bluuebunny/crossref_metadata_2025 huggingface dataset:
Endogenous Ciliary Neurotrophic Factor Is a Lesion Factor for Axotomized Motoneurons in Adult Mice
Authors: Sendtner Michael , Götz Rudolf , Holtmann Bettina , Thoenen Hans
Published: April 2018

Ciliary neurotrophic factor (CNTF) is an abundant cytosolic molecule in myelinating Schwann cells of adult rodents. In newborn animals in which CNTF is not yet expressed, exogenous CNTF that is locally administered very effectively protects motoneurons from degeneration by axotomy. To evaluate whether endogenous CNTF, released after nerve injury from the cytosol of Schwann cells, supports motoneuron survival, we transected the facial nerve in 4-week-oldpmnmice. In this mouse mutant a rapidly progressing degenerative disease of motoneurons starts by the third postnatal week at the hindlimbs and progresses to the anterior parts of the body, leading to death by the seventh to eighth week. Apoptotic death of motoneurons can be observed during this period, as revealed by TUNEL staining. In 6-week-old unlesionedpmnmice ∼40% of facial motoneurons have degenerated. Facial nerve lesion dramatically increased the number of surviving motoneurons inpmnmice. This protective effect was absent inpmnmice lacking endogenous CNTF. Quantitative analysis of leukemia inhibitory factor (LIF) mRNA expression revealed that the dramatic upregulation seen in wild-type mice after peripheral nerve lesion did not occur inpmnmice. Therefore, endogenous LIF cannot compensate for the lack of CNTF inpmncrossbred with CNTF knock-out mice. Thus, endogenous CNTF released from lesioned Schwann cells supports the survival of axotomized motoneurons under conditions in which motoneurons are in the process of rapid degeneration.
A Postsynaptic Excitatory Amino Acid Transporter with Chloride Conductance Functionally Regulated by Neuronal Activity in Cerebellar Purkinje Cells
Authors: Kataoka Yosky , Morii Hiroshi , Watanabe Yasuyoshi , Ohmori Harunori
Published: April 2018

Excitatory amino acid (EAA) neurotransmitters induce postsynaptic depolarization by activating receptor-mediated cation conductances, a process known to underlie changes in synaptic efficacy. Using a patch-clamp method, we demonstrate here an EAA-dependent postsynaptic anion conductance mediated by EAA transporters present on cerebellar Purkinje cell bodies and dendrites in culture. This transporter-mediated current was modulated by neuronal activity: it exhibited facilitation for >20 min after transient depolarization accompanied by Ca2+influx. Evidence is presented suggesting that the transporter facilitation is mediated by arachidonate release after Ca2+-dependent activation of phospholipase A2, which exists in Purkinje cells. This postsynaptic reuptake system may represent a novel modulatory mechanism of synaptic transmission as well as prevent neuronal excitotoxicity.
Morphogenesis of the Node of Ranvier: Co-Clusters of Ankyrin and Ankyrin-Binding Integral Proteins Define Early Developmental Intermediates
Authors: Lambert Stephen , Davis Jonathan Q. , Bennett Vann
Published: April 2018

AnkyrinG480/270 kDa and three ankyrin-binding integral membrane proteins (neurofascin, NrCAM, and the voltage-dependent sodium channel) colocalize within a specialized domain of the spectrin–actin network found at axonal segments of nodes of Ranvier in myelinated axons. Before myelination in embryonic nerves, ankyrinG480/270 kDa and the related ankyrin isoform ankyrinB440 kDa are co-expressed along with NrCAM in an abundant, continuous distribution along the length of axons. This study has resolved intermediate stages in the developmental transition from a continuous distribution of ankyrinG480/270 kDa in all axons to a highly polarized localization at the node of Ranvier in the developing rat sciatic nerve. The first detected event is formation of clusters containing the cell adhesion molecules neurofascin and NrCAM at sites independent of myelin-associated glycoprotein (MAG)-staining Schwann cell processes. Subsequent steps involve recruitment of ankyrinG480/270 kDa and the voltage-dependent sodium channel to cluster sites containing cell adhesion molecules, and elaboration of MAG-staining Schwann cell processes adjacent to these cluster sites. Formation of the mature node of Ranvier results from the fusion of asynchronously formed pairs of clusters associated with MAG-positive Schwann cells flanking the site of presumed node formation. Studies with the hypomyelinating mutant mousetremblerdemonstrate that the elaboration of compact myelin is not required for the formation of these clustered nodal intermediates. Clustering of neurofascin and NrCAM precedes redistribution of ankyrinG480/270 kDa and the voltage-dependent sodium channel, suggesting that the adhesion molecules define the initial site for subsequent assembly of ankyrin and the voltage-dependent sodium channel.
Cell Coupling and Uncoupling in the Ventricular Zone of Developing Neocortex
Authors: Bittman Kevin , Owens David F. , Kriegstein Arnold R. , LoTurco Joseph J.
Published: April 2018

