bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and molecular genetic characteristics of classical homocystinuria
Authors: Gusina A. A. , Zinovik A. V. , Naumchik I. N. , Kulak V. D. , Motiuk I. N. , Boisha A. S. , Miasnikov S. O. , Stalybko N. S. , Gusina N. B. 
Published: March 2022

Classical homocystinuria is caused by a genetic mutation in the CBS gene, which leads to low levels or absence of an enzyme called cystathionine beta-synthase.The purpose of the study was to analyze the clinical features and molecular and genetic data of patients with classical homocystinuria in Belarus.The study group included patients with classical homocystinuria and their healthy siblings (3 probands and 2 siblings) from three unrelated families. Diagnosis of homocystinuria was made on a quantitative determination of the total homocysteine level in plasma. The next-generation sequencing was performed for the molecular genetic analysis of the CBS gene. The presence of the identified variants in probands and their siblings was confirmed by the Sanger sequencing.All probands had specific clinical signs of classic homocystinuria: ectopia lentis, skeletal pathology, intellectual, psychiatric, behavioural problems and seizures (in 2 of 3 probands).Homozygous missense-mutations c.430G>C (p.Glu144Gln, rs121964966), c.473C>T p.(Ala158Val, rs1376851289) and 1064C>T p.(Ala355Val, rs772384826) were identified in proband 1, 2 and 3 respectively. Healthy siblings of probands 1 and 3 were the heterozygous carriers of the corresponding mutations.Classical homocystinuria is a very rare disease in the Republic of Belarus. All cases of the disease in Belarus are caused by very rare mutations not registered in the neighboring countries and are the result of marriages between the relatives or the natives of the same area. We have described for the first time the phenotypic manifestations of the p.Glu144Gln and p.Ala355Val mutations, expanded the description of the spectrum of clinical manifestations of the Ala158Val substitution, and assessed the clinical significance of the identified variants in accordance with the modern criteria.
Evaluation of Bacillus thuringiensis Subsp. Israelensis and Bacillus sphaericus Combination Against Culex pipiens in Highly Vegetated Ditches
Authors: Virgillito Chiara , Manica Mattia , Marini Giovanni , Rosà Roberto , Della Torre Alessandra , Martini Simone , Drago Andrea , Baseggio Alberto , Caputo Beniamino 
Published: March 2022

ABSTRACT
Among the few mosquito larvicides available in the market, Bacillus thuringiensis subsp. israelensis (Bti) and B. sphaericus (Bs) represent the most environmentally safe alternatives. The combination of the 2 products is known to overcome their specific limitations by producing a synergistic effect. The aim of the study was to assess the effect and persistence of a single treatment with a granular Bti + Bs formulation on highly vegetated ditches in northeastern Italy that represents the primary rural larval sites for Culex pipiens, the primary vector of the West Nile virus in Europe. The analysis takes into account the nonlinear temporal effects on the population dynamics of larvae and pupae. The results showed a dramatic reduction in mosquito larval abundance 24 h posttreatment (93%) and was effective against larvae up to 22 days (100%). The residual effect after 28 days was 99.5%, and a limited residual effect was observed after 39 days (31.2%). A reduction in pupal density was observed after 4 days (70%) and was &gt;98% from days 14 to 28 posttreatment, persisting for up to 39 days (84% after 39 days). The results demonstrate the effective use of the Bti + Bs formulation against Cx. pipiens in vegetated ditches in rural areas. Our modeling framework provides a flexible statistical approach to predict the residual effect of the product over time, in order to plan a seasonal intervention scheme.
The Impact of Adulticide on Culex Abundance and Infection Rate in North Shore of Cook County, Illinois
Authors: Sass Danielle , Li Bo , Clifton Mark , Harbison Justin , Xamplas Christopher , Smith Rebecca 
Published: March 2022

