Evaluación de las matemáticas emergentes de 0 a 3 años

A first exploration of the assessment of emergent mathematics, which refers to the first mathematics of an intuitive and informal nature developed by children under 3 years of age, is carried out. In order to obtain data, firstly, a semi-structured interview was conducted with a teacher at a nursery school and, secondly, an educational proposal implemented in a group of 12 children aged 1-2 years was documented and interpreted mathematically, based on the rubric "Acquisition of informal mathematical knowledge from 0 to 3 years" (ACMI 0-3). The results show: a) the lack of teacher education in strategies and resources for the assessment of emergent mathematics; b) the validity of the rubric used to document and mathematically interpret the children's actions, showing that most actions are associated with the recognition of sensory qualities and the relative position of objects. It is concluded that it is necessary to provide disciplinary and pedagogical knowledge, both in pre-service and in-service education, so that nursery school professionals can promote and assess emergent mathematics effectively.Se realiza una primera exploración de la evaluación de las matemáticas emergentes, que se refieren a las primeras matemáticas de naturaleza intuitiva e informal que desarrollan los niños menores de 3 años. Para la obtención de datos, en primer lugar, se ha realizado una entrevista semiestructurada a una maestra de una Escuela Infantil y, en segundo lugar, se ha documentado e interpretado matemáticamente una propuesta educativa implementada en un grupo de 12 niños de 1-2 años, a partir de la rúbrica “Adquisición de conocimientos matemáticos informales de 0 a 3 años” (ACMI 0-3). Los resultados muestran: a) la carencia de formación por parte de los docentes en cuanto a las estrategias y recursos para la evaluación de las matemáticas emergentes; b) la validez de la rúbrica utilizada para documentar e interpretar matemáticamente las acciones de los niños, evidenciándose que la mayoría de acciones se asocian al reconocimiento de las cualidades sensoriales y la posición relativa de los objetos. Se concluye que es necesario proporcionar conocimientos disciplinares y didácticos, tanto en la formación inicial como continua, para que las profesionales de la Escuela Infantil puedan promover y evaluar las matemáticas emergentes de manera eficaz

Multidisciplinary models for pregnancy care in patients with rheumatic diseases: Clinical experiences and experts opinion

Pregnancy care; Rheumatic diseasesCuidado del embarazo; Enfermedades reumáticasCura de l'embaràs; Malalties reumàtiquesObjectives: To describe different models of multidisciplinary pregnancy care for patients with inflammatory and autoimmune rheumatic diseases, and the steps to follow concerning their implementation. Methods: A qualitative study was conducted including: (1) a comprehensive literature search in PUBMED focused on multidisciplinary care models; (2) structured interviews with seven rheumatologists from multidisciplinary pregnancy clinics for patients with inflammatory and autoimmune rheumatic diseases. Data were collected related to the hospitals, medical departments, populations cared for, and multidisciplinary care models (type, material, and human resources, professional requirements, objectives, referral criteria, agendas, protocols, responsibilities, decision-making, research and educational activities, multidisciplinary clinical sessions, initiation/start, planning, advantages/disadvantages, and barriers/facilitators for implementation); (3) a nominal meeting group in which the results of searches and interviews were analyzed and the recommendations for the implementation of the multidisciplinary care models defined. Results: We analyzed seven models of multidisciplinary care in pregnancy, implemented 3-10 years ago, which can all be summarized by two different subtypes: parallel (patients are assessed the same day in the involved medical services) and preferential (patients are assessed on different days in the involved medical services) circuits. The implementation of a specific model results rather from an adaptation to the hospital’s and professionals’ circumstances. Correct planning and good harmony among professionals are key points to implementing a model. Conclusion: Different multidisciplinary care models have been implemented for patients with inflammatory and autoimmune rheumatic diseases during pregnancy. They pretend to improve care, system efficiency, and collaboration among specialists and should be carefully implemented.The project was supported by UCB Pharma

Editors' corner: Anales de pediatría annual report.

Anales de Pediatría continúa siendo la única publicación científica pediátrica española reconocida con factor de impacto internacional y una de las 2 únicas revistas pediátricas españolas (junto a Cirugía Pediátrica) indexada en el National Center for Biotechnology Information. Anales es la revista científica pediátrica más importante publicada en castellano. Esta primacía, sin duda debida al trabajo y constancia de todos los Comités Editoriales que nos han precedido, creemos que se ha afianzado en los últimos años con 2 hechos fundamentales: la mejora de los tiempos en la gestión de los manuscritos y su publicación bilingüe en inglés