Cells within the ventricular zone (VZ) of developing neocortex are coupled together into clusters by gap junction channels. The specific role of clustering in cortical neurogenesis is unknown; however, clustering provides a means for spatially restricted local interactions between subsets of precursors and other cells within the VZ. In the present study, we have used a combination of 5-bromo-2′-deoxyuridine (BrDU) pulse labeling, intracellular biocytin labeling, and immunocytochemistry to determine when in the cell cycle VZ cells couple and uncouple from clusters and to determine what cell types within the VZ are coupled to clusters. Our results indicate that clusters contain radial glia and neural precursors but do not contain differentiating or migrating neurons. In early neurogenesis, all precursors in S and G2phases of the cell cycle are coupled, and approximately half of the cells in G1are coupled. In late neurogenesis, however, over half of the cells in both G1and S phases are not coupled to VZ clusters, whereas all cells in G2are coupled to clusters. Increased uncoupling in S phase during late neurogenesis may contribute to the greater percentage of VZ cells exiting the cell cycle at this time. Consistent with this hypothesis, we found that pharmacologically uncoupling VZ cells with octanol decreases the percentage of VZ cells that enter S phase. These results demonstrate that cell clustering in the VZ is restricted to neural precursors and radial glia, is dynamic through the cell cycle, and may play a role in regulating neurogenesis.
Axonal Transport Blockade in the Neonatal Rat Optic Nerve Induces Limited Retinal Ganglion Cell Death
Authors: Fagiolini Michela , Caleo Matteo , Strettoi Enrica , Maffei Lamberto
Published: April 2018

Optic nerve section in the newborn rat results in a rapid apoptotic degeneration of most axotomized retinal ganglion cells (RGCs). This massive process of neuronal death has been ascribed mainly to the interruption of a trophic factor supply from target structures rather than to the axonal damage per se. To distinguish between these two possibilities, we induced a reversible axonal transport blockade in the developing optic nerve by topical application of a local anesthetic (lidocaine). Light and electron microscopy showed no alterations in the fine structure of treated optic nerves. Retinae of treated and control rats were stained with cresyl violet and examined at different times after surgery. We found that axonal transport blockade induced only a limited number of pyknotic RGCs. Degeneration of these cells was completely prevented by inhibiting protein synthesis during lidocaine application. We conclude that the rapid degeneration of RGCs after axotomy can be ascribed only partly to the loss of retrogradely transported trophic factors.
Aβ Deposition Is Associated with Neuropil Changes, but not with Overt Neuronal Loss in the Human Amyloid Precursor Protein V717F (PDAPP) Transgenic Mouse
Authors: Irizarry Michael C. , Soriano Ferdie , McNamara Megan , Page Keith J. , Schenk Dale , Games Dora , Hyman Bradley T.
Published: April 2018

The PDAPP transgenic mouse overexpresses human amyloid precursor protein V717F (PDAPP minigene) and develops age-related cerebral amyloid-β protein (Aβ) deposits similar to senile plaques in Alzheimer’s disease. We find age-related cortical and limbic Aβ deposition that begins at 8 months and progresses to cover 20–50% of the neuropil in cingulate cortex, entorhinal cortex, and hippocampus of 18-month-old heterozygotic animals. The regional patterns of transgene expression and amyloid deposition suggest that Aβ deposits occur at the terminals of overexpressing neurons. Amyloid deposition is associated with dystrophic neurites and extensive gliosis. However, stereological analysis shows that there is no overt neuronal loss in entorhinal cortex, CA1 hippocampal subfield, or cingulate cortex through 18 months of age. In addition, there is no apparent loss of mRNA encoding neuronal synaptic, cytoskeletal, or metabolic proteins. Thus, widespread Aβ deposition in 18-month-old heterozygotic mice produces neuritic alterations and gliosis without widespread neuronal death.
GDNF Protection against 6-OHDA: Time Dependence and Requirement for Protein Synthesis
Authors: Kearns Cecilia M. , Cass Wayne A. , Smoot Kyle , Kryscio Richard , Gash Don M.
Published: April 2018

Glial cell line-derived neurotrophic factor (GDNF) injected intranigrally protects midbrain dopamine neurons against 6-hydroxydopamine (6-OHDA) toxicity. The timing between GDNF administration and exposure to 6-OHDA is critical in achieving optimal protection. When injected 6 hr before an intranigral injection of 6-OHDA, GDNF provides complete protection as measured by the number of surviving neurons in the substantia nigra of adult rats. The surviving neuronal population decreases by ∼50% with 12 and 24 hr separating GDNF and 6-OHDA administrations. In controls with 6-OHDA lesions, there is <10% survival of nigral dopamine neurons. No significant increase in survival is seen with either concurrent injections of GDNF and 6-OHDA or 1 hr GDNF pretreatment. Based on HPLC measurements, striatal and midbrain dopamine levels are at least twofold higher on the lesioned side in animals receiving GDNF 6 hr before a 6-OHDA lesion compared with vehicle recipients. Protein synthesis is necessary for GDNF-induced neuroprotective effects because cycloheximide pretreatment that inhibits protein synthesis also blocks neuroprotection.
Obstacle Avoidance and a Perturbation Sensitivity Model for Motor Planning
Authors: Sabes Philip N. , Jordan Michael I.
Published: April 2018

