P. Hermann, R. Henneberg, A. Nascimento, M. Leonart
{"title":"Erythrocyte Oxidative Stress Markers in Children: A Clinical Laboratory Experience","authors":"P. Hermann, R. Henneberg, A. Nascimento, M. Leonart","doi":"10.1055/s-0035-1554780","DOIUrl":"https://doi.org/10.1055/s-0035-1554780","url":null,"abstract":"Abstract Because oxidative damage to erythrocytes plays an important pathophysiologic role in many diseases and there are some difficulties in obtaining reference intervals for children in many laboratory tests, this work intends to describe the determination of oxidative stress parameters and the results obtained for children. A total of 280 blood samples were obtained from children between 8 and 11 years old, without any hematological disease. Samples were analyzed for seven oxidative stress parameters, methemoglobin, reduced glutathione, TBARS, percentage of hemolysis and activity of the enzymes G6PD, superoxide dismutase, and catalase. To draw a comparison, the same parameters were analyzed in children with sickle cell disease. Because all results presented normal distribution in the Kolmogorov-Smirnov, data were expressed as 2.5 and 97.5th accumulated percentiles and the statistical analysis between male and female children was performed using Student t-test. A p value <0.05 was considered significant. Except for hemolysis, no significant difference was observed on oxidative stress parameters, in normal children, separated by sex. Except for reduced glutathione and superoxide dismutase, significant differences were observed in all parameters between normal children and those with sickle cell disease. Oxidative stress parameters can be determined in children using simple laboratory methods with small volumes of blood and the establishment of reference intervals is necessary to improve clinical decisions.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"05 1","pages":"028 - 033"},"PeriodicalIF":0.0,"publicationDate":"2015-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0035-1554780","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58070450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Tondo, M. Casado, M. O'Callahan, I. Jordan, L. Altimira, B. Pérez-Dueñas, A. García-Alix, À. García-Cazorla, A. Ormazabal, R. Artuch
{"title":"Cerebrospinal Fluid Selenium Concentrations in Pediatric Patients with Neurologic Disorders","authors":"M. Tondo, M. Casado, M. O'Callahan, I. Jordan, L. Altimira, B. Pérez-Dueñas, A. García-Alix, À. García-Cazorla, A. Ormazabal, R. Artuch","doi":"10.1055/s-0035-1554782","DOIUrl":"https://doi.org/10.1055/s-0035-1554782","url":null,"abstract":"Abstract We aimed to study the cerebrospinal fluid (CSF) selenium (Se) status in pediatric patients with neurologic disorders and to assess the relationship between CSF Se concentrations, proteins, glutathione peroxidase (GPx) activity, and patient clinical data. We analyzed CSF Se by inductively coupled plasma-mass spectrometry (ICP-MS) in 212 patients with several neurologic conditions. Student's t-test was applied to compare CSF Se values from patients with those of controls. Single and multiple correlation studies were applied to assess the association between the different variables. Nineteen patients presented with abnormal CSF Se concentrations when compared with our reference values established for the different groups of age (group 1: 1–30 days; 1.8–4.7 µg/L; group 2: 1–36 months; 0.68–3.0 µg/L; group 3: 4–18 years; 0.73–2.13 µg/L) associated with different clinical conditions. Ten had decreased CSF Se levels and nine had increased levels. A positive correlation between CSF Se and GPx activity (r = 0.586; p <0.001), as well as with total proteins (r = 0.387; p <0.001) and with albumin (r = 0.898; p <0.001), was observed. Impaired Se concentrations affected 9% of patients with neurologic dysfunction. The positive correlation observed between CSF Se and CSF albumin values suggests that this protein markedly contributes to total Se concentrations. Also, the positive correlation observed between CSF Se values and GPx activity suggests that it may be a biomarker for antioxidant status in the CSF.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"05 1","pages":"015 - 020"},"PeriodicalIF":0.0,"publicationDate":"2015-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0035-1554782","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58070524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Sert, D. Çimen, D. Arslan, E. Aypar, H. Vatansev, F. Kaya, C. Kılıçaslan, E. Aslan, O. Guvenc, F. Gun, B. Oran, D. Odabaş
{"title":"Serum Asymmetric Dimethylarginine Levels in Patients with Acute Rheumatic Fever","authors":"A. Sert, D. Çimen, D. Arslan, E. Aypar, H. Vatansev, F. Kaya, C. Kılıçaslan, E. Aslan, O. Guvenc, F. Gun, B. Oran, D. Odabaş","doi":"10.1055/s-0035-1554781","DOIUrl":"https://doi.org/10.1055/s-0035-1554781","url":null,"abstract":"Abstract Asymmetric dimethylarginine (ADMA) is an analogue of l-arginine, a naturally occurring product of metabolism found in human circulation. It is an endogenous inhibitor of nitric oxide production. Acute rheumatic fever (ARF) is a delayed immunologically mediated autoimmune sequel of throat infection by group A β-hemolytic streptococci. As serum ADMA levels have not previously been assessed in patients with ARF, we aimed to investigate ADMA levels in patients with ARF during the acute stage and after anti-inflammatory treatment and compared results with healthy control subjects. The study population consisted of 34 children with ARF (30 