ANTIEPILEPTIC DRUG THERAPY AND SERUM CARNITINE LEVELS IN CHILDREN PRIOR TO AND FOLLOWING TREATMENT

Background:The physiologic function of carnitine, oxidation of fatty acid and lipid  metabolism, is severely affected in carnitine deficiency, secondary forms of which lead to renal tubular disorders and chronic renal failure. Reduction in serum carnitine has been frequently reported in patients and experimental animals treated with antiepileptic drugs, one of which, valproic acid has consistently been found to cause the deficiency; the antiepileptic drugs, valproic acid, has consistently been found to cause the deficiency. Previous results, however, regarding the effects of other antiepileptic drugs have been less consistent.  Considering the controversial results available in lterarure, the aim of this study was to determine the effect of Valproic acid, Carbamazepine and Phenobarbital on serum carnitine levels in epileptic children.Methods:In the present study, serum carnitine levels were randomly monitored before and six months after therapy in 39 epileptic patients receiving the antiepileptic drugs mentioned. Patient blood samples were taken before and six months after treatment and L-carnitine level was determined using the UV enzymatic test (Rouche Kit) spectronic Genesis 2, 340 nm.Results:Results showed a significant fall in the L-carnitine levels of epileptic children taking these drugs (P< 0.01).Conclusion:Considering the reducing effect of antiepileptic drugs on serum carnitine levels, it is recommended that a carnitine supplement be administered in pediatric epileptic patients to prevent the deficiency and related consequences caused by such therapies.Keywords:Carnitine ,Epilepsy - in children ,Valproic Acid, Phenobarbital, Carbamazepin

Spatially clustered type I interferon responses at injury borderzones

Sterile inflammation after myocardial infarction is classically credited to myeloid cells interacting with dead cell debris in the infarct zone1,2. Here we show that cardiomyocytes are the dominant initiators of a previously undescribed type I interferon response in the infarct borderzone. Using spatial transcriptomics analysis in mice and humans, we find that myocardial infarction induces colonies of interferon-induced cells (IFNICs) expressing interferon-stimulated genes decorating the borderzone, where cardiomyocytes experience mechanical stress, nuclear rupture and escape of chromosomal DNA. Cardiomyocyte-selective deletion of Irf3 abrogated IFNIC colonies, whereas mice lacking Irf3 in fibroblasts, macrophages, neutrophils or endothelial cells, Ccr2-deficient mice or plasmacytoid-dendritic-cell-depleted mice did not. Interferons blunted the protective matricellular programs and contractile function of borderzone fibroblasts, and increased vulnerability to pathological remodelling. In mice that died after myocardial infarction, IFNIC colonies were immediately adjacent to sites of ventricular rupture, while mice lacking IFNICs were protected from rupture and exhibited improved survival3. Together, these results reveal a pathological borderzone niche characterized by a cardiomyocyte-initiated innate immune response. We suggest that selective inhibition of IRF3 activation in non-immune cells could limit ischaemic cardiomyopathy while avoiding broad immunosuppression

Prediction of response to treatment in children with epilepsy

Abstract Objective: This study was conducted to predict the response to treatment in patients treated with anti-epilepsy drugs. Material and Methods: This analytical questionnaire-based study was conducted in 2014 among 128 patients with epilepsy admitted to Mofid Children's Hospital, Tehran, Iran. The inclusion criteria were children 2 months to 12 yr of age with epilepsy and patients who experienced fever and seizure attacks at least once were excluded from the study. Patients were followed up for 6 months and the response to their treatment was recorded. The good response to treatment was defined as the absence of seizure with two drugs during follow up. Results: Seventy-two patients (56.3%) were boys. The age of the first seizure was under 2 yr old in 90 patients (70.3%). History of febrile convulsion, family history of epilepsy and history of asphyxia was found in 16 (12.5%), 41 (32%), and 27 (21.1%) patients, respectively. Seizure etiology was idiopathic in 90 patients (70.3%), and the number of seizures was 1-2 in 36 patients (28.1%). Overall, 57 patients (44.5%) had cerebral lesion according to CT scan or MRI, and EEG was abnormal in 101 patients (78.9%). In 6-month follow-up, 40 patients (31.3%) responded well to the treatment and 88 patients (68.8%) responded poorly to the treatment. History of asphyxia (OR = 6.82), neonatal jaundice (OR = 2.81) and abnormal EEG (OR = 0.19) were effective factors in response to treatment. Conclusion: Abnormal EEG is an effective factor in treatment response in the children studied. Key Words: Pediatric, Anti-seizure drug, Response to treatment, Children, Epileps

Hexamine adsorption study on activated carbon from aqueous solutions for application in treatment of hexamine industrial wastewater

