bluuebunny/crossref_metadata_2025 huggingface dataset:
Molecular Basis for the Inactivation of Ca2+- and Voltage-Dependent BK Channels in Adrenal Chromaffin Cells and Rat Insulinoma Tumor Cells
Authors: Xia Xiao-Ming , Ding Jiu Ping , Lingle Christopher J.
Published: April 2018

Large-conductance Ca2+- and voltage-dependent potassium (BK) channels exhibit functional diversity not explained by known splice variants of the singleSloα-subunit. Here we describe an accessory subunit (β3) with homology to other β-subunits of BK channels that confers inactivation when it is coexpressed withSlo. Message encoding the β3 subunit is found in rat insulinoma tumor (RINm5f) cells and adrenal chromaffin cells, both of which express inactivating BK channels. Channels resulting from coexpression ofSloα and β3 subunits exhibit properties characteristic of native inactivating BK channels. Inactivation involves multiple cytosolic, trypsin-sensitive domains. The time constant of inactivation reaches a limiting value ∼25–30 msec at Ca2+of 10 μmand positive activation potentials. UnlikeShakerN-terminal inactivation, but like native inactivating BK channels, a cytosolic channel blocker does not compete with the native inactivation process. Finally, the β3 subunit confers a reduced sensitivity to charybdotoxin, as seen with native inactivating BK channels. Inactivation arises from the N terminal of the β3 subunit. Removal of the β3 N terminal (33 amino acids) abolishes inactivation, whereas the addition of the β3 N terminal onto the β1 subunit confers inactivation. The β3 subunit shares with the β1 subunit an ability to shift the range of voltages over which channels are activated at a given Ca2+. Thus, the β-subunit family of BK channels regulates a number of critical aspects of BK channel phenotype, including inactivation and apparent Ca2+sensitivity.
Bipotent Cortical Progenitor Cells Process Conflicting Cues for Neurons and Glia in a Hierarchical Manner
Authors: Park John K. , Williams Brenda P. , Alberta John A. , Stiles Charles D.
Published: April 2018

Neurons and glia of the cerebral cortex are thought to arise from a common, multipotent progenitor cell that is instructed toward alternate fates by extracellular cues. How do these cells behave when confronted with conflicting cues? We show here that nestin-positive neuroepithelial (NE) cells from embryonic day 14 rat cortex coexpress surface receptor proteins for ciliary neurotrophic factor (CNTF) and platelet-derived growth factor (PDGF). Both sets of these receptor proteins are functional in NE cells, as shown by ligand-dependent activation of downstream signal-generating proteins. Transient (30′) exposure to CNTF instructs NE cells toward an astrocyte fate. Brief exposure to PDGF initiates neuronal differentiation. However, when challenged with conflicting cues, PDGF is dominant to CNTF. Moreover, CNTF-treated NE cells can be “redirected” by a subsequent exposure to PDGF to form neurons instead of astrocytes, whereas the converse is not true. The asymmetric relationship between CNTF and PDGF indicates that these two growth factors act on a common progenitor cell that has, at a minimum, two fates available to it rather than separate populations of precommitted neuroblasts and astroblasts. This bipotent progenitor cell processes conflicting cues for neurons and glia in a hierarchical manner.
Stimulus History Alters Behavioral Responses of Neuronal Growth Cones
Authors: Diefenbach Thomas J. , Guthrie Peter B. , Kater Stanley B.
Published: April 2018

Generally, it is assumed that growth cones respond to a specific guidance cue with a single, specific, and stereotyped behavior. However, there is evidence to suggest that previous exposure to a given cue might alter subsequent responses to that cue (Snow and Letourneau, 1992; Shirasaki et al., 1998). We therefore tested the hypothesis that growth cone responses to stimuli are dependent on the history of previous stimulation. Growth cones of chick dorsal root ganglion neurons were exposed to well characterized stimuli: (1) contact with a laminin-coated bead, which causes growth cone turning, or (2) electrical stimulation, which causes growth cone collapse. Although the expected behavioral responses were observed after the initial stimulation, strikingly different responses to a subsequent stimulation were observed. Growth cones that had recovered from electrical stimulation-induced collapse rapidly developed insensitivity to a second identical electrical stimulation. Growth cones that previously turned in response to contact with a laminin-coated bead responded to a second bead with a “stall” or cessation in outgrowth. This stimulus history dependence of growth cone behavior could be generalized across dissimilar stimuli: after contact with a laminin-coated bead, growth cones failed to collapse in response to electrical stimulation. The calcium/calmodulin-dependent protein kinase II (CaMKII) was implicated in this history dependence by pharmacological experiments. Together, these results demonstrate that growth cones can alter their behavioral response rapidly to a given stimulus in a manner dependent on previous history and that knowledge of past events in growth cone navigation may be required to predict future growth cone behavior.
Directional Guidance of Oligodendroglial Migration by Class 3 Semaphorins and Netrin-1
Authors: Spassky Nathalie , de Castro Fernando , Le Bras Barbara , Heydon Katharina , Quéraud-LeSaux Françoise , Bloch-Gallego Evelyne , Chédotal Alain , Zalc Bernard , Thomas Jean-Léon
Published: April 2018