A novel obstacle avoidance paradigm was used to investigate the planning of human reaching movements. We explored whether the CNS plans arm movements based entirely on the visual space kinematics of the movements, or whether the planning process incorporates specific details of the biomechanical plant to optimize the trajectory plan. Participants reached around an obstacle, the tip of which remained fixed in space throughout the experiment. When the obstacle and the start and target locations were rotated about the tip of the obstacle, the visually specified task constraints retained a rotational symmetry. If movements are planned in visual space, as indicated from a variety of studies on planar point-to-point movements, the resulting trajectories should also be rotationally symmetric across trials. However, systematic variations in movement path were observed as the orientation of the obstacle was changed. These path asymmetries can be accounted for by a class of models in which the planner reduces the likelihood of collision with the obstacle by taking into account the anisotropic sensitivity of the arm to external perturbations or uncertainty in joint level control or proprioception. The model that best matches the experimental results uses planning criteria based on the inertial properties of the arm.
A Polysynaptic Feedback Circuit in Rat Visual Cortex
Authors: Johnson Randall R. , Burkhalter Andreas
Published: April 2018

Feedback connections from extrastriate cortex to primary visual cortex (V1) in the primate may provide “top-down” information that plays a role in visual attention and object recognition. Our work in a rodent model of corticocortical circuitry demonstrates that feedback pathways synapse preferentially with pyramidal cells in V1 (Johnson and Burkhalter, 1996) and favor excitation over inhibition in cortical microcircuits (Shao and Burkhalter, 1996).

To investigate the polysynaptic circuits activated by feedback inputs, we studied chains of neurons postsynaptic to feedback connections using a combination of axonal tract tracing and anterograde degeneration. This approach enabled independent labeling of local collaterals of forward-projecting neurons in V1 and feedback connections from extrastriate lateromedial (LM) visual area to V1. Postsynaptic targets were identified in the electron microscope after retrograde transport of biotinylated dextran amine (BDA) to identify dendrites of forward-projecting neurons (i.e., from V1 to LM) and postembedding immunogold labeling to identify GABAergic interneurons.

The results show that feedback connections provide strong monosynaptic input to forward-projecting neurons in V1. These neurons in turn make local connections that preferentially form synapses with other pyramidal cells (∼97%), many of which were identified as forward-projecting neurons. This indicates that feedback pathways provide input directly to neurons which make the reciprocal forward connection, and that feedback-recipient forward-projecting neurons are strongly interconnected. The function of these excitatory networks within V1 may be to amplify feedback activity and provide a circuit for modulation of striate cortical activity by top-down influences.
Modified Origins of Cortical Projections to the Superior Colliculus in the Deaf: Dispersion of Auditory Efferents
Authors: Butler Blake E. , Sunstrum Julia K. , Lomber Stephen G.
Published: April 2018

Following the loss of a sensory modality, such as deafness or blindness, crossmodal plasticity is commonly identified in regions of the cerebrum that normally process the deprived modality. It has been hypothesized that significant changes in the patterns of cortical afferent and efferent projections may underlie these functional crossmodal changes. However, studies of thalamocortical and corticocortical connections have refuted this hypothesis, instead revealing a profound resilience of cortical afferent projections following deafness and blindness. This report is the first study of cortical outputs following sensory deprivation, characterizing cortical projections to the superior colliculus in mature cats (N= 5, 3 female) with perinatal-onset deafness. The superior colliculus was exposed to a retrograde pathway tracer, and subsequently labeled cells throughout the cerebrum were identified and quantified. Overall, the percentage of cortical projections arising from auditory cortex was substantially increased, not decreased, in early-deaf cats compared with intact animals. Furthermore, the distribution of labeled cortical neurons was no longer localized to a particular cortical subregion of auditory cortex but dispersed across auditory cortical regions. Collectively, these results demonstrate that, although patterns of cortical afferents are stable following perinatal deafness, the patterns of cortical efferents to the superior colliculus are highly mutable.

SIGNIFICANCE STATEMENTWhen a sense is lost, the remaining senses are functionally enhanced through compensatory crossmodal plasticity. In deafness, brain regions that normally process sound contribute to enhanced visual and somatosensory perception. We demonstrate that hearing loss alters connectivity between sensory cortex and the superior colliculus, a midbrain region that integrates sensory representations to guide orientation behavior. Contrasting expectation, the proportion of projections from auditory cortex increased in deaf animals compared with normal hearing, with a broad distribution across auditory fields. This is the first description of changes in cortical efferents following sensory loss and provides support for models predicting an inability to form a coherent, multisensory percept of the environment following periods of abnormal development.
Understanding Parental Preferences for Participants in Medical Decision-making for Their Hospitalized Children
Authors: Dadlez Nina M. , Bisono Gabriela M. , Williams Camille Y. , Rosenthal Susan L. , Hametz Patricia A.
Published: April 2018

OBJECTIVES:

Family-centered care promotes parental engagement in medical decision-making for hospitalized children. Little is understood about parental preferences and factors influencing the desire to involve extended family in decision-making. We explored parent and family member interest in participation in medical decision-making.

METHODS:

Parents of hospitalized children ≤7 years old admitted to the inpatient service were interviewed regarding preferences for self-, other parent, and extended family involvement in decision-making. Scores were calculated for each potential participant on a scale of 1 to 5 (5 indicating that parents strongly agreed with participation). Associations of decision-making preferences with parental age, education, language, and health; the involvement of a child with chronic illness; and the level of clinical acuity were assessed with χ2 tests, Wilcoxon rank tests, and the Spearman correlation.

