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Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves
- Benedikt Mayr, Takuya Osawa, Helena Staehler, Thibault Schaeffer, Christoph Röhlig, Julie Cleuziou, Alfred Hager, Peter Ewert, Jürgen Hörer, Rüdiger Lange, Masamichi Ono
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- Cardiology in the Young , First View
- Published online by Cambridge University Press:
- 03 April 2024, pp. 1-9
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Objectives:
Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge.
Methods:We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation.
Results:Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9–18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively.
Conclusions:In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.
Electronic State of Iron Complexes in the Interlayer of Hydrotalcite-Like Materials
- Satoshi Idemura, Eiichi Suzuki, Yoshio Ono
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- Clays and Clay Minerals / Volume 37 / Issue 6 / December 1989
- Published online by Cambridge University Press:
- 02 April 2024, pp. 553-557
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Infrared and Mössbauer spectroscopic studies were made on hydrotalcite-like materials, Mg6Al2(OH)16(NO3)2·4H2O, anion exchanged in aqueous solution of K3Fe(CN)6, K4Fe(CN)6·3H2O, or Na2Fe(CN)5NO·2H2O. The material anion-exchanged in aqueous solution of K3Fe(CN)6 gave infrared (IR) absorption bands at 2120 and 2040 cm-1 in the C≡N stretching region, suggesting that part of the ferrate(III) complex was reduced to ferrate(II) complex on the intercalation chiefly because pure K3Fe(III)(CN)6 and K4Fe(II)(CN)6·3H2O gives bands at 2120 and 2040 cm-1, respectively. On the intercalation of Fe(CN)64-, no change in the oxidation state of iron was observed. These features were confirmed by Mössbauer spectroscopy. In the IR spectra of material anion-exchanged in aqueous solution of Na2Fe(CN)5NO·2H2O, the intensity of bands due to N≡O (1940 cm-1) and C≡N stretching was much less than that observed for Na2Fe(CN)5NO·2H2O, indicating that most of N≡O ligand was eliminated during the intercalation. Four bands were observed in the C≡N stretching region: a band at 2143 cm-1 was assigned to C≡N groups in Fe(CN)5NO2-; a band at 2120 cm-1 was tentatively assigned to Fe(CN)5H2O2-; bands at 2040–2050 cm-1 were assigned to Fe(CN)5H2O3-.
Properties of Hexacyanocobaltate(III)-Exchanged Hydrotalcite-Like Minerals
- Eiichi Suzuki, Satoshi Idemura, Yoshio Ono
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- Clays and Clay Minerals / Volume 37 / Issue 2 / April 1989
- Published online by Cambridge University Press:
- 02 April 2024, pp. 173-178
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Hydrotalcite-like minerals, containing Mg2+-Al3+-Xn− (Xn− = NO3−, Cl−, SO42−, or CrO42−), Zn2+-Al3+-NO3−, or Zn2+-Cr3+-NO3− ions, were intercalated with Co(CN)63− by an anion exchange method. For most of the minerals examined, the degree of anion exchange was 79–90%. X-ray powder diffraction patterns of the ion-exchanged samples revealed two peaks at about 8° and 11°2θ (CuKα radiation) attributable to reflections from expanded and non-expanded interlayers, respectively. The intensity of the peak at about 8°2θ was a linear function of the degree of anion exchange. The amount of hexane adsorbed onto the anion-exchanged sample increased linearly with the increase in degree of anion exchange. From these results, anions near the edge of the interlayers appear to have been preferentially replaced by Co(CN)63− at a low degree of anion exchange, whereas those at some distance from the edge were replaced only at a high degree of anion exchange. The adsorption capacity of the Co(CN)63−-exchanged sample for hydrocarbons was: hexane ≃ 2-methylpentane ≫ cyclohexane > methylcyclohexane. The adsorption of chain hydrocarbons and cyclic hydrocarbons was different.
Impact of early postoperative haemodynamic and laboratory parameters on outcome after the Fontan procedure
- Chiara Di Padua, Takuya Osawa, Birgit Waschulzik, Gunter Balling, Thibault Schaeffer, Helena Staehler, Nicole Piber, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
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- Cardiology in the Young , First View
- Published online by Cambridge University Press:
- 29 January 2024, pp. 1-8
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Objective:
To identify early postoperative haemodynamic and laboratory parameters predicting outcomes following total cavopulmonary connection.
Methods:Patients who underwent total cavopulmonary connection between 2012 and 2021 were evaluated. Serial values of mean pulmonary artery pressure, mean arterial pressure, peripheral oxygen saturation, and lactate levels were collected. The influence of these variables on morbidities was analyzed. Cut-off values were calculated using the receiver operating characteristic analysis.
Results:A total of 249 patients were included. All patients had previous bidirectional cavopulmonary shunt. Median age and weight at total cavopulmonary connection were 2.2 (1.8–2.7) years and 11.7 (10.7–13.4) kg, respectively. All patients were extubated in the ICU at a median of 3 (2–5) hours after ICU admission. Postoperative pulmonary artery pressure, around 12 hours after extubation, was significantly associated with chest tube drainage (p = 0.048), chylothorax (p = 0.021), ascites (p = 0.016), and adverse events (p = 0.028). Receiver operating characteristic analysis revealed a cut-off value of 13–15 mmHg for chest tube drainage and chylothorax and 17 mmHg for ascites and adverse events. Mean arterial pressure 1 hour after extubation was associated with prolonged chest tube drainage (p = 0.015) and adverse events (p = 0.008). Peripheral oxygen saturation 6 hours after extubation (p = 0.003) was associated with chest tube duration and peripheral oxygen saturation 1 hour after extubation (p < 0.001) was associated with ascites. Lactate levels on 2nd postoperative day (p = 0.022) were associated with ascites and lactate levels on 1st postoperative day (p = 0.009) were associated with adverse events.