ABSTRACT
Mosquito surveillance is critical to reduce the risk of West Nile virus (WNV) transmission to humans. In response to surveillance indicators such as elevated mosquito abundance or increased WNV levels, many mosquito control programs will perform truck-mounted ultra-low volume (ULV) adulticide application to reduce the number of mosquitoes and associated virus transmission. Despite the common use of truck-based ULV adulticiding as a public health measure to reduce WNV prevalence, limited evidence exists to support a role in reducing viral transmission to humans. We use a generalized additive and fused ridge regression model to quantify the location-specific impact of truck-mounted ULV adulticide spray efforts from 2010 to 2018 in the North Shore Mosquito Abatement District (NSMAD) in metropolitan Chicago, IL, on commonly assessed risk factors from NSMAD surveillance gravid traps: Culex abundance, infection rate, and vector index. Our model also takes into account environmental variables commonly associated with WNV, including temperature, precipitation, wind speed, location, and week of year. Since it is unlikely ULV adulticide spraying will have the same impact at each trap location, we use a spatially varying spray effect with a fused ridge penalty to determine how the effect varies by trap location. We found that ULV adulticide spraying has an immediate temporary reduction in abundance followed by an increase after 5 days. It is estimated that mosquito abundance increased more in sprayed areas than if left unsprayed in all but 3 trap locations. The impact on infection rate and vector index were inconclusive due to the large error associated with estimating trap-specific infection rates.
Evaluation of Alternative Power Sources for Operating CDC Mosquito Surveillance Traps
Authors: Cilek J. E. , Fajardo J. D. , Weston J. R. , Sallam M. 
Published: March 2022

ABSTRACT
Several portable, rechargeable lithium-ion (Li-Ion) cell phone power banks were compared with standard 6-V gel lead-acid batteries as alternative power sources for operating mosquito surveillance equipment. In laboratory trials, ToughTested® (TT)16000 and 24000, Goal Zero Venture™ 70, and Griffin Survivor® units either met or exceeded that of sealed 6-V batteries when operating the Centers for Disease Control and Prevention (CDC) suction light traps (with incandescent light on) for an average of 24 h. No significant difference was found when continually operating traps powered by either the TT16000 or Goal Zero Venture 70 units compared with 6-V batteries (at approximately 57 h). The TT24000 unit was the only Li-Ion power bank that exceeded this threshold at an average of approximately 73 h. In field studies, there was no significant difference in species diversity or abundance of mosquitoes among the above 4 power sources when operating CDC light traps for 24 h compared with 6-V batteries. Our results indicate that portable Li-Ion cell phone power banks ≥10,050 mAh may be suitable replacements for 6-V gel lead-acid batteries when operating these light traps, especially if weight and size constraints influence the extent of mosquito surveillance during deployments.
Initial Survey of Mosquito Control Programs with Set Action Thresholds and Associated Surveillance Characteristics
Authors: Aryaprema Vindhya S. , Steck Madeline R. , Peper Steven T. , Farooq Muhammad , Xue Rui-De , Qualls Whitney A. 
Published: March 2022

ABSTRACT
The establishment of action thresholds is becoming critical in mosquito control management to implement effective proactive control measures using limited available resources. As a part of a large-scale study to identify different mosquito control action thresholds used in different geographical regions, we conducted an initial survey to identify mosquito control programs that claim to have set action thresholds and to investigate their associated program characteristics. We identified 68% (USA), 60% (mainland Australia), 78% (Asia), 35% (Africa), and 50% (US military units) of the responding programs, which perform mosquito control, have set mosquito control action thresholds. More than 50% of the programs that have not set mosquito control action thresholds already collect basic surveillance information as do the programs with thresholds. Further investigations with the selected mosquito control programs from this initial survey will help develop guidelines on establishing action thresholds by identifying different types of actual action thresholds used by programs in different geographical settings and other related information.
Authors: Amaliyah Aam 
Published: March 2022

Penelitian ini bertujuan untuk mengetahui terdapat atau tidaknya perbedaan kemampuan pemecahan masalah matematika dengan model pembelajaran Realistic Mathematic Education (RME). Jenis penelitian yang dilakukan adalah penelitian eksperimen dengan desain quasi experimental design dan bentuk nonequivalent control group design. Penelitian ini dilaksanakan di SDN Poris Pelawad 3 Cipondoh Kota Tangerang Kelas IV pada semester genap tahun pelajaran 2018/2019. Sampel yang diteliti sebanyak 40 siswa yang terdiri dari 20 siswa kelas eksperimen dan 20 siswa kelas kontrol. Hasil dari penelitian menjelaskan bahwa terdapat perbedaan kemampuan pemecahan masalah matematika antar kelas kontrol dengan kelas eksperimen.
Pelatihan Da’I Muda Sumatera Utara
Authors: Syafaruddin Syafaruddin , Siahaan Dira Nadira Amelia , Assingkily Muhammad Saleh , Siregar Tarmiji 
Published: March 2022