Oligodendrocytes, the myelin-forming cells of the CNS, are generated from multiple foci distributed along the developing neural tube. Little is known about the endogenous guidance cues controlling the migration of oligodendrocyte precursor cells (OPCs) from their site of emergence toward their final destination, mainly the future white matter tracts. During embryonic development, the optic nerve is populated by OPCs originating in the diencephalon that migrate from the chiasm toward the retina. Here we show that OPCs migrating into the embryonic optic nerve express the semaphorin receptors neuropilin-1 and -2, as well as deleted in colorectal cancer (DCC) and, to a lesser extend unc5H1, two of the netrin-1 receptors. Using a functional migration assay, we provide evidence that Sema 3A and netrin-1 exert opposite chemotactic effects, repulsive or attractive, respectively, on embryonic OPCs. In addition, we show that Sema 3F has a dual effect, chemoattractive and mitogenic on embryonic OPCs. The localization of cells expressing Sema 3A, Sema 3F, and netrin-1 is consistent with a role for these ligands in the migration of OPCs in the embryonic optic nerve. Altogether, our results suggest that the migration of OPCs in the embryonic optic nerve is modulated by a balance of effects mediated by members of the semaphorin and netrin families.
Functional Repair of the Corticospinal Tract by Delayed Transplantation of Olfactory Ensheathing Cells in Adult Rats
Authors: Keyvan-Fouladi Naghmeh , Raisman Geoffrey , Li Ying
Published: April 2018

Adult rats were trained to use their forepaws to retrieve a piece of food. Destruction of the dorsal corticospinal tract on one side at the level of the first cervical segment abolished the use of the ipsilateral forepaw for retrieval for at least 6 months after operation. Where a variable amount of the corticospinal tract was spared, there was a proportionate persistence of retrieval. In lesioned rats that had shown no retrieval for 8 weeks after operation, a suspension of olfactory ensheathing cells was injected into the lesion site. Starting between 1 and 3 weeks after transplantation, all rats with transplants bridging the lesion site resumed retrieval by the ipsilateral forepaw. Biotin dextran anterograde tracing shows regenerating corticospinal axons crossing the bridge, traveling caudally for ∼10 mm in the distal part of the corticospinal tract and forming terminal arborizations in the spinal gray matter. Functional recovery can occur when only ∼1% of the corticospinal tract axons are present.
Hybrid Sterility in Fish Caused by Mitotic Arrest of Primordial Germ Cells
Authors: Yoshikawa Hiroyuki , Xu Dongdong , Ino Yasuko , Yoshino Tasuku , Hayashida Takao , Wang Junjie , Yazawa Ryosuke , Yoshizaki Goro , Takeuchi Yutaka
Published: April 2018

Sterility in hybrid animals is widely known to be due to a cytological mechanism of aberrant homologous chromosome pairing during meiosis in hybrid germ cells. In this study, the gametes of four marine fish species belonging to the Sciaenid family were artificially fertilized, and germ cell development was examined at the cellular and molecular levels. One of the intergeneric hybrids had gonads that were testis-like in structure, small in size, and lacked germ cells. Specification of primordial germ cells (PGCs) and their migration toward genital ridges occurred normally in hybrid embryos, but these PGCs did not proliferate in the hybrid gonads. By germ cell transplantation assay, we showed that the gonadal microenvironment in hybrid recipients produced functional donor-derived gametes, suggesting that the germ cell-less phenotype was caused by cell autonomous proliferative defects of hybrid PGCs. This is the first evidence of mitotic arrest of germ cells causing hybrid sterility in animals.
Contraception for Adolescents and Young Adults in the Inpatient Setting: The Providers’ Perspective
Authors: Goldstein Rachel L. , Carlson Jennifer L. , Halpern-Felsher Bonnie
Published: April 2018

OBJECTIVES:

To assess pediatric providers’ attitudes and barriers to initiating a contraceptive method in the inpatient setting.

METHODS:

Pediatric attending physicians and trainees from 5 academic institutions were surveyed about contraceptive prescribing practices, attitudes, and potential barriers to initiating contraception in the inpatient setting.

RESULTS:

In 2016, 271 pediatric providers (34.0% were attending physicians, 18.1% fellows, and 47.9% residents) completed the survey; the response rate was 19.2%. Most participants practiced in both inpatient and outpatient settings (95.7% and 85.0%, respectively). More providers felt confident screening for sexual activity among young adults as compared with adolescents (71.9% vs 65.6%). The same was true for discussing contraceptive options (44.0% vs 38.8%, respectively). Inpatient providers reported seeing adolescents and young adults privately, discussing confidentiality, and asking about sex less than half of the time. More than 80% of providers agreed that it would be appropriate to initiate a contraceptive method for inpatients; 35.8% had done so, and 85.2% indicated that having additional consultation would increase initiation of a contraceptive method in the hospital (88.1% felt similarly for long-active reversible contraception methods). General barriers to initiating contraception included insufficient training, insufficient exposure to adolescents and young adults to maintain skills, and lack of time. Barriers specific to the inpatient setting included concerns about follow-up, confidentiality, and interference with the treatment plan.

CONCLUSIONS:

Initiation of a contraceptive method in the inpatient setting is acceptable to providers. In our findings, it is suggested that strategies are needed to enhance provision of these services by addressing confidentiality concerns and educating providers.
Focused Training for the Handover of Critical Patient Information During Simulated Pediatric Emergencies
Authors: Lautz Andrew J. , Martin Kelly C. , Nishisaki Akira , Bonafide Christopher P. , Hales Roberta L. , Hunt Elizabeth A. , Nadkarni Vinay M. , Sutton Robert M. , Boyer Donald L.
Published: April 2018

OBJECTIVES:

Miscommunication has been implicated as a leading cause of medical errors, and standardized handover programs have been associated with improved patient outcomes. However, the role of structured handovers in pediatric emergencies remains unclear. We sought to determine if training with an airway, breathing, circulation, situation, background, assessment, recommendation handover tool could improve the transmission of essential patient information during multidisciplinary simulations of critically ill children.

