Journal of pediatric biochemistry最新文献

{"title":"The Cause of Pyloric Stenosis of Infancy: Primary Hyperacidity and Biochemistry Combined","authors":"I. Rogers","doi":"10.1055/s-0036-1597606","DOIUrl":"https://doi.org/10.1055/s-0036-1597606","url":null,"abstract":"Abstract The cause of pyloric stenosis (PS) of infancy is at present unknown. A theory of causation is proposed, which is consistent with all the known clinical features of this condition. It is based on the knowledge that babies with PS are hypersecretors of acid, which predates the development of PS and is an inherited constitutional feature. This acidity becomes dangerously high due to a temporary insensitivity of the negative feedback between gastrin and gastric acidy within the first few weeks of life. Normal babies with normal acidity levels will also experience peak acidity at that time but not dangerously so. Acid entering the duodenum causes contraction of the pyloric sphincter. Hyperacidity will naturally lead to repeated pyloric sphincter contractions with resulting work hypertrophy of the sphincter and PS. Should the baby with PS survive beyond the age of approximately 6 weeks, the matured negative feedback between gastrin and acid will ensure that dangerous hyperacidity is kept in check. Thus, the passage of time allows the physiological control of hyperacidity. When associated with an age-related widening of the pyloric canal control, a long-term cure is the natural outcome. This theory explains satisfactorily all the known and hitherto unexplained features of this condition.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"06 1","pages":"146 - 151"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1597606","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"57955871","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":"Association of Thiol Disulfide Homeostasis with Childhood Asthma","authors":"Buket Kaya, M. Aydın, M. Donma, Muhammet Demirkol, C. Bicer, O. Erel","doi":"10.1055/s-0036-1597607","DOIUrl":"https://doi.org/10.1055/s-0036-1597607","url":null,"abstract":"Abstract The aim of this study is to evaluate the oxidative stress level in asthmatic children in terms of the disulfide/thiol ratio, to research the role the disulfide/thiol ratio plays in the pathogenesis of the malady, and to determine the usefulness of the disulfide/thiol ratio as an indicator of the oxidation stress situations in patients with asthma. We report that the disulfide/thiol ratio and disulfide levels were significantly higher in the asthmatic group than in the control group. All in all, high levels of disulfide/thiol ratios in asthmatic children are thought to play roles in the pathogenesis of the disease.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"06 1","pages":"152 - 155"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1597607","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"57955907","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":"Use of Off-Label Targeted Therapies in Refractory Sarcomas: Analysis of Pediatric Data from the French Registry Observatoire de l'Utilisation des Thérapies Ciblées dans les Sarcomes","authors":"N. Garnier, A. Bertrand, C. Libbrecht, N. Corradini, Hélène Pacquement, Jean-Claude Gentet, Cyril Lervat, M. Girodet, L. Bouclier, I. Ray-coquard, P. Marec-Bérard","doi":"10.1055/s-0036-1597609","DOIUrl":"https://doi.org/10.1055/s-0036-1597609","url":null,"abstract":"Abstract Targeted therapies (TT) are used in pediatric patients based on the data from adult literature. In 2008, the French Sarcoma Study Group and the Bone Tumor Group (Groupe Sarcome Français-Groupe d'étude des Tumeurs Osseuses) opened Observatoire de l'Utilisation des Thérapies Ciblées dans les Sarcomes (OUTC'S), a national registry of targeted off-label sarcomas therapies. All patients registered in the OUTC'S database and treated in pediatric oncology units were included in this analysis. We describe TTs using off-label and off clinical trial practices for children with sarcoma. We analyzed TT tolerability and efficacy for 34 patients with osteosarcoma (n = 20), Ewing sarcoma (n = 9), clear cell sarcoma (n = 1), synovialsarcoma (n = 1), epithelioid sarcoma (n = 1), myofibroblastic tumor (n = 1), and desmoid tumor (n = 1) who were registered from six pediatric centers. In total, 38 different TT courses were administered. The median age was 15 years (5–23) and 18.5 years (7–27) at diagnosis and at the time of starting TT, respectively. The decision to initiate TT was taken in a multidisciplinary board in 92% of the cases. TT included sirolimus (alone or in association with other treatments), sunitinib, sorafenib, cetuximab, imatinib, and crizotinib. The median duration of treatment was 109 days (21–515). Of the 34 patients, 6 had a partial response with a median response duration of 3.72 months (2.08–30.8) and 10 had a stabilization of the disease with a median duration of 3.63 months (0.75- 16.89). In our cohort, overall survival and progression-free survival were 8.68 months (95% confidence interval [CI]: 5.85–11.50) and 3.29 months (95% CI: 2.69–3.88), respectively. Grades 3 and 4 toxicities were reported for seven patients (26%) and were most commonly hematological. Patients under 15 years of age did not show severe toxicity. Hence, TT is an acceptable therapeutic option for refractory pediatric sarcomas. It is very important to continue collecting data and develop phase I/II protocols.