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Inherited neurotransmitter disorders are a group of rare nervous system diseases frequently diagnosed in children.The disorders are caused by biosynthesis, breakdown or transport detects of neurotransmitters or cofactors essential in their biosynthesis.They can be classified as primary and secondary disorders.The clinical phenotypes of primary inherited neurotransmitter disorders include developmental delay, dyskinesia, schizophrenia, and epilepsy.Among them, epilepsy is the main clinical phenotype.Gamma-aminobutyric acid, glutamate, acetylcholine, biogenic amine and other neurotransmitters are involved in the epileptogenesis.The epilepsy related to primary inherited neurotransmitter disorders has diverse phenotypes, from mild seizures to severe early onset epileptic encephalopathy.An inherited neurotransmitter disorder should be suspected in children with epilepsy if the following features are present: (1) early onset epileptic encephalopathies associated with developmental impairment, autonomic dysfunctions or movement disorders; (2) frequent occurrence of such peculiar electroencephalogram patterns as burst suppression, hypsarrhythmia, and diffused/focal/multifocal electroencephalogram abnormalities; (3) neuroradiological signs of metabolic intoxication; (4) detection of specific cerebrospinal fluid biomarkers.Early identification, diagnosis and treatment is of great significance in reducing the incidence, lowering the mortality rate, and improving the prognosis of patients with epilepsy related to primary inherited neurotransmitter disorders.
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The synchronous abnormal discharge of neurons leads to epileptic seizures.However, in addition to neurons, microglia, as the main immune cells in the brain, plays an important role in the development and maintenance of neural circuits.Microglia is involved in early epileptic seizures, which can be mediated by increasing inflammatory cytokines and chemokines.Microglia can regulate the abnormal neurogenesis after epileptic seizures, promote the death of neurons after seizures, and cause neurodegeneration.Moreover, it can also affect synaptic pruning after seizures, eliminate synapses by phagocytosis or stripping, destroy the balance between synaptic excitation and inhibition, and aggravate seizures.Microglia plays an important role in the development of epilepsy.However, whether microglia participates in the occurrence of epilepsy still needs to be further studied.
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This commentary was based on the paper "Calorie Restriction with or without Time-Restricted Eating in Weight Loss" (TREATY trial) published recently in N Engl J Med, with the aim to introduce the study design of TREATY trial and discuss the main findings along with its clinical applicability. This trial offers additional evidence on the time-restricted eating and provides insights for future research.
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Objective To investigate the therapeutic effects of emodin on acute cholestatic hepatitis and mec-hanism thereof. Methods Fifty Sprague - Dawley (SD)rats were randomly divided into 5 groups and were treated with emodin,ursodeoxycholic acid,dexamethasone,or normal saline respectively for 4 days. On the fifth day gastric perfusion of alpha - naphthylisothiocyanate(ANIT)was performed to establish models of choiestatic hepatitis. Four to six hours after the establishment of model the above mentioned agents were given continuously. Forty - eight hours after the model establishment blood samples were collected from abdominal aorta to examine the total bilirubin(TB),direct bilirubin (DB),alanine aminotransferase(ALT),total bile acid(TBA),aspartate aminotransferase(AST),alkaline phosphatase (ALP),gamma glutamine transferase(GGT). Specimen of liver was collected to undergo pathological examination. Real - time PCR was used to detect the mRNA expression of farnesoid X receptor(FXR),small heterodimer partner (SHP ),bile salt export pump (BSEP ),uridine diphosphate glucuronosyltransferase 2 family polypeptide B4 (UGT2B4). Results The serum levels of total bilirubin (TB),direct bilirubin (DB),alanine aminotransferase (ALT),total bile acids (TBA),aspartate aminotransferase (AST),and alkaline phosphatase (ALP)of the model group were respectively (68. 1 ± 26. 1)μmol/ L,(46. 3 ± 20. 1)μmol/ L,(483 ± 228)U/ L,(159. 1 ± 57. 9)μmol/L,(2. 0 ± 0. 5)U/ L,(996 ± 382)U/ L,(324 ± 120)U/ L. The levels of TB,DB,ALT,TBA,AST,ALP of the emodin group were respectively (15. 0 ± 8. 7)μmol/ L,(10. 8 ± 3. 9)μmol/ L,(147 ± 71)U/ L,(60. 1 ± 22. 7)μmol/ L, (295 ± 104)U/ L,(222 ± 59)U/ L,and were all significantly lower than those of the model group (all P < 0. 05). The levels of TB,DB,ALT,TBA,AST,GGT,ALP of the emodin group were all significantly lower than those of the ursode-oxycholic acid group (all P < 0. 05). The levels of TB,DB,ALT,TBA,GGT,AST were all significantly lower than those of the dexamethasone group (all P < 0. 01). The expression levels of FXR,SHP,BSEP,UGT2B4 mRNA in the emodin group (1. 087 ± 0. 285,0. 892 ± 0. 390,0. 902 ± 0. 149,1. 785 ± 0. 403)were all significantly higher than those of the model group (0. 152 ±0. 088,0. 559 ±0. 194,0. 561 ±0. 123,0. 177 ±0. 039,all P <0. 05). Conclusions By decreasing the levels of TB,DB,ALT,TBA,AST,ALP and reducing pathological changes,emodin has a protective effect on cholestatic hepatitis. It has better effects than ursodeoxycholic acid and dexamethasone. These effects may be due to promoting FXR,SHP,BSEP and UGT2B4 expression.