METHODS:

We conducted a prospective, randomized, intervention study with first-year pediatric residents at a quaternary academic children’s hospital. Baseline and second handovers were recorded for residents in the intervention group (n = 12) and residents in the control group (n = 8) during multidisciplinary simulations throughout the academic year. The intervention group received handover education after baseline handover observation and a cognitive aid before second handover observation. Audio-recorded handovers were scored by using a Delphi-developed assessment tool by a blinded rater.

RESULTS:

There was no difference in baseline handover scores between groups (P = .69), but second handover scores were significantly higher in the intervention group (median 12.5 [interquartile range 12–13] versus median 7.5 [interquartile range 6–8] in the control group; P < .01). Trained residents were more likely to include a reason for the call (P < .01), focused history (P = .02), and summative assessment (P = .03). Neither timing of the second observation in the academic year nor duration between first and second observation were associated with the second handover scores (both P > .5).

CONCLUSIONS:

Structured handover training and provision of a cognitive aid may improve the inclusion of essential patient information in the handover of simulated critically ill children.
Autoimmune Pancreatitis and Ulcerative Rectocolitis in an Adolescent
Authors: Cousin Elie , Cousin Ianis , Aziz Karim , Chailloux Pascal , Breton Estelle
Published: April 2018

Autoimmune pancreatitis (AIP) is rare in teenagers and difficult to diagnose. There are no clear and established diagnostic criteria in the pediatric population to distinguish subtype 1 and subtype 2. Here, we report the case of a 16-year-old white French teenager admitted to the pediatric emergency service with more than 1 year’s history of pain originating from the epigastric and the right hypochondriac regions, with bloody diarrhea. After exclusion of pancreatic cancer and other common causes of acute pancreatitis, the diagnosis of AIP was suspected. Biological analyses revealed acute pancreatitis with severe cholestasis and an elevated level of serum immunoglobulin G4. Magnetic resonance cholangiography revealed a voluminous pancreas presenting a typical “sausage-like” aspect. Anatomopathological analyses of the liver biopsy specimen revealed a biliary obstruction due to pancreatic involvement without the typical aspect of chronic destructive cholangitis. Corticotherapy and immunosuppressive treatment proved effective after 1 week of treatment. Without a pancreatic biopsy specimen, the distinction between AIP type 1 and 2 could not be made clearly in this case. The succession of clinical observations could allow clinicians to recognize, treat, and manage AIP in children.
Erythropoietic Protoporphyria: Initial Diagnosis With Cholestatic Liver Disease
Authors: Coffey Amy , Leung Daniel H. , Quintanilla Norma M.
Published: April 2018

The porphyrias are a group of rare metabolic disorders that result from defects in heme biosynthesis. Erythropoietic protoporphyria (EPP) is the most common inherited porphyria in children and is diagnosed in most individuals after the onset of cutaneous manifestations. Hepatobiliary disease affects the minority of individuals with EPP and usually manifests in patients with an established diagnosis of EPP. We report on a classic but rare case of EPP that masqueraded as cholestasis. An 8-year-old boy was referred to the Hepatology Clinic after an abrupt onset of jaundice with a longstanding history of dermatitis. The diagnosis of EPP was established with liver biopsy, which revealed dense, dark-brown pigment in hepatocytes and Kupffer cells that, on polarization, displayed bright-red birefringence and centrally located Maltese crosses. Plasma total porphyrins and erythrocyte protoporphyrin were elevated and confirmed a diagnosis of EPP. We hope to raise awareness of this diagnosis among pediatricians, hepatologists, and pathologists and increase the consideration of EPP in patients with cholestatic liver disease and chronic dermatitis.
Pediatric Malignant Atrophic Papulosis
Authors: Huang Yung-Chieh , Wang Jiaan-Der , Lee Fang-Yi , Fu Lin-Shien
Published: April 2018

Malignant atrophic papulosis (MAP), also known as Degos disease, is an extremely rare disease that is characterized by its unique skin presentation (namely, central, porcelain-white atrophic lesions with a telangiectatic rim). MAP has the following 2 variants: cutaneous MAP is manifested in the skin alone, whereas systemic MAP affects the gastrointestinal tract, central nervous system, lungs, and other internal organs. Some patients who presented with only cutaneous symptoms at first may develop systemic symptoms several years later. Although the exact pathologic mechanisms are unclear, Magro et al suggested in a recent study that MAP is a vascular injury syndrome that involves complement component C5b–9 complex deposition and high expression of interferon-α. The prognosis of systemic MAP is poor and typically fatal within a few years. Nonetheless, because the C5b–9 complex is detected in MAP, some researchers have suggested combined treatment with eculizumab (a humanized monoclonal antibody against C5) and treprostinil (a prostacyclin analog). Here, we report on a girl with systemic MAP who had severe central nervous system involvement and responded to eculizumab.
Introduction to Transitions in the Life Course of Autism and Other Developmental Disabilities
Authors: Kuo Alice A. , Anderson Kristy A. , Crapnell Tara , Lau Lynette , Shattuck Paul T.
Published: April 2018

The Health Care Transitions Research Network for Autism Spectrum Disorder and other Developmental Disabilities and the Life Course Research Network, both funded by the Maternal and Child Health Bureau, invited articles for this Supplement. Our goal in this Supplement is to highlight and explore developmental and transition-related challenges over the life course of individuals on the autism spectrum and other neurodevelopmental disabilities, discuss the clinical and practice implications of these issues, highlight gaps in knowledge, and identify directions for future research.
Stakeholder Perspectives on Research and Practice in Autism and Transition
Authors: Kuo Alice A. , Crapnell Tara , Lau Lynette , Anderson Kristy A. , Shattuck Paul
Published: April 2018

OBJECTIVES:

Individuals with autism spectrum disorder (ASD) are reported to experience significant challenges during the transition to adulthood. Although recent evidence indicates that individuals with ASD experience poor outcomes in adulthood, little is understood about the contributing factors. In this qualitative study, we investigated the barriers to and needs in research and practice in the transition to adulthood among individuals with ASD.