Conclusions:Higher pulmonary artery pressure, lower mean arterial pressure, lower peripheral oxygen saturation, and higher lactate in early postoperative period, around 12 hours after extubation, predicted in-hospital and post-discharge adverse events following total cavopulmonary connection.
Characterization and Assessment of Natural Amazonian Clays for Cosmetics-Industry Applications
- Antonio Claudio Kieling, Cláudia Cândida Silva, Sérgio Duvoisin Júnior, José Costa de Macedo Neto, Miécio de Oliveira Melquíades, Gilberto Garcia del Pino, Yago Ono de Souza Moreira, Túlio Hallak Panzera, Maria das Graças da Silva Valenzuela, Francisco Rolando Valenzuela Diáz
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- Clays and Clay Minerals / Volume 70 / Issue 5 / October 2022
- Published online by Cambridge University Press:
- 01 January 2024, pp. 780-795
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Clays are abundant materials in the Amazon region and have been used historically by ancient Amazonian people to produce ceramic and cosmetics products. The current study aimed to evaluate the potential of four clays from the metropolitan area of Manaus, each with a different color, for cosmetics applications. Two clays were collected in the Ponta Negra region (red and gray in color) in Manaus, one in Careiro (white), and one in Itacoatiara (black). After drying in an oven for 24 h at 105°C, the four clays were characterized by X-ray fluorescence (XRF), X-ray diffraction (XRD), infrared (IR) spectroscopy, thermogravimetry (TGA), differential thermal analysis (DTA), differential scanning calorimetry (DSC), scanning electron microscopy (SEM), particle-size analysis, and detection of microorganisms. The amounts of Al, Si, Cl, K, Ca, Ti, Cr, Fe, Zn, P, and S in all samples were below the limits for use in cosmetics. The main phases identified were kaolinite 1A, quartz, gibbsite, and the rare kaolinite 2M. Approximately 40 wt.% of each sample was in the < 20 μm particle-size range. Analyses by SEM revealed pseudo-hexagonal kaolinite structures with nano-islands and nanocrystallites. The low toxicity, mineralogic compositions, and particle-size findings suggest that Amazonian clays are promising for cosmetics applications.
27 Assessing Differences in Academic Achievement Among a National Sample of Children with Epilepsy Before and During the COVID-19 Pandemic
- Brandon Almy, Lauren Scimeca, David Marshall, Brittany L. Nordhaus, Erin Fedak Romanowski, Nancy McNamara, Elise Hodges, Madison M. Berl, Alyssa Ailion, Donald J. Bearden, Katrina Boyer, Crystal M. Cooper, Amanda M. Decrow, Priscilla H. Duong, Patricia Espe-Pfeifer, Marsha Gabriel, Jennifer I. Koop, Kelly A. McNally, Andrew Molnar, Emily Olsen, Kim E. Ono, Kristina E. Patrick, Brianna Paul, Jonathan Romain, Leigh N. Sepeta, Rebecca L.H. Stilp, Greta N. Wilkening, Mike Zaccariello, Frank Zelko
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 28-29
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The COVID-19 pandemic significantly disrupted schools and learning formats. Children with epilepsy are at-risk for generalized academic difficulties. We investigated the potential impact of COVID-19 on learning in those with epilepsy by comparing achievement on well-established academic measures among school-age children with epilepsy referred prior to the COVID-19 pandemic and those referred during the COVID-19 pandemic.
Participants and Methods:This study included 466 children [52% male, predominately White (76%), MAge=10.75 years] enrolled in the Pediatric Epilepsy Research Consortium Epilepsy (PERC) Surgery database project who were referred for surgery and seen for neuropsychological testing. Patients were divided into two groups based on a proxy measure of pandemic timing completed by PERC research staff at each site (i.e., “were there any changes to typical in-person administration [of the evaluation] due to COVID?”). 31% of the sample (N = 144) were identified as having testing during the pandemic (i.e., “yes” response), while 69% were identified as having testing done pre-pandemic (i.e., “no” response). Of the 31% who answered yes, 99% of administration changes pertained to in-person testing or other changes, with 1% indicating remote testing. Academic achievement was assessed by performance measures (i.e., word reading, reading comprehension, spelling, math calculations, and math word problems) across several different tests. T-tests compared the two groups on each academic domain. Subsequent analyses examined potential differences in academic achievement among age cohorts that approximately matched grade level [i.e., grade school (ages 5-10), middle school (ages 11-14), and high school (ages 15-18)].
Results:No significant differences were found between children who underwent an evaluation before the pandemic compared to those assessed during the pandemic based on age norms across academic achievement subtests (all p’s > .34). Similarly, there were no significant differences among age cohorts. The average performance for each age cohort generally fell in the low average range across academic skills. Performance inconsistently varied between age cohorts. The youngest cohort (ages 5-10) scored lower than the other cohorts for sight-word reading, whereas this cohort scored higher than the middle cohort (ages 11-14) for math word problems and reading comprehension. There were no significant differences between the two pandemic groups on demographic variables, intellectual functioning, or epilepsy variables (i.e., age of onset, number of seizure medications, seizure frequency).
Conclusions:Academic functioning was generally equivalent between children with epilepsy who underwent academic testing as part of a pre-surgical evaluation prior to the pandemic compared to those who received testing during the pandemic. Additionally, academic functioning did not significantly differ between age cohorts. Children with epilepsy may have entered the pandemic with effective academic supports and/or were accustomed to school disruptions given their seizure history. Replication is needed as findings are based on a proxy measure of pandemic timing and the extent to which children experienced in-person, remote, and hybrid learning is unknown. Children tested a year into the pandemic, after receiving instruction through varying educational methods, may score differently than those tested earlier. Future research can address these gaps. Although it is encouraging that academic functioning was not disproportionately impacted during the pandemic in this sample, children with epilepsy are at-risk for generalized academic difficulties and continued monitoring of academic functioning is necessary.
