Busulfan with 400 centigray of total body irradiation and higher dose fludarabine: An alternative regimen for hematopoietic stem cell transplantation in pediatric acute lymphoblastic leukemia.

BACKGROUND: Hematopoietic stem cell transplantation can be curative for children with difficult-to-treat leukemia. The conditioning regimen utilized is known to influence outcomes. We report outcomes of the conditioning regimen used at the Alberta Children's Hospital, consisting of busulfan (with pharmacokinetic target of 3750 µmol*min/L/day ±10%) for 4 days, higher dose (250 mg/m2 ) fludarabine and 400 centigray (cGy) of total body irradiation. PROCEDURE: This retrospective study involved children receiving transplant for acute lymphoblastic leukemia (ALL). It compared children who fell within the target range for busulfan with those who were either not measured or were measured and fell outside this range. All other treatment factors were identical. RESULTS: Twenty-nine children (17 within target) were evaluated. All subjects engrafted neutrophils with a median [interquartile range] time of 14 days [8-30 days]. The cumulative incidence of acute graft-versus-host disease was 44.8% [95% confidence interval, CI: 35.6%-54.0%], while chronic graft-versus-host disease was noted in 16.0% [95% CI: 8.7%-23.3%]. At 2 years, the overall survival was 78.1% [95% CI: 70.8%-86.4%] and event-free survival was 74.7% [95% CI: 66.4%-83.0%]. Cumulative incidence of relapse was 11.3% [95% CI: 5.1%-17.5%]. There were no statistically significant differences in between the group that received targeted busulfan compared with the untargeted group. CONCLUSION: Our conditioning regiment for children with ALL resulted in outcomes comparable to standard treatment with acceptable toxicities and significant reduction in radiation dose. Targeting busulfan dose in this cohort did not result in improved outcomes.

LNK/SH2B3 as a novel driver in juvenile myelomonocytic leukemia.

Mutations in five canonical Ras pathway genes (NF1, NRAS, KRAS, PTPN11 and CBL) are detected in nearly 90% of patients with juvenile myelomonocytic leukemia (JMML), a frequently fatal malignant neoplasm of early childhood. In this report, we describe seven patients diagnosed with SH2B3-mutated JMML, including five patients who were found to have initiating, loss of function mutations in the gene. SH2B3 encodes the adaptor protein LNK, a negative regulator of normal hematopoiesis upstream of the Ras pathway. These mutations were identified to be germline, somatic or a combination of both. Loss of function of LNK, which has been observed in other myeloid malignancies, results in abnormal proliferation of hematopoietic cells due to cytokine hypersensitivity and activation of the JAK/STAT signaling pathway. In vitro studies of induced pluripotent stem cell-derived JMML-like hematopoietic progenitor cells (HPCs) also demonstrated sensitivity of SH2B3- mutated HPCs to JAK inhibition. Lastly, we describe two patients with JMML and SH2B3 mutations who were treated with the JAK1/2 inhibitor ruxolitinib. This report expands the spectrum of initiating mutations in JMML and raises the possibility of targeting the JAK/STAT pathway in patients with SH2B3 mutations.

Mechanistic understanding of the combined immunodeficiency in complete human CARD11 deficiency.

