bluuebunny/crossref_metadata_2025 huggingface dataset:
Estimation of the relative DNA content in species of the genus Spiraea, sections Chamaedryon and Glomerati by flow cytometry
Authors: Kostikova V. A. , Voronkova M. S. , Mitrenina E. Yu. , Kuznetsov A. A. , Ernst A. S. , Veklich T. N. , Shabanova(Kobozeva) E. V.
Published: December 2019

The relative DNA content was studied in seven species of the genus Spiraea L., section Chamaedryon Ser., and in two species, section Glomerati Nakai, from 28 natural populations growing in Asian Russia. The cell nuclei were isolated from a leaf tissue. The relative intensity of fluorescence was measured using flow cytometry of propidium iodide-stained nuclei. The analysis was performed using a CyFlowSpace device (Germany, Sysmex Partec) with a laser radiation source of 532 nm. Fresh leaves of Solanum lycopersicum cv. ‘Stupice’ were used as an internal standard. Data on the relative DNA content are presented for the first time for S. flexuosa Fisch ex Cambess. (0.42–0.47 pg), S. ussuriensis Pojark. (0.49–0.52; 0.85 pg), S. alpina-Pall. (0.49–0.51 pg), S. media Schmidt. (0.45; 0.98–1.01 pg), S. trilobata L. (0.46 pg), S. hypericifolia L. (0.49–0.52 pg) and S. aquilegifolia Pall. (0.48–0.51 pg). Mesophytic species of the genus Spiraea growing in the forest zone (S. chamaedryfolia L. and S. media) exhibit a 2-fold higher relative DNA content (C-value) compared to xerophytic species (S. hypericifolia, S. crenata L., S. aquilegifolia and S. trilobata) and species confined only to mountain areas (S. alpinа, S. trilobata and S. aquilegifolia).
Ecological substantiation of the normalization of the state of the air environment in the uninsulated barn in the hot period
Authors: Mylostyvyi R. V. , Chernenko O. M. , Izhboldina O. O. , Puhach A. M. , Orishchuk O. S. , Khmeleva O. V.
Published: December 2019

The results of the assessment of the temperature-humidity regime in the modern uninsulated barn in the summer period with external temperature fluctuations from +16.6°С to +37.2°С are presented. It was established that the indoor climate was significantly related to the state of the environment (r=0.95; p 0.001). Significant differences were found in terms of the temperature-humidity index (1.6–5.1 units) in different parts of the building, which may affect the state of comfort of the animals. The use of additional (except natural) ventilation in order to normalize the air environment should be carried out taking into account the time of day and the area of the room.
Adoption and intensity of adoption of beekeeping technology by farmers: The case of Sheko Woreda of Bench-Maji Zone, South West Ethiopia
Authors: Albore A. , Anshiso D. , Abraham D.
Published: December 2019

Beekeeping delivers the rural people with sources of income, dietary use, pollination service and social benefit. In spite of the benefits obtained from beekeeping, farmer’s resistivity to adopt the recommended modern beekeeping technological components (packages) is among the major problem. Therefore, the objectives of this study were to examine the intensity of adoption of beekeeping technology package, and identify factors influencing adoption and intensity of adoption of beekeeping technology package in Sheko district. Stratified sampling procedure was used to select sample households. A total of 200 household heads were selected randomly from adopters and non-adopters categories of four potential beekeeping localities. Date was collected by using household survey and key informant interview and analyzed by using censured Tobit model. From sampled households 68% of respondents who have bee hive; the majority of them fall under medium adopters’ category. Variations in intensity of adoption of bee hive components were observed among adopter category. The output of censured Tobit model was indicate that six variables such as total livestock unit, apiary site visit by farmer, perceived cost of technology by farmer, distance of market center from home, knowledge of farmers for bee management and availability of bee hive technology were found to have significant influence on adoption and intensity of adoption of bee hive technological package. Therefore, agricultural policy and development interventions should give emphasis to the improvement of institutional support system so as to achieve wider adoption and intensity of adoption for small scale farmers.
Industrial characteristics and consumer properties of Chaenomeles Lindl. fruits
Authors: Lykholat Y. V. , Khromykh N. O. , Lykholat T. Y. , Didur O. O. , Lykholat O. A. , Legostaeva T. V. , Kabar A. M. , Sklyar T. V. , Savosko V. M. , Kovalenko I. M. , Davydov V. R. , Bielyk Yu. V. , Volyanik K. O. , Onopa A. V. , Dudkina K. A. , Grygoryuk I. P.
Published: December 2019