METHODS:

Thirteen researchers, including service providers, family members, and an individual with ASD participated in 30- to 60-minute, semistructured, open-ended telephone interviews. Interviews were transcribed, and data were analyzed by using an inductive approach to identify themes related to barriers to and needs in the transition to adulthood for youth with ASD.

RESULTS:

Stakeholders identified the need for transition planning and preparation to begin earlier and for systems to better accommodate the interests and varying abilities of individuals with ASD. Stakeholders also felt that parent and service provider expectations and perceptions influence early opportunities and experiences offered throughout the transition process.

CONCLUSIONS:

This study reveals the multilevel barriers to and needs in the transition to adulthood and the need for interagency and multidisciplinary collaboration and research to address the varying levels of needs, abilities, and multisector challenges.
Transition of Individuals With Autism to Adulthood: A Review of Qualitative Studies
Authors: Anderson Kristy A. , Sosnowy Collette , Kuo Alice A. , Shattuck Paul T.
Published: April 2018

Many young adults with autism spectrum disorder experience poor transition outcomes in key areas, including postsecondary employment, higher education, health care, social connectedness, and independent living, yet we lack a clear understanding of the specific factors that impact these outcomes. We reviewed qualitative research in which the perspectives of youth and young adults with autism spectrum disorder, parents, services providers, and other stakeholders were gathered to identify barriers and facilitators to optimal outcomes. Findings revealed that poor transition outcomes are influenced by several factors, including poor person-environment fit, uncertainty about the roles of parents, and the lack of comprehensive or integrated services. These findings also revealed the aspects of familial, organizational, and policy contexts that may be targeted for interventions. Finally, stakeholders emphasized that supports should be individualized and focused on the changing aspects of the young adult’s social and physical environment rather than behavior change. We discuss implications for policy and practice and provide recommendations for further research.
Psychiatric and Medical Conditions in Transition-Aged Individuals With ASD
Authors: Davignon Meghan N. , Qian Yinge , Massolo Maria , Croen Lisa A.
Published: April 2018

BACKGROUND AND OBJECTIVES:

Children with autism spectrum disorder (ASD) have a variety of medical and psychiatric conditions and an increased use of health care services. There is limited information about the prevalence of psychiatric and medical conditions in adolescents and young adults with ASD. Our objective was to describe the frequency of medical and psychiatric conditions in a large population of diverse, insured transition-aged individuals with ASD.

METHODS:

Participants included Kaiser Permanente Northern California members who were enrolled from 2013 to 2015 and who were 14 to 25 years old. Individuals with ASD (n = 4123) were compared with peers with attention-deficit/hyperactivity disorder (n = 20 615), diabetes mellitus (n = 2156), and typical controls with neither condition (n = 20 615).

RESULTS:

Over one-third (34%) of individuals with ASD had a co-occurring psychiatric condition; the most commonly reported medical conditions included infections (42%), obesity (25%), neurologic conditions (18%), allergy and/or immunologic conditions (16%), musculoskeletal conditions (15%), and gastrointestinal (11%) conditions. After controlling for sex, age, race, and duration of Kaiser Permanente Northern California membership, most psychiatric conditions were significantly more common in the ASD group than in each comparison group, and most medical conditions were significantly more common in the ASD group than in the attention-deficit/hyperactivity disorder and typical control groups but were similar to or significantly less common than the diabetes mellitus group.

CONCLUSIONS:

Although more research is needed to identify factors contributing to this excess burden of disease, there is a pressing need for all clinicians to approach ASD as a chronic health condition requiring regular follow-up and routine screening and treatment of medical and psychiatric issues.
Inherited Immunodeficiency: A New Association With Early-Onset Childhood Panniculitis
Authors: Bader-Meunier Brigitte , Rieux-Laucat Frédéric , Touzot Fabien , Frémond Marie-Louise , André-Schmutz Isabelle , Fraitag Sylvie , Bodemer Christine
Published: April 2018

We report on 4 children who presented with aseptic panniculitis associated with inherited immunodeficiency. Three patients had a B-cell immunodeficiency resulting from mutations in the TRNT1 and NF-κb2 genes (no mutation was found in the third patient), and 1 had a T-cell deficiency (mutation in the LCK gene). Panniculitis occurred before the age of 2 years in the 4 patients and preceded the onset of recurrent infections because of immunodeficiency in 2. It presented either as nodules, which resolved spontaneously within 1 to 2 weeks (3 patients), or chronic ulcerative lesions (1 patient) associated with unexplained fever and elevated acute phase reactants, without evidence of infection or high-titer autoantibodies. Febrile nodules relapsed in 2 patients, and recurrent attacks of unexplained fever (without relapse of panniculitis) occurred in the third. Skin biopsy revealed predominantly lympho-histiocytic or septal neutrophilic panniculitis in 1 and 3 patients, respectively. Panniculitis was associated with dermal involvement in the 4 patients. Patients with B-cell deficiency received monthly intravenous immunoglobulin replacement. Two patients who underwent bone marrow transplant died of bone marrow transplant-related complications. The 2 remaining patients had persistent, mild autoinflammatory disease, which did not require specific treatment. In these cases, the need for careful immunologic evaluation of patients who present with unexplained panniculitis, especially early-onset panniculitis before the age of 2 years, is highlighted.
Clinical Benefit of NMDA Receptor Antagonists in a Patient With ATP1A2 Gene Mutation
Authors: Ueda Keisuke , Serajee Fatema , Huq Ahm M.
Published: April 2018