BACKGROUND: Germline pathogenic variants impairing the caspase recruitment domain family member 11 (CARD11)-B cell chronic lymphocytic leukemia/lymphoma 10 (BCL10)-MALT1 paracaspase (MALT1) (CBM) complex are associated with diverse human diseases including combined immunodeficiency (CID), atopy, and lymphoproliferation. However, the impact of CARD11 deficiency on human B-cell development, signaling, and function is incompletely understood. OBJECTIVES: This study sought to determine the cellular, immunological, and biochemical basis of disease for 2 unrelated patients who presented with profound CID associated with viral and fungal respiratory infections, interstitial lung disease, and severe colitis. METHODS: Patients underwent next-generation sequencing, immunophenotyping by flow cytometry, signaling assays by immunoblot, and transcriptome profiling by RNA-sequencing. RESULTS: Both patients carried identical novel pathogenic biallelic loss-of-function variants in CARD11 (c.2509C>T; p.Arg837∗) leading to undetectable protein expression. This variant prevented CBM complex formation, severely impairing the activation of nuclear factor-κB, c-Jun N-terminal kinase, and MALT1 paracaspase activity in B and T cells. This functional defect resulted in a developmental block in B cells at the naive and type 1 transitional B-cell stage and impaired circulating T follicular helper cell (cTFH) development, which was associated with impaired antibody responses and absent germinal center structures on lymph node histology. Transcriptomics indicated that CARD11-dependent signaling is essential for immune signaling pathways involved in the development of these cells. Both patients underwent hematopoietic stem cell transplantations, which led to functional normalization. CONCLUSIONS: Complete human CARD11 deficiency causes profound CID by impairing naive/type 1 B-cell and cTFH cell development and abolishing activation of MALT1 paracaspase, NF-κB, and JNK activity. Hematopoietic stem cell transplantation functionally restores impaired signaling pathways.

Survival outcomes of children with relapsed or refractory myeloid leukemia associated with Down syndrome.

Children with Down syndrome (DS) are at a significantly higher risk of developing acute myeloid leukemia, also termed myeloid leukemia associated with DS (ML-DS). In contrast to the highly favorable prognosis of primary ML-DS, the limited data that are available for children who relapse or who have refractory ML-DS (r/r ML-DS) suggest a dismal prognosis. There are few clinical trials and no standardized treatment approach for this population. We conducted a retrospective analysis of international study groups and pediatric oncology centers and identified 62 patients who received treatment with curative intent for r/r ML-DS between year 2000 to 2021. Median time from diagnosis to relapse was 6.8 (range, 1.1-45.5) months. Three-year event-free survival (EFS) and overall survival (OS) were 20.9 ± 5.3% and 22.1 ± 5.4%, respectively. Survival was associated with receipt of hematopoietic stem cell transplantation (HSCT) (hazard ratio [HR], 0.28), duration of first complete remission (CR1) (HR, 0.31 for > 12 months) and attainment of remission after relapse (HR, 4.03). Patients who achieved complete remission (CR) before HSCT, had an improved OS and EFS of 56.0 ± 11.8% and 50.5 ± 11.9%, respectively compared to those who underwent HSCT without CR (3-year OS and EFS of 10.0 ± 9.5%). Treatment failure after HSCT was predominantly because of disease recurrence (52%) followed by treatment-related mortality (10%). The prognosis of r/r ML-DS remains dismal even in the current treatment period and serve as a reference point for current prognostication and future interventional studies. Clinical trials aimed at improving the survival of patients with r/r ML-DS are needed.

'Teratoid' Hepatoblastoma: An Intriguing Variant of Mixed Epithelial-Mesenchymal Hepatoblastoma.

Liver neoplasms are quite rare in childhood. They often involve 6.7 cases per 10 million children aged 18 years or younger. Hepatoblastoma (HB) is the most frequent tumor, but this neoplasm's rarity points essentially to the difficulty of performing biologic studies and large-scale therapeutic trials. On the pathological ground, HB is separated into an entirely epithelial neoplasm or a mixed neoplasm with epithelial and mesenchymal components. This last category has been further subdivided into harboring teratoid features or not. The 'teratoid' HB includes a mixture of components with heterologous origin. The heterologous components include neuroectoderm, endoderm, or melanin-holding cells with or without mesenchymal components. The most important criterium for the teratoid component is neuroepithelium, melanin, and, more recently, a yolk-sac-like component and neuroendocrine components. The mesenchymal components include muscle, osteoid, and cartilage, which are most often observed mainly in 'teratoid' neoplasms. The teratoid component or mesenchymal components are diagnosed with biopsies. They appear more prominent after chemotherapy due to the response and shrinkage of epithelial elements and non- or low-responsive components of mixed HB. This review focuses on the clinical, radiological, and pathological findings of HB with teratoid features.

Extensive Pelvic Plexiform Neurofibroma Presenting As Clitoromegaly in a 3-Year-Old Female: Presentation and Management with MEK Inhibitor.