The comparative analysis of the fruit and seed capacity of Chaenomeles Lindl., grew in the botanical garden of the Oles Honchar Dnipro National University, was carried out. It is shown that all studied taxa undergo a complete vegetation cycle. According to our observations, the duration of the growing season is as follows: the smallest in Chaenomeles japonica, the longest in C. × superba and C. maulei, the longest in C. speciosa, C. cathayensis and C. californica. Seed length in Chaenomeles plants ranged from 4.2 ± 0.10 mm (C. japonica var. maulei) to 8.5 ± 0.25 mm (C. сathayensis). Seed widths varied from 4.1 ± 0.32 mm (C. cathayensis) to 11.1 ± 0.10 mm (C. japonica var. maulei). According to the results obtained, the average number of seeds in one fruit of different Chaenomeles species differed, decreasing in the following order: C. cathayensis, C. speciosa > C. × californica > C. × superba > C. japonica > C. japonica var. maulei. Most of the Chaenomeles genus representatives were found to be in a good condition: C. japonica var. maulei vitality was estimated at 7 balls, C. japonica and C. × superba – 6 balls. This index is slightly lower for C. speciosa, C. × californica and C. cathayensis as 5 and 4 balls, respectively. It should be noted that the highest vitality was shown by C. japonica var. maulei, which was introduced in the Botanical Garden of DNU in 1955. In research, considerable attention has been paid to the antioxidant system, which is a powerful mechanism preventing the development of avalanche-free and radical peroxide reactions in living organisms. The antioxidant capacity of Chaenomeles fruits, determined in the range from 565.8 ± 15.7 (C. speciosa) to 1121.7 ± 27.5 (C. cathayensis) mg UAE / 100 g DW, can be considered quite high. The overall antioxidant capacity was highest for C. cathayensis fruits (1121.7 ± 27.5 mg AE / 100 g DW), which exceeded the indexes of other species by 1.2–2.0 times. The presence of antioxidants in raw materials and finished products provides for the prevention of their deterioration, reduction of losses, increase the shelf life and release of high quality products, retaining for a long time the characteristic features inherent in fresh, complete products. High taste qualities are characteristic of C. speciosa and C. × californica. Considering the large assortment of Chaenomeles, representatives differing in their biological, physiological and nutritional properties, we consider it promising to introduce their use in the food industry to obtain functional products with high consumer properties, namely, high content of antioxidants, catering for a variety of dishes or for exotic flavors, as well for direct consumption by the population of useful fruits with different flavors.
The effect of cadmium loading on protein synthesis function and functional state of laying hens’ liver
Authors: Gutyj B. , Ostapiuk A. , Kachmar N. , Stadnytska O. , Sobolev O. , Binkevych V. , Lutytska N. V. , Petryshak R. , Petryshak O. , Kulyaba O. , Naumyuk A. , Nedashkivsky V. , Nedashkivska N. , Magrelo N. , Golodyuk I. , Nazaruk N. , Binkevych O.
Published: December 2019

In the article are considered issues related to the study of the effect of cadmium on the body of birds, in particular laying hens. The effect of cadmium sulfate at doses of 2.0 and 4.0 mg/kg body weight on protein synthesis function and functional state of the liver were investigated of cross-laying Hysex white hens, of 78 weeks old. Three groups of chickens were formed: two experimental and one control groups. The laying hens of the experimental groups were subjected to a cadmium load. Laying chickens of the first experimental group were given cadmium sulfate at a dose of 2.0 mg/kg body weight. Laying chickens of the second experimental group were given cadmium sulfate at a dose of 4.0 mg/kg body weight. Laying chickens in the control group were in the normal diet without cadmium. Increased activity of aminotransferases, in particular alanine and aspartate aminotransferases in their serum of hens, was observed during cadmium binge drinking in doses of 2.0 and 4.0 mg/kg body weight, respectively. These changes indicate a violation of the functional state of the liver of laying hens under conditions of cadmium load. In the study of protein synthesis of the liver of laying hens under conditions of cadmium loading, it was found that cadmium sulfate in the above doses contributed to a significant decrease in the level of total protein by 14, 21 and 30 days of the experiment. The reduction of total protein in the blood of laying hens was due to a decrease in the level of albumin, which in the second experimental group, respectively, decreased to 28.16 ± 0.75%, whereas in the first – 29.62 ± 0.98%. In the study of the level of globulins, it is possible to increase this indicator in both experimental groups of poultry for 21 days. Drinking with water cadmium sulfate at a dose of 4.0 mg/kg body weight was associated with a more likely decrease in total protein and albumin than drinking cadmium sulfate at a lower dose.
Dynamics of macrozoobenthos in the Eastern Sivash during different salinity regimes
Authors: Antonovskiy A. G. , Degtyarenko E. V. , MArushkina E. A. , Matsyura A. V.
Published: December 2019

In current paper we presented the dynamics of the macrozoobenthos in Eastern Sivash depending on temporal and spatial salinity fluctuations from pleiomesohaline to polyhaline and hyperhaline. We shown that the highest species richness and biomass were observed in polyhaline conditions at the lowest salinity values, and the maximum density was in pleiomesohaline conditions. The lowest species riches and biomass were observed in hyperhaline conditions.
Soil acid-base buffering in the step agriculture lands
Authors: Ozhovan O. O. , Mikhaylyuk V. I.
Published: December 2019

Modern studies of chernozem soils in the northwestern Black Sea region have shown intense dehumification due to disruption of biogeochemical cycles caused by anthropogenic removal of organic matter and associated biophile elements. At former time, the humus zoning was typical for southern chernozems and now we noted the smoothing of this zoning. Acid-base buffering has a significant role in counteracting degradation processes, the buffering parameters could be integral indicators of soil chemicals balance. We studied the automorphic soils (arable chernozems) of the northwestern Black Sea region, their humus state, particle size distribution, and acid-base buffering parameters. We studied ordinary modal and micellar-carbonate chernozems on arable land and 40-year-old fallows, southern chernozems removed from irrigation 15 years ago and southern carbonate chernozems of the second floodplain terrace of the Danube River. We determined the neutralization index, degree of buffering capacity in the acid and alkaline ranges, equilibrium coefficient, and sodium absorption ratio to characterize the acid-base buffering. The studied soils belong to stable buffer agriculture lands by their acid-base buffer ability. We determined that soils have the parameters of chernozem type: low humus content, humate and humate-fulvate type of humus, average content of insoluble residue, high degree of humification, heavy-medium loam particle size distribution, and neutral soil solution. The acid-base buffer capacity is characterized by average values, the buffer capacity in the acid interval increases with depth, while decreases in the alkaline interval. We revealed a significant correlation between the ability to counteract the acid load and the content of physical clay. We believe that the agricultural use of southern chernozems leads to a decrease in soil resistance to acidification.
Migration of antibiotics residual quantities in aquatic ecosystems
Authors: Voitsitskiy V. M. , Danchuk V. V. , Midyk S. Vu. , Kepple O. Yu. , Danchuk Z. V. , Shevchenko L. V.
Published: December 2019