Mutations in the ATP1A2 gene cause familial hemiplegic migraine type 2, alternating hemiplegia of childhood, and cerebellar function deficits, epilepsy, and mental retardation. These symptoms are likely related to glutamatergic hyperexcitability. Our patient is a 12-year-old boy with a history of complex partial seizures, attention-deficit/hyperactivity disorder, and fine motor difficulty. During early childhood, he had episodes of a self-resolving right-sided hemiparesis and focal epilepsy. His seizures did not respond to several antiepileptic medications but stopped after he received valproate. His intermittent episodes of hemiplegia persisted. Additionally, he had pronounced bilateral fine motor impairment and significant executive deficits that gradually worsened. The whole exome sequencing revealed a de novo missense mutation in the ATP1A2 gene and a maternally inherited POLG gene mutation of unknown clinical significance. We hypothesized that glutamatergic excitotoxicity due to the ATP1A2 mutation contributed to the pathogenesis of our patient’s condition. He was started on N-methyl-D-aspartate receptor antagonists (memantine and dextromethorphan), as well as coenzyme Q10. One year later, he showed significant improvement in sustained attention, learning efficiency, general cognitive efficiency, and fine motor dexterity. We postulate that N-methyl-D-aspartate receptor antagonists were effective for behavioral, cognitive, and cerebellar symptoms in our patient with ATP1A2 gene mutation.
Topical Hypochlorite and Skin Acidification Improves Erythroderma of Omenn Syndrome
Authors: Wat Margaret , Olicker Arielle , Meyerson Howard , Nedorost Susan , Paller Amy S. , Cooper Kevin
Published: April 2018

We describe a case of Omenn syndrome displaying exudative erythroderma and other characteristic features, including alopecia, absent B and naïve T cells, hyper immunoglobulin E levels, and eosinophilia. A pathogenic recombination-activating RAG1 homozygous genetic mutation confirmed the diagnosis. She required frequent antibiotics at both treatment and prophylactic doses, which alone did not control her erythroderma, but her high risk of infection precluded the use of systemic agents such as cyclosporine, which would further suppress her already severely compromised immune system. Thrice-weekly topical dilute hypochlorite compresses, combined with skin acidification with a low pH emollient, were initiated to control inflammation and for cutaneous bacterial prophylaxis. She demonstrated a marked improvement in her erythroderma within days after treatment initiation. Further improvement continued with the addition of systemic corticosteroids, with resolution of erythroderma after her first dose. This case reveals for the first time that dilute topical hypochlorite and skin pH restoration holds promise to control severe dermatitis associated with immunodeficiency and inflammatory syndromes with minimal side effects.
HLA-A*31:01 and Oxcarbazepine-Induced DRESS in a Patient With Seizures and Complete DCX Deletion
Authors: Kim Hyun , Chadwick Laura , Alzaidi Yasir , Picker Jonathan , Poduri Annapurna , Manzi Shannon
Published: April 2018

Oxcarbazepine is an antiepileptic drug (AED) commonly used as a first-line treatment option for focal epilepsy. Several AEDs, including carbamazepine, oxcarbazepine, and phenytoin are associated with various delayed-hypersensitivity reactions such as drug reaction with eosinophilia and systemic symptoms, Stevens-Johnson syndrome, or toxic epidermal necrolysis. The Food and Drug Administration–approved label for oxcarbazepine currently presents information regarding a pharmacogenomic association with the HLA antigen allele HLA-B*15:02 and hypersensitivity reactions in certain ancestry groups with a high incidence of this allele. However, unlike carbamazepine, screening for the presence of this allele is not routinely recommended before administration of oxcarbazepine. In practice, even with carbamazepine, HLA antigen testing is not always performed before initiating treatment because of lack of physician awareness of the recommendations and because of the desire to initiate treatment without delay. We present the clinical course of a pediatric patient with focal epilepsy refractory to several AEDs who developed drug reaction with eosinophilia and systemic symptoms after oxcarbazepine administration. The pharmacogenomic testing for various HLA antigen alleles was performed post hoc, and results were evaluated for structural similarities between AEDs and their molecular associations with HLA antigen proteins. In addition, we review the population-wide prevalence of various hypersensitivity reactions to AEDs and associated HLA antigen alleles. Finally, we discuss the potential utility of preemptive pharmacogenomic screening of patients before pharmacological treatment of epilepsy to assess the risk of developing hypersensitivity reactions.
Neonatal Adenovirus Infection Complicated by Hemophagocytic Lymphohistiocytosis Syndrome
Authors: Censoplano Nina , Gorga Stephen , Waldeck Kate , Stillwell Terri , Rabah-Hammad Raja , Flori Heidi
Published: April 2018

Two infants with disseminated adenoviral infections are described. Both these infants had a similar clinical course and were also diagnosed with secondary hemophagocytic lymphohistiocytosis (HLH). Previous reports of immunocompromised adults with adenovirus-associated HLH are in the literature; however, this is the first report that we are aware of with this pathology occurring in infants. These cases are used to demonstrate the importance of thinking about HLH in patients who are diagnosed with adenovirus and exhibit prolonged fevers that are unresponsive to antimicrobial agents with hepatosplenomegaly and cytopenias.
The Case for the Case Report
Authors: Malatack J. Jeffrey
Published: April 2018

