bluuebunny/crossref_metadata_2025_split huggingface dataset:
Giant nerves in chronic inflammatory polyradiculoneuropathy
Authors: Grimm Alexander , Schubert Victoria , Axer Hubertus , Ziemann Ulf 
Published: July 2016

ABSTRACTIntroduction: Nerve enlargement (NE) is described in inflammatory and inherited neuropathies. It is commonly multifocal and moderate in the former and homogeneous and generalized in the latter. We describe 4 cases of massive NE in inflammatory neuropathies. Methods: Patients presented with symptoms of polyneuropathy that progressed over months to years. Nerve conduction studies (NCS), laboratory analysis, nerve MRI, and nerve ultrasound were performed. Results: NCS revealed demyelinating neuropathy in all with multifocal conduction blocks or increased terminal latency indices. MRI/ultrasound revealed extensive NE in the roots and nerves. Detailed diagnostics including biopsies, positron emission tomography‐computed tomography, and genetic testing revealed no other pathology. Chronic inflammatory demyelinating polyneuropathy variants were diagnosed in all, and immunotherapies were successfully initiated. Conclusions: MRI and ultrasound contributed to diagnosis and therapy. All patients had giant NE in common, which strongly suggested inherited neuropathy. However, the final diagnosis was inflammatory neuropathy. Impressive NE can occur in immune‐mediated neuropathies and should be carefully differentiated from inherited neuropathies. Muscle Nerve 55: 285–289, 2017
Models of energy homeostasis in response to maintenance of reduced body weight
Authors: Rosenbaum Michael , Leibel Rudolph L. 
Published: July 2016

ObjectiveTo test three proposed models for adaptive thermogenesis in compartments of energy expenditure following different degrees of weight loss. Specifically, (1) there is no adaptive thermogenesis [constant relationship of energy expenditure (EE) to metabolic mass]. (2) There is a fixed degree of adaptive thermogenesis once fat stores are below a “threshold.” (3) The degree of adaptive thermogenesis is proportional to weight loss.MethodsThe relationship between weight loss and EE was examined in 17 inpatient subjects with stable weight and obesity studied at usual weight and again following a 10% and a 20% weight loss.ResultsFollowing initial weight loss (10%), resting (REE) and non‐resting (NREE) EE were significantly below those predicted on the basis of the amount and composition of weight lost. Further reductions below predicted values of NREE but not REE occurred following an additional 10% weight loss. Changes in body weight, composition, and/or energy stores were significantly correlated with changes in EE.ConclusionsAll models are applicable to the decline in EE following weight loss. The disproportionate decline in REE is consistent with a threshold model (no change with further weight loss) while the disproportionate decline in NREE is largely reflective of the degree of weight loss.
A meta‐analysis of the neuropsychological effects of chemotherapy in the treatment of childhood cancer
Authors: Pierson Cory , Waite Erin , Pyykkonen Ben 
Published: July 2016

BackgroundLong‐term neuropsychological deficits associated with pediatric cancers and the related treatments have been consistently reported. Whole brain cranial radiation therapy (CRT) is associated with neurocognitive impairment. As a result, physicians are reticent to use CRT in favor of systemic or intrathecal chemotherapy, which have a less clear impact on cognition.ProcedureThe current meta‐analysis examined post‐treatment neuropsychological performance of children diagnosed with cancer and treated with chemotherapy to better understand the impact of chemotherapy upon cognition. Relevant test scores from 18 empirical studies were utilized and analyzed in comparison to normative data yielding 199 unique effect sizes across nine neurocognitive domains.ResultsChildren diagnosed with cancer, who received chemotherapy, demonstrated deficits in attentional capacity (g = −0.277). These deficits are noted in the context of relatively unaffected performance in other domains. When examining potential moderators, those tested more than 5 years after completion of treatment demonstrated better attentional performance than those tested within 5 years of treatment completion.ConclusionsThese deficits in attentional capacity have implications related to the academic success of these children. Given the potential for remediation strategies within this domain, neuropsychological assessment can be an integral aspect of long‐term care plans of survivors of childhood cancer.
Pituitary disorders as a predictor of apathy and executive dysfunction in adult survivors of childhood brain tumors
Authors: Fox Michelle E. , King Tricia Z. 
Published: July 2016