RESULTS:

There were 116 participants. Parents’ median level of interest in participation in decision-making was as follows: self (4.3; interquartile range [IQR]: 4–4.6); other parent (3.6; IQR: 2.7–4), and family (2.0; IQR: 1.7–2.7). Parents with better physical health (P < .001) and those in a relationship with the other parent (P < .001) were more likely to desire involvement of the other parent in medical decision-making. This was also true for those who faced higher acuity scenarios. Parents <35 years old (P < .01) and those who were interviewed in Spanish (P = .03) were more likely to desire participation of extended family members.

CONCLUSIONS:

Parents of hospitalized children want to participate in medical decision-making. Desire for the involvement of other family members is complex; therefore, discussions regarding parental preferences are necessary.
Acceptance of Routine HIV Testing by Hospitalized Adolescents and Young Adults
Authors: Bhalakia Avni M. , Talib Hina J. , Choi Jaeun , Watnick Dana , Bochner Risa , Futterman Donna , Gross Elissa
Published: April 2018

BACKGROUND AND OBJECTIVES:

Youth carry a disproportionate burden of new HIV infections. With our study, we aimed to characterize HIV testing experiences among adolescents and young adults admitted to a children’s hospital that is located in a high HIV-prevalent community and implemented routine HIV testing for all patients ≥13 years of age.

METHODS:

A total of 120 patients aged 13 to 24 years old who were admitted to our hospital and had a documented offer of routine HIV testing on admission were invited to complete a self-administered survey that asked about sex, race and/or ethnicity, HIV risk behaviors, and attitudes toward routine HIV testing in the hospital. Date of birth, admission diagnosis, and verification of HIV testing and results were collected by chart review.

RESULTS:

Study participants (N = 99) were 17.4 ± 2.3 years old, 52% female, 47% Hispanic, and 29% African American. Additional characteristics include the following: 65% had previous sexual activity, 11% had a history of sexually transmitted infections, and 12% were worried about their risk for HIV. Forty-seven percent of participants accepted HIV testing, with older patients (P < .01) and those reporting previous sexual activity (P < .01) and a previous HIV test (P < .01) being more likely to accept testing. A total of 96% of participants agreed that the hospital is a good place to offer HIV testing.

CONCLUSIONS:

Our findings support offering routine HIV testing to youth admitted to children’s hospital. Given the high incidence of new and undiagnosed HIV infections among youth, additional venues for HIV testing are essential.
The Effect of Malnutrition on the Risk of Unplanned 7-Day Readmission in Pediatrics
Authors: Ehwerhemuepha Louis , Bendig Donald , Steele Caroline , Rakovski Cyril , Feaster William
Published: April 2018

BACKGROUND:

Malnutrition is known to be associated with higher morbidity and a risk factor of readmissions in the adult population. In this study, we explore the effect of malnutrition in pediatrics because it may differ from the adult population.

METHODS:

Data for all inpatient encounters at a tertiary children’s hospital within a 2-year period corresponding to 19 702 visits were obtained. The data included demographics, socioeconomic status, registered dietitian diagnosis of malnutrition, and variables of the LACE readmission model. We excluded all neonates and patients older than 21 years. A multivariable logistic model was obtained by implementing best subset regression on these variables, controlling for demographics and socioeconomic status, and considering all possible 2-way statistical interactions between malnutrition and the variables for demographics and socioeconomic status.

RESULTS:

We discovered a statistical interaction effect between a patient’s age and malnutrition status (P value = .002) with respect to odds of unplanned 7-day readmission. It is indicated in this interaction term that patients who were malnourished had higher odds of readmission than patients who were not malnourished. Furthermore, younger patients who were malnourished were at increased odds of readmission than their older peers, whereas among patients who were not malnourished, younger patients were at reduced odds of readmission.

CONCLUSIONS:

The statistical interaction effect revealed that a patient’s risk of readmission is jointly modified by the patient’s age and malnutrition status. This finding advances our understanding of the complex picture of the simultaneous risk factor of unplanned 7-day readmissions in pediatrics.
Human Milk Banking: An Indian Experience
Authors: Nangia Sushma , Sachdeva Ruchika Chugh , Sabharwal Vandana
Published: April 2018

As part of integrated newborn care, human milk banks can reduce death and illness as well as lower health-care costs for infants born prematurely, especially with birthweights less than 1,500 g, and for infants born in resource-limited settings without access to their mother’s milk. Promotion of human milk banks is of special significance in India which has the highest burden of such infants. About 50 milk banks are insufficient to meet the needs of vulnerable infants. The government of India has acknowledged the role that human milk banking can play in reducing neonatal mortality and morbidity, and launched the “National Guidelines on Lactation Management Centers in Public Health Facilities” in 2017 with a vision to make breast milk universally available for all infants. The government is now working on an implementation strategy to scale up the lactation management center model (promotion of breastfeeding, kangaroo mother care, and donor human milk) for all newborn care units and delivery centers in the country. However, for effective expansion, it will be important to ensure improved government ownership, mandate availability of standard operating procedures at all facilities, stringent systems of quality control, standardized accreditation, and a robust monitoring system. Local evidence on the effectiveness of the comprehensive lactation management center (CLMC) model and knowledge, practices, and perceptions of human milk banking are limited. There is a need for rigorous implementation, process research, and technology innovation, along with a robust regulatory framework to prevent commercialization. In addition, attitude changes of mothers, maternal influencers, and health-care providers are all essential to successfully expand the CLMC model.
Health Care and Societal Costs of Bronchopulmonary Dysplasia
Authors: Lapcharoensap Wannasiri , Lee Henry C. , Nyberg Amy , Dukhovny Dmitry
Published: April 2018