patients with carditis and 4 patients without carditis) and 31 healthy control subjects. Erythrocyte sedimentation rate and C-reactive protein values were significantly higher and serum ADMA values were lower, but not statistically significant in patients with ARF during the acute stage when compared with controls. Serum C-reactive protein and erythrocyte sedimentation rate values were significantly decreased in patients with ARF after the treatment when compared with baseline and ADMA levels were increased after the treatment compared with baseline, but this change was not statistically significant. Our study has demonstrated that resolution of acute inflammation in patients with ARF may lead to a mild increase in serum concentration of ADMA. Comprehensive prospective and observational studies are required to confirm our findings and to assess potential interactions between ARF and ADMA levels.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"05 1","pages":"021 - 027"},"PeriodicalIF":0.0,"publicationDate":"2015-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0035-1554781","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58070457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Alzahrani, N. Mohammed, Humaid Al-Swat, R. Karam
{"title":"Measurement of Serum Osteopontin and Interleukin-17 in Asthmatic Children in Saudi Arabia","authors":"A. Alzahrani, N. Mohammed, Humaid Al-Swat, R. Karam","doi":"10.1055/s-0035-1554779","DOIUrl":"https://doi.org/10.1055/s-0035-1554779","url":null,"abstract":"Abstract Cytokines play an important role in the pathophysiology of asthma by affecting airway remodeling and allergic reactions. We aimed to measure the serum concentrations of osteopontin (OPN) and interleukin (IL)-17 in a group of asthmatic and normal children to test the association of these two cytokines with the severity of asthma in Saudi children. Our study included 50 asthmatic and 50 normal children. Serum cytokines were measured by ELISA technique. Pulmonary function tests were performed in the asthmatic children. Serum levels of OPN and IL-17 were significantly increased in asthmatic children in comparison to control group. Also, the concentrations of OPN and IL-17 were significantly associated with asthma severity. There was a significant positive correlation between OPN and IL-17 levels. In conclusion, our study suggests that OPN and IL-17 may play a role in childhood asthma and are strongly related to the disease severity.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"05 1","pages":"034 - 037"},"PeriodicalIF":0.0,"publicationDate":"2015-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0035-1554779","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58070441","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Persistent Deficiency for 40% of Toddlers Who Were Vitamin D Deficient as Neonates, Which Cannot Be Assessed by Examining Symptoms of Rickets","authors":"P. Hogeman, M. Hoevenaar-Blom, J. Wielders","doi":"10.1055/s-0035-1554731","DOIUrl":"https://doi.org/10.1055/s-0035-1554731","url":null,"abstract":"Abstract We studied a group of 74 toddlers, mean age 16 months, diagnosed with severe vitamin D deficiency at birth (cord blood <20 nmol/L 25OH vitamin D for neonates). Of 74 initially deficient toddlers, 30 did not reach sufficiency at 50 nmol/L level of serum vitamin D, suggesting persistent vitamin D deficiency over on average 16 months. Boys remained deficient more often than girls. Even in severely deficient toddlers (25OH vitamin D <30 nmol/L), no clinical evidence of symptoms of rickets, growth and development retardation, or abnormal serum calcium levels was observed.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"05 1","pages":"012 - 014"},"PeriodicalIF":0.0,"publicationDate":"2015-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0035-1554731","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58070370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Serum Asymmetric Dimethylarginine Levels in Patients with Acute Rheumatic Fever: Inflammation and Endothelial Dysfunction","authors":"Mahboob Alam","doi":"10.1055/s-0035-1554730","DOIUrl":"https://doi.org/10.1055/s-0035-1554730","url":null,"abstract":"Endothelial dysfunction is considered to be an important and early step in the initiation and progression of atherosclerosis. 1 Nitric oxide is produced by the metabolism of L -arginine via endothelial NO synthase (eNOS) to maintain appropriate endothelial function (i.e., intracellular messaging, anti-in-fl ammatory, antithrombotic, and antiapoptotic effects). 1 Serum asymmetric dimethylarginine (ADMA) is an endogenous analog of L -arginine. 2 Studies have found an association between elevated ADMA levels and endothelial dysfunction in humans, which may partly be related to eNOS uncoupling that results in increased eNOS-derived superoxide production in human vessels. 2 Serum ADMA levels have been associated with coronary atherosclerosis in patients with clinical risk factors as well as in healthy individuals. 3 – 5 Plasma levels of ADMA have been associated with mortality and a worsened clinical outcome in diabetic patients with estab-lished coronary atherosclerotic disease. 6 The relationship between serum ADMA levels and acute rheumatic fever (ARF) is not well de fi ned. ARF is a clinical state of heightened in fl ammation driven by an autoimmune reac-tion. Sert and collogues in the accompanying article “ Serum Asymmetric Dimethylarginine Levels in Patients with Acute Rheumatic Fever ” have reported the results of their case control study exploring the relationship between markers of in fl ammation (erythrocyte sedimentation rate [ESR], serum C-reactive protein [CRP] level, serum ADMA) and disease activity. 