Colours

Treballs de l'alumnat del Grau de Comunicació Audiovisual, Facultat de Biblioteconomia i Documentació, Universitat de Barcelona, Metanarratives. Curs: 2018-2019, Tutor: Rovira, Josep (Rovira Cuadrench). // Director: Roger Centeno; Càmera:Laia Hervàs; Aj. càmera: Julia Calvo; Il·luminador: Julia Calvo i Helena Alsina; Direcció artística: Roger Centeno; Música: Muddy Waters; Postproducció: Helena Alsina. Equip artístic: Roger Centeno, Alba Hernández, Nerea Molina, Mar Mora, Kenny Pereyra i Albeto Rabal. La música utilitzada és de Muddy Waters i no tenim els drets.Nos hemos decidido por el concepto de la libertad sexual dentro de la comunidad LGBT. Tomando como referente la bandera y sus colores, asignaremos a cada color un sentimiento negativo que ha podido sentir en algún momento una persona del colectivo. Los sentimientos o conceptos que hemos decidido son: la opresión, exposición, debilidad, impotencia, miedo y prejuicios. Somos conscientes que esto pasa en parte por la sociedad y el sistema por el cual se rige y en el que hemos sido educados. En este punto, lo relacionamos también con Foucault y la teoría que explica en su libro Vigilar y castigar. Nacimiento de una prisión. Explica 4 fases por las que ha pasado el castigo de los que él llama “indeseables”, estas etapas se pueden emplear en cualquiera de los procesos o movimientos que alguna vez han sido oprimidos, como es la libertad sexual. Queremos reflejar también está sensación que puede sentir el colectivo lgbt en relación a la exposición y vigilancia a la que se ven sometidos por ser “indeseables” para el sistema

Changes in Treg and Breg cells in a healthy pediatric population

The interpretation of clinical diagnostic results in suspected inborn errors of immunity, including Tregopathies, is hampered by the lack of age-stratified reference values for regulatory T cells (Treg) in the pediatric population and a consensus on which Treg immunophenotype to use. Regulatory B cells (Breg) are an important component of the regulatory system that have been poorly studied in the pediatric population. We analyzed (1) the correlation between the three immunophenotypic definitions of Treg (CD4+CD25hiCD127low, CD4+CD25hiCD127lowFoxP3+, CD4+CD25hiFoxP3+), and with CD4+CD25hi and (2) the changes in Treg and Breg frequencies and their maturation status with age. We performed peripheral blood immunophenotyping of Treg and Breg (CD19+CD24hiCD38hi) by flow cytometry in 55 healthy pediatric controls. We observed that Treg numbers varied depending on the definition used, and the frequency ranged between 3.3–9.7% for CD4+CD25hiCD127low, 0.07-1.6% for CD4+CD25hiCD127lowFoxP3+, and 0.24-2.83% for CD4+CD25hiFoxP3+. The correlation between the three definitions of Treg was positive for most age ranges, especially between the two intracellular panels and with CD4+CD25hi vs CD4+CD25hiCD127low. Treg and Breg frequencies tended to decline after 7 and 3 years onwards, respectively. Treg’s maturation status increased with age, with a decline of naïve Treg and an increase in memory/effector Treg from age 7 onwards. Memory Breg increased progressively from age 3 onwards. In conclusion, the number of Treg frequencies spans a wide range depending on the immunophenotypic definition used despite a good level of correlation exists between them. The decline in numbers and maturation process with age occurs earlier in Breg than in Treg

Recurrent invasive pneumococcal disease in children: Underlying clinical conditions, and immunological and microbiological characteristics.

Purpose Clinical, immunological and microbiological characteristics of recurrent invasive pneumo-coccal disease (IPD) in children were evaluated, differentiating relapse from reinfection, in order to identify specific risk factors for both conditions. Methods All patients<18 years-old with recurrent IPD admitted to a tertiary-care pediatric center from January 2004 to December 2011 were evaluated. An episode of IPD was defined as the presence of clinical findings of infection together with isolation and/or pneumococcal DNA detection by Real-Time PCR in any sterile body fluid. Recurrent IPD was defined as 2 or more episodes in the same individual at least 1 month apart. Among recurrent IPD, we differentiated relapse (same pneumococcal isolate) from reinfection. Results 593 patients were diagnosed with IPD and 10 patients died. Among survivors, 23 episodes of recurrent IPD were identified in 10 patients (1.7%). Meningitis was the most frequent form of recurrent IPD (10 episodes/4 children) followed by recurrent empyema (8 episodes/4 children). Three patients with recurrent empyema caused by the same pneumococcal clone ST306 were considered relapses and showed high bacterial load in their first episode. In contrast, all other episodes of recurrent IPD were considered reinfections. Overall, the rate of relapse of IPD was 0.5% and the rate of reinfection 1.2%. Five out of 7 patients with rein- fection had an underlying risk factor: cerebrospinal fluid leak (n = 3), chemotherapy treatment (n = 1) and a homozygous mutation in MyD88 gene (n = 1). No predisposing risk factors were found in the remainder. Conclusions recurrent IPD in children is a rare condition associated with an identifiable risk factor in case of reinfection in almost 80% of cases. In contrast, recurrent IPD with pleuropneumonia is usually a relapse of infection