The uncontrolled antimicrobial agents use leads to the spread of antibiotic-resistant pathogenic strains. The ingress of antibacterial drugs into surface water through sewage from livestock premises, people's houses, hospitals and during medical and agronomic activities exacerbates this problem. Antimicrobials in surface waters have several ways of developing the process: lethal interaction of antibiotics with hydrobionts, elimination of sensitive taxa and alteration of trophic chains; neutralization of antimicrobials (self-destruction of the molecule, hydrolysis, sorption, abiotic and biotic transformation); non-lethal interaction of antibiotics and hydrobionts with the acquisition of antibiotic resistance signs). There are following indicators proposed to predict the antimicrobial agents impact on the aquatic ecosystem: accumulation factor; the amount of substance per unit mass of plant or animal in one cubic meter of water (mg or mcg per 1m3); the maximum amount of a substance that can be contained in an ecosystem without disturbing its basic trophic properties. The basic principles of a dynamic chamber model construction for studying the effect of antibiotics on the ecosystem are based on the following basic statements: 1) the trophic chain is divided into the chambers in which substance is instantaneously mixed in all parts of the chamber in the same way in any direction; 2) the transfer of the substance from one chamber to another occurs according to the laws of first order kinetics, which is described by the system of differential equations. In this case, transition coefficients of a substance between the chambers are constant. It is advisable to use a dynamic chamber model to analyze the migration pathways of substances in a freshwater non-flowing reservoir. For instance, its simplified version consists of the 3 chambers, namely: water – sediments (silt) – biota. It is advisable to use a stationary chamber model for analysing the pathways of antibiotics which enter into the sea with the river flows.
The contents of artificial and natural radionuclides in tissues of the Percidae fish from the Dnipro Reservoir
Authors: Ananieva T. , Shapovalenko Z.
Published: December 2019

The contents of artificial and natural radionuclides of 137Cs, 90Sr, 226Ra, 232Th, and 40K had been investigated in muscle and bone tissues, gill petals, and scales of common perch Perca fluviatilis and pikeperch Sander lucioperca from two fishing areas of the Dnipro (Zaporizhzhia) Reservoir. The specific radionuclide activities were measured with the SEG-001 “AKP-C” and SEB-01-150 scintillation spectrometers of gamma- and beta-radiation energy in Bq/kg of wet weight. The radioactive elements of both natural and artificial origins were concentrated greatly in common perch tissues compared to pikeperch. The bone and muscle tissues contained the maximal levels of radionuclides, while respectively smaller amounts of radionuclides were found in gills and scales. The radionuclide levels in the tissues of investigated fish species were significantly higher in the Dnipro Reservoir area with weak flowage and slow water exchange.
Determining the relationship between energy consumption and economic growth in Pakistan
Authors: Ali F. , Khan Kh. A. , Raza A.
Published: December 2019

Energy is substantial for economic development. This study aims to unveil the causal relationship and long-term association between economic growth and energy consumption in Pakistan. The Granger-Causality test finds that; natural gas consumption, electricity consumption and coal consumption have uni-directional causal relationship with economic growth as (GC, EC and CC→GDP), however, GDP growth rate, natural gas consumption and coal consumption unilaterally Granger causes Inflation (GDP, GC and CC→CPI) and lastly coal consumption→natural gas consumption (GC), Electricity consumption (EC)→GC. The ARDL estimations delineate natural gas consumption and oil consumption having a positive and negative association with GDP growth rate may have significant long term impacts respectively on the the economic growth of Pakistan.
Peculiarities of seasonal changes of taxonomic structure and number of weevil-beetles (Coleoptera, Curculionidae) of herptobios in Kharkiv urbocenoses (Ukraine)
Authors: Komaromi N.A. , Putchkov A.V. , Nazarenko V.Yu.
Published: December 2019