Plexiform neurofibroma (PN) involvement of the external genitalia in patients with neurofibromatosis type I (NF1) is a rare cause of nonhormonal clitoromegaly. We present a 3-year-old female with known NF1 who presented with clitoromegaly. She was identified with an extensive pelvic mass involving the bladder wall, perineum, labia, clitoris, rectum, and sacral foramina. A partial cystectomy was performed, and histopathology was consistent with PN. She has been initiated on a mitogen activated protein kinase enzyme kinase inhibitor, trametinib, which has been effective in achieving partial radiographic response of the bladder mass over 5 months. Additionally, she has experienced clinical response to trematinib with resolution of urinary urgency and frequency since initiating treatment.

Neurodevelopmental phenotypes in individuals with pathogenic variants in CHAMP1.

De novo pathogenic variants in CHAMP1 (chromosome alignment maintaining phosphoprotein 1), which encodes kinetochore-microtubule associated protein on 13q34, cause a rare neurodevelopmental disorder. We enrolled 14 individuals with pathogenic variants in CHAMP1 that were documented by exome sequencing or gene panel sequencing. Medical history interviews, seizure surveys, Vineland Adapted Behavior Scales Second Edition, and other behavioral surveys were completed by primary caregivers of available participants in Simons Searchlight. Clinicians extracted clinical data from the medical record for two participants. We report on clinical features of 14 individuals (ages 2-26) with de novo predicted loss-of-function variants in CHAMP1 and compare them with previously reported cases (total n = 32). At least two individuals have the same de novo variant: p.(Ser181Cysfs*5), p.(Trp348*), p.(Arg398*), p.(Arg497*), or p.(Tyr709*). Common phenotypes include intellectual disability/developmental delay, language impairment, congenital and acquired microcephaly, behavioral problems including autism spectrum disorder, seizures, hypotonia, gastrointestinal issues of reflux and constipation, and ophthalmologic issues. Other rarely observed phenotypes include leukemia, failure to thrive, and high pain tolerance. Pathogenic variants in CHAMP1 are associated with a variable clinical phenotype of developmental delay/intellectual disability and seizures.

Classic Hodgkin lymphoma post-transplant lymphoproliferative disorders (PTLD) are often preceded by discordant PTLD subtypes.

Classic Hodgkin lymphoma (CHL) is the rarest post-transplant lymphoproliferative disorder (PTLD) subtype. Few cases of patients with metachronous discordant PTLD episodes including CHL-PTLD have been reported, but the incidence of and risk factors for this phenomenon are unknown. Patients with CHL-PTLD were identified from an institutional PTLD database. Of 13 patients identified with CHL-PTLD six (46%) had antecedent non-CHL-PTLD: three had polymorphic PTLD, two monomorphic PTLD, and one nondestructive PTLD. Patients with prior metachronous non-CHL-PTLD were younger at transplant and had a longer latency time to CHL-PTLD post-transplant. The prevalence of EBV seronegativity at transplant was high in both groups, but prolonged high-level EBV DNAemia only occurred in some with metachronous non-CHL-PTLD. In conclusion, patients with CHL-PTLD have metachronous non-CHL-PTLD diagnoses with discordant histology more commonly than previously recognized. Primary EBV infection with chronically elevated EBV viral loads may represent unique risk factors for CHL-PTLD following an initial non-CHL-PTLD event.

Preliminary Development of the Food Allergy Coping and Emotions Questionnaires for Children, Adolescents, and Young People: Qualitative Analysis of Data on IgE-Mediated Food Allergy from Five Countries.