7 This study in an elegant way has demonstrated (although in a small sample) a statistical association","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"05 1","pages":"001 - 001"},"PeriodicalIF":0.0,"publicationDate":"2015-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0035-1554730","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58070353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Oxidative Stress and Overview of Pediatric Disease Biomarkers","authors":"Ahmet Ayaz","doi":"10.1055/s-0035-1554784","DOIUrl":"https://doi.org/10.1055/s-0035-1554784","url":null,"abstract":"Abstract Free radicals are small molecules enabled to react with other biological molecules. The antioxidant system in metabolism is responsible for balancing antioxidant levels and the amount of free radicals. Excessive free radicals production will result in oxidative stress. A certain amount of reactive oxygen species are required to maintain normal physiological activity. However, elevated oxidative stress levels will damage molecules and produce enzymatic malfunction. Several pediatric diseases are associated with increased oxidative stress. A biomarker is a specific molecule that acts as an indicator for a specific condition. There are some oxidative stress biomarkers currently in uses and further analysis of their application is required. Protein molecules may serve as potential biomarker according to proteomics analysis.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"05 1","pages":"008 - 011"},"PeriodicalIF":0.0,"publicationDate":"2015-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0035-1554784","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58070553","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"New ideas in inborn errors of metabolism in children and infants","authors":"A. Gropman","doi":"10.1055/s-0036-1586479","DOIUrl":"https://doi.org/10.1055/s-0036-1586479","url":null,"abstract":"In order to highlight the importance of inborn errors of metabolism (IEMs) in children and to update the journal readers regarding the state of the art in diagnosis, management and treatment, we have compiled a special issue of the Journal of Pediatric Biochemistry comprising various types of inborn errors of small molecules (organic acidemias), large molecules (lysosomal disorders), mtDNA disorders, and metabolic muscle disease. One additional section is devoted to educate the reader regarding how neuroimaging can be used to study the IEMs. Each expert shares current knowledge with the readers of this journal. This Special Issue will provide information on IEMs from a clinical, diagnostic, biochemical and therapeutic perspective.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"04 1","pages":"181 - 182"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1586479","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58159927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Lysosomal storage disorders","authors":"P. Tanpaiboon","doi":"10.1055/s-0036-1586483","DOIUrl":"https://doi.org/10.1055/s-0036-1586483","url":null,"abstract":"Abstract Lysosomes are cytoplasmic organelles that play a major role in cellular metabolic salvage, necessary for cellular homeostasis. Besides, degrading several macromolecules in metabolic salvage process, lysosomes also involve in several cellular processes e.g. cell apoptosis and intracellular signaling. Lysosomal storage disorder (LSD) is a group of inherited metabolic disorders, which can present at any age from prenatal to adult. Pathology/pathophysiology usually engages several organ systems. Majority of diseases in this group involve neurological system causing neurodegenerative manifestation. Biomarkers are not only available but also useful for disease screening and monitoring. Diagnosis should be confirmed by enzyme analysis and/or molecular analysis. Although treatment is available in some diseases, the outcomes are not favorable in selected patients, especially when present with neurological symptoms. Understanding the complexity of LSD is important for patients' care and development of new treatment. Appropriate genetic counseling should be provided to every patient.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"04 1","pages":"217 - 229"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1586483","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58160132","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Metabolic muscle disorders in infants and children","authors":"C. Rocha","doi":"10.1055/s-0036-1586484","DOIUrl":"https://doi.org/10.1055/s-0036-1586484","url":null,"abstract":"Abstract Metabolic myopathies refer to a group of heterogeneous hereditary muscle disorders associated with known enzymatic defects. These conditions affect the ability of muscle fibers to maintain adequate energy and adenosine triphosphate (ATP) concentrations. Conventionally these diseases are grouped into abnormalities of lipid, glycogen, purine or mitochondrial metabolism. This review will focus on current diagnosis and management of pediatric patients presenting with a suspected metabolic myopathy.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"04 1","pages":"231 - 248"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1586484","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58160148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}