Selección de pruebas del lenguaje y análisis crítico de su aplicación en población infantil con discapacidad auditiva

The objective of this study has been to select the tests that allow us to study in the best possible way - from a formal point of view - the different aspects of the language of the child with hearing loss. Although there is not a consensus, we consider that in the case of a child with hearing loss, it is essential to analyze all aspects of the language. This will provide us a deep knowledge of their language development, which it is not homogenous in all areas. The language tests that we chose and analyzed were: PPVT-III Peabody. Test de Vocabulario en Imágenes; Evaluación fonológica del habla infantil (L. Bosch); Registro Fonológico Inducido (M. Monfort); Evaluación de la Discriminación Auditiva y Fonológica (EDAF); Escala Magallanes de Lectura y Escritura (EMLE-TALE-2000); Prueba de Lenguaje Oral de Navarra-Revisado (PLON-R); Batería de Lenguaje Objetiva y Criterial Screening-Revisado (BLOC S-R); Test de Comprensión de Estructuras Gramaticales (CEG), y el Subtest de Vocabulario del WISC-IV. This assessment is complemented by a questionnaire addressed to those who perform tests and the opinion of expert speech therapists in the practical field. In general terms, all the tests have been appropriate depending on their purpose. The tests that are corrected better are EDAF, PPVT-III Peabody and BLOC-SR. The test that is corrected worse is the phonological record of Laura Bosch. For us, the procedure of application of PPVT-III Peabody and PLON-R is ideal for children with hearing loss, presenting major difficulties the EDAF and BLOC-SR and being very difficult to implement, the phonological record of Laura Bosch and the EMLE-TALE-2000. We believe that the selected tests are enough to evaluate the development of the language in the child with hearing impairment, when we apply them correctly according to the age of development and take into account the specific modifications that we adopt in this populationEl objetivo de este trabajo consiste en seleccionar aquellas pruebas que mejor permitan examinar, desde un punto de vista formal, el lenguaje del niño con hipoacusia. Dado que el desarrollo lingüístico de las personas con discapacidad auditiva no avanza de forma homogénea en todas sus dimensiones, consideramos fundamental analizar todos los aspectos del lenguaje. Las pruebas escogidas, según criterios fijados en nuestro estudio, han sido: PPVT-III Peabody. Test de Vocabulario en Imágenes; Evaluación fonológica del habla infantil (L. Bosch); Registro Fonológico Inducido (M. Monfort); Evaluación de la Discriminación Auditiva y Fonológica (EDAF); Escala Magallanes de Lectura y Escritura (EMLE-TALE-2000); Prueba de Lenguaje Oral de Navarra-Revisado (PLON-R); Batería de Lenguaje Objetiva y Criterial Screening-Revisado (BLOC S-R); Test de Comprensión de Estructuras Gramaticales (CEG), y el Subtest de Vocabulario del WISC-IV. Esta evaluación se complementa con un cuestionario dirigido a quienes aplican los test y con la opinión de expertos logopedas en el terreno práctico. Como resultados, en general, todas las pruebas se han mostrado adecuadas según su finalidad. Las que mejor se corrigen son EDAF, PPVT-III Peabody y BLOC-SR y la que peor, la Evaluación fonológica del habla infantil de Laura Bosch. Para nosotros, el procedimiento de aplicación de PPVT-III Peabody y PLON-R es ideal para niños con hipoacusia, presentando mayores dificultades el EDAF y BLOC-SR y siendo más difíciles de aplicar, el Registro fonológico de Laura Bosch y el EMLE-TALE-2000. Consideramos que esta selección de pruebas es adecuada, siempre y cuando tengamos en cuenta la edad de desarrollo del niño con discapacidad auditiva y las modificaciones específicas que debemos adoptar en esta població

Metodología aplicada en el estudio del desarrollo del lenguaje en niños con detección temprana de la hipoacusia neonatal