Despite significant technological advances and increasing survival of premature infants, bronchopulmonary dysplasia (BPD) continues to be the most prevalent major morbidity in surviving very low-birthweight infants. Infants with BPD are often sicker, require longer stays in the NICU, and accumulate greater hospital costs. However, care of the infant with BPD extends beyond the time spent in the NICU. This article reviews the costs of BPD in the health-care setting, during the initial hospitalization and beyond, and the long-term neurodevelopmental impact of BPD, as well as the impact on a family caring for a child with BPD.
Use of Insulin Glargine in the Management of Neonatal Hyperglycemia in an ELBW Infant
Authors: Hwang Min J. , Newman Robert , Philla Katherine , Flanigan Elizabeth
Published: April 2018

Neonatal hyperglycemia is common in extremely low birth weight (ELBW) infants because of physiologic stress, exogenous glucose infusion, and postnatal corticosteroid therapy for hypotension, adrenal insufficiency, and pulmonary immaturity. The use of long-acting insulin glargine has been described in the treatment of transient neonatal diabetes in the premature infant, but in these reports is a lack of regard to its use in the treatment of iatrogenic neonatal hyperglycemia. We present the case of an ELBW infant with significant hyperglycemia that was refractory to usual treatment but demonstrated a favorable response to long-acting subcutaneous insulin glargine. The pharmacokinetics on regular insulin and long-acting insulin are different. Regular insulin is broken down into biologically active monomers after subcutaneous injection, and long-acting insulin forms microprecipitates and is gradually released to the body at a neutral physiologic pH after subcutaneous injection. Pharmacokinetics of both regular insulin and long-acting insulin are not clear in ELBW infants. However, with further research on long-acting insulin, it can be used safely to achieve consistent euglycemia with once-daily administration in neonatal hyperglycemia.
Lung Volume Reduction Surgery for Respiratory Failure in Infants With Bronchopulmonary Dysplasia
Authors: Sohn Bongyeon , Park Samina , Park In Kyu , Kim Young Tae , Park June Dong , Park Sung-Hye , Kang Chang Hyun
Published: April 2018

Lung volume reduction surgery (LVRS) can be performed in patients with severe emphysematous disease. However, LVRS in pediatric patients has not yet been reported. Here, we report our experience with 2 cases of pediatric LVRS. The first patient was a preterm infant girl with severe bronchopulmonary dysplasia, pulmonary hypertension, and hypothyroidism. The emphysematous portion of the right lung was removed via sternotomy and right hemiclamshell incision. The patient was discharged on full-time home ventilator support for 3 months after the surgery. Since then, her respiratory function has improved continuously. She no longer needs oxygen supplementation or ventilator care. Her T-cannula was removed recently. The second patient was also a preterm infant girl with bronchopulmonary dysplasia. She was born with pulmonary hypertension and multiple congenital anomalies, including an atrial septal defect. Despite receiving the best supportive care, she could not be taken off the mechanical ventilator because of severe hypercapnia. We performed LVRS on the right lung via thoracotomy. She was successfully weaned off the mechanical ventilator 1 month after the surgery. She was discharged without severe complications at 3 months after the operation. At present, she is growing well with the help of intermittent home ventilator support. She can now tolerate an oral diet. Our experience shows that LVRS can be considered as a treatment option for pediatric patients with severe emphysematous lung. It is especially helpful for discontinuing prolonged mechanical ventilator care for patients with respiratory failure.
Family Characteristics and Children’s Receipt of Autism Services in Low-Resourced Families
Authors: Karp Elizabeth A. , Dudovitz Rebecca , Nelson Bergen B. , Shih Wendy , Gulsrud Amanda , Orlich Felice , Colombi Costanza , Kuo Alice A.
Published: April 2018

OBJECTIVES:

Parents of children with autism spectrum disorder (ASD) face competing demands when caring for their child and fulfilling family commitments. It remains unknown whether family obligations and parental stress might decrease the use of intervention services for young children with ASD.

METHODS:

The current study is a secondary analysis of baseline date from a published randomized control trial with 147 low-resourced caregiver-child dyads. Demographic information, data on service use, maternal employment, parent’s perception of their child’s development, and parental stress were collected for primary caregivers of 2- to 5-year-old children with ASD from 5 sites. Multiple logistic regressions of accessing any intervention services or more than 1 services on familial characteristics were performed, controlling for demographic and contextual variables.

RESULTS:

Twenty-five percent of children were receiving no intervention service; 26% were receiving 1 service; and 49% were receiving 2 or more services. Perceived developmental delay and not having a sibling in the home were associated with higher odds of receiving intervention services. Children were more likely to receive more than 1 service if their parents had at least a college education and low levels of stress.