Whether it's raising new hypotheses, finding individual case exceptions to current paradigms, providing alerts to untoward outcomes, or suggesting new therapies to be further tested, the case report remains a viable form of medical publication.
La universidad vista por Unamuno: las funciones del rector y de los claustros
Authors: Maroco dos Santos Emanuel José
Published: April 2018

El hecho de que Unamuno sea un escritor autobiográfico nos permite determinar su concepción de rector, no solo sobre la base de lo que nos dice acerca de dicho cargo, sino también a partir de su experiencia, lo que es particularmente sugestivo, ya que la idea sui generis que tenía de dicho cargo se encarna en la realidad social que lo rodea.Por ejemplo, un texto que permitiría acceder a la experiencia de Unamuno como rector esel discurso que pronunció en el “Paraninfo de la Universidadde Salamanca el 1 de octubre de 1931, al inaugurar, en calidad de rector, el curso académico de 1931-1932” (cf. Unamuno 1971d 395).
Filosofía de la praxis como crítica de la hegemonía en Antonio Gramsci
Authors: Gómez Gutiérrez Juan José
Published: April 2018

Se exploran las relaciones entre hegemonía y filosofía de la praxis en A. Gramsci. Se examina la influencia de la filosofía hegeliana sobre estas nociones en los Cuadernos de la cárcel. Asimismo, se estudia la crítica a estas nociones a la luz del feminismo de la diferencia y del debate sobre hegemonía, universalidad y contingencia iniciado por E. Laclau, J. Butler y S. Žižek. Se concluye que concebir la hegemonía desde la perspectiva de la filosofía de la praxis significa que la acción político-intelectual, según Gramsci, no solo implica la formación de una hegemonía alternativa, sino una crítica políticamente operante a la pretensión de universalidad del discurso hegemónico a partir de la exposición de su contingencia histórica.
Kant on Free Will and Theoretical Rationality
Authors: Wolt Daniel
Published: April 2018

The focus of this essay is Kant’s argument in the Groundwork for the Metaphysics of Morals (GMS) III that regarding oneself as rational implies regarding oneself as free. After setting out  an interpretation of how the argument is meant to go (§§1-2), I argue that Kant fails to show that regarding oneself as free is incompatible with accepting universal causal determinism (§3). However, I argue that the argument succeeds in showing that regarding oneself as rational is inconsistent with accepting universal causal determinism if one accepts a certain, plausible view of the explanation of events (§4).
KERUGIAN SUMBER DAYA IKAN AKIBAT PRAKTIK MARK DOWN KAPAL PENANGKAP IKAN DI INDONESIA
Authors: Firdaus Maulana , Dewitasari Yesi , Pramoda Radityo , Koeshendrajana Sonny
Published: April 2018

Dampak praktik Illegal Unreported and Unregulated Fishing (IUUF) telah mengakibatkan terganggunya pengelolaan pemanfaatan perikanan yang berkelanjutan dan menimbulkan kerugian ekonomi. Praktik mark down ukuran kapal penangkapan ikan merupakan salah satu penyalahgunaan perizinan dalam konteks praktik IUUF. Penelitian ini bertujuan untuk mengkaji nilai kerugian sumber daya ikan (deplesi sumber daya) akibat praktik “mark down” ukuran kapal penangkap ikan yang dilakukan di Indonesia. Penelitian dilaksanakan pada bulan Maret – April 2017 bersifat ‘desk study’ dan dilengkapi dengan kajian literature terkait. Data sekunder dan primer digunakan dalam penelitian ini. Analisis data dilakukan melalui Pendekatan Surplus Produksi Model Schaefer digunakan dalam penelitian ini. Nilai kerugian sumber daya ikan diketahui berdasarkan nilai deplesinya. Nilai deplesi sumber daya menggunakan pendekatan The Net Price Method. Hasil penelitian menunjukkan bahwa adanya praktik “mark down” telah menyebabkan deplesi sumber daya ikan atau pengurangan aset sumber daya ikan di perairan Indonesia. Besarnya nilai deplesi sumber daya pada tahun 2015 mencapai 9,83 trilyun rupiah dan diprediksi pada tahun 2020 meningkat menjadi 14,55 trilyun rupiah. Kajian merekomendasikan perlunya percepatan pengukuran ulang kapal perikanan dan penerapan sangsi yang tegas terhadap pelanggar sehingga tata kelola pemanfaatan sumber daya perikanan tangkap yang baik yang mampu mewujudkan pengelolaan perikanan yang berkelanjutan di Indonesia. Tittle: Fish Resources Losses Due to Mark Down Fishing Vessel Practiced in IndonesiaThe impact of IUU fishing has resulted in management disorder of sustainable fisheries and it caused economic loss. Markdown in vessels size is one type of manipulation practices of license in IUU fishing. This study aims to analyze the loss value of fish resources (resource depletion) due to the “markdown” practices in Indonesia. The study was basically a desk study completmenting with relevant literatures review during March – April 2015. Primary and secondary data were used in this study. Data were analyzed using the Schaefer surplus production model approached. Loss value of fish resources was estimated in terms of depletion resource value using the Net Price Method. The research found that “mark down” has led to depletion or reduction of fish resources in Indonesian waters. The estimated value of resource depletion in 2015 reached 9.83 trillion rupiahs and it is predicted to rise into 14.55 trillion rupiahs in 2020. The research suggests the need to accelerate the process of re-measuring the size of fishing vessel as well as to impose sanctions for the disobedience of the rules, so that Indonesia could have a good governance in fisheries resource management with sustainable fisheries resources.
Minor defect correlation with dynamic elastic properties of polypropylene fiber-reinforced concrete
Authors: Gupta Rishi , El-Newihy Adham , Shah Mandeep
Published: April 2018