Case report : characterizing the role of the STXBP2-R190C monoallelic mutation found in a patient with hemophagocytic syndrome and Langerhans cell histiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disorder. HLH can be considered as a threshold disease depending on the trigger and the residual NK-cell cytotoxicity. In this study, we analyzed the molecular and functional impact of a novel monoallelic mutation found in a patient with two episodes of HLH. A 9-month-old child was diagnosed at 2 months of age with cutaneous Langerhans cell histiocytosis (LCH). After successful treatment, the patient developed an HLH episode. At 16 month of age, the patient went through an HSCT losing the engraftment 5 months later concomitant with an HLH relapse. The genetic study revealed a monoallelic mutation in the STXBP2 gene (.pArg190Cys). We transfected COS7 cells to analyze the STXBP2-R190C expression and to test the interaction with STX11. We used the RBL-2H3 cell line expressing STXBP2-WT-EGFP or R190C-EGFP for degranulation assays. Mutation STXBP2-R190C did not affect protein expression or interaction with syntaxin-11. However, we have demonstrated that STXBP2-R190C mutation diminishes degranulation in the RBL-2H3 cell line compared with the RBL-2H3 cell line transfected with STXBP2-WT or nontransfected. These results suggest that STXBP2-R190C mutation acts as a modifier of the degranulation process producing a decrease in degranulation. Therefore, under homeostatic conditions, the presence of one copy of STXBP2-R190 could generate sufficient degranulation capacity. However, it is likely that early in life when adaptive immune system functions are not sufficiently developed, an infection may not be resolved with this genetic background, leading to a hyperinflammation syndrome and eventually develop HLH. This analysis highlights the need for functional testing of new mutations to validate their role in genetic susceptibility and to establish the best possible treatment for these patients

Acute and long-term immune responses to SARS-CoV-2 infection in unvaccinated children and young adults with inborn errors of immunity

PurposeTo describe SARS-CoV-2 infection outcome in unvaccinated children and young adults with inborn errors of immunity (IEI) and to compare their specific acute and long-term immune responses with a sex-, age-, and severity-matched healthy population (HC).MethodsUnvaccinated IEI patients up to 22 years old infected with SARS-CoV-2 were recruited along with a cohort of HC. SARS-CoV-2 serology and ELISpot were performed in the acute phase of infection (up to 6 weeks) and at 3, 6, 9, and 12 months.ResultsTwenty-five IEI patients (median age 14.3 years, min.-max. range 4.5-22.8; 15/25 males; syndromic combined immunodeficiencies: 48.0%, antibody deficiencies: 16.0%) and 17 HC (median age 15.3 years, min.-max. range 5.4-20.0; 6/17 males, 35.3%) were included. Pneumonia occurred in 4/25 IEI patients. In the acute phase SARS-CoV-2 specific immunoglobulins were positive in all HC but in only half of IEI in whom it could be measured (n=17/25): IgG+ 58.8% (10/17) (p=0.009); IgM+ 41.2% (7/17)(p&lt;0.001); IgA+ 52.9% (9/17)(p=0.003). Quantitative response (index) was also lower compared with HC: IgG IEI (3.1 ± 4.4) vs. HC (3.5 ± 1.5)(p=0.06); IgM IEI (1.9 ± 2.4) vs. HC (3.9 ± 2.4)(p=0.007); IgA IEI (3.3 ± 4.7) vs. HC (4.6 ± 2.5)(p=0.04). ELISpots positivity was qualitatively lower in IEI vs. HC (S-ELISpot IEI: 3/11, 27.3% vs. HC: 10/11, 90.9%; p=0.008; N-ELISpot IEI: 3/9, 33.3% vs. HC: 11/11, 100%; p=0.002) and also quantitatively lower (S-ELISpot IEI: mean index 3.2 ± 5.0 vs. HC 21.2 ± 17.0; p=0.001; N-ELISpot IEI: mean index 9.3 ± 16.6 vs. HC: 39.1 ± 23.7; p=0.004). As for long term response, SARS-CoV-2-IgM+ at 6 months was qualitatively lower in IEI(3/8, 37.5% vs. 9/10 HC: 90.0%; p=0.043), and quantitatively lower in all serologies IgG, M, and A (IEI n=9, 1.1 ± 0.9 vs. HC n=10, 2.1 ± 0.9, p=0.03; IEI n=9, 1.3 ± 1.5 vs. HC n=10, 2.9 ± 2.8, p=0.02; and IEI n=9, 0.6 ± 0.5 vs. HC n=10, 1.7 ± 0.8, p=0.002 –respectively) but there were no differences at remaining time points.ConclusionsOur IEI pediatric cohort had a higher COVID-19 pneumonia rate than the general age-range population, with lower humoral and cellular responses in the acute phase (even lower compared to the reported IEI serological response after SARS-CoV-2 vaccination), and weaker humoral responses at 6 months after infection compared with HC