BackgroundThe relationship between apathy and endocrine dysfunction, both frequent outcomes of neurological insult, has not yet been investigated in brain tumor survivors. The present study aimed to assess the relationship between pituitary disorders and apathy and other facets of executive function in long‐term adult survivors of childhood brain tumors and to differentiate between apathy and depression in this population.ProcedureSeventy‐six adult survivors of childhood brain tumors at least 5 years past diagnosis participated. An informant completed the Frontal Systems Behavior Scale (FrSBe), and 75 of the 76 participants completed a Structured Clinical Interview for the DSM‐IV‐TR (SCID). Information on neuroendocrine dysfunction was obtained through medical chart review.ResultsClinically significant levels of apathy on the FrSBe were identified in 41% of survivors. Pituitary dysfunction significantly explained 9% of the variance in apathy scores and affected whether an individual presented with clinical levels of apathy. Pituitary dysfunction predicted higher levels of executive dysfunction but did not impact whether a participant reached clinical levels of executive dysfunction. A past major depressive episode (MDE) significantly predicted current apathy but showed no relationship with pituitary disorders. Radiation treatment predicted pituitary dysfunction but not the differences in apathy or executive functions.ConclusionsApathy and executive dysfunction in survivors of childhood brain tumors are strongly predicted by pituitary dysfunction, and individuals with pituitary dysfunction are more likely to present with clinical levels of apathy as adults. Clinical levels of apathy may present absent of current depression, and pituitary dysfunction impacts apathy uniquely.
A detailed proteomic profiling of plasma membrane from zebrafish brain
Authors: Smidak Roman , Aradska Jana , Kirchberger Stefanie , Distel Martin , Sialana Fernando J. , Wackerlig Judith , Mechtcheriakova Diana , Lubec Gert 
Published: July 2016

Zebrafish (Danio rerio) is a well‐established model organism in developmental biology and disease modeling. In recent years, an increasing amount of studies used zebrafish to analyze the genetic changes underlying various neurological disorders. The brain plasma membrane proteome represents the major subsets of signaling proteins and promising drug targets, but is often understudied due to traditional experimental difficulties including problems with solubility, detergent removal, or low abundance. Here, we report a comprehensive dataset of the proteins identified in the enriched plasma membrane of the zebrafish brain by applying sequential trypsin/chymotrypsin digestion with multidimensional LC‐MS/MS. A total number of 97 017 peptide groups corresponding to 9201 proteins were identified. These were annotated in various molecular functions or neurological disorders. The dataset of the current study provides a useful data source for further utilizing zebrafish in basic and clinical neuroscience.
Models and metrics to assess humanitarian response capacity
Authors: Acimovic Jason , Goentzel Jarrod 
Published: July 2016

AbstractThe race to meet vital needs following sudden onset disasters leads response organizations to establish stockpiles of inventory that can be deployed immediately. These government or non‐government organizations dynamically make stockpile decisions independently. Even though the value of one organization's stock deployment is contingent on others' decisions, decision makers lack evidence regarding sector capacity to assess the marginal contribution (positive or negative) of their action. To our knowledge, there exist no metrics describing the system capacity across many agents to respond to disasters. To address this gap, our analytical approach yields new humanitarian logistics metrics based on stochastic optimization models. Our study incorporates empirical data on inventory stored by various organizations in United Nations facilities and in their own warehouses to offer practical insights regarding the current humanitarian response capabilities and strategies. By repositioning inventory already deployed, the system could respond to disasters in the same expected time with a range of 7.4%–20.0% lower cost for the items in our sample.
Analgesia for acute gingivostomatitis: a national survey of pediatric emergency physicians
Authors: MacLellan Joe , Ali Samina , Curtis Sarah , Baserman Jason , Dixon Andrew 
Published: July 2016