66 Neuropsychological Profile of ROHHAD Syndrome: A Case Study
- Ivana Cernokova, Ronnise D Owens, Eva del Valle Martinez, Nicole Semaan, Coralie Bergeron, Donald J Bearden, Kim E Ono
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, p. 742
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Rapid Onset Obesity with Hypoventilation, Hypothalamic Dysfunction, Autonomic Dysregulation (ROHHAD) is a rare and often progressive syndrome with unknown etiology and only 100 cases reported to date. The syndrome is characterized by generally normal development followed by rapid onset of pain, muscle weakness, personality changes, and developmental regression. Associated chronic pain and fatigue result in difficulty concentrating, slow information processing, and executive function challenges. Only one study has examined the neuropsychological profile of pediatric patients with this syndrome.
Participants and Methods:Our patient was a 10-year-old, right-handed male with a history of ROHHAD syndrome, focal epilepsy, mild neurocognitive disorder, autism spectrum disorder (ASD), and attention-deficit/hyperactivity disorder (ADHD) who underwent two comprehensive neuropsychological evaluations at our medical center.
Results:Findings across multiple evaluations showed solid verbal skills and difficulty processing visual-spatial and nonverbal information, as well as problems with attention, executive functioning, and adaptive skills, and psychosocial functioning consistent with his diagnoses of ADHD and ASD. He exhibited fine-and gross-motor challenges associated with hypotonia. Chronic fatigue contributed to his challenges with attention and information processing. These findings are generally consistent with previous research examining the neuropsychological profile associated with ROHHAD syndrome.
Conclusions:Results from our case study highlight the complexity and challenges associated with ROHHAD syndrome. Consistent with available information, etiology of our patients’ neuropsychological weakness and functional decline is unclear. Yearly neuropsychological evaluations are recommended for these patients to update interventions based on their variable abilities. More research is needed to firmly establish the neuropsychological profile in youth of varying ages afflicted with this syndrome.
39 Neurodevelopmental Complexity of a Patient with Perinatal Right Middle Cerebral Artery Stroke and Infantile Spasms
- Ronnise D Owens, Ivana Cernokova, Eva del Valle Martinez, Nicole Semaan, Coralie Bergeron, Donald J Bearden, Kim E Ono
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 38-39
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A rich literature exists on cognitive changes related to focal injury in the adult brain. In contrast, the developing brains of children are less understood. In contrast to adult cases, 20% to 25% of perinatal strokes result in language disorder regardless of lesion lateralization. Existing literature suggests children with perinatal stroke may present with a range of executive functioning and visuospatial processing difficulties. Gross and fine motor challenges are also likely to occur. Furthermore, these children have an increased prevalence of autism spectrum disorder (ASD) and carry the highest risk for epilepsy. Despite growing research on neurodevelopmental profiles in patients with perinatal stroke, published literature is limited.
Participants and Methods:Our study examines neurodevelopment of a 2-year-old, right-handed male with a history of perinatal ischemic right middle cerebral artery (MCA) stroke, infantile spasms, and left hemiparesis following right hemispherectomy for seizure management who underwent two neurodevelopmental evaluations at our medical center over approximately 3 years.
Results:Findings from the patient’s evaluation with the Mullen Scales of Early Learning revealed overall cognitive ability in the low average range (SS = 89, 23rd percentile); however, notable variability was seen in his performance. His receptive language was average (SS = 98, 45th %tile) and consistent with previous evaluation results, and he has made gains in visual reception (from SS = 75, 5th %tile to SS = 91, 27th %tile) and expressive language (from SS = 55, 0.1st %tile to SS = 70, 2nd %tile). In addition, his gross motor was exceptionally low (SS = 55, 0.1st %tile) and consistent with previous evaluation results. Fine motor was low average (SS = 84, 14th %tile).
Conclusions:Our patient showed cognitive gains in language and visual reception since his prior evaluation despite history of right MCA stroke and right hemispherectomy. Improvements are likely due to a combination of early brain plasticity and intensive therapies he has received. Consistent with published findings in this population, he experienced seizures associated with his stroke. Our results add to the limited literature on neurodevelopmental challenges associated with perinatal stroke and progress that can be made when appropriate supports are provided early and consistently.
63 A Literature Review of Cortical Mapping, fMRI, and Standards of Care in Pediatric Epilepsy Surgical Workup
- Ivana Cernokova, Ronnise D Owens, Eva del Valle Martinez, Nicole Semaan, Coralie Bergeron, Donald J. Bearden, Kim E Ono
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, p. 471
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Epilepsy includes recurrent, unprovoked seizures and affects 470,000 children in the US, of which 7% have drug-resistant epilepsy due to failing two or more antiseizure medication trials. For some patients with drug-resistant epilepsy, surgery has been successful in reducing seizure burden. Functional MRI (fMRI) and intracranial mapping of neurocognitive functions, especially language, are increasingly done to assess potential functional loss from epilepsy surgery. However, these procedures vary by medical institute. The purpose of this review was to examine published literature on fMRI and intracranial mapping procedures for pediatric epilepsy surgery workup toward development of a standardized protocol that can be used across institutes as a guide to standard-of-care best practices for predicting loss of function associated with epilepsy surgery.
Participants and Methods:Our literature review includes information from 8 electronic databases for peer-reviewed, English language studies of evaluation for pediatric epilepsy surgery candidacy. Thirty-one studies were selected based on inclusion criteria. Only studies including fMRI and intracranial mapping conducted with pediatric patients being worked up for epilepsy surgery were selected.