CONCLUSIONS:

Factors including perceived developmental level, parental stress, and caring for siblings may play a role in accessing services for children with ASD. Results reveal that competing family needs may be barriers to service use. Mothers of children with ASD with multiple children in the home, low levels of education, and high levels of stress may need additional supports or alternative service delivery models.
Social-Ecological Correlates in Adult Autism Outcome Studies: A Scoping Review
Authors: Anderson Kristy A. , Roux Anne M. , Kuo Alice , Shattuck Paul T.
Published: April 2018

The transition into adulthood is a critical period in the life course that shapes later outcomes. Many adults on the autism spectrum fare poorly across a wide range of quality of life indicators. Understanding the multilevel factors that influence transition outcomes is necessary to develop strategies that promote better outcomes. In this scoping review, we characterize the use of social-ecological factors in adult autism outcome studies, identify understudied areas of research, and provide recommendations for future research. We conducted a literature search for studies in which the relationship between social-ecological factors and transition outcomes among transition-age youth with autism was assessed. We organized variables used in studies across 5 levels of influence: family-, interpersonal-, institutional-, community-, and policy-level factors. Our findings reveal that both breadth and depth of social-ecological factors usage in autism outcomes studies is limited because of the narrow inclusion of variables across social-ecological levels, the overreliance on a limited number of national data sets, and the overall lack of variation in research design. We propose 9 recommendations to inform the development of multilevel studies.
A Health Care Transition Curriculum for Primary Care Residents: Identifying Goals and Objectives
Authors: Kuo Alice A. , Ciccarelli Mary R. , Sharma Niraj , Lotstein Debra S.
Published: April 2018

BACKGROUND AND OBJECTIVES:

The transition from pediatric to adult health care is a vulnerable period for youth with special health care needs. Although successful transitions are recognized as critical for improving adult outcomes and reducing health care utilization and cost, an educational gap in health care transitions for physicians persists. Our aim with this project was to develop a national health care transition residency curriculum for primary care physicians, using an expert-based, consensus-building process.

METHODS:

Medical professionals with expertise in health care transition were recruited to participate in a survey to assist in the development of a health care transition curriculum for primary care physicians. By using a modified Delphi process, curricular goals and objectives were drafted, and participants rated the importance of each objective, feasibility of developing activities for objectives, and appropriateness of objectives for specified learners. Mean and SDs for each response and percent rating for the appropriateness of each objective were calculated.

RESULTS:

Fifty-six of 246 possible respondents participated in round 1 of ratings and 36 (64%) participated in the second round. Five goals with 32 associated objectives were identified. Twenty-five of the 32 objectives (78%) were rated as being appropriate for “proficient” learners, with 7 objectives rated as “expert.” Three objectives were added to map onto the Got Transition guidelines.

CONCLUSIONS:

The identified goals and objectives provide the foundation and structure for future curriculum development, facilitating the sharing of curricular activities and evaluation tools across programs by faculty with a range of expertise.
Social Support, Well-being, and Quality of Life Among Individuals on the Autism Spectrum
Authors: Kapp Steven K.
Published: April 2018

Social support plays a critical role in the quality of life (adaptive functioning and subjective well-being) of individuals on the autism spectrum across the lifespan.
The Use of Pyridostigmine in a Child With Chronic Intestinal Pseudo-Obstruction
Authors: Choudhury Ahamodur , Rahyead Abdul , Kammermeier Jochen , Mutalib Mohamed
Published: April 2018

Chronic intestinal pseudo-obstruction is a rare disorder that affects the motility of the gastrointestinal tract. It results in acute or subacute intestinal obstruction symptoms in the absence of mechanical lesion. It can lead to intestinal failure in children with significant strain on nutrition, growth, and development. There is no universally agreed protocol for management of chronic intestinal pseudo-obstruction in children, and there is wide variation in clinical practice.
NALCN Dysfunction as a Cause of Disordered Respiratory Rhythm With Central Apnea
Authors: Campbell Jamie , FitzPatrick David R. , Azam Tara , Gibson Neil A. , Somerville Laura , Joss Shelagh K. ,   Deciphering Developmental Disorders Study, Urquhart Don S.
Published: April 2018

The sodium leak channel nonselective protein (NALCN) is a regulator of the pacemaker neurons that are responsible for rhythmic behavior (including respiration), maintaining the resting membrane potential, and are required for action potential production. NALCN-null mice show early death associated with disrupted respiratory rhythms, characterized by frequent and profound apneas. We report 3 children (2 siblings) with compound heterozygous mutations in NALCN associated with developmental impairment, hypotonia, and central sleep-disordered breathing causing apneas. Supplemental oxygen normalized the respiratory rhythm. NALCN mutations have been previously reported to cause severe hypotonia, speech impairment, and cognitive delay as well as infantile neuroaxonal dystrophy and facial dysmorphism. Nonsynonymous changes in the 2 affected extracellular loops may be responsible for the deleterious effect on the stability of the respiratory rhythm. Although oxygen is known to be a stabilizer of respiratory rhythm in central apnea in children, its role in NALCN dysfunction requires further investigation.
Recanalization of Occluded Central Veins in a Parenteral Nutrition–Dependent Child With No Access
Authors: Sullivan Patrick M. , Merritt Russell , Pelayo Juan Carlos , Ing Frank F.
Published: April 2018