AbstractObjectivesGingivostomatitis is a common, painful pediatric presentation, and yet, few studies are available to guide management. We aimed to describe pediatric emergency physicians’ current practice patterns, with respect to analgesic use in children with acute gingivostomatitis, in order to inform future studies.MethodsA national survey was conducted at all 15 national academic pediatric centres.Electronic surveys were distributed to pediatric emergency physicians using a modified Dillman protocol; non-respondents received paper surveys via post. Data were collected regarding demographic characteristics, clinical behaviour, factors that may influence practice, and future directions.ResultsResponse rate was 74% (150/202). Most physicians (72%) preferred the combination of acetaminophen and ibuprofen to either agent alone (ibuprofen 19%, acetaminophen 7%). The preferred second-line analgesics were oral morphine (48%, 72/150) and compounded topical formulas (42%, 64/150). The most commonly cited compounded agent was Benadryl plus Maalox (23%, 35/150). Clinical experience with a medication had the greatest influence on practice pattern, with 52% (78/149) strongly agreeing. The most commonly cited barrier to adequate analgesia was difficulty in the administration of topical or oral medication to children.ConclusionsAs with many other painful conditions, the combination of acetaminophen and ibuprofen was preferred, followed by either agent alone. Oral morphine and topical compounded agents were also frequently prescribed. Regardless of patient age, physicians preferred oral morphine as a second-line agent to treat pain from severe gingivostomatitis. Future research will focus on determining which analgesic and route (oral or topical) is the most effective and best-tolerated choice.
Disaster Preparedness Among Older Japanese Adults With Long-Term Care Needs and Their Family Caregivers
Authors: Wakui Tomoko , Agree Emily M. , Saito Tami , Kai Ichiro 
Published: July 2016

AbstractObjectiveIn the 2011 Great East Japan Earthquake, as in Hurricanes Katrina and Rita in the United States, older individuals were at the greatest risk of mortality. Much concern has been raised about developing plans to reduce these risks, but little information has been provided about preparedness, and the key role played by caregivers has been largely unexplored. The aims of this study were thus to examine the preparedness of family caregivers of older adults with long-term care needs and to identify the characteristics of older adults and their caregivers that are associated with poor preparedness and greater concern about disasters.MethodsShortly after the Great East Japan Earthquake, the second wave of the Fukui Longitudinal Caregiver Study was administered to the family caregivers of older Japanese individuals with long-term care needs. The sample included 952 caregivers from 17 municipalities in Fukui prefecture. Logistic regression analyses were used to identify the factors associated with self-assessed preparedness, evacuation planning, and caregivers’ concerns about preparedness.ResultsThe majority (75%) of the caregivers had no concrete plans for evacuation in an emergency, and those caring for persons with dementia were 36% less likely to have any plan. In multivariate models, caregivers who were more experienced and wealthier and who reported more family and community support were more likely to feel well prepared. Caregivers with poor health or limited financial resources or who were responsible for older persons with mobility difficulties reported higher levels of anxiety about their disaster preparedness.ConclusionsThis study indicates that most caregivers are ill prepared to respond in emergencies and that caregiver resources, community support, and the needs of older care recipients influence both preparedness and concern about disasters. Education for caregivers and the development of community support programs could provide important sources of assistance to this vulnerable group. (Disaster Med Public Health Preparedness. 2017;11:31–38)
Spectroscopic Pulsational Frequency and Mode Determination of the γ Doradus Star HD 189631
Authors: Davie M. W. , Pollard K. R. , Cottrell P. L. , Brunsden E. , Wright D. J. , De Cat P. 
Published: July 2016

AbstractWe present improvement and confirmation of identified frequencies and pulsation modes for the γ Doradus star HD 189631. This work improves upon previous studies by incorporating a significant number of additional spectra and precise determination of frequencies. Four frequencies were identified for this star: 1.6774 ± 0.0002 d−1, 1.4174 ± 0.0002 d−1, 0.0714 ± 0.0002 d−1, and 1.8228 ± 0.0002 d−1 which were identified with the modes (l,m) = (1, +1), (1, +1), (2, −2), and (1, +1) respectively. These findings are in agreement with the most recent literature. The prevalence of (l,m) = (1, +1) modes in γ Doradus stars is starting to become apparent and we discuss this result.
Type 1 Diabetes and Prediabetic State in a Monozygotic Triplet
Authors: Krokowski M. , Abel M. , Teodorczyk A. , Szadkowska A. , Pyrżak B. , Rymkiewicz-Kluczyńska B. , Bodalski J. 
Published: July 2016