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"06 1","pages":"136 - 145"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1597609","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"57955982","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":"The Postnatal Growth Delay in Preterm Infants and Survey of the Risk Factors","authors":"O. Starets, T. Khimenko, V. Mykhaylenko","doi":"10.1055/s-0036-1597815","DOIUrl":"https://doi.org/10.1055/s-0036-1597815","url":null,"abstract":"Abstract The aim was to analyze the factors that are associated with growth delay (GD) and malnutrition in preterm infants during the first year of life. The trial included 150 preterm children. The data of life history and anthropometry were studied. The trial showed statistically significant correlation of GD in preterm babies with intrauterine growth restriction, prolonged respiratory care and tube feeding, formula feeding, anemia of prematurity, and severe asphyxia at birth. To optimize health care in preterm infants, it is necessary to consider the factors that correlate with GD and give the recommendations how to prevent or treat pathological and deficit conditions.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"22 1","pages":"156 - 158"},"PeriodicalIF":0.0,"publicationDate":"2016-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1597815","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"57956080","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":"Laboratory medicine in pediatric lupus","authors":"A. Cava","doi":"10.3233/JPB-2010-0008","DOIUrl":"https://doi.org/10.3233/JPB-2010-0008","url":null,"abstract":"Pediatric lupus encompasses a broad variety of clinical manifestations of a systemic disease. Routine laboratory testing, autoantibodies, and complement levels can confirm the diagnosis and help monitoring and therapy of the disease, to ultimately improve outcomes. Additionally, tests can help to identify subsets of patients with specific risk factors and/or the involvement of selected organs/systems. The availability, refinement and accuracy of laboratory tests are instrumental to improve the diagnosis, treatment and prognosis of pediatric lupus patients.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"01 1","pages":"045-051"},"PeriodicalIF":0.0,"publicationDate":"2016-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.3233/JPB-2010-0008","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"70093922","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":"Fetal Programming, Maternal Nutrition, and Oxidative Stress Hypothesis","authors":"S. Perrone, M. Tataranno, A. Santacroce, Carlotta Bracciali, M. Riccitelli, M. Alagna, M. Longini, E. Belvisi, F. Bazzini, G. Buonocore","doi":"10.1055/s-0036-1593811","DOIUrl":"https://doi.org/10.1055/s-0036-1593811","url":null,"abstract":"Abstract Fetal programming occurs when the normal pattern of fetal development is disrupted by an abnormal stimulus or an “insult” during intrauterine life, which leads to adaptations by the fetus to allow its survival but could finally result in permanent structural and physiological changes with long-term consequences in adulthood. The availability of nutrients, hormones, and respiratory gases is the principal determinant of fetal growth and offspring's subsequent health. Fetal nutrient and oxygen availability depend on the rate of transfer across the “placental barrier.” Nutritional status of the mother is also important: both maternal undernutrition and/or overnutrition during early gestation may increase the incidence of cardiovascular and metabolic disorders in the offspring in later life. Oxidative stress has been supposed to be the link between adverse intrauterine environment and later elevated risks of chronic diseases. It is an important initiating mechanism underlying the programming process due to suboptimal nutrition. Antioxidant vitamins, proteins, and trace elements can be compromised under condition of poor maternal nutrition leading to oxidant/antioxidant imbalance during pregnancy. On the other hand, maternal overnutrition is associated to chronic inflammatory states that increase free radicals' production. Developing dietary strategies to optimize maternal nutrition is necessary to supply the fetus with appropriate substrates and to avoid fetal redox status disruption.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"06 1","pages":"96 - 102"},"PeriodicalIF":0.0,"publicationDate":"2016-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1593811","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58177936","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":"Neonatal Brain Hemorrhage: The Role of NonProtein-Bound Iron","authors":"S. Perrone, S. Negro, M. Tataranno, A. Santacroce, Carlotta Bracciali, M. Longini, F. Proietti, F. Bazzini, E. Belvisi, G. Buonocore","doi":"10.1055/s-0036-1593755","DOIUrl":"https://doi.org/10.1055/s-0036-1593755","url":null,"abstract":"Abstract Intraventricular hemorrhage (IVH) in very preterm infants is a common disease, which can induce long-term consequences. Oxidative stress (OS) occurs easily in preterm newborns due to an imbalance between high free radical (FR) production and the low antioxidant shield, not completely developed at birth. Nonprotein-bound iron (NPBI) concentration in cord blood has been found to be highly predictive for the risk of poor neurodevelopmental outcome. However, at present, no data exist about the exact mechanisms associated with IVH induced by iron-mediated FRs. We propose the hypothesis that hypoxia or ischemia-induced releasing of NPBI is a key regulating event that initiates a vicious circle of excessive FR generation, which in turn participates in edema development, inflammatory reaction, and endothelial injury. This suggests that developing effective neuroprotective strategies for preterm infants requires a detailed understanding of OS reactions and glial responses.