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IgA vasculitis (IgAV) is a common systemic small-sized vessel vasculitis.It's characterized by non-thrombocytopenic palpable purpura,arthralgia/arthritis,bowel angina,and nephritis,but some details of etiology and pathogenesis is not very clear.The disease course is usually benign and self-limited,however,it is necessary to discuss the therapy of severe or chronic cases,especially there is not enough evidence-based basis for using key drugs to IgAV.Excellent clinical trails is expected to establish scientific system of prediction and evaluation of disease course,which will be helpful to individualize treatment of IgAV.
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Recent studies show that resveratrol is characterized with its antioxidative and anti-inflammatory effects, and plays an important role in the prevention and treatment of colorectal cancer through modulating intracellular carbohydrate and ceramide metabolism, activating tumor suppressors such as activator of transcription 3 and peroxisome proliferators-activated receptor γ, down-regulating the expression of kirsten rat sarcoma viral oncogene and inhibiting the epithelial-to-mesenchymal transition process of cancer cells.In addition,resveratrol exhibits its broad application prospects in combination therapy.
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Objective To investigate the coronary arterial lesions (CAL) in Kawasaki disease (KD) in Jilin Province and Inner Mongolia Autonomous Region from 1999 through 2012 and evaluate the influence of glucocorticoids (GCs) on prognosis.Methods We performed a retrospective study among patients of KD in Jilin Province and Inner Mongolia Autonomous Region from 1999 to 2012,in order to observe CAL in KD,the incidence of sequelae and the influence of CGs on disease outcome.Comparison between groups was carried out by Chi-square test.Results Eight hundred and ten KD cases were reported from 32 hospitals and 787 patients accepted an Echo examination,as the main objective of this study.Fivehundred and forty were boys and 247 were girls,with the male to female ratio of 2.19:1,of which 498 cases (63.3％) had CAL.Most CAL occurred in patients at 58 day to 14 year old (2.7±2.4).The most common abnormality was CAL including dilatation (49.5％) and stenosis (13.4％).Compared incomplete to complete KD,the result showed that the occurrence of CAL was similar between these two groups,no significant differences could be found between different gender.Nineteen (2.4％) of them had cardiac sequelae and the male/female ratio was 3.67:1.The younger the age,the higher the incidence of cardiac sequelae.The most frequent cardiac sequelae was coronary artery dilatation.GCs therapy could increase the occurrence of CAL [80.2％(97/121) vs 62.6％ (419/666),x2=8.71,P＜0.05].Conclusion CAL is not different between patients with complete or incomplete KD and the occurrence of CAL is not associated with gender,but with age.The younger the age,the higher the incidence of CAL and the higher the incidence of cardiac sequelae.It is possible that patients treated with GCs are prone to CAL.
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Objective To investigate the effect of hypertension on carotid atherosclerosis ,and the clinical significance of BFI technique in the diagnosis of carotid atherosclerosis .Methods Using BFI technique and CDFI technique to detect 198 cases of pa-tients with hypertension(hypertension groups) and 200 cases with normal blood pressure(control group) of carotid internal-media thickness(IMT) and the number of atherosclerotic plaque .To explore the relationship between hypertension and carotid atheroscle-rosis .Results IMT and the detection rate of plaque of hypertension groups was significantly higher than which of control group (P<0 .01) .In hypertension groups ,the higher of blood pressure levels ,the higher carotid IMT and the detection rate of plaque(P<0 .01 or P<0 .05) .The detection rate of plaque in BFI technique was higher than which in CDFI technique (P<0 .01) .Conclusion hypertension can aggravate carotid atherosclerosis ,and the higher blood pressure levels ,the more obvious of carotid atherosclerosis . BFI technique is useful for the detection of carotid atherosclerosis .