Central venous thromboses are common and pose challenges in the care of chronically ill pediatric patients. Among patients with intestinal failure (most commonly because of short bowel syndrome) who depend on parenteral nutrition, progressive loss of central venous access sites is a potentially fatal complication. We present the case of a 5-year-old girl with parenteral nutrition-dependent short bowel syndrome and no remaining standard central venous access sites despite medical anticoagulation, in whom angioplasty and stent implantations were used to reconstruct chronically occluded central veins. The patient presented with a bloodstream infection necessitating tunneled central venous line removal from the left internal jugular vein. All other standard access sites had known occlusions. The right iliofemoral vein (RIFV) and infrarenal inferior vena cava were recanalized and dilated with high-pressure balloons. The left internal jugular line was removed and a line was placed in the now-patent RIFV for antimicrobial therapy. After treatment, the RIFV line was removed and the vessels were stented open for future access. The occluded left innominate vein was recanalized and dilated to allow a new tunneled line to be placed. At 10 months, the line was functional and uninfected and the RIFV and inferior vena cava stents were patent without in-stent restenosis. We propose a new paradigm that uses these techniques to prevent access site exhaustion in patients who do not respond to anticoagulation therapy. This approach may reduce morbidity and mortality in patients with chronic access needs and the need for intestinal transplantation in patients with intestinal failure.
Histoplasmosis Myocarditis in an Immunocompetent Host After a Recreational Mud Run
Authors: Scott Briana L. , Sherwin Jennifer I. , Rehder Kyle J. , Campbell Michael J. , Ozment Caroline P.
Published: April 2018

Mud runs are an increasingly popular recreational fitness activity across the United States, combining a running race through an obstacle course with submersion in mud. Recent reports estimate 4 million people have participated in these types of events over the last 5 years. We describe an atypical case of myocarditis and multiorgan failure from disseminated histoplasmosis in a previously healthy pediatric patient, likely acquired during participation in a mud run. Although cases of histoplasmosis-associated endocarditis and pericarditis have been reported in the literature, cases of histoplasmosis myocarditis are rare.
Unusual Presentations of Dystrophinopathies in Childhood
Authors: Allen Nicholas M. , Ewer Alice , Nakou Vasiliki , Konstantoulaki Ele , Wraige Elizabeth , Gowda Vasantha , Jungbluth Heinz
Published: April 2018

X-linked recessive mutations in the dystrophin gene are one of the most common causes of inherited neuromuscular disorders in humans. Duchenne muscular dystrophy, the most common phenotype, and Becker muscular dystrophy are often recognizable by certain clinical features; however, less frequent presentations require a higher degree of suspicion. In this article, we describe a series of 6 children (4 boys, 2 girls) referred to a tertiary pediatric neuromuscular clinic for isolated elevated creatine kinase levels (range: 720–7000 IU/L) identified on initial assessment for otherwise unexplained transaminase elevations (n = 2), a social communication disorder (n = 3), and exertional myalgia and/or rhabdomyolysis (n = 1). There was no preceding family history of neuromuscular disease. One boy had an additional history of severe cerebral palsy and cyclical vomiting, and 1 girl had a history of maternal hepatitis C. There was no significant weakness at presentation, and the majority remained stable over a prolonged period of follow-up (age range at last follow-up: 9–16 years). All 6 children were found to carry dystrophin gene mutations resulting in milder phenotypes. This series highlights that dystrophinopathies may not uncommonly present with features distinct from the classic Duchenne muscular dystrophy and Becker muscular dystrophy phenotypes in both boys and girls. Pediatricians should be aware of such atypical presentations to initiate a timely and adequate diagnostic process. Establishing the correct genetic diagnosis of a dystrophinopathy is important to allow appropriate genetic counseling, to implement relevant surveillance and management strategies, and to avoid unnecessary investigations in search of an incorrect alternative diagnosis.
Safety and Immunogenicity of a Recombinant Influenza Vaccine: A Randomized Trial
Authors: Dunkle Lisa M. , Izikson Ruvim , Patriarca Peter A. , Goldenthal Karen L. , Cox Manon , Treanor John J.
Published: April 2018

OBJECTIVES:

The recombinant influenza vaccine is well established in adults ≥18 years of age for preventing seasonal influenza disease. In this randomized controlled trial, we compared the safety and immunogenicity of the quadrivalent, recombinant influenza vaccine (RIV4) versus the inactivated influenza vaccine in children and adolescents 6 to 17 years of age.

METHODS:

Two age cohorts were enrolled sequentially: 159 subjects aged 9 to 17 years and, after reviewing for safety, 60 children aged 6 to 8 years. Enrollment of the younger children was halted prematurely at the onset of the influenza season. Subjects in each cohort were randomly assigned 1:1 to the RIV4 or inactivated vaccine. Hemagglutination inhibition antibody titers were obtained before and 28 days after vaccination. Tolerability and safety were monitored for 7 days and 6 months after vaccination, respectively.

RESULTS:

Both vaccines were well tolerated in both age groups, and long-term follow-up revealed no vaccine-related adverse events. Overall, immunogenicity (geometric mean titers and seroconversion rate differences) provided comparable antibody responses to most antigens in both vaccines in the older subjects. Low responses to the influenza B Victoria lineage in both vaccines made interpretation difficult. Immunogenicity in younger children was similar, but the truncated sample size was insufficient to support noninferiority comparisons.