A total of 54 species in 35 genera of Curculionidae are registered in the urbocenoses of Kharkiv city. Only three species, Exomias pellucidus, Otiorhynchus raucus and Ot. ovatus, are recorded as dominants. Some other species (Urometopus nemorum, Sciaphobus squalidus and Glocianus punctiger) are sporadically noted in some sites as subdominants. The total number of species turned out to be higher in the herpetobios of plantings at periphery (31), slightly lower in private farmlands of the city (21), parks and plantings of the center (17 each), but minimal in suburban forests (11 species). The highest quantity of specimens were observed for the plantations of the center and margins of city: nevertheless, minimal quantity of specimens was registered at parks and woods. The maximum number of species (including all cenoses) was recorded from the end of April to first decade of May (28 species). From May to the end of June 15–18 species were registered. Ten species were recorded at July, and only six species at August. A slight rise was noted from the end of August to mid-September (11 species). In biotopes where Exomias pellucidus appeared to be the monodominant, the seasonal dynamic density of weevils reached a peak at late May or early June, but the significant decline was observed from early June to July. At the sites with several dominants, two or three peaks were registered: at spring (May) and at mid-summer (end of June or beginning of July). Furthermore, the number of weevils decreased gradually, but some species of Curculionidae were rather abundant even from the end of summer to September–October. The spring increase is reasoned by high density of species in the genus Otiorhynchus; the peak in early summer (maximum) is caused by the increased activity of majority of dominant species; the autumn peak is also caused by the high number of species of the genus Otiorhynchus. The level of sex index (by the example of E. pellucidus) differed significantly at all plots. It was higher for the plantations at the periphery of the city (0.70), and it was minimal at private farmlands (0.20). Relative conjugacy of sex index and dynamic density were not observed: the maximum abundances of quantity of Curculionidae were recorded 20–30 days earlier than the maximal numbers of the sex index.
Májátültetés Wilson-kóros betegekben, 1996–2017
Authors: Németh Dániel , Folhoffer Anikó , László Szilvia Bianka , Kóbori László , Görög Dénes , Fehérvári Imre , Fazakas János , Gerlei Zsuzsanna , Máthé Zoltán , Szalay Ferenc
Published: December 2019

Absztrakt:

Bevezetés: A Wilson-kór a rézanyagcsere ritka, kezelés nélkül
fatális, öröklődő megbetegedése. Bár a diagnosztika és a kezelés jelentős
fejlődésen ment át az elmúlt években, számos beteg esetében ma is májátültetésre
van szükség. Célkitűzés: A vizsgálat célja a Magyarországon
Wilson-kór miatt májátültetésen átesett betegek adatainak összegyűjtése és
feldolgozása volt. Módszer: Retrospektív módon vizsgáltuk a
Semmelweis Egyetemen 1996 és 2017 között Wilson-kór miatt májátültetésen átesett
24 beteg adatait. A Wilson-kór diagnózisa minden esetben a nemzetközi, lipcsei
pontrendszeren alapult. A heveny májelégtelenség felállításához a King’s
College-kritériumrendszert használtuk. A májátültetések a Semmelweis Egyetem
Transzplantációs és Sebészeti Klinikáján történtek, első alkalommal 1996-ban.
Eredmények: Az átlagéletkor 26 év volt, a nő/férfi arány
13/11. 12 beteg heveny májelégtelenség miatt, 12 beteg dekompenzált cirrhosis
miatt esett át májátültetésen. Egy beteg krónikus rejekció miatt
retranszplantációra került. Három heveny májelégtelen beteg az Eurotransplant
segítségével kapott új májat. A várólistán eltöltött átlagos idő 3 nap volt a
heveny májelégtelen betegek, míg 320 nap a dekompenzált májbetegek esetében. Az
ötéves túlélés 66% volt, azonban a 2002 után transzplantáltak esetében 80%, ami
a tanulási folyamatot és a májátültetés elérhetőségének javulását jelezheti. A
diagnosztika nehézségei ellenére a betegek többségében (21/24 beteg) már a műtét
előtt ismert volt a Wilson-kór. Következtetések: Bár a
Wilson-kór diagnosztikája és kezelése jelentős fejlődésen ment át az elmúlt évek
során, ma is számos esetben van szükség májátültetésre. A betegek megfelelő
kiválasztása és a transzplantáció időzítése jelentősen javítja a betegek
túlélését. Orv Hetil. 2019; 160(51): 2021–2025.
A cukorbetegség és a daganatos thrombocytosis túlélésre gyakorolt hatásának vizsgálata emlőtumoros nőkben
Authors: Somogyi Anikó , Herold Magdolna , Lohinszky Júlia , Harsányi László , Herold Zoltán
Published: December 2019

Absztrakt:

Bevezetés: Számos daganatban igazolták, hogy mind a
thrombocytosis, mind a 2-es típusú cukorbetegség negatívan befolyásolja a
tumoros betegek túlélését. A két tényező szerepét együttesen emlőtumorokban
eddig még nem vizsgálták. Célkitűzés: Vizsgálatunk célja a
Semmelweis Egyetem II. sz. Belgyógyászati Klinika, illetve az I. sz. Sebészeti
Klinika szakambulanciáin 2014−2017 között emlőtumor miatt kezelt
betegpopulációban a thrombocytosis és a 2-es típusú diabetes gyakoriságának és a
daganatos betegség kimenetelére gyakorolt önálló és együttes hatásának felmérése
volt. Vizsgáltuk az emlőtumor diagnózisakor a laboratóriumi és anamnesztikus
adatokat a cukorbeteg és nem cukorbeteg csoportokban, túléléselemzéssel a
thrombocytosis és/vagy a 2-es típusú cukorbetegség túlélést befolyásoló hatását.
Módszer: Retrospektív vizsgálatunkban 274, emlőtumoros
beteg adatait elemeztük. A betegeket legkésőbb 2018. 12. 31-ig, az utolsó
egyetemi megjelenésükig vagy haláluk bekövetkeztéig követtük.
Eredmények: Emelkedett thrombocytaszámokat (400 G/l
feletti) a betegek ~5%-ában figyeltünk meg. A betegek közel ötöde 2-es típusú
cukorbeteg volt (52 fő). A cukorbetegek szignifikánsan idősebbek voltak a tumor
felismerésekor (nem cukorbeteg: 56,8 ± 13,8 év, cukorbeteg: 67,8 ± 11,0 év,
p<0,0001). A nem cukorbetegeknél gyakoribb a tripla negatív (p = 0,0366),
illetve a T1-es stádiumú tumor (50%), míg cukorbetegekben a T2-es stádium a
leggyakoribb (51,9%). A túlélési modell alapján az emlőtumoros betegek rövidebb
túlélési idejét prognosztizálja, ha 2-es típusú cukorbetegségben is szenvednek
(p = 0,0032). Vizsgálatunkban a betegek túlélését a thrombocytosis nem
befolyásolta. Következtetés: A 2-es típusú cukorbetegekben az
emlőtumor diagnosztizálásakor súlyosabb klinikai stádium valószínűsíthető, és
rövidebb túlélés prognosztizálható. Javasoljuk, hogy a cukorbeteg nők
kontrollvizsgálata alkalmával a nők figyelmét fel kell hívni a mammográfiai
szűrővizsgálatok fontosságára, illetve 30 éves kor felett a diabetest
rizikótényezőnek kell tekinteni, és a betegek szűrővizsgálata legalább kétévente
javasolt. Orv Hetil. 2019; 160(51): 2012–2020.
A temática HIV/AIDS e os medicamentos antirretrovirais no Ensino Médio: o entretecer da educação sexual e o ensino de química
Authors: Máximo Junior Nilton , Orlandi Renata , Brondani Patrícia Bulegon , Oliveira Aldo Sena
Published: December 2019

Há um expressivo aumento de infecções pelo vírus HIV na juventude, portanto abordar a temática HIV/AIDS é essencial no contexto educacional brasileiro. O objetivo deste trabalho foi promover reflexões acerca da temática HIV/AIDS por meio de uma sequência didática voltada à educação sexual no contexto do ensino de química. O projeto foi desenvolvido com 22 estudantes do terceiro ano do Ensino Médio, no período noturno. Dividiu-se a sequência didática em três etapas: (i) a descrição escrita de uma pessoa soropositiva e a exibição do documentário “Positivas”; (ii) uma aula com uma atividade experimental sobre o ciclo viral e; (iii) uma aula sobre os medicamentos antirretrovirais. Como resultado, possibilitou aos estudantes um espaço para reflexões e discussões ao estigma atrelado à soropositividade, questões sobre a sexualidade e a identificação da importância do conhecimento de química para a produção dos medicamentos antirretrovirais.
Launching the Journal of International Child Neurology Association (JICNA)
Authors: Newton Charles R.J.C , Hameed Biju A
Published: December 2019

The International Child Neurology Association has started a peer-reviewed child neurology journal, the Journal of International Child Neurology Association (JICNA). The Journal website at http://jicna.org is up and running, and journal has already been receiving submissions. We would like to thank the brave authors who have submitted their initial papers to JICNA. The papers will be deposited on the web, and is currently indexed in CrossRef, Google Scholar and Directory of Open Access Journals (DOAJ).All manuscripts submitted to JICNA will undergo full peer review and fast publication. JICNA implements a continuous publication workflow, from 2013 where full citation details are provided for all published articles from the first date of publications whilst maintaining the archives in a number of issues per volume. Under the current licence scholars, scientists, government officials, and the general public can view, download, print and redistribute any article without a subscription. At present JICNA does not charge any article processing and publication fees.In addition the content in JICNA will also be integrated into ICNApedia (http://icnapedia.org) the Child Neurology Knowledge Environment published by the International Child Neurology Association (ICNA).  This would allow readers to access the interesting and useful material on this platform. We aim to get the Journal indexed in Medline within the year and also deposit in other repositories including PubMed Central. In addition there are robust arrangements in place to ensure permanent preservation of, and access to, all content published. But to achieve these goals, the journal needs to be in existence for more than a year and published at least thirty peer-reviewed papers.JICNA will only flourish if it receives papers to publish, and is read and referenced by child neurologists all over the world. We have started and now we look to the ICNA membership to contribute to this journal, and be involved in a new and exciting publication in child neurology helping to disseminate current research as widely as possible. Please start submitting your papers to JICNA via the website: http://jicna.org/index.php/journal/about/submissions#onlineSubmissionsYou will need to register as an author, and follow the author guidelines for submission. If you having any difficulties please contact either of us directly. We look forward to receiving your manuscripts.
Carpal tunnel syndrome in pediatric mucopolysaccharidoses
Authors: Jadhav Trupti Mangesh , Kornberg Andrew J , Peters Heidi , Lee Joy , Ryan Monique M
Published: December 2019