<p>Da'wah Islamiyah is the education of Islamic values which is taught through oral, written, and behavior or actions. To that end, Al-Ittihadiyah has responded to the need for da'wah cadres as “Islamic educators” in society by training the younger generation in training activities for young da'i. This paper aims to analyze the role of the DPW Al-Ittihadiyah in fostering young da'i in North Sumatra. The data were collected by means of interview techniques, observation, and documentation review. Furthermore, the data were analyzed using reduction techniques, data display, and conclusion drawing. The data validity was tested through triangulation and member check. The results of this study indicate that the role of Al-Ittihadiyah in fostering young preachers in North Sumatra has been carried out well through preachers' training every year since 2017. This is indicated by 3 aspects, namely (1) input in the form of efforts to produce young preachers. AlIttihadiyah through youth preachers training, (2) a process in the form of training activities with Islamic da'wah practices and materials for 3x24 hours, and (3) the output in the form of delegating young preachers to the Tanah Karo area for 7x24 hours. </p><p> </p>
Peningkatan Afeksi Anak Usia Dini melalui Dongeng dengan Alat Peraga
Authors: Wahyudi Muhammad 
Published: March 2022

<p>Through the Fairy Tale Method with props, where children are invited to develop their own children's affections. Furthermore, teachers play a role in orienting children to understand the moral messages contained in, a fairy tale that is conveyed by using a media that is more interesting, so that images that can enhance children's affection in the process carried out in Early Childhood Class Yasmin TK DR. Cipto Mangunkusumo, Bandar Jawa Village, Bandar District, Simalungun Regency. To obtain data through testing and observation with data collection tools such as observation sheets for children and teachers and the test as an evaluation material at the end of each cycle that aims to determine changes in the ability of children to develop affection in the fairytale method. There are several conclussions: The level of affection of children in Cycle I there were 18 children 66.67%) reached the level of completeness ≥ 70, this means the child had not reached classical completeness and was in the Fair criteria. While in Cycle II there were 23 children who had reached mastery levels ≥ 70 (85.18%). So in Cycle II an increase in affection which has reached the Good category.</p>
How much residual deformity is acceptable according to SRS-22r and satisfaction scores after posterior spinal fusion for Lenke type 1 and 2 curves in adolescent idiopathic scoliosis?
Authors: Mimura Tetsuhiko , Ikegami Shota , Kuraishi Shugo , Uehara Masashi , Oba Hiroki , Takizawa Takashi , Munakata Ryo , Hatakenaka Terue , Kamanaka Takayuki , Miyaoka Yoshinari , Koseki Michihiko , Takahashi Jun 
Published: March 2022

OBJECTIVE
In recent years, it has become possible to predict postoperative correction and residual deformity in adolescent idiopathic scoliosis (AIS) surgery based on the technique used and extent of fixation. However, the recommended degree of correction has not yet been established. In this study, the authors aimed to clarify the extent to which a residual postoperative deformity would be acceptable according to Scoliosis Research Society (SRS)–22r and satisfaction scores after AIS surgery.

METHODS
Overall, 92 patients who underwent posterior spinal fusion for Lenke type 1 or 2 AIS were retrospectively included. The Patient Acceptable Symptom State (PASS) cutoff values for each SRS-22r domain were calculated using receiver operating characteristic (ROC) curves to obtain predictive values of treatment satisfaction 2 years after surgery. Multivariate logistic regression analysis was performed with deformity parameters and demographic data as explanatory variables, and achieving the PASS cutoff value of each SRS-22r domain and treatment satisfaction were objective variables. Cutoff values were calculated using ROC analysis.

RESULTS
The PASS cutoff values for SRS-22r domains were 3.69 (area under the ROC curve [AUC] 0.86) for self-image, 4.25 (AUC 0.82) for mental health, and 4.22 (AUC 0.82) for the subtotal. The residual main thoracic Cobb angle was not remarkably related to SRS-22r or treatment satisfaction. The residual thoracolumbar/lumbar (TL/L) Cobb angle was significantly associated with treatment satisfaction, with a cutoff value of 12.5° (AUC 0.75). The parameters of deformity that were significantly associated with achieving the PASS cutoff value for self-image were the TL/L Cobb angle and main thoracic apical vertebral translation, although their respective AUCs were < 0.7.

CONCLUSIONS
In patients with Lenke type 1 and 2 AIS, the residual postoperative TL/L Cobb angle was significantly associated with achieving the PASS cutoff values for self-image and treatment satisfaction. Satisfaction with treatment was more likely when the TL/L Cobb angle was ≤ 12.5°.
Increased Serum Beta-Secretase 1 Activity is an Early Marker of Alzheimer’s Disease
Authors: Nicsanu Roland , Cervellati Carlo , Benussi Luisa , Squitti Rosanna , Zanardini Roberta , Rosta Valentina , Trentini Alessandro , Ferrari Clarissa , Saraceno Claudia , Longobardi Antonio , Bellini Sonia , Binetti Giuliano , Zanetti Orazio , Zuliani Giovanni , Ghidoni Roberta 
Published: March 2022