BACKGROUND: We have previously developed a food allergy-specific developmental model, that explained emotions and coping styles, among children aged 6 to 15years in Ireland. OBJECTIVE: The objective of this study was to investigate the usefulness of the developmental model in a large multicountry data set, including any mediators of coping style, and to use the findings to generate an item pool that will form the basis for 3 age-appropriate self-report questionnaires to measure coping and emotions. METHODS: We conducted deductive thematic analysis on secondary data from interviews with 274 participants aged 6 to 23 years, and 119 parents from Australia, Ireland, Italy, the UK, and the USA. Analysis was undertaken across the entire data set. RESULTS: The Food Allergy Coping and Emotions (FACE) model has 5 major themes: (1) experiences and emotions, (2) search for normality, (3) management and coping, (4) "external mediators," and (5) "internal mediators" (between emotions and coping). These themes were present across countries, but differed according to age. CONCLUSIONS: Early-life experiences provide the foundation for later cognitions and behaviors. The expanded FACE developmental model is useful in explaining emotions and coping styles across different age groups and countries. These data will also be used to generate an age-specific bank of items for the development of 3 (age-specific self-report, and parent proxy) questionnaires to assess emotions and coping in food allergy. Findings provide insight into how particular styles of coping develop and vary from patient to patient and may also guide clinician-patient communication and the development of individualized management strategies.

Estudio fenomenológico sobre la relación entre el envejecimiento activo y la terapia ocupacional en centros de día y residencias de la ciudad de A Coruña / Phenomenological study on the relationship between active aging and occupational therapy in careday centers and residences of the city of A Coruña

Objetivos: el Envejecimiento Activo se centra en la participación de las personas mayores en los asuntos sociales, económicos, culturales, espirituales y cívicos, y no únicamente en la capacidad de estar activos física y mentalmente. La Terapia Ocupacional, con sus conocimientos y herramientas, puede tener un papel fundamental en la promoción del Envejecimiento Activo. El objetivo es estudiar la relación entre laTerapia Ocupacional y la promoción del Envejecimiento Activo, a partir de las percepciones de terapeutas ocupacionales que trabajan en recursos de atención a personas mayores de la ciudad de A Coruña. Metodología: El estudio se enmarca en la investigación cualitativa, dentro del paradigma fenomenológico, utilizando como técnicas de recogida de datos la entrevista semi estructurada y el cuaderno de campo. La muestra del estudio está conformada por 10 profesionales de terapia ocupacional que ejercen su trabajo en centros de día y residencias de A Coruña. Resultados: A pesar de la importancia de la Terapia Ocupacional en los programas de Envejecimiento Activo, se observa en los participantes un desconocimiento importante sobre el fenómeno, que queda patente en la dificultad para justificar su práctica profesional, así como los beneficiosque aporta la disciplina a las personas mayores. Conclusiones: Se considera muy importante incluir información y educación en los planes de estudio y programas de formación de trabajadores sanitarios, dando a conocer los principios básicos del Envejecimiento Activo. También es muy importante continuar desarrollando trabajos que profundicen en la relación entre el Envejecimiento Activo y laTerapia Ocupacional.

Objectives: the Active Aging focuses on the participation of older people in social, economic, cultural, spiritual and civic affairs, not only in the ability to be physically and mentally active. Occupational Therapy with knowledge and tools can play a key role in promoting Active Aging. The aim was to study the relationship between Occupational Therapy and promoting Active Aging, from the perceptions of occupational therapists working in care resources at people over the city of A Coruna. Methodology: The study is part of qualitative research, within the phenomenological paradigm, using semistructured interview and field notebook as data collection techniques. The sample of the study is formed by 10 professionals of occupational therapy who work in care day centers and residences of A Coruña. Results: Despite the importance of Occupational Therapy in Active Aging programs, we can see in participants a significant lack of knowledge about the phenomenon, which is evident in the difficulty to justify their practice, as well as the benefits of the discipline for elderly. Conclusions: It is considered very important to include information and education in the curricula and training programs for health workers, revealing the basic principles of Active Aging. It is also very important to continue to work unwrapping delve in to the relationship between Active Aging and Occupational Therapy.