BackgroundCarpal tunnel syndrome (CTS) is rare in children but is a recognised complication of the mucopolysaccharidoses (MPS). Clinicians should have a low threshold of suspicion for CTS in this group as symptoms may be atypical or minimal. If untreated, CTS can cause significant loss of hand function. We present findings in 11 children with mucopolysaccharidoses and suspected CTS, and propose guidelines for screening for CTS in children with these disorders.MethodsClinical and electrodiagnostic data of 11 children with mucopolysaccharidoses, who were suspected on clinical grounds to have CTS, was reviewed. All subjects underwent motor and sensory conduction studies of bilateral median and ulnar nerves. The presence of carpal tunnel syndrome and its severity was determined. Subsequent details of intervention(s) and recurrence were noted.ResultsThree children had MPS I, five had MPS II, one had MPS III and two had MPS IV. Seven had motor and three sensory features referable to median nerve compression. Nine of the eleven children (2/3 with MPS I, 5/5 with MPS II, 0/1 with MPS III, 2/2 with MPS IV) had median neuropathies at the wrist, (eight bilateral, one unilateral) which were mild in three, moderate in five, and severe in one. Three children presented with symptoms at five years age. Six underwent median nerve decompression. Four of these had recurrent symptoms several years after surgery, which was confirmed on nerve conduction studies in two cases. To the best of our knowledge, this is the first report of carpal tunnel syndrome in MPS IV. ConclusionSome children with mucopolysaccharidoses experience early development of at least moderately severe carpal tunnel syndrome. We recommend screening for median neuropathies at the wrist from age 5 years for children with mucopolysaccharidoses, particularly types I, II and IV, regardless of their symptoms of CTS, and of the treatment received for mucopolysaccharidosis.
Non-syndromic patients with infantile spasms and malformations of cortical development: absence of seizures in siblings
Authors: Tiwari Vijay N , Chugani Harry T , Kupsky William J , Huq AHM M
Published: December 2019

Background: Parents of children with infantile spasms undergoing epilepsy surgery typically inquire about the risk of other children for a similar affliction.  Here, we determined whether non-syndromic patients with infantile spasms and malformation of cortical development (MCD) have a family history of seizures, particularly in siblings.Patients and methods: We selected 29 children with intractable infantile/epileptic spasms who underwent surgery (mean age at surgery:  4.4±3.8 years; 12 males, 17 females; age range 0.8-14.9 years). A pathological diagnosis of MCD was confirmed in all the patients. Family (including parents, and siblings) history of seizures was acquired in all patients through neurology chart review/telephonic interview.Results: Pathological diagnosis of migration disorder (16) and cortical dysplasia (9) was made in these patients.  Diagnosis of hemimegalencephaly, dysembryoplastic neuroepithelial tumor (DNET), lissencephaly type1 and porencephaly was also confirmed in 4 different patients. None of the siblings in any family (total number of siblings in the group = 30) were affected by infantile spasms or other types of seizures. The maternal aunt of one patient and mother of another patient had a history of childhood seizures for short durations.Conclusions: Our retrospective study showed that patients with infantile spasms with MCD who underwent resective surgery have low sibling recurrence risk for seizures.  Our study suggests that de novo mutations and non-genetic or epigenetic factors are major causes of infantile spasms due to MCD.
Management of Children with Status Epilepticus
Authors: Wilmshurst Jo M
Published: December 2019

Status epilepticus is defined as generalised convulsions lasting 30 minutes or longer, which are either continuous, or where there is failure regain consciousness between seizures. The longer the time taken to gain control of seizures the worse the neurological outcome for the child and the harder it is to terminate the seizures. The outcome is further influenced by the underlying aetiology. Treatment of status epilepticus consists of four stages - pre-hospital treatment, emergency department, in-hospital treatment (ward or high care) and anaesthesia (ICU). There are numerous protocols available world-wide. Most are based on the available facilities and the anecdotal preferences of the units involved. Beyond the first level of intervention there are no large evidence based guidelines which identify the optimal intervention. Newer agents are increasingly being used, but studies to assess the true efficacy of these are not available. Further, protocols differ between resource poor countries compared to equipped countries where the capacity to provide intensive care support and expensive medical interventions is limited. There are two targets in the management of status epilepticus namely the rapid identification of the underlying aetiology as this affects treatment and prognosis and the early initiation towards terminating status epilepticus which decreases morbidity and mortality.
Vitamin and Cofactor Responsive Encephalopathies and Seizures
Authors: Tein Ingrid
Published: December 2019

Early diagnosis and treatment vitamin and/or cofactor responsive encephalopathies and seizures is critical for both seizure control and cerebral development and to prevent the kindling of intractable seizures with secondary brain injury. Recognition of these specific disorders is key to their management given their essential requirement for specific cofactors and their reduced responsiveness to standard anticonvulsant therapy. The overall goals of this review are; (1) To provide recognition of the clinical phenotypes of selected treatable metabolic etiologies of early-onset encephalopathies with seizures; (2) To highlight the appropriate diagnostic investigations for each, and (3) To  outline the effective treatment strategies. Each condition will be described followed by an approach to vitamin-responsive infantile-onset seizure management. A list of vitamin/cofactor responsive neurological conditions is provided in Table 1.
Non-invasive brain stimulation in childhood epilepsy
Authors: Ghosh Soumya , Nagarajan Lakshmi
Published: December 2019