The efficient rehabilitation of aging civil infrastructure requires innovative and emerging materials along with the proper implementation of structural health monitoring (SHM). Prior to identifying a strategic SHM technique, the understanding of defects in structures is vital. Common defects in concrete include consolidation problems and the development of microcracks during consolidation or stress induction. Monitoring the dynamic characteristics of concrete can play an essential role in detecting real-time and early stages of deterioration. Much research is focused on detecting large defects; however, not much information is available on the detection of minor defects in composites such as fiber-reinforced concrete. This study focuses on testing and monitoring of the dynamic elastic behavior of concrete using a non-destructive resonant frequency approach. The change in dynamic elastic properties of normal concrete under flexural and compression loading is analyzed. Moreover, an initial attempt to monitor the change in the elastic behavior when polypropylene fibers are added as reinforcement is also investigated. Experimental results show a decrease in the dynamic modulus of elasticity when minor defects are present and when polypropylene fibers are added to plain concrete mixture.
12 – 14 YAŞ GRUBU BAYAN HENTBOLCULARA UYGULANAN 8 HAFTALIK PLİOMETRİK ANTRENMANLARIN SEÇİLMİŞ BAZI KUVVET PARAMETRELERİNE ETKİSİ
Authors: PANCAR Zarife , BİÇER Mürsel , ÖZDAL Mustafa
Published: April 2018

Bu çalışma, sekiz haftalık pliometrik
antrenman programının, 12- 14 yaş kadın hentbolcuların seçilmiş bazı kuvvet
parametrelerine etkilerinin incelenmesi amacı ile yapılmıştır. Çalışmaya
katılan hentbolcular, deney (n=14, yaş: 13,07±0,83 yıl) ve kontrol (n=14, yaş:
13,07± 0,83 yıl) grubu olmak üzere iki gruba ayrılmıştır. Her iki gruba da
çalışma öncesi ve sonrasında yaş, boy, ağırlık, dikey sıçrama, durarak uzun
atlama, 30 sn mekik testi, 30 sn şınav testi uygulanmıştır. Deney grubuna sekiz
haftalık, haftada üç gün pliometrik antrenman programı hentbol antrenmanı ile
birlikte uygulanmıştır. Kontrol grubu ise düzenli olarak hentbol
antrenmanlarına devam etmiştir. Verilerin analizi için bağımlı t-testi ve
bağımsız t-testi kullanılmıştır. Elde ettiğimiz veriler doğrultusunda, deney ve
kontrol grubu arasında dikey sıçrama, durarak uzun atlama, 30 sn mekik testi,
30 sn şınav testi, açısından deney grubu lehine anlamlılık saptanmıştır
(p<0,05). Deney grubunun ön test ve son testi karşılaştırıldığında dikey
sıçrama, durarak uzun atlama, 30 sn mekik testi, 30 sn şınav testi açısından
anlamlı bir artış tespit edilmiştir (p<0,05). Kontrol grubunda ise yalnızca
durarak uzun atlama ve 30 sn şınav testleri açısından anlamlılığa rastlanmıştır
(p<0,05). Sonuç olarak, sekiz haftalık pliometrik antrenman programının
12-14 yaş kadın hentbolcuların kuvvet özelliklerini olumlu yönde etkilediği
söylenebilir.
Lecciones desde África: Impacto socioeconómico del enclave turístico en Livingstone, Zambia
Authors: Ngwira Percy Mabvuto
Published: April 2018

El presente artículo emplea el paradigma de dependencia para ilustrar los impactos socioeconómicos del turismo de enclave en países en desarrollo utilizando la ciudad de Livingstone en Zambia (África) como caso de estudio. Utilizando fuentes primarias y secundarias, este estudio revela que los inversionistas extranjeros y los turistas internacionales dominan principalmente la industria del turismo en Livingstone. El dominio del sector por parte de inversionistas extranjeros ha resultado en el desarrollo de un paradigma de dependencia de la actividad turística para contribuir significativamente al desarrollo socioeconómico de la ciudad de Livingstone. A pesar de ser acreditado como un sector importante que contribuye al desarrollo socioeconómico y al alivio de la pobreza, el enfoque del paradigma de dependencia del desarrollo turístico en Livingstone ha conducido a vínculos más débiles con la economía doméstica, lo que ha tenido un impacto mínimo en el crecimiento económico general de la ciudad. Además, el turismo en Livingstone se ha vuelto poco competitivo desde una perspectiva comercial debido al dominio de la propiedad extranjera de las principales empresas turísticas que han creado carteles y oligopolios en el negocio, creando así barreras para que las empresas locales compitan efectivamente. Debido a su naturaleza, el sector turístico en Livingstone no se puede declarar como sostenible. Para abordar los problemas del turismo de enclave y promover un desarrollo turístico más inclusivo y beneficioso en Livingstone y en otros destinos turísticos de países en desarrollo, es necesario adoptar políticas y estrategias que garanticen la plena participación de las comunidades locales y un retorno doméstico de las significativas ganancias del turismo. Las estrategias también deben garantizar que el desarrollo turístico tenga fuertes vínculos con el resto de actividades socioeconómicas locales.
Turismo sostenible y emprendimiento social. El pueblo mágico de Tequila, México
Authors: Saiz Alvarez Jose Manuel
Published: April 2018