Vivencias ocupacionales de personas recientemente jubiladas en el entorno urbano de la provincia de A Coruña / Occupational experiences of recently retired people in the urban envinronment of the province of Coruña

Objetivos: en la actualidad, se ha podido observar un envejecimiento progresivo de la población, lo que ha aumentado el interés acerca de la jubilación, ya que la duración de esta será cada vez más prolongada y afectará a los sistemas laborales, sanitarios y educativos, así como a la propia organización de la sociedad. El objetivo de este estudio es explorar y conocer las vivencias ocupacionales de los participantes en su etapa de jubilación. Método: Se ha utilizado la investigación cuantitativa y cualitativa, empleándose como métodos de recogida de datos un cuestionario, una entrevista semiestructurada y un diario de campo. La muestra estuvo conformada por 13 personas jubiladas. Resultados: Existen inconsistencias entre los resultados obtenidos en el cuestionario y lo que los participantes refieren en la entrevista, ya que en el cuestionario otorgan a la jubilación un significado positivo mientras en sus discursos se refleja lo contrario. Además, presentan limitaciones para comprender el concepto oficial de jubilación e identificarse con él. Por último, ha destacado la influencia del clima en el desempeño ocupacional y la controversia existente en cómo se entiende el ocio según el entorno en el que viva la persona (urbano o rural). Conclusiones: Se concluye que la distribución del tiempo es uno de los cambios más significativos de la jubilación, existiendo contradicciones en cuanto al significado que se le otorga a este concepto. Los resultados obtenidos también reflejan la necesidad de la preparación previa a la jubilación, aunque inicialmente muchos participantes no identifiquen esta necesidad como propia.

Aims: nowadays has been observed a progressive aging of the population which has increased the interest about retirement, as the duration of this will be longer and will affect labour, health and education systems, as well as the proper organization of society. The objective of this study is to explore and learn about the occupational experiences of older recently retired in the urban environment of the province of A Coruña. Methods: It has been used for the study the quantitative and qualitative research, being used as methods of data collection a questionnaire, a semistructured interview and a field journal homemade. The sample consisted of 13 retired people. Results: Thereare inconsistencies between the results of the questionnaire and refer participants in the interview, because the retirement questionnaire give a positive meaning while in his speech is the opposite. Furthermore, they have limitations to understand the official concept of retirement and identify with him. Finally, it highlighted the influence of climate on occupational performance and the controversy about how leisure is understood as the environment in which the person lives (urban or rural). Conclusions: We conclude that the distribution of time is one of the most significant changes in retirement, existing contradictions about the meaning given to it. The results also reflect the need for pre - retirement preparation, although initially many participants do not identify this need as their own.

Ante la desesperanza del desplazamiento: un hijo sano, el mayor anhelo de la mujer gestante / While coping with displacement despair: a healthy child/baby, the pregnant woman’s main yearning

Introducción: Entre los países del mundo Colombia ocupa el tercer lugar con más desplazados que, en los últimos quince años son casi dos millones de personas, cuyo mayor porcentaje corresponde a mujeres. Esta ®feminización¼ del desplazamiento, trae consigo para la mujer la ruptura de las relaciones de pareja, la pérdida de su compañero, de los bienes y medios de subsistencia y la convierte en jefe de hogar. Lleva a la ruptura de las redes de apoyo comunitario, a la destrucción de la cohesión social y de la solidaridad y altera la identidad colectiva. Respecto de la salud, muchas gestantes desplazadas no asisten a control prenatal y el cuidado de sí durante este proceso, se relaciona con las tradiciones, creencias y valores que traen pero en el marco de contexto que la situación de desplazadas les confiere. Con el desplazamiento hay gran heterogeneidad cultural, que tiene que ver con las experiencias previas de la mujer y su familia en torno al cuidado durante la gestación. Objetivo: A partir de sus propios conceptos se exploró qué significa el cuidado de sí y del hijo por nacer en un grupo de mujeres gestantes desplazadas, que viven en el barrio Pampas de Mirador, Cali, con el intento de descubrir las dinámicas y la forma como reconstruyen, en el nuevo entorno social, las prácticas de cuidado que se relacionan con la gestación. Métodos: Por medio de la etnoenfermería, y para recolectar los datos, se utilizaron entrevistas en profundidad.