Transcranial Magnetic Stimulation (TMS) and Transcranial Direct Current Stimulation (tDCS) are new neurophysiological techniques that allow neurologists and neuroscientists to investigate brain function and neural networks in normal humans as well as in those with neurological and neuropsychiatric disorders.In epilepsy, these techniques reveal abnormal excitability of the brain in focal and generalized epilepsy. Different patterns of excitatory and inhibitory changes detected by TMS have the potential to be used in the clinic for evaluating patients with epilepsy and to help with diagnosis, monitoring and treatment.Repetitive TMS (rTMS) and tDCS have the ability to modulate cortical excitability over prolonged periods and are being trialled for the treatment of epilepsy. However, further studies are needed to find optimal stimulation paradigms that reliably reduce seizures, and to confirm long term benefits and safety of these interventions.There are fewer TMS and tDCS studies in children and it's not clear if patterns of excitability changes are similar to those seen in adults or if there are unique patterns in childhood epilepsies. Continuing interventional trials assessing safety and efficacy of TMS and tDCS offer hope to children with treatment resistant epilepsies.
Focal cortical dysplasias
Authors: Guerreiro Marilisa M , Coan Ana Carolina
Published: December 2019

Focal cortical dysplasias (FCDs) are localized malformations of cortical development associated with refractory epilepsies. Seizure onset most commonly occurs in the first decade of life and the clinical presentation varies according to the localization of the lesion. Neuropsychological impairment often accompanies the high seizure burden. Drug- resistant seizures can be controlled in a significant percentage of patients if adequate surgical treatment is possible. Although some clinical, electroencephalographic and neuroimaging characteristics of the FCDs are well understood, the differences among the histological subtypes of FCDs remains to be better determined.
Schizencephaly and intractable epilepsy: an FDG-PET study
Authors: Govil-Dalela Tuhina , Kumar Ajay , Konka Praneetha , Chugani Harry T
Published: December 2019

Background: To assess the role of 2-deoxy-2(18F)-fluoro-D-glucose positron emission tomography (FDG-PET) scans in the comprehensive evaluation and surgical decision-making in patients with schizencephaly. Methods: We evaluated 11 patients (8M) with schizencephaly (mean follow-up: 4.5 years), including detailed clinical, MRI, FDG-PET, EEG, surgical and neuropathology data. Results: Eight patients had unilateral and three had bilateral clefts on MRI. Mean age at seizure onset was 20 months, with seizure being frequent in 10 and rare in one. Multiple seizure types were noted, with complex partial seizures being the most common (n=8) followed by infantile spasms (n=6). FDG-PET showed larger area of involvement than MRI in all the patients which corresponded better with the electrophysiological changes. Five patients (with unilateral disease on MRI) underwent epilepsy surgery (4 hemispherectomy and 1 multilobar resection). Two patients with focal defect on MRI underwent hemispherectomy due to larger area of abnormality revealed by FDG-PET.  One patient was excluded from the surgery due to bilateral abnormalities on FDG PET. Six patients (4 with surgery) were seizure-free at last follow-up (average seizure-free duration: 70 months). One patient who underwent hemispherectomy due to apparently unilateral disease on both video-EEG and MRI but having bilateral abnormality on PET continued to have seizures. ACTH treatment had only a brief (1 month to 1 year) or no response in the six infantile spasms patients. Conclusions: FDG-PET typically shows a much larger area of involvement than MRI thus supplementing MRI in defining the full extent of malformation and assessing the functional integrity of the contralateral hemisphere. FDG-PET may prove to be a useful tool to aid in surgical decision-making and predicting surgical outcome, as patients with contralateral abnormality on FDG-PET may have poor surgical outcomes. When the malformation is unilateral with an intact contralateral hemisphere, surgery (usually hemispherectomy) may be curative of the epilepsy.
Deletion at 2q24.3-31.1 resulting in severe Epileptic Encephalopathy and Episodic Autonomic Storming
Authors: Flynn Maureen A , Douglass Laurie , Rana Mandeep , Mian Asim , Milunsky Jeff M
Published: December 2019

We describe an 11.5-year-old male who has profound intellectual disability, treatment resistant epilepsy with temperature sensitive seizures, and paroxysms of autonomic storming, atypical brain magnetic resonance imaging, and microcephaly.  He has a 5.7 Mb deletion at 2q24.3-31.1, which includes the SCN1A gene.  This is the first report of periodic and recurrent autonomic storming associated with Dravet syndrome, which is both prevented and treated with clonidine.  Comparison to other reported individuals with the same deletion and genotype-phenotype correlations are discussed.
JICNA in its Second year
Authors: Newton Charles , Hameed Biju A
Published: December 2019

The year 2017 represents the third year of the Journal of International Child Neurology Association (JICNA). In 2015 we assessed 9 papers and accepted 6 papers for publication. In 2016, we assessed 8 papers and accepted 7 papers for publication. To date we published 7 research papers, 5 review papers and 2 case reports. There have been considerable challenges in setting up a journal. These challenges include identifying reviewers who produce timely reviews, persuading people to submit research based papers, copy editing and issues with the software. We have sorted out the latter two issues. Papers are sent out to at least two reviewers, often one reviewer produces a time review, but chasing the other review is often time consuming, and review and thus publication. We would really encourage members of International Child Neurology Association (ICNA) to let us know if they would be willing to review papers in a timely manner and on what subjects they would be willing to review papers. We would like to invite members of ICNA to consider becoming section editors for the journal. This does involve a commitment, and we have suggested the following criteria:contribute at least two papers (one research and one review);review at least two papers within 2 weeks;be prepared to help people from low resource areas to analyse and write up their research;and have acknowledged expertise in the area of section (reference from another person in the field). This does represent considerable commitment in terms of time and energy. Please contact us if you would like to be and can commit to be a section editor.The JICNA is the journal of ICNA, and was set up with the following aims: i) to promote child neurology throughout the world, in particular encourage papers from low and middle income countries, who find it difficult to have their papers accepted by the more established journals; ii) to provide peer review scientifically sound papers on child neurology; iii) to be open access, so that papers can be accessed from anywhere in the world; iv) to be free as long as feasible, so to encourage papers from low and middle income countries. In order to achieve these goals it is important that members of ICNA contribute to making this a rigorous and vibrant journal. We encourage all members to contribute to this journal, by submitting papers (particularly research papers), reviewing papers in a timely manner and if they have the time and energy put themselves forward as a section editor.
Batten disease and parents: marital quality, support, and communication
Authors: Cozart Elizabeth J , Augustine Erika F , Mink Jonathan W , Thatcher Alyssa R , Adams Heather R
Published: December 2019