El programa “Pueblos Mágicos” en México tiene como objetivo garantizar la sostenibilidad turística del territorio para combatir la pobreza, preservar el rico y único patrimonio cultural e histórico existente y promover la inclusión de la población indígena. Según datos del CONEVAL (2017), más de 55,3 millones de personas viven en pobreza (46,2% de la población) y cerca de 11,4 millones están en pobreza extrema en México. Sin embargo, el Pueblo Mágico de Tequila, del estado de Jalisco, es una excepción, por lo que el objetivo de esta investigación será analizar las causas de este triunfo. Para ello se utiliza una metodología cuali-cuantitativa basada en la técnica de la observación y el análisis de contenido para analizar la sostenibilidad turística de la población. Se realiza, además, un análisis DAFO para tener así un análisis holístico del tema a analizar. Las principales conclusiones son: [1] Dados los buenos resultados conseguidos, se ha de continuar con la política de protección tequilera siguiendo una doble perspectiva público-privada; [2] La excesiva protección del tequila evita el desarrollo de otras bebidas destiladas dentro de la región; [3] La producción y la comercialización del tequila contribuye a la sostenibilidad del turismo gastronómico y de naturaleza en la región tequilera; y [4] El tequila constituye una pieza clave dentro de la Política Turística de México al 2040, lo que garantiza su continuidad y evita el fortalecimiento de bebidas sustitutivas al tequila.
Percepción y planificación del turismo sostenible en comunidades indígenas de México
Authors: Lievano Torres Kristian , Mazó Quevedo María Lyssette , Torres Méndez Fabiola
Published: April 2018

La investigación destaca la necesidad de salvaguardar las riquezas culturales de los pueblos indígenas, más aún cuando en sus comunidades se promueven y llevan a cabo actividades turísticas que ponen en riesgo su identidad como culturas autóctonas preservadoras de sus tradiciones y lengua de origen. Tal es el caso de las comunidades Mayas Chontales en el municipio de Nacajuca, Tabasco, México. Donde se preservan costumbres ancestrales como la lengua chontal y la elaboración de artesanías. El trabajo tiene como objetivo analizar el grado de participación activa de los pobladores en cuatro comunidades pertenecientes al corredor Bijí Yokot’an, donde la población Maya Chontal funge como parte de la oferta turística. El diseño metodológico es con enfoque mixto. La recolección de información incluyó: observación directa, entrevistas exploratorias y encuestas con sondeos de opinión. Para el cálculo de la muestra se realizó un muestreo aleatorio en cada comunidad utilizando la estimación de una proporción, y con base en los resultados del muestreo se aplicaron 304 cuestionarios en las comunidades objeto de estudio. Los resultados obtenidos indican que en dichas comunidades no se promueve la práctica del turismo sostenible. Incluso, hay artesanos quienes permanecen como espectadores del producto turístico desarrollado en sus comunidades. Por tanto, el trabajo demuestra la ausencia de una oferta turística planificada, así como la falta de toma de conciencia y participación en la mayoría de los habitantes en la zona.
Saving Preferences and Financial Literacy of Self Help Group Members: A Study of Uttar Pradesh
Authors: Ruhela Sandhya , Prakash Ajai
Published: April 2018

Microfinance is the provision of financial services to the rural and underprivileged sections of the society to engage in income-generating activities for their own growth and progress. This paper examines the income generating activities of Self-Help Groups (SHGs) for empowerment of women. It also analyses the saving preferences of SHG members and examines the financial literacy of these members after joining the SHGs. The analysis reveals that the different types of income generating activities started by SHG members include embroidery work, dairy, flower and bangle making, jute work, stitching work, vegetable cultivation and own shop. Among these, ‘own shop’ is the most prominent income generating activity followed by ‘stitching work’. In terms of saving preference, ‘own business development’ is their first saving preference for sustainable livelihood; ‘education of children’ is their saving preference for future of their children and ‘repairing of house’ is their saving preference for immediate expenditure. The analysis in terms of financial literacy reveals that financial literacy of SHG members regarding ration card, voter card, bank account and their phone number has increased after joining of SHGs.
Environmental Kuznets Curve: An Empirical Analysis in the Indian Context
Authors: Gambhir Sakshi
Published: April 2018

The relationship between economic growth and environmental quality has been much under dispute. According to the EKC (Environmental Kuznets Curve) hypothesis, environmental damage increases in the early stages of economic growth, but diminishes once nations reach higher levels of income. While the notion EKC is well established, there is controversy about its shape, incidence and determinants. In this paper, we model EKC with the variables of GDP and CO2 emissions (aggregate and per capita) using alternative model specifications to bridge the gap between conventional and modern EKC literature. We also place the theoretical construct of EKC into a policy-oriented framework by incorporating the impact of four global policy periods namely, liberalisation, globalisation, world recovery and global financial crisis. We substantiate a cubic form of EKC in the Indian context for the time period 1991 to 2014. With aggregate CO2 emissions as the dependent variable, the linear, quadratic and cubic terms are all significant with the expected signs, which confirm an N-shaped EKC for India. Even with per capita emissions as the dependent variable, existence of an N-shaped EKC is established. In this case however, evidence on the cubic term is rather weak which points towards the difference in socio-psychological factors that influence the revival of upturn in the case of India. The policy period analysis does not show any distinct results, which could be due to contradictory effects on different variables and volatility in these variables.