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"06 1","pages":"88 - 91"},"PeriodicalIF":0.0,"publicationDate":"2016-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1593755","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58178040","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":"Antioxidant Effects of Lutein in Neonatal Period","authors":"M. Tei, G. De Bernardo, F. Bazzini, A. Santacroce, E. Belvisi, M. Riccitelli, Carlotta Bracciali, G. Buonocore, S. Perrone","doi":"10.1055/s-0036-1593812","DOIUrl":"https://doi.org/10.1055/s-0036-1593812","url":null,"abstract":"Abstract During fetal life, a right balance between oxidants and antioxidants is required for survival and growth of the fetus. At birth, an overproduction of free radicals (FRs) together with poor antioxidant defenses may be detrimental for developing organs and tissues. Protecting the newborn infant against perinatal oxidative stress (OS) is a health care priority, and therefore the search for new, safe, and efficacious antioxidants has been a major quest during the last decade. Due to its antioxidant and anti-inflammatory properties, lutein, a compound belonging to the xanthophyll family of carotenoids, is one of the most promising molecules with clinical application during neonatal age. Lutein is not synthesized by humans, hence the intake primarily depends on diet. In the neonatal period, fresh human milk is the main source of lutein, though lutein-enriched infant formulas are now available. Oral supplementation represents an alternative source of lutein, and it has been demonstrated to decrease plasma biomarkers of OS and increase antioxidant capacity in the first days of life.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"06 1","pages":"110 - 113"},"PeriodicalIF":0.0,"publicationDate":"2016-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1593812","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58177948","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":"Diabetes or Obesity in Pregnancy and Oxidative Stress in the Offspring","authors":"A. Santacroce, G. Bernardo, S. Negro, Carlotta Bracciali, M. Alagna, M. Tei, F. Bazzini, E. Belvisi, G. Buonocore, S. Perrone","doi":"10.1055/s-0036-1593759","DOIUrl":"https://doi.org/10.1055/s-0036-1593759","url":null,"abstract":"Abstract During normal gestations, oxidant molecules have many physiological functions, which are summarized in controlling cellular fate and signaling, thus playing a crucial role in pregnancy development. Oxidative stress (OS) arises when the production of reactive oxygen species (ROS) overwhelms the intrinsic antioxidant defenses. OS is implicated in the pathophysiology of many complications of human pregnancy, such as gestational diabetes mellitus (GDM), in which both free-radical production and antioxidant defenses are disrupted. The mechanisms of such kind of relation between OS and gestational diabetes have been analyzed during the years. Hyperglycemia leads to an increased production of ROS through different metabolic pathways. Thus, when ROS production increases, transcription factors (TFs) such as nuclear factor-κB will become activated and lead to insulin resistance due to the impairment of insulin signaling. Moreover, TFs may also induce proinflammatory cytokine expression, such as interleukin-6, tumor necrosis factor-α, and monocyte chemoattractant protein-1, which are able to cause insulin resistance directly or indirectly. Placental tissue, fetuses and preterm infants are greatly susceptible to OS damage because of the organ and functional immaturity. ROS were first suggested to be related to diabetes-induced teratogenicity by Eriksson and Borg. Indeed, during the period of organogenesis, ROS attack on DNA represents the first step involved in mutagenesis, carcinogenesis, and fetal programming. Therefore, pregnancies complicated with GDM have miscarriage, PROM, and other anomaly rates higher than nondiabetic gestation. Furthermore, children of GDM mothers are at high risk to develop obesity and type 2 diabetes later in life. The management of OS, along with tight glycemic control, could be beneficial, both preconceptionally and during pregnancy, in women with GDM. However, whether an antioxidant supplementation or a diet rich in antioxidants can prevent the consequences of OS in the offspring or not is yet to be elucidated.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"50 1","pages":"92 - 95"},"PeriodicalIF":0.0,"publicationDate":"2016-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1593759","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58178122","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":"Enzyme Activities in Erythrocytes of Term and Preterm Newborns","authors":"E. Belvisi, Carlotta Bracciali, M. Ognean, M. Tei, S. Negro, F. Carra, F. Proietti, G. Buonocore, S. Perrone","doi":"10.1055/s-0036-1593814","DOIUrl":"https://doi.org/10.1055/s-0036-1593814","url":null,"abstract":"Abstract The decrease of erythrocyte antioxidant enzyme activity should be considered as a possible cause of hemolysis when there is no evidence of an immune-mediated hemolytic anemia, no consumptive red blood cell disorder, no morphologic or laboratory data to suggest a problem of the red cell membrane, and no evidence of a quantitative or qualitative defect in hemoglobin synthesis. Glutatione has a pilot role in orchestrating the redox balance when there is an excess reactive oxygen species production or defective antioxidant defenses that may have deleterious consequences in various perinatal diseases. Neonatal erythrocytes are a target of extracellular free radicals and in the same time generators of free radicals through Fenton reaction. The complex mechanism underlying the increased oxidative stress susceptibility of neonatal erythrocytes requires further investigation.","PeriodicalId":89425,"journal":{"name":"Journal of pediatric biochemistry","volume":"06 1","pages":"114 - 118"},"PeriodicalIF":0.0,"publicationDate":"2016-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-0036-1593814","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58178007","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}