Background: The neuronal ceroid lipofuscinoses, collectively known as Batten disease, are rare pediatric neurodegenerative disorders resulting in blindness, progressive motor impairment, dementia, and premature death. This study identified services and support accessed by parents of children with Batten disease and assessed parental marital quality. Methods: Parents completed a survey to identify services their affected children received, and their own uses of social support, information, and online resources. We used the Dyadic Adjustment Scale to evaluate parental marital quality. Results: 29 parents of 23 Batten-affected children completed the survey. 18 children (78%) received receiving in-home nursing or paraprofessional care.  Over 90% of parents used the internet and social media to learn or talk about Batten disease; their preferred learning method was from other parents of children with Batten disease.  Batten parent marital quality was significantly lower than that reported in studies of the general population or parents of children with chronic, but non-neurodegenerative, disease. Conclusions: Parents expressed an interest in and preference for parent-to-parent communication for support and information about Batten disease. Further work is needed to understand factors associated with the significantly lower marital quality among Batten parents.
Child Neurology in India: Challenges and Opportunities
Authors: Sahu Jitendra
Published: December 2019

India is a vast and populous country. The spectrum of childhood neurological disorders in India differs from the developed countries. Central nervous system infections like pyogenic meningitis, viral encephalitis, tuberculous meningitis, neurocysticercosis, rabies and cerebral malaria constitute important cause of neurological morbidity. Epilepsy, development delay, cerebral palsy and autism spectrum disorder are important non-infectious causes of neurologic disability. The load of these disorders is enormous. However, it is important that a significant proportion of these disorders are potentially preventable or treatable. With scarcity of child neurologists in India, child neurology as a sub-speciality of paediatrics is in its formative years. This review describes the brief overview of burden of some important childhood neurological disorders and status of child neurology in India.
Hypochondroplasia and epilepsy: the neurological spectrum of FGFR3 mutations
Authors: Van der Poest Clement Emma A , Yang Edward , Holm Ingrid , Peters Jurriaan M
Published: December 2019

Hypochondroplasia is a disorder caused by FGFR3 mutations, commonly featuring short limbs and stature. Thanatophoric dysplasia, achondroplasia and Muenke syndrome are genetically related conditions with skeletal dysplasia and temporal lobe dysgenesis. We present a 6-month old boy with hypochondroplasia and epilepsy. His brain MRI revealed bilateral temporal lobe dysplasia, with redundant sulci and a rotated hippocampus. The neuroimaging findings of our case provide direct insight in the pathogenesis of the cerebral dysgenesis in FGFR3-related disorders. In addition, through this case we review the neurological spectrum of FGFR3 mutations.
Diagnosing neurodegeneration with brain iron accumulation before iron starts to accumulate
Authors: Hoogwijs Ine
Published: December 2019

Introduction

Neurodegeneration with brain iron accumulation (NBIA) consists of a heterogeneous group of disorders with brain iron accumulation as common radiological endpoint. Mutations in multiple genes have been associated with NBIA. We present 2 cases with a different type of NBIA, in whom the diagnosis was confirmed before brain iron accumulation became evident on MRI.

 Case description

The first patient was referred because of frequent falls at the age of 4 years. She had an ataxic gait and weak Achilles tendon reflexes. Two years later, pyramidal and more prominent cerebellar signs became evident. A skin and muscle biopsy revealed intra-axonal spheroids in the peri-and endomysial myelinated nerve bundles as well as in the motor endplates, which led to the diagnosis of PLA2G6-associated neurodegeneration (PLAN). Brain iron accumulation occurred at follow-up MRI at 9 years of age.

The second patient was referred because of developmental stagnation and detection of elevated liver enzymes at 3 months of age. Seizures started at 15 months of age, and were refractory to treatment with multiple anti-epileptic drugs. Molecular genetic testing using an epilepsy gene panel revealed a mutation in the WDR45 gene, a known cause of beta-propeller protein-associated neurodegeneration (BPAN). Brain MRI at 14 months of age showed diffuse hypomyelination in the absence of BIA.

 Discussion

This report highlights that NBIA can be suspected on a clinical basis and confirmed by genetic testing before iron accumulation becomes present on brain MRI. Early diagnosis will provide a longer timeframe for potential disease modulating treatments in the future.
Progressive chorea and dystonia associated with a large arteriovenous malformation
Authors: Raskin Jeffrey S , Parnes Mered , Lam Sandi
Published: December 2019

A 9-year-old male with intellectual disability and epilepsy presenting with a progressive movement disorder characterized by chorea and dystonia primarily affecting the left face, arm, and leg and attributed to an arteriovenous malformation centered within the right thalamus with bilateral extension into the basal ganglia, midbrain and pons is reported.