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Progress on molecular mechanism of mercury induced neurotoxicity in children

International Journal of Pediatrics.2013;40(5):489-493. doi:10.3760/cma.j.issn.1673-4408.2013.05.015

Mercury is one of the ubiquitous environmental neurotoxins which causes adverse effects on the development of children's neural system.Considering the severe neurotoxicity of mercury,finding and minimizing the sources of mercury exposure is essential for children's health.Amongst the mercury compounds,the toxicity of organic mercury is the most severe.Methyl mercury (MeHg) is the most detrimental and represents a major source of human exposure of mercury.Recent studies on molecular mechanisms of MeHg neurotoxicity points out that MeHg mainly mediates its toxic effects through the impairment of intraceilular calcium homeostasis,alteration of glutamate homeostasis,and oxidative stress.These concepts provide the biochemical interpreting of MeHg neurotoxicity.This review provides the current information on mercury,including environmental sources,neurotoxicity,and molecular mechanisms of MeHg-induced neurotoxicity.It will be helpful to find some effective ways of interfering children mercury exposure.

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The mechanism of action of probiotics on intestinal mucosal barrier

Nan LIN

International Journal of Pediatrics.2013;40(5):493-496. doi:10.3760/cma.j.issn.1673-4408.2013.05.016

Intestinal mucosal barrier has the function to isolate intestinal contents and internal environment,prevent foreign antigens and pathogens invading to lamina propria mueosae and can maintain organic homeostasis and normal activities.Impairment of the intestinal barrier is a key event in various gastrointestinal diseases.Probiotics are live microorganisms which ingested and reach the intestines in sufficient numbers to confer benefit to the host.It can improve or prevent many gastrointestinal diseases.The main reason is probiotics can maintain intestinal barrier function in various paths.This paper reviews its mechanisms of action.

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Advances of the LHX3 and LHX4 LIM-homeodomain transcription factors

Limin WANG ; Guiling LI

International Journal of Pediatrics.2013;40(5):518-521. doi:10.3760/cma.j.issn.1673-4408.2013.05.022

The LHX3 and LHX4 LIM-homeodomain transcription factors play essential roles in pituitary gland.Nervous system developments play certain roles in some cancers,leukemia,and deafness disease.Mutations in the genes encoding these regulatory proteins are closely associated with combined hormone deficiency diseases in human and animal models,and related to nervous system defect,tumors,leukemia and deafness.Analysis of these diseases and the biochemical properties of the LHX3 and LHX4 protein will facilitate a better understanding of the pathogenesis of these diseases.LHX3 and LHX4 are reviewed in this paper.

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Research progress of genetic mechanism in Henoch-Schonlein purpura of children

Li WANG

International Journal of Pediatrics.2013;40(5):497-500. doi:10.3760/cma.j.issn.1673-4408.2013.05.017

The research on the clinical characteristic and epidemiology in Henoch-Schonlein purpara of children indicates that genetic factors are closely connected with the disease and pathological process.In recent years,molecular biology studies show that C1 GALT1 gene,IL gene,vasomotor and endothelial function regulation genes,angiotensin-converting enzyme gene,angiotensinogen gene,MEFV gene and so on,which have aberrant IgAl giycosylation,are closely related with pathogenesis of Henoch-Schonlein purpura in children.The paper reviews the progress of genetic mechanism associated with Henoch-Schonlein purpura in recent years.

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Progress in maple syrup urine disease

Guoqing FAN

International Journal of Pediatrics.2013;40(5):514-517. doi:10.3760/cma.j.issn.1673-4408.2013.05.021

Maple syrup urine disease (MSUD) is a rare inherited disorder of branched-chain amino acid metabolism presenting with life threatening encephalopathy and maple syrup odor in urine in affected individuals.Blood levels of branched-chain amino acids(BCAA) significantly increase.It is classified into 5 forms according to the clinical course,and classified 4 molecular phenotypes based on the affected locus of the branched chain αr-ketoacid dehydrogenase complex.Treatment of MSUD is divided into acute decompensation stage treatment and life-long dietary restriction treatment.

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Adrenocortical hormone in the treatment of pediatric epilepsy

Jianmin ZHONG

International Journal of Pediatrics.2013;40(5):522-525. doi:10.3760/cma.j.issn.1673-4408.2013.05.023

Adrenocortical hormone (ACH) has been used in the treatment of the epilepsies,especially for epileptic encephalopathy,for over 70 years.ACH is clearly established as fast line treatment of infantile spasm and the Landau-Kleffner syndrome.This is typically as monotherapy in infantile spasm,but often in conjunction with anti-epileptic drugs in Landau-Kleffner syndrome,though the mechanism of ACH for epilepsies is not clear.However,in view of these potential adverse side effects,the use of ACH in the treatment of the epilepsies in children must be determined on a risk-benefit basis.

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Autoimmune encephalitis

Jiong QIN

International Journal of Pediatrics.2013;40(5):526-528. doi:10.3760/cma.j.issn.1673-4408.2013.05.024

Autoimmune encephalitis is a recently defined heterogeneous group of disorders probably resulting from an immune reaction against neuronal antigens.The characteristic clinical features include cognitive decline,seizures and behavioral disturbances.Autoimmune encephalitis,often improved with immune-modulatory therapies,is an important cause of rapidly progressive cognitive decline and behavioral problems.The recent knowledge about the syndromes was reviewed,and the clinical manifestations,diagnosis and treatment strategy were focused.

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Progress on treatment of primary nocturnal enuresis

Huiying DENG

International Journal of Pediatrics.2013;40(5):501-504. doi:10.3760/cma.j.issn.1673-4408.2013.05.018

Primary nocturnal enuresis is a common childhood disorder.This review discusses the progress on the treatment of primary nocturnal enuresis in children.Alarm interventions for nocturnal enuresis and desmopressin are the first-line therapy of primary nocturnal enuresis.In therapy-resistant cases,anticholinergic treatment combined with desmopressin can be tried.In situations when all other treatments have failed,imipramine treatment is warranted,provided the cardiac risks are taken into account.More research is needed regarding both the above drugs and non-pharmacological therapies on children with therapy-resistant enuresis.

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Research progress of mitochondrial disease

Yuanyuan HUANG

International Journal of Pediatrics.2013;40(5):505-510. doi:10.3760/cma.j.issn.1673-4408.2013.05.019

Mitochondrial disease is a set of multi-systematic and heterogeneous disease which invades in the central nervous system and muscles tissue adequately,mainly refers to the genetic defects that causes mitochondrial structure and function defect.Mitochondrial disease occupies an important position in the human genetics.At present,the research of mitochondrial disease and the related genetic mutation has become the focus in the genetics.This review is to discuss the mitochondrial disease epidemiology,molecular biology and genetics,clinical manifestations and diagnosis.

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Effects of Di(2-ethylhexyl) phthalate on development and metabolism in children

Qingsong ZHAO ; De WU

International Journal of Pediatrics.2013;40(5):511-513. doi:10.3760/cma.j.issn.1673-4408.2013.05.020

With the development of modem technology,more and more plastic products are widely used in various fields,while bringing significant convenience to the society,it also leads to more and more environmental estrogen.The influence of plasticizer is increasing,especially on the development of children.Extensive contacting with plasticizer is considered to be closely related with gonadal digenesis,obesity and other problems in children.This review focuses on the latest research results about the basic and clinical experiments of Di (2-ethylhexyl) phthalate (DEHP),which illuminate the effects of DEHP on children's growth and metabolism,and lay foundations for rational clinical intervention and scientific plasticizer's application.

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