Results:Our review revealed that the most common task used in fMRI and intracranial mapping procedures is visual-object naming, but type of naming tasks and the way they are administered varies widely across medical institutes and includes published measures and those created on site. Variability makes examining findings across studies and designing best practice for these procedures challenging.
Conclusions:Creating gold-standard procedures for fMRI and intracranial mapping administration for epilepsy surgery evaluations is critical in optimizing treatment and functional outcomes for our pediatric patients. Our review is an initial step in this process.
11 Sociodemographic, Cultural, and Linguistic Considerations for Clinical Neuropsychological Assessment with Japanese and Japanese American Patients in the United States
- Daryl Fujii, Erin T. Kaseda, Aya Haneda, Hirofumi Kuroda, Sayaka Machizawa, Yoko Okamura, Kim Ono, Torricia Yamada, Nicholas S. Thaler
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 426-427
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Japanese-Americans are the sixth largest Asian ethnicity in the United States. They represent a highly heterogeneous population due to their history of immigration dating back to the late 19th century. In comparison to the total Japanese-American population, there are very few neuropsychologists of Japanese descent who are familiar with the culture. The Asian Neuropsychological Association lists 16 licensed members of Japanese descent, and only 7 practice outside of Hawaii or California. These numbers suggest that unless non-Japanese neuropsychologists are knowledgeable of the culture, test translations, and appropriate norms, it would be challenging for many Japanese-Americans to receive culturally and linguistically competent neuropsychological services. The aim of the present study is to provide guidance for conducting neuropsychological assessments with Japanese-Americans with the goal of facilitating competent culturally-informed services to this population.
Participants and Methods:Pertinent facets of Japanese culture as identified in the ECLECTIC framework and demographics of the U.S. Japanese-American population, and the available literature on neuropsychological tests that are translated into Japanese and normed with Japanese samples, was reviewed by authors with knowledge of Japanese language and culture. Literature published in both English and Japanese were included for review.
Results:Psychological testing is a Western technology fraught with the behavioral expectations and values of the culture in which it was developed. Thus, these tests may be biased against persons coming from cultures that differ from the West. Recommendations for providing neuropsychological services to Japanese-Americans are presented with an aim of maximizing test fairness by addressing the following issues: comfort with the testing situation, test biases, accessibility, and validity. Given the emphasis on education, Japanese-Americans should be familiar and comfortable with cognitive testing, although they may experience undue pressure to perform well to avoid shame. Japanese-Americans may experience discomfort disclosing personal information during the interview, particularly if the evaluation is perceived to be psychiatric in nature, as there is a strong stigma associated with mental illness that could bring shame to the family. Japanese communication styles are indirect in nature, where the message is implied and what is “not said” is just as important as what is directly conveyed. Accessibility issues will primarily impact first generation Japanese-Americans who are native Japanese speakers. Another characteristic that may impact responding is hesitancy for guessing when unsure. Recommendations for providing culturally competent neuropsychological assessment given these considerations will be presented and expanded upon in detail. Finally, an online database of translated and normed neuropsychological tests by cognitive domain has been created and will be presented.
Conclusions:Providing neuropsychological services to Japanese-Americans can be challenging as Japanese culture is significantly different from western culture. In addition, Japanese-Americans are heterogenous with salient issues of English proficiency and acculturation. Information to individualize a conceptual understanding of Japanese-Americans, translated and normed tests, and recommendations to maximize fairness in testing are presented to assist clinical neuropsychologists provide competent services to Japanese-Americans.
3 Latent Wechsler Profiles in Presurgical Pediatric Epilepsy
- Madison M Berl, Erin T Kaseda, Jennifer I Koop, Brandon Almy, Alyssa Ailion, Donald J Bearden, Katrina Boyer, Crystal M Cooper, Amanda M DeCrow, Priscilla H Duong, Patricia Espe-Pfeifer, Marsha Gabriel, Elise Hodges, David Marshall, Kelly A McNally, Andrew Molnar, Emily Olsen, Kim E Ono, Kristina E Patrick, Brianna Paul, Jonathan Romain, Leigh N Sepeta, Rebecca LH Stilp, Greta Wilkening, Michael Zaccariello, Frank Zelko, PERC Epilepsy Surgery Database Project
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 308-310
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The Pediatric Epilepsy Research Consortium (PERC) Epilepsy Surgery Database Project is a multisite collaborative that includes neuropsychological evaluations of children presenting for epilepsy surgery. There is some evidence for specific neuropsychological phenotypes within epilepsy (Hermann et al, 2016); however, this is less clear in pediatric patients. As a first step, we applied an empirically-based subtyping approach to determine if there were specific profiles using indices from the Wechsler scales [Verbal IQ (VIQ), Nonverbal IQ (NVIQ), Processing Speed Index (PSI), Working Memory Index (WMI)]. We hypothesized that there would be at least four profiles that are distinguished by slow processing speed and poor working memory as well as profiles with significant differences between verbal and nonverbal reasoning abilities.
Participants and Methods:Our study included 372 children (M=12.1 years SD=4.1; 77.4% White; 48% male) who completed an age-appropriate Wechsler measure, enough to render at least two index scores. Epilepsy characteristics included 84.4% with focal epilepsy (evenly distributed between left and right focus) and 13.5% with generalized or mixed seizure types; mean age of onset = 6.7 years, SD = 4.5; seizure frequency ranged from daily to less than monthly; 53% had structural etiology; 71% had an abnormal MRI; and mean number of antiseizure medications was two. Latent profile analysis was used to identify discrete underlying cognitive profiles based on intellectual functioning. Demographic and epilepsy characteristics were compared among profiles.