Introduction: Colombia occupies the third place in the world as a country with more displacements approaching two million persons in the last fifteen years and the major percentage corresponds to women. This ®feminization¼ of the displacement, brings with itself the break of couple relations, the loss for the wife of her companion, of the goods and means of subsistence turning her into a household keeper. It takes to break the community support networks, the destruction of the social cohesion and of the solidarity altering the collective identity. With relation to health, a high number of displaced pregnant women do not attend pregnancy health controls and their self care during this process is related to the traditions, beliefs and values that they bring with them into the context that the displacement situation awards them. The displacement brings a great cultural heterogeneity related to the previous experiences of these women’s self care and their families’ during pregnancy. Objective: this research is related to the meaning of the pregnant mother’s self care and her child to be born using a group of displaced women living in Pampas del Mirador neighborhood in the city of Cali, and based in their own beliefs. It tries to discover the dynamics and the way they reconstruct the practices of the care related to pregnancy, within their new social tissue. Method: Ethno nursing includes deep interviews. Nine displaced pregnant women participated in the survey besides two general reporters. The size of the survey was taken by applying the criteria of data saturation. Findings: The big concern for women is that the child is born well and this means for them that they will achieve this provided they develop the same care they saw their mothers to practice, their aunts and the great majority of women of their land.

Mirando el cuidado cultural desde la óptica de Leininger / Cultural care observation from Leininger’s perspective

Introducción: Comprender el cuidado desde la perspectiva de Leininger, implica aprender de las personas sus formas de vida, sus contextos, sus sentimientos, sus preocupaciones, sus creencias, prácticas y valores, en sucesos tan diversos como maternidad, paternidad, nacimiento, adolescencia, adultez, vejez, enfermedad y muerte, con el fin primordial de ofrecer un cuidado coherente con la cultura. Investigación y práctica en enfermería transcultural: Este aprendizaje sobre el cuidado que los individuos adquieren, necesariamente se desarrolla cuando se interactúa con las personas a las que se cuida; la interacción requiere preparación, sensibilidad y pensamiento crítico, para descubrir y captar las distintas significaciones que las personas dan a sus vidas y sus propios cuidados. Reflexión final: La investigación se configura en una puerta de entrada que permite percibir esas perspectivas y también en un puente que aproxima al profesional de enfermería a la cotidianidad de las personas para aprender de ellas.

Introduction: Understanding care from Leininger’s perspective implies learning about people’s living ways, their environments, their feelings, their worries, their beliefs, their practices as well as their values in different events, such as motherhood, fatherhood, birth, adolescence, adultness, elderliness, diseases and death, with the main objective of offering a care agreeable and compatible to their culture. Research and practice in transcultural nursing: This learning related to the care that persons have by themselves, necessarily indicates or suggests an interaction with them. However, an interaction requires preparation, sensitiveness and a critical thought in order to discover and to grasp all of the different meanings that people give to their lives and their own care. Reflection: This investigation has been configured as an entry door to get those points of view and as a bridge to approximate the professional nursing towards quotidian people’s lives in order to obtain knowledge from them.

El cuidado de sí de la embarazada diabética como una via para asegurar un hijo sano / Self-care of diaetic pregnant women, a way of ensuring a healthy baby

Esta investigación etnográfica focalizada exploró las creencias, los valores, los hábitos y los padrones comportamentales de las embarazadas diabéticas atendidas en el Hospital Doctor Enrique Tejera, quienes residen en tres comunidades urbanas pobres de la ciudad de Valencia - Venezuela, con el objetivo de encontrar el significado del cuidado de si para dichas grávidas. Se utilizó como referencial teórico el Modelo del Análisis de la Salud y la Teoría Antropológica de la Salud, siendo fundamentales para revelar aquello que estaba implícito en el comportamiento de las mujeres con relación a su cuidado. Surgieron tres temas: la salud y la enfermedad - valores culturales del cuidado de si; las prácticas del cuidado de la salud y cuidándose en el embarazo garantiza tener un hijo más saludable. Los temas culturales revelaron que las creencias, los valores, los hábitos y los patrones culturales comportamentales, conforme las tres maneras de acción identificadas, pueden ser preservadas, acomodadas y re-estructuradas para proporcionar un cuidado culturalmente congruente...