The adsorption of hexamine onto powdered activated carbon from aqueous solutions was studied in a fixed bed system. Langmuir, Freundlich, Redlich–Peterson and Toth isotherm models were used to fit the experimental data and isotherm parameters were determined. The results revealed that the adsorption isotherm models fitted the data in the order of Langmuir > Toth > Redlich–Peterson > Freundlich. Lagergren pseudo-first order kinetic model was found to correlate well with the experimental data. The effects of solution pH, temperature, initial hexamine concentration and added salts concentration on the adsorption capacity and the rate of adsorption were studied. The results indicate that the rate of adsorption increases and then decreases as temperature of the hexamine solution increases, however, the adsorption capacity decreases. The addition of low concentration of salt significantly increases the adsorption capacity of activated carbon. The results showed that the activated carbon has potential for the adsorption of hexamine from industrial hexamine wastewater

Removal of Urotropine from Industrial Wastewater by Acidic Cation Exchange Resins

The industrial wastewater produced by urotropine plants is considered as a major environmental polluting factor and hence its treatment is required. In this work, strongly acidic cation exchange resins including C100H (Purolite), Amberlite IR120 and Amberlyst 15W were used for removing of urotropine from wastewater. Optimum conditions for the three resins and regeneration were studied. The required amounts of resins for efficient exchange of urotropine and amount of acid for regenerating process of exhausted resins were determined and the potential of these resins in removing of urotropine and ammonia from the wastewater were compared. It was found that C100H resin has a higher capability in removing of urotropine in comparison with tow other resins. This capability of C100H was 2 and 7 times greater than Amberlite IR120 and Amberlyst 15W, respectively. The comparison of results with other methods indicates that this method reduces urotropine concentration more effectively. The simple and fast conductometric method has been developed for determination of urotropine in wastewater in the presence of formaldehyde and ammonia without any interference

Neurofilament-Light in Former Athletes: A Potential Biomarker of Neurodegeneration and Progression

BACKGROUND/OBJECTIVE: This study aimed to evaluate serum neurofilament light chain (NF-L) levels in former professional contact sports athletes with multiple concussions (ExPro) as a potential biomarker of neurodegeneration and predictor of white-matter (WM) abnormality progression. METHODS: Concentrations of NF-L in the serum of fifty-two cognitively normal ExPro and twenty-one healthy controls (HC) with no history of concussions were measured using single molecule array (Simoa) technology. Both groups underwent neuroimaging at the time of serum collection. Eighteen of the participants in the ExPro underwent follow-up imaging after 2 years. RESULTS: Levels of serum NF-L were not significantly different between the ExPro and HC. However, in the ExPro group, NF-L levels were positively correlated with the mean diffusivity (MD) of corpus callosum (CC) and fornix, and total ventricular volume. Moreover, NF-L levels in the ExPro group at the first visit were positively correlated with the amount of increase in CC MD at the 2-year follow-up. CONCLUSIONS: NF-L levels reflect neuronal changes in the ExPro group and predict the extent of decrease in white matter integrity over time. Serum NF-L might be a biomarker of neurodegeneration and WM abnormality progression in ExPro

Characterising the spectrum of autosomal recessive hereditary hearing loss in Iran

Background Countries with culturally accepted consanguinity provide a unique resource for the study of rare recessively inherited genetic diseases. Although hereditary hearing loss (HHL) is not uncommon, it is genetically heterogeneous, with over 85 genes causally implicated in non-syndromic hearing loss (NSHL). This heterogeneity makes many gene-specific types of NSHL exceedingly rare. We sought to define the spectrum of autosomal recessive HHL in Iran by investigating both common and rarely diagnosed deafness-causing genes. Design Using a custom targeted genomic enrichment (TGE) panel, we simultaneously interrogated all known genetic causes of NSHL in a cohort of 302 GJB2- negative Iranian families. Results We established a genetic diagnosis for 67 of probands and their families, with over half of all diagnoses attributable to variants in five genes: SLC26A4, MYO15A, MYO7A, CDH23 and PCDH15. As a reflection of the power of consanguinity mapping, 26 genes were identified as causative for NSHL in the Iranian population for the first time. In total, 179 deafness-causing variants were identified in 40 genes in 201 probands, including 110 novel single nucleotide or small insertion-deletion variants and three novel CNV. Several variants represent founder mutations. Conclusion This study attests to the power of TGE and massively parallel sequencing as a diagnostic tool for the evaluation of hearing loss in Iran, and expands on our understanding of the genetics of HHL in this country. Families negative for variants in the genes represented on this panel represent an excellent cohort for novel gene discovery