Results:Based on class enumeration procedures, a 3-cluster solution provided the best fit for the data, with profiles characterized by generally Average, Low Average, or Below Average functioning. 32.8% were in the Average profile with mean index scores ranging from 91.7-103.2; 47.6% were in the Low Average profile with mean index ranging from 80.7 to 84.5; and 19.6% were in the Below Average profile with mean index scores ranging from 55.0-63.1. Across all profiles, the lowest mean score was the PSI, followed by WMI. VIQ and NVIQ represented relatively higher scores for all three profiles. Mean discrepancy between indices within a profile was as large as 11.5 IQ points. No demographics or epilepsy characteristics were significantly different across cognitive phenotypes.
Conclusions:Latent cognitive phenotypes in a pediatric presurgical cohort were differentiated by general level of functioning; however, across profiles, processing speed was consistently the lowest index followed by working memory. These findings across phenotypes suggest a common relative weakness which may result from a global effect of antiseizure medications and/or the widespread impact of seizures on neural networks even in a largely focal epilepsy cohort; similar to adult studies with temporal lobe epilepsy (Hermann et al, 2007). Future work will use latent profile analysis to examine phenotypes across other domains relevant to pediatric epilepsy including attention, naming, motor, and memory functioning. These findings are in line with collaborative efforts towards cognitive phenotyping which is the aim of our PERC Epilepsy Surgery Database Project that has already established one of the largest pediatric epilepsy surgery cohorts.
26 The Importance of Executive Functioning for Academic Achievement Among a National Sample of Children with Epilepsy
- Brandon Almy, David Marshall, Brittany L. Nordhaus, Erin Fedak Romanowski, Nancy McNamara, Elise Hodges, Madison M. Berl, Alyssa Ailion, Donald J. Bearden, Katrina Boyer, Crystal M. Cooper, Amanda M. Decrow, Priscilla H. Duong, Patricia Espe-Pfeifer, Marsha Gabriel, Jennifer I. Koop, Kelly A. McNally, Andrew Molnar, Emily Olsen, Kim E. Ono, Kristina E. Patrick, Brianna Paul, Jonathan Romain, Leigh N. Sepeta, Rebecca L.H. Stilp, Greta N. Wilkening, Mike Zaccariello, Frank Zelko
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 26-27
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Children with epilepsy are at greater risk of lower academic achievement than their typically developing peers (Reilly and Neville, 2015). Demographic, social, and neuropsychological factors, such as executive functioning (EF), mediate this relation. While research emphasizes the importance of EF skills for academic achievement among typically developing children (e.g., Best et al., 2011; Spiegel et al., 2021) less is known among children with epilepsy (Ng et al., 2020). The purpose of this study is to examine the influence of EF skills on academic achievement in a nationwide sample of children with epilepsy.
Participants and Methods:Participants included 427 children with epilepsy (52% male; MAge= 10.71), enrolled in the Pediatric Epilepsy Research Consortium (PERC) Epilepsy Surgery Database who had been referred for surgery and underwent neuropsychological testing. Academic achievement was assessed by performance measures (word reading, reading comprehension, spelling, and calculation and word-based mathematics) and parent-rating measures (Adaptive Behavior Assessment System (ABAS) Functional Academics and Child Behavior Checklist (CBCL) School Performance). EF was assessed by verbal fluency measures, sequencing, and planning measures from the Delis Kaplan Executive Function System (DKEFS), NEPSY, and Tower of London test. Rating-based measures of EF included the 'Attention Problems’ subscale from the CBCL and 'Cognitive Regulation’ index from the Behavior Rating Inventory of Executive Function (BRIEF-2). Partial correlations assessed associations between EF predictors and academic achievement, controlling for fullscale IQ (FSIQ; A composite across intelligence tests). Significant predictors of each academic skill or rating were entered into a two-step regression that included FSIQ, demographics, and seizure variables (age of onset, current medications) in the first step with EF predictors in the second step.
Results:Although zero-order correlations were significant between EF predictors and academic achievement (.29 < r’s < .63 for performance; -.63 < r’s < -.50 for rating measures), partial correlations controlling for FSIQ showed fewer significant relations. For performance-based EF, only letter fluency (DKEFS Letter Fluency) and cognitive flexibility (DKEFS Trails Condition 4) demonstrated significant associations with performance-based academic achievement (r’s > .29). Regression models for performance-based academic achievement indicated that letter fluency (ß = .22, p = .017) and CBCL attention problems (ß = -.21, p =.002) were significant predictors of sight-word reading. Only letter fluency (ß = .23, p =.006) was significant for math calculation. CBCL Attention Problems were a significant predictor of spelling performance (ß = -.21, p = .009) and reading comprehension (ß = -.18, p =.039). CBCL Attention Problems (ß = -.38, p <.001 for ABAS; ß = -.34, p =.002 for CBCL School) and BRIEF-2 Cognitive Regulation difficulties (ß = -.46, p < .001 for ABAS; ß = -.46, p =.013 for CBCL School) were significant predictors of parent-rated ABAS Functional Academics and CBCL School Performance.
Conclusions:Among a national pediatric epilepsy dataset, performance-based and ratings-based measures of EF predicted performance academic achievement, whereas only ratings-based EF predicted parent-rated academic achievement, due at least in part to shared method variance. These findings suggest that interventions that increase cognitive regulation, reduce symptoms of attention dysfunction, and promote self-generative, flexible thinking, may promote academic achievement among children with epilepsy.