Razonamiento analógico verbal y no verbal en niños preescolares con trastorno específico del lenguaje / Verbal and non-verbal analogical reasoning in children with specific language impairment

Los niños con transtornos específicos del lenguaje (TEL) suelen presentar dificultades en diversos aspectos de la cognición verbal y no verbal. Una de las habilidades cognitivas poco estudiadas en estos niños es el razonamiento analógico. Incluso no ha sido estudiado en niños preescolares con TEL. Por ello, el propósito de este trabajo es estudiar algunos aspectos del razonamiento analógico verbal y no verbal en niños preescolares con TEL. Se comparó el desempeño de un grupo de niños preescolares con TEL moderado y un grupo control de niños sin TEL en diversas tareas de razonamiento analógico verbal y no verbal. El rendimiento de los niños con TEL moderado es inferior en ambos lados tipos de razonamiento. El manejo de las analogias verbales en niños con TEL moderado es inferior en tres de las cinco estudiadas. El manejo de tres de las cuatro analogias no verbales es inferior en los niños con TEL moderado. Se discuten las implicancias clínicas y se ofrecen sugerencias para el estudio del tema en el contexto de la relación-cognición

El efecto de recencia y el efecto de metría en la memoria de trabajo fonológica en niños preescolares con TEL moderado / Recency and measurement effect in the phonological memory in children with moderate specific lenguage impairment

El propósito del presente trabajo fue investigar algunos aspectos de la memoria en niños preescolares con trastorno específico del lenguaje TEL moderado. Se estudió el efecto de recencia y el efecto de latencia y su relación con la memoria de trabajo fonológica tanto en el procesamiento de palabras inventadas logotomas como en palabras de uso frecuente. En primer lugar, se investigó si el efecto de recencia, presente en sujetos sin trastornos del lenguaje, estaba conservado en niños con TEL. En segundo lugar, se investigó si el efecto de recencia era afectado por la metría cantidad de sílabas de las palabras en niños con TEL. Los resultados sugieren, en primer lugar, que el efecto de recencia se conserva en los niños con TEL tanto en logotomas como en palabras conocidas y, en segundo lugar, que el efecto de recencia es afectado por la metría en logotomas, pero no en palabras conocidas. Se discuten las implicancias para la problemática cognitiva del TEL

Importancia de la instruccion en el desarrollo de la tecnica correcta de la lactancia materna / Importance of intruction in developing the right technic of breast feeding

Este trabajo tiene como fin mejorar la calidad de la lactancia materna en primipara, a traves de la ensenanza de una tecnica correcta, practica que le proporciona beneficios tanto a la madre como al hijo. El universo, lo conformaron las madres primiparas, a las que se les llevo el control prenatal en el centro de salud no. 6 de Bogota, durante el periodo comprendido entre el 11 de marzo y el 31 de marzo de 1987. La muestra fue escogida durante el mismo periodo utilizando un muestreo no probabilistico. Para el logro de este fin se realizo un estudio practicamente descriptivo, para su desarrollo se consideraron 2 etapas a saber: la analitica y la evaluativa. Se realizo la etapa descriptiva a traves de un pretest, donde se identifica el nivel de conocimientos de las madres, sobre aspectos especificos de la lactancia materna. En la etapa analitica se analizaron los resultados obtenidos; con base en estos se elaboro el programa educativo, teniendo en cuenta los aspectos impostantes que permitieron el logro de los objetivos.La tercera etapa evalua los conocimientos adquiridos por las madres a quienes se les impartio la instruccion; se determina conclusiones y se formula recomendaciones al respecto.La metodologia utilizada consistio en la realizacion de una visita domiciliaria a cada madre, con el fin de impartir la instruccion, la cual fue evaluada por medio de un post-test al finalizar la misma que contemplaba los mismos aspectos del pre-test.Los resultados mas relevantes que se obtuvieron al finalizar el trabajo fueron:los conocimientos sobre la lactancia materna,son...