AbstractType 1 diabetes mellitus (IDDM) results from a chronic process of autoimmune destruction of ß cells of the Langerhans islets. The presence of autoantibodies (ICA, GADA, anti-IA2, IAA) in serum preceds the clinical onset of the disease. Genetic predisposition for IDDM is connected with HLA, CTLA-4 and insulin gene region.The aim of the study was the genetic and immunological analysis of a triplet. One of them developed Type 1 diabetes mellitus. We analysed HLA class II, CTLA-4 and insulin gene polymorphisms in the whole family. Besides, we investigated immunological status of three brothers.All patients present identical genotype for VNTR loci: D1S80, D17S5 and Apo B, as well as for HLA-DRB1, — DQA1, — DQB1, CTLA-4 gene and all studied insulin gene polymorphisms. That proves their monozigosity. The triplet presents strong genetic predisposition for IDDM. The two patients without overt diabetes have increased levels of ICA, GADA, IA2 and IAA.
From the Research on Electrophoretical Mobility of Nuclei in Twins
Authors: Rębacz E. 
Published: July 2016

AbstractI studied 8 pairs of male twins and 14 pairs of female twins. The aim of this paper is to demonstrate the evaluation of development age in pairs of twins examined through the electrophoretical mobility of nuclei. EMN index in pairs of male twins, the index figures do not change dramatically between the ages of 8 – 12. EMN index in pairs of female twins rises from 9 – year – old to 11 – year – old pair. In this paper I want to indicate the studies on development age, which began at The Twins Festival in Szczecin in 1998.
A Comparative Study of Twinning and Triplet Rates in 17 Countries, 1972-1996
Authors: Imaizumi Y. 
Published: July 2016

AbstractSecular changes in twinning and triplet rates were analyzed using vital statistics in Austria, the Czech Republic, the Slovak Republic, England and Wales, Germany, the Netherlands, Switzerland, Denmark, Finland, Norway, Sweden, Canada, Australia, Hong Kong, Israel, Japan, and Singapore during the period from 1972 to 1996. Among those 17 countries, the twinning and triplet rates in the Czech Republic and in the Slovak Republic remained constant from 1972 to 1994, whereas these rates increased significantly year by year in the other 15 countries during the examined period in each country. Twinning rates increased from 1.2-fold in Austria to 2-fold in Denmark from 1972 to 1996. As for triplets, the rate increased from 3-fold in Denmark to 9-fold in Norway during that period. With one exception, that being the Slovak Republic, the triplet rate was highest in the Scandinavian countries, followed by the other European and Asian countries. The rising twinning and triplet rates have been attributed to the higher proportion of mothers treated with ovulation-inducing hormones and partially attributed to IVF.
Ultrasound Examination in Twin Pregnancy and Late Fetal Death
Authors: Rydhstroem H. , Walles B. 
Published: July 2016

AbstractObjective. To test the hypothesis that the (case) twin later to succumb in utero has biparietal diameter measurements (by ultrasound) different from those (control) twins surviving the perinatal period.Material and methods. Information from the Medical Birth Registry, National Board of Health and Welfare, Stockholm, was used to identify all births in a defined population in southern Sweden with about 20,000 deliveries each year. In 3,019 twin pregnancies between 1973 and 1989, one or both twins were stillborn in 47 cases (gestational duration ≥28 weeks, birthweight ≥500g.). For each case pregnancy, two control pregnancies were selected, the matching criteria being: same delivery unit, same parity (0, I, II, III+), similar year of delivery (± 1 year) and maternal age (± 5 years). Data on ultrasound examinations were extracted from the original medical records. Screening in early second trimester started at one of the units as early as 1973 and at the latest of 12 units in 1982.Results. There was no obvious difference between cases and controls in intra-pair discordant biparietal diameter (BPD) measured in early second trimester. Nor was there any evident difference in the rate of deviant BPD between cases and controls. In all, 8% of dead male and 24% of dead female fetuses were by definition small-for-gestational age (<-2 standard deviations).Conclusions. No significant difference was seen between cases and controls regarding deviating biparietal diameters. Abdominal diameter may be a better predictor of subsequent fetal death (not analysed in this study), though only about 15% of all dead twins were deemed small-for-gestational age.
Trends of Twinning Rates in Ten Countries, 1972-1996
Authors: Imaizumi Y. 
Published: July 2016