38 Fine Motor Skills in Pediatric Frontal Lobe Epilepsy are Associated with Executive Dysfunction and ADHD Symptomatology
- Moshe Maiman, Madison Berl, Jennifer I Koop, Donald J Bearden, Katrina Boyer, Crystal M Cooper, Amanda M Decrow, Priscilla H. Duong, Patricia Espe-Pfeifer, Marsha Gabriel, Elise Hodges, Kelly A McNally, Andrew Molnar, Emily Olsen, Kim E Ono, Kristina E Patrick, Brianna Paul, Jonathan Romain, Leigh N Sepeta, Rebecca LH Stilp, Greta N Wilkening, Mike Zaccariello, Frank Zelko, Clemente Vega, Trey Moore, Szimonetta Mulati, Phillip Pearl, Jeffrey Bolton, Alyssa Ailion
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- Journal of the International Neuropsychological Society / Volume 29 / Issue s1 / November 2023
- Published online by Cambridge University Press:
- 21 December 2023, pp. 37-38
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Objective:
Pediatric patients with frontal lobe epilepsy (FLE) have higher rates of attention deficit hyperactivity disorder (ADHD), as well as executive functioning (EF) and fine motor (FM) challenges. Relations between these constructs have been established in youth with ADHD and are supported by FM and EF skill involvement in frontal-subcortical systems. Still, they are not well understood in pediatric FLE. We hypothesized that poorer FM performance would be related to greater executive dysfunction and ADHD symptomatology in this group.
Participants and Methods:47 children and adolescents with FLE (AgeM=12.47, SD=5.18; IQM=84.07; SD=17.56; Age of Seizure OnsetM=6.85, SD=4.64; right-handed: n=34; left-handed: n=10; Unclear: n=3) were enrolled in the Pediatric Epilepsy Research Consortium dataset as part of their phase I epilepsy surgical evaluation. Participants were selected if they had unifocal FLE and completed the Lafayette Grooved Pegboard (GP). Seizure lateralization (left-sided: n=19; right-sided: n=26; bilateral: n=2) and localization were established via data (e.g., EEG, MRI) presented at a multidisciplinary team case conference. Patients completed neuropsychological measures of FM, attention, and EF. Parents also completed questionnaires inquiring about their child’s everyday EF and ADHD symptomatology. Correlational analyses were conducted to examine FM, EF, and ADHD relations.
Results:Dominant hand (DH) manual dexterity (GP) was related to parent-reported EF (Behavior Rating Inventory of Executive Function, Second Edition [BRIEF-2]-Global Executive Composite [GEC]: r(15) =-.70, p<.01, d=1.96). While not statistically significant, medium to large effect sizes were found for GP DH and parent-reported inattention (Behavior Assessment System for Children, Third Edition [BASC-3]-Attention Problems: r(12)=-.39, p=.17, d=.85) and hyperactivity/impulsivity (BASC-3-Hyperactivity: r(11)= -.44, p=.13, d=.98), as well as performance-based attention (Conners Continuous Performance Test, Third Edition -Omission Errors: r(12)=-.35, p=.22, d=.41), working memory (Wechsler Intelligence Scale for Children - Fifth Edition [WISC-V]-Digit Span [DS]: r(19)=.38, p=.09, d=.82) and cognitive flexibility (Delis-Kaplan Executive Function System (D-KEFS) Verbal Fluency Category Switching: r(13)=.46, p=.08, d=1.04); this suggests that these relations may exist but that our study was underpowered to detect them. Non-dominant hand (NDH) manual dexterity was related to performance-based working memory (WISC-V-DS: r(19)=.50, p<.01, d=1.12) and cognitive flexibility (D-KEFS-Trails Making Test Number-Letter Switching: r(17)=.64, p<.01, d=1.67). Again, while underpowered, medium to large effect sizes were found for GP NDH and parent-reported EF (BRIEF-2 GEC: r(15) =-.45, p=.07, d=1.01) and performance-based phonemic fluency (D-KEFS-Letter Fluency: r(13)=.31, p=.20, d=.65).
Conclusions:Our findings suggest that FM, EF, and ADHD are related in youth with FLE; however, these relations appear to vary by skill and hand. We posit that our findings are due in part to the frontal-cerebellar networks given their anatomic proximity between frontal motor areas and the dorsolateral prefrontal cortex - as well as their shared functional involvement in these networks. Future studies should evaluate the predictive validity of initial FM skills for later executive dysfunction and ADHD symptomatology in FLE. If such relations emerge, contributions of early FM interventions on EF development should be examined. Further replication of these findings with a larger sample is warranted.
Reynolds number dependence of turbulent kinetic energy and energy balance of 3-component turbulence intensity in a pipe flow
- Marie Ono, Noriyuki Furuichi, Yoshiyuki Tsuji
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- Journal:
- Journal of Fluid Mechanics / Volume 975 / 25 November 2023
- Published online by Cambridge University Press:
- 10 November 2023, A9
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Measurement data sets are presented for the turbulence intensity profile of three velocity components ($u$, $v$ and $w$) and turbulent kinetic energy (TKE, $k$) over a wide range of Reynolds numbers from $Re_\tau =990$ to 20 750 in a pipe flow. The turbulence intensity profiles of the $u$- and $w$-component show logarithmic behaviour, and that of the $v$-component shows a constant region at high Reynolds numbers, $Re_\tau >10\,000$. Furthermore, a logarithmic region is also observed in the TKE profile at $y/R=0.055\unicode{x2013}0.25$. The Reynolds number dependences of peak values of $u$-, $w$-component and TKE fit to both a logarithmic law (Marusic et al., Phys. Rev. Fluids, vol. 2, 2017, 100502) and an asymptotic law (Chen and Sreenivasan, J. Fluid Mech., vol. 908, 2020, R3), within the uncertainty of measurement. The Reynolds number dependence of the bulk TKE $k^+_{bulk}$, which is the total amount of TKE in the cross-sectional area of the pipe also fits to both laws. When the asymptotic law is applied to the $k^+_{bulk}$, it asymptotically increases to the finite value $k^+_{bulk}=11$ as the Reynolds number increases. The contribution ratio $\langle u'^2\rangle /k$ reaches a plateau, and the value tends to be constant within $100< y^+<1000$ at $Re_\tau >10\ 000$. Therefore, the local balance of each velocity component also indicates asymptotic behaviour. The contribution ratios are balanced in this region at high Reynolds numbers as $\langle u'^2\rangle /k\simeq 1.25$, $\langle w'^2\rangle /k\simeq 0.5$ and $\langle v'^2\rangle /k \simeq 0.25$.
