,the demand for energy sources *** fossil fuels have traditionally satisﬁed this demand,their environmental inﬂuence and limited reserves require *** fuel combustion contributes substantially to greenhouse gas emissions,with a pressing need to halve these emissions by 2030 and target net-zero by *** energy sources,contributing currently to 29%of global electricity,are viewed as promising *** wind energy's potential,Zheng's team developed a novel method to harness even low wind speeds using well-aligned nanoﬁbers and an innovative“drop wind generator”.This system,combining moisture-saturated ionic liquid 3-methyl-1-octylimidazolium chloride with speciﬁc nanoﬁber arrays,exploits wind-inducedﬂows for energy *** study highlights the vast untapped potential of low-speed wind as a sustainable energy source potentially for electronics.

关键词： Wind energy Low-speed wind Ionic liquid Electronic devices

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