CONCLUSIONS:

Despite low responses to influenza B lineages in both vaccines, the RIV4 provided safety and immunogenicity that were comparable to those of the licensed inactivated vaccine in pediatric subjects, which was most convincing in those aged 9 to 17 years. Future confirmatory clinical efficacy trials may be used to support the recombinant influenza vaccine as an alternative for the pediatric age group of ≥6 years.
Divinización del mercado, teodicea liberal. Una respuesta no eurocéntrica
Authors: Polo Blanco Jorge
Published: April 2018

Se examinan los supuestos del liberalismo económico (en sus versiones más puras y extremas), a través de los cuales este divinizó el mercado y lo convirtió en una instanciatotalizadora exenta de toda crítica. El neoliberalismo ha hecho del mercado un locus donde el ser y el deber ser se reconcilian en perfecta unidad. De esta manera, el mercado deviene en el único y verdadero sujeto del razonar: solo él piensa y todos sus movimientos son contenidos de lo racional. Al comparar la obra de Friedrich von Hayek con la de Hegel y Leibniz, se ve que estos autores comparten una misma estructura de pensamiento que, en última instancia, nos remite al ámbito de la teodicea.
Estatus metodológico de las Lecciones de fenomenología de la conciencia interna del tiempo
Authors: Kretschel Verónica
Published: April 2018

Aunque las Lecciones de fenomenología de la conciencia interna del tiempo tienen pocas referencias al marco metódico, ciertos pasajes apuntan a la reducción fenomenológica, de modo que su status se diferenciaría del de las Investigaciones lógicas y se acercaría al de Ideas i. Se ha señalado que aun si la reducción es empleada en este texto, no lo sería en su “versión madura”. Se busca determinar el estatus metodológico de las Lecciones a partir de una comparación entre los desarrollos metódicos y las descripciones fenomenológicas que se presentan allí y aquellos consignados en Investigaciones e Ideas, teniendo como idea regulativa el esclarecimiento del papel del tiempo en la ampliación del dominio de la fenomenología como conjunto.
La exhortación de las cualidades sensibles Una descripción existencial del mundo inmediato a partir del pensamiento de Heidegger
Authors: Johnson Felipe
Published: April 2018

Se discute la posibilidad de comprender la presencia sensible del mundo y de una caracterización existencial de las cualidades sensibles según las cuales este comparecedesde el punto de vista de los análisis heideggerianos del existir humano o Dasein. Pese a las ocasionales referencias a dicho problema en el pensamiento de Heidegger, se enfatiza que la relación fáctica del existir con el ente inmediato radica en un hallarse concernido del propio Dasein. Se analiza el papel de las cualidades sensibles como una exhortación a establecer tratos fácticos con lo ente que permiten al existirconcreto vivir en una confrontación con las cosas de su entorno, entendiendo que el ente intramundano solo es tal en el interior de una totalidad remitiva. Se advierte que el problema de lo sensible en términos existenciales puede ser descrito desde la manera de ser del Dasein, como quien es exhortado por las cosas entre las cuales vive.
STUDY OF SEDIMENTATION IN THE WATER OF PATIMBAN PORT DEVELOPMENT PLAN USING COMPUTATION MODELING
Authors: Wibowo Mardi
Published: April 2018

The developmentof the international port of Patimban in Subang is very urgent. The port is expected to support the Tanjung Priok port in Jakarta which is unable to meet the loading and unloading needs of business operators, especially in West Java. In port development planning one of the main problems of physical chemical aspects is sedimentation and erosion process. The purpose of this modeling is to know the sedimentation and erosion pattern around the location plan of port development. The result of modeling is expected to be the initial consideration in designing the Patimban Port. Modeling of sediment transport is done by using MIKE21 Sand Transport module software by entering geophysical data of field survey result such as bathymetry, sea level, river flow and characteristic of existing sediment. Modeling is done both in west season and east season for 1 year (October 2016-September 2017).Based on the results of this modeling is known that the existing conditions in parairan construction plan ponds occur sedimentation sedimentation of 12.74 cm / m th while when the port has been built a little erosion -0.34 cm / year. Whereas in the existing shipping lane plan there is sedimentation 14,41 cm / year and when the port has been built there is sedimentation of 6,06 cm / year.
DAYA DUKUNG SPASIAL KAWASAN PERTAMBAKAN MELALUI INTERPRETASI CITRA SATELIT ALOS AVNIR-2 STUDI KASUS KABUPATEN MAROS SULAWESI SELATAN
Authors: Amri Syahrial Nur , Arifin Taslim
Published: April 2018

Calculation of carrying capacity of aquaculture area is important to optimize the coastal area utilization. The calculations of the spatial carrying capacity require the availability of accurate spatial data, and one of the best spatial data providers today is through the use of satellite imagery. This study aims to determine the ability of the pond land in support of the coastal community activities and regional development. This research covers two stages: 1) spatially identifies the pond area through the interpretation of ALOS AVNIR-2 satellite imagery with the masking technique; 2) Calculating the value of Carrying Capacity Ratio (CCR)  to know the ability of land to coastal community activities. The results show that the pond areas in Maros District identified as 9.621,60 hectares, and the CCR analysis shows a value of 2,79 or > 1. The value indicates the ability of pond land which is still relatively able to meet the needs of community activities. Bontoa sub-district is the region with the lowest CCR value (1,73), which means that the available of pond land is almost unable to fully support the community's activities. Management strategies are needed in the form of increased productivity through the intensification patterns, and / or suppressing the population growth.