Impact of calorie intake and weight gain after Norwood procedure on the outcome of stage II palliation
- Helena Staehler, Thibault Schaeffer, Bettina Ruf, Paul Philipp Heinisch, Chiara Di Padua, Melchior Burri, Nicole Piber, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
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- Journal:
- Cardiology in the Young / Volume 34 / Issue 4 / April 2024
- Published online by Cambridge University Press:
- 06 November 2023, pp. 876-883
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Background:
This study aimed to assess the impact of caloric intake and weight-for-age-Z-score after the Norwood procedure on the outcome of bidirectional cavopulmonary shunt.
Methods:A total of 153 neonates who underwent the Norwood procedure between 2012 and 2020 were surveyed. Postoperative daily caloric intake and weight-for-age-Z-score up to five months were calculated, and their impact on outcome after bidirectional cavopulmonary shunt was analysed.
Results:Median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Modified Blalock-Taussig shunt was used in 95 patients and right ventricle to pulmonary artery conduit in 58. Postoperatively, total caloric intake gradually increased, whereas weight-for-age-Z-score constantly decreased. Early and inter-stage mortality before stage II correlated with low caloric intake. Older age (p = 0.023) at Norwood, lower weight (p < 0.001) at Norwood, and longer intubation (p = 0.004) were correlated with low weight-for-age-Z-score (< –3.0) at 2 months of age. Patients with weight-for-age-Z-score < –3.0 at 2 months of age had lower survival after stage II compared to those with weight-for-age-Z-score of –3.0 or more (85.3 versus 92.9% at 3 years after stage II, p = 0.017). There was no difference between inter-stage weight gain and survival after bidirectional cavopulmonary shunt between the shunt types.
Conclusion:Weight-for-age-Z-score decreased continuously throughout the first 5 months after the Norwood procedure. Age and weight at Norwood and intubation time were associated with weight gain. Inter-stage low weight gain (Z-score < –3) was a risk for survival after stage II.
Replacement of pulmonary venous stent during Fontan operation
- Shin Ono, Sho Torii, Hideaki Ueda
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- Journal:
- Cardiology in the Young / Volume 33 / Issue 12 / December 2023
- Published online by Cambridge University Press:
- 12 October 2023, pp. 2670-2672
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Post-operative pulmonary venous stenosis is a poor prognostic factor in single-ventricle haemodynamics. Implantation of a drug-eluting stent is a therapeutic option. However, due to their small size, they inevitably become inadequate as the patient grows. We present the first case, to the best of our knowledge, of the replacement of a small-diameter stent with a large-diameter stent during Fontan surgery.
Tachyarrhythmia after the total cavopulmonary connection: incidence, prognosis, and risk factors
- Cornelius Bohn, Thibault Schaeffer, Magdalena Cuman, Helena Staehler, Chiara Di Padua, Paul Philipp Heinisch, Nicole Piber, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
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- Journal:
- Cardiology in the Young / Volume 34 / Issue 4 / April 2024
- Published online by Cambridge University Press:
- 21 September 2023, pp. 713-721
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Objective:
The purpose of this study is to evaluate the incidence and outcomes regarding tachyarrhythmia in patients after total cavopulmonary connection.
Methods:A retrospective analysis of 620 patients who underwent total cavopulmonary connection between 1994 and 2021 at our institution was performed. Incidence of tachyarrhythmia was depicted, and results after onset of tachyarrhythmia were evaluated. Factors associated with the onset of tachyarrhythmia were identified.
Results:A total of 52 (8%) patients presented with tachyarrhythmia that required medical therapy. Onset during hospital stay was observed in 27 patients, and onset after hospital discharge was observed in 32 patients. Freedom from late tachyarrhythmia following total cavopulmonary connection at 5, 10, and 15 years was 97, 95, and 91%, respectively. The most prevalent late tachyarrhythmia was atrial flutter (50%), followed by supraventricular tachycardia (25%) and ventricular tachycardia (25%). Direct current cardioversion was required in 12 patients, and 7 patients underwent electrophysiological study. Freedom from Fontan circulatory failure after onset of tachyarrhythmia at 10 and 15 years was 78% and 49%, respectively. Freedom from occurrence of decreased ventricular systolic function after the onset of tachyarrhythmia at 5 years was 85%. Independent factors associated with late tachyarrhythmia were dominant right ventricle (hazard ratio, 2.52, p = 0.02) and weight at total cavopulmonary connection (hazard ratio, 1.03 per kilogram; p = 0.04). Type of total cavopulmonary connection at total cavopulmonary connection was not identified as risk.
Conclusions:In our large cohort of 620 patients following total cavopulmonary connection, the incidence of late tachyarrhythmia was low. Patients with dominant right ventricle and late total cavopulmonary connection were at increased risk for late tachyarrhythmia following total cavopulmonary connection.