AbstractTrends of twinning rates were analyzed using vital statistics in Austria, Finland, Norway, Sweden, Canada, Australia, Hong Kong, Israel, Japan, and Singapore during the period from 1972 to 1996. The twinning rates increased significantly year by year in each country. During these periods, the twinning rate increased by twenty percent in Austria and Canada, and by sixty percent in Norway and Sweden. The twinning rate was 1.6 times higher in Sweden than in Hong Kong in 1972 and by eighty percent in Israel in 1995. Twinning rates were higher in European countries, Canada, and Australia than in Asia. The variations of the twinning rates among countries were not only due to biological factors, but also to assisted reproductive techniques. In Australia, the overall twinning rate was 1.3 times higher in the nuptial births (14.1 per 1000 births) than in the ex-nuptial births (10.7) during the period 1994-1996. As for maternal age, twinning rates in Sweden increased year by year for maternal age groups except the youngest and the oldest age groups. In Sweden, the rising twinning rate has been attributed to the higher proportion of mothers (for the 25-39 year old age groups) treated with ovulation-inducing hormones and attributed to in-vitro fertilization.
Balanced X; 22 Translocation in a Patient with Premature Ovarian Failure
Authors: Banerjee N. , Kriplani A. , Takkar D. , Kucheria K. 
Published: July 2016

AbstractA case of balanced X; autosome translocation 46, X, t (X; 22) (q 24; q 13) in a 25-year-old female with secondary amenorrhea and premature ovarian failure (POF) is described. The relationship between balanced X; autosome translocation [t (X; A)] and varied phenotypic expression observed in these women is discussed. This case highlights the importance of early recognition of these women in order to give them the best chance of conception in their oligohypomenorrhic phase before complete loss of gonadal function.
Very Early and Simple Determination of Chorionic and Amniotic Type in Twin Gestations by High-Frequency Transvaginal Ultrasonography
Authors: Malinowski W. 
Published: July 2016

AbstractObjective. The aim was to determine the chorionic and amniotic types in multifetal pregnancies with transvaginal ultrasonography at very early stage of gestation.Study design. Twenty-one spontaneous multifetal pregnancies were scanned transvaginally before 8 weeks' gestation (four of them from 4th week). The chorionic and amniotic type was determined ultrasonographically. All twin gestations had postpartum pathologic evaluation of the placenta and histologic determination of the chorionic and amniotic type.Results. Ultrasonographic evaluation of the 21 pregnancies demonstrated 20 twin and 1 triplet gestation. Four of the twin pregnancies were monochorionic-diamniotic. Triplet was monochorionic-triamniotic (spontaneously aborted in 8th week of gestation). In all 20 twin pregnancies, transvaginal ultrasonography correctly predicted the chorionic and amniotic type before 8 weeks of gestation.Conclusion. Transvaginal ultrasonography allows a reliable, simple and rapid determination; the dichorionic twin pregnancy in 4 weeks, monochorionic in 5 weeks, and differentiation of mono-or diamniotic in 7 weeks of gestation.
Contributions of Mouse Genetic Studies to Genomic Imprinting
Authors: Cattanach B.M. 
Published: July 2016

Maternal and paternal disomies and equivalent duplications for specific chromosome regions can readily be generated in the mouse. Most do not result in abnormality but for 10 regions distributed over 6 chromosomes developmental anomalies ranging from early embryonic lethalities to characteristic phenotypic abnormalities occur. With certain chromosome regions, maternal or paternal duplication leads to different and, in some cases, opposite anomalies so that, in total, 15 different imprinting effects can be distinguished. These observations have provided key evidence on the occurrence of imprinting in mammals.On the basis of the established homologies between mouse and human chromosomes, it is possible to predict which segments of the human genome are subject to equivalent imprinting. In this regard it is significant that candidate imprinting effects for the two classical examples of imprinting in humans, namely the Prader-Willi and Angelman syndromes, have been found in the mouse.Current studies are aimed at reducing the size of the imprinting regions with the objective of facilitating identification of the genes involved. Furthermore, the developmental profiles of genes already identified as being subject to imprinting are being determined.A new approach involves the analysis of a novel mutation that causes growth retardation and cranial abnormalities and which shows the inheritance pattern of an imprinted gene, as originally predicted by Hall (Am J Hum Genet 46: 857, 1990). It is anticipated that the mutation will represent a deletion and will lie within one of the recognised imprinting regions.