Background: Beta-site APP cleaving enzyme 1 (BACE1) is the rate-limiting enzyme in amyloid-β (Aβ) plaques formation. BACE1 activity is increased in brains of patients with Alzheimer’s disease (AD) and mild cognitive impairment (MCI) and plasma levels of BACE1 appears to reflect those in the brains. Objective: In this work, we investigated the role of serum BACE1 activity as biomarker for AD, estimating the diagnostic accuracy of the assay and assessing the correlation of BACE1 activity with levels of Aβ1 - 40, Aβ1 - 42, and Aβ40/42 ratio in serum, known biomarkers of brain amyloidosis. Methods: Serum BACE1 activity and levels of Aβ1 - 40, Aβ1 - 42, were assessed in 31 AD, 28 MCI, diagnosed as AD at follow-up (MCI-AD), and 30 controls. The BACE1 analysis was performed with a luciferase assay, where interpolation of relative fluorescence units with a standard curve of concentration reveals BACE1 activity. Serum levels of Aβ1 - 40, Aβ1 - 42 were measured with the ultrasensitive Single Molecule Array technology. Results: BACE1 was increased (higher than 60%) in AD and MCI-AD: a cut-off of 11.04 kU/L discriminated patients with high sensitivity (98.31%) and specificity (100%). Diagnostic accuracy was higher for BACE1 than Aβ40/42 ratio. High BACE1 levels were associated with worse cognitive performance and earlier disease onset, which was anticipated by 8 years in patients with BACE1 values above the median value (> 16.67 kU/L). Conclusion: Our results provide new evidence supporting serum/plasma BACE1 activity as an early biomarker of AD.
Interval-valued pointwise sustaining degree fuzzy reasoning methods based on the left-continuous t-representable t-norms
Authors: Luo Minxia , Xu Donghui 
Published: March 2022

In this paper, the concept of α (x, y)-interval-valued pointwise sustaining degree based on the left-continuous t-representable t-norms is put forward. And then, as a general extension based on the interval-valued pointwise sustaining degree, the interval-valued α (x, y)-full implication triple I method model, the interval-valued α (x, y)-quintuple implication principle models and the interval-valued α (x, y)-similarity measure method models are given. Moreover, the interval-valued R -type α (x, y)-fuzzy reasoning solutions with triple I method, quintuple implication principle and similarity measure method are given. Some existing results are special cases of the main conclusions in this paper.
Trastorno de Evitación y Restricción de la Ingesta de Alimentos (ARFID): Lo que el pediatra debe saber
Authors: De Toro Valeria , Aedo Karina , Urrejola Pascuala 
Published: March 2022

Los problemas en la alimentación durante la infancia han sido descritos a lo largo del tiempo por diversos autores. El diagnóstico de Trastorno de Evitación y Restricción de la Ingesta de Alimentos, (ARFID, de sus siglas en inglés) fue incluido en el Manual Diagnóstico y Estadístico de Trastornos Mentales, 5ta Edición (DSM-5) el año 2013, como un nuevo diagnóstico dentro de los Trastornos Alimentarios y de Ingesta (TAI) para describir un grupo de pacientes que tienen conductas alimentarias evitativas o restrictivas que no están motivadas por una alteración de la imagen corporal o un deseo de adelgazar. Este trastorno puede manifestarse en una pérdida de peso significativa y/o deficiencia nutricional y/o una marcada interferencia en el funcionamiento psicosocial. Se describen tres formas de presentación que pueden ocurrir en conjunto o de manera independiente: (i) Niños con aversiones sensoriales (selectivos), que rechazan algunas comidas por sus sabores, texturas, olores o formas; (ii) otros que presentan inapetencia, baja ingesta o poco interés en alimentarse (ingesta limitada); (iii) y los que rechazan algunos alimentos o dejan de comer después de un evento traumático asociado (aversivos). Debido a la incorporación reciente del diagnóstico al DSM-5, existe aún escasa evidencia respecto a su enfrentamiento. El propósito de esta revisión es clarificar criterios diagnósticos, y exponer un manejo progresivo de enfrentamiento y tratamiento, con un enfoque multidisciplinario, sin ahondar en la resolución de las causas médicas orgánicas.
Nefropatía lúpica en pacientes pediátricos
Authors: Moral Larraz Ana , Cuenca Carcelén Sandra , Aparicio López Cristina , Clemente Garulo Daniel , López Robledillo Juan Carlos , De Lucas Collantes Carmen 
Published: March 2022