Brady-arrhythmias requiring permanent pacemaker implantation during and after staged Fontan palliation
- Cornelius Bohn, Thibault Schaeffer, Helena Staehler, Paul Philipp Heinisch, Nicole Piber, Magdalena Cuman, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
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- Journal:
- Cardiology in the Young / Volume 34 / Issue 3 / March 2024
- Published online by Cambridge University Press:
- 27 July 2023, pp. 524-530
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Background:
Brady-arrhythmia requiring pacemaker implantation remains one of the Fontan-specific complications before and after total cavopulmonary connection.
Methods:A retrospective analysis of 620 patients who underwent total cavopulmonary connection between 1994 and 2021 was performed to evaluate the incidence of brady-arrhythmia and the outcomes after pacemaker implantation. Factors associated with the onset of brady-arrhythmia were identified.
Results:A total of 52 patients presented with brady-arrhythmia and required pacemaker implantation. Diagnosis included 16 sinus node dysfunctions, 29 atrioventricular blocks, and 7 junctional escape rhythms. Pacemaker implantation was performed before total cavopulmonary connection (n = 16), concomitant with total cavopulmonary connection (n = 8), or after total cavopulmonary connection (n = 28, median 1.8 years post-operatively). Freedom from pacemaker implantation following total cavopulmonary connection at 10 years was 92%. Twelve patients needed revision of electrodes due to lead dysfunction (n = 9), infections (n = 2), or dislocation (n = 1). Lead energy thresholds were stable, and freedom from pacemaker lead revision at 10 years after total cavopulmonary connection was 78%. Congenitally corrected transposition of the great arteries (odds ratio: 6.6, confidence interval: 2.0–21.5, p = 0.002) was identified as a factor associated with pacemaker implantation before total cavopulmonary connection. Pacemaker rhythms for Fontan circulation were not a risk factor for survival (p = 0.226), protein-losing enteropathy/plastic bronchitis (p = 0.973), or thromboembolic complications (p = 0.424).
Conclusions:In our cohort of patients following total cavopulmonary connection, freedom from pacemaker implantation at 10 years was 92% and stable atrial and ventricular lead energy thresholds were observed. Congenitally corrected transposition of the great arteries was at increased risk for pacemaker implantation before total cavopulmonary connection. Having a pacemaker in the Fontan circulation had no adverse effect on survival, protein-losing enteropathy/plastic bronchitis, or thromboembolic complications.
Development of Standard Operation Procedures for Receiving International Emergency Medical Teams. Cooperation Between the United States National Disaster Medical System (NDMS) Disaster Medical Assistant Team (DMAT) and Japan DMAT
- Akinori Wakai, Yoshiki Toyokuni, Yuichi Koido, Tatsuo Ono, Tatsuhiko Kubo
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- Journal:
- Prehospital and Disaster Medicine / Volume 38 / Issue S1 / May 2023
- Published online by Cambridge University Press:
- 13 July 2023, p. s33
- Print publication:
- May 2023
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Introduction:
Japan DMAT and US DMAT have been conducting several tabletop exercises to prepare for major earthquake disasters in Japan. Japan is predicting overwhelming disasters on Japanese soil soon, which needs efficient and optimum use of resources in medical assistance, including additional support from the US. The Japanese government established a large-scale Earthquake/Tsunami Disaster Emergency Response protocol in 2020. However, this protocol does not include any standard operation procedure (SOP) to receive an international medical team. The purpose of this study is to establish the SOP of receiving medical assistance from US-DMAT based on the WHO International Emergency Team (EMT) initiative through tabletop exercises.
Method:Collaborated with the Office of the Administration for Strategic Preparedness and Response (ASPR) of the United States Health and Human Services, tabletop exercises assuming that a large-scale earthquake occurred during the hosting of the 2025 Osaka Expo was conducted utilizing an online meeting system.
Results:A provisional SOP was composed. Even though Japan had several disaster medical assistance collaborations with US DMAT and is well-familiarized with the Classification and Minimum Standards for Emergency Medical Teams", many issues need to be prepared to accept US DMAT.
Conclusion:Numerous procedures need to be conducted to receive US DMAT assistance during a large-scale earthquake in Japan. With this SOP, receiving US medical team assistance will be conducted promptly, eventually saving many lives. This SOP can be modified for other international teams' acceptance in Japan. It could reference other countries seeking to have SOP for receiving international medical team assistance shortly.
Introduction of Japan Disaster Medical Assistant Team as a National Emergency Medical Team
- Yuichi Koido, Hisayoshi Kondo, Akinori Wakai, Tatsuo Ono, Yoshiki Toyokuni
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- Journal:
- Prehospital and Disaster Medicine / Volume 37 / Issue S2 / November 2022
- Published online by Cambridge University Press:
- 22 November 2022, p. s81
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- November 2022
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Background/Introduction:
Japan is a country with many disasters. Japan’s disaster medical system has improved significantly over the past 30 years by gaining experience in various disasters. Japan is implementing one of the unique disaster medical teams raised from experience and needs.
Objectives:Introducing the Japan Disaster Medical Assistance Team (DMAT) system and discussing how the domestic disaster medical team should be.
Method/Description:Referring to the actual response to domestic disasters and the development of disaster medical teams in Japan.
Results/Outcomes:The National Emergency Medical Teams (National-EMT), Japan DMAT, was established in 2005. Currently, DMAT is 1,754 teams and over 15,862 members were registered. The team usually consists of five to six personnel, including two doctors, two nurses, and two logisticians. Each team carries standardized equipment. Japan DMAT will not set up a field hospital in the disaster area. Japan DMAT will bring a team to the Disaster Base Hospitals (DBHs) and start supporting the affected hospital operation first. Then, if there are other affected patients within DBH’s medical jurisdiction, then the team will mobile and support each hospital and clinic for further medical assistance.
Conclusion:Having National-EMT in your own country is necessary for disaster-prone countries. Still, it is also required to consider what type of EMT needs to be established, referring already existing emergency medical system of your country.