La nefropatía lúpica es una manifestación precoz en el desarrollo del lupus eritematoso sistémico que empeora la morbimortalidad de estos pacientes.Objetivo: Estudiar la forma de presentación de los pacientes con nefropatía lúpica, las características clínicas, inmunológicas y su relación con la histopatología renal.Pacientes y Método: Estudio retrospectivo en menores de 18 años, con nefropatía lúpica, seguidos en un hospital infantil de tercer nivel en Madrid entre enero 2012 y mayo 2020. Se registraron datos demográficos, clínicos, de laboratorio (hemograma, función renal, función hepática, proteínas, ionograma, glucemia, ácido úrico, lactato deshidrogenasa, coagulación y sistemático de orina), inmunológicos (inmunoglobulinas, anticuerpos antinucleares, complemento y anticoagulante lúpico), y su clasificación histológica. Se realizó análisis descriptivo y de asociaciones entre variables, con un p significativo < 0,05.Resultados: Se incluyeron 16 pacientes (11 mujeres), mediana de edad de presentación de 10,6 ± 2,3 años (5,7-15,3). La mediana de tiempo entre el inicio de los síntomas y el compromiso renal fue 6,3 meses ± 10,5 (rango 0-33,6). El 37,5% de los pacientes tuvo compromiso renal como manifestación inicial. El 50% presentó artralgias o artritis previo al diagnóstico, y el 25% fiebre y síntomas constitucionales (astenia, anorexia, y/o pérdida de peso). La forma más frecuente de compromiso renal fue microhematuria asociada a proteinuria en rango no nefrótico. En el estudio anatomo-patológico renal predominaron, según la clasificación ISN/RPS 2003, los grados III (46,6%) y IV (33,3%).Conclusiones: Seis pacientes presentaron compromiso renal al inicio siendo más frecuente el compromiso musculoesquelético. La mayoría (86,6%) presentaron nefritis lúpica avanzada en el estudio histológico al diagnóstico. El compromiso inmunológico fue el único marcador que se correlacionó con el compromiso sistémico.
Trombosis de senos venosos en pediatría. Serie de casos de un hospital terciario
Authors: Rubio Atienza Yolanda , Torrejon Rodriguez Laura , Marco Hernandez Ana , Tomás Vila Miguel 
Published: March 2022

La trombosis de senos venosos (TSV) es una entidad poco frecuente en pediatría, probablemente subdiagnosticada y potencialmente grave, descrita como causa de ictus en la infancia.Objetivo: Describir la presentación clínica, factores de riesgo, tratamiento y evolución de pacientes pediátricos con TSV.Pacientes y Método: Estudio retrospectivo de pacientes ingresados en un hospital de referencia, con diagnóstico de TSV, con edad comprendida entre un mes y diecisiete años, desde enero de 2011 hasta diciembre de 2019. Se revisaron los siguientes datos: edad al diagnóstico, sexo, signos y síntomas de presentación, mecanismos predisponentes, estudio de trombofilias, tratamiento y duración de este, protocolo de seguimiento, secuelas a largo plazo y mortalidad. Se dividió la muestra en dos grupos de edad por sus diferencias en cuanto a la presentación clínica: niños pequeños entre 1 mes y 5 años, y niños mayores y adolescentes entre 6 y 17 años.Resultados: 17 pacientes fueron diagnosticados de TSV, 45% mujeres, con mediana de edad de 4,5 años. Los síntomas más frecuentes en niños mayores (6-17 años) fueron cefalea (80%) y diplopía (60%). En menores de 5 años la presentación clínica más frecuente fue ataxia cerebelosa (42%), asintomáticos (34%) y cefalea (25%). En un 23,5% del total la TSV fue un hallazgo casual en la neuroimagen. 13 pacientes presentaron un antecedente destacable: 53% una otitis media complicada con mastoiditis, un 6% un traumatismo craneoencefálico grave, un 6% resección de una lesión ocupante de espacio cerebral. En el 23% idiopáticos. En un 23% existían factores protrombóticos. El tratamiento de elección en todos los pacientes fue heparina de bajo peso molecular. Durante el seguimiento a corto plazo el 11,8% presentaron déficits neurológicos autolimitados. Un paciente presentó paresia del sexto par craneal a largo plazo. No hubo fallecimientos ni recurrencias del episodio en nuestra serie.Conclusiones: La TSV es una entidad infrecuente y se suele manifestar con signos y síntomas de hipertensión intracraneal. Se trata de una enfermedad potencialmente grave y un diagnóstico y tratamiento precoz puede contribuir a minimizar las secuelas a largo plazo.