Introduction and objective: Given that the disabling potential causing neonatal hearing impairment decreases with early detection and intervention, we have drawn up a research project, with which we intend to know the degree of development of the language of children and girls than from the universal hearing screening, have been diagnosed for us of prelingual hearing loss in the last 15 years and analyze the determining variables and which are modifiable. The object of this communication will be to present the methodology that we use. Method: We assume the data stored on our ENT service, including 282 children with hearing loss. We have had the opportunity to create a research group in which we agree ENT and language specialists so we have resources sufficient for the study. Results: We describe the elements that make up this project in relation to the team and to its development. After applying inclusion/exclusion criteria, we have selected a group of 45 children between 3 and 15 years, defining their auditory characteristics. Through specific tests, adapted to the age, we will study the different aspects of the language; and through a structured interview parents, we try to determine the variables that influence the rehabilitator process. Finally, the data will be analyzed statistically. Discussion: The variability and the low prevalence of infant hearing loss, make it difficult studies with sufficient population to obtain statistically significant results. However, we believe that the group of children and the methodology selected will allow us to learn more about the influential in the development of the language variables. Conclusions: The hearing screening program allowed us to earlier intervention, which should improve the levels of language of children detected with hearing loss. Although the standard development of communication depends on other factors difficult to determine, through the presented protocol we equate these findings, validating the process of screening/diagnosis and intervention of our environment.Introducción y objetivo: Dado que el potencial discapacitante que causa la deficiencia auditiva neonatal disminuye con una detección e intervención tempranas, hemos elaborado un proyecto de investigación, con el que nos proponemos conocer el grado de desarrollo del lenguaje de los niños y niñas que procedentes del cribado auditivo universal, han sido diagnosticados/as por nosotros de hipoacusia prelingual en estos últimos 15 años y analizar las variables determinantes y las que son modificables. El objeto de esta comunicación consistirá en presentar la metodología que vamos a utilizar. Método: Partimos de los datos almacenados en nuestro Servicio de ORL, que comprende a 282 niños con hipoacusia. Hemos tenido la oportunidad de crear un grupo de investigación en el que coincidimos especialistas de la audición infantil y del lenguaje, por lo que contamos con medios suficientes para el estudio. Resultados: Describimos los elementos que configuran este proyecto, en relación al equipo de trabajo y a su desarrollo. Tras aplicar unos criterios de exclusión/inclusión, hemos seleccionado a un grupo de 45 niños entre 3 y 15 años, definiendo sus características auditivas. Mediante pruebas específicas, adaptadas a la edad, estudiaremos los diferentes aspectos del lenguaje; y a través de una entrevista estructurada realizada a los padres, intentaremos determinar las variables que influyen en el proceso re-habilitador. Finalmente, los datos serán analizados estadísticamente. Discusión: La variabilidad y la escasa prevalencia de la hipoacusia infantil, dificultan la realización de estudios con población suficiente para obtener resultados estadísticamente significativos. Sin embargo, creemos que el grupo de niños seleccionado y la metodología utilizada nos permitirán conocer mejor las variables influyentes en el desarrollo del lenguaje. Conclusiones: El programa de cribado auditivo universal ha permitido una intervención más precoz, lo que debería mejorar los niveles de lenguaje de los niños detectados/as con hipoacusia. Aunque el desarrollo normalizado de la comunicación depende de otros factores difíciles de determinar, a través del protocolo presentado pretendemos equiparar estos resultados, validando el proceso de cribado/diagnóstico e intervención de nuestro medio

Assessment of plasma chitotriosidase activity, CCL18/PARC concentration and NP-C suspicion index in the diagnosis of Niemann-Pick disease type C : A prospective observational study

Niemann-Pick disease type C (NP-C) is a rare, autosomal recessive neurodegenerative disease caused by mutations in either the NPC1 or NPC2 genes. The diagnosis of NP-C remains challenging due to the non-specific, heterogeneous nature of signs/symptoms. This study assessed the utility of plasma chitotriosidase (ChT) and Chemokine (C-C motif) ligand 18 (CCL18)/pulmonary and activation-regulated chemokine (PARC) in conjunction with the NP-C suspicion index (NP-C SI) for guiding confirmatory laboratory testing in patients with suspected NP-C. In a prospective observational cohort study, incorporating a retrospective determination of NP-C SI scores, two different diagnostic approaches were applied in two separate groups of unrelated patients from 51 Spanish medical centers (n = 118 in both groups). From Jan 2010 to Apr 2012 (Period 1), patients with ≥2 clinical signs/symptoms of NP-C were considered 'suspected NP-C' cases, and NPC1/NPC2 sequencing, plasma chitotriosidase (ChT), CCL18/PARC and sphingomyelinase levels were assessed. Based on findings in Period 1, plasma ChT and CCL18/PARC, and NP-C SI prediction scores were determined in a second group of patients between May 2012 and Apr 2014 (Period 2), and NPC1 and NPC2 were sequenced only in those with elevated ChT and/or elevated CCL18/PARC and/or NP-C SI ≥70. Filipin staining and 7-ketocholesterol (7-KC) measurements were performed in all patients with NP-C gene mutations, where possible. In total across Periods 1 and 2, 10/236 (4%) patients had a confirmed diagnosis o NP-C based on gene sequencing (5/118 [4.2%] in each Period): all of these patients had two causal NPC1 mutations. Single mutant NPC1 alleles were detected in 8/236 (3%) patients, overall. Positive filipin staining results comprised three classical and five variant biochemical phenotypes. No NPC2 mutations were detected. All patients with NPC1 mutations had high ChT activity, high CCL18/PARC concentrations and/or NP-C SI scores ≥70. Plasma 7-KC was higher than control cut-off values in all patients with two NPC1 mutations, and in the majority of patients with single mutations. Family studies identified three further NP-C patients. This approach may be very useful for laboratories that do not have mass spectrometry facilities and therefore, they cannot use other NP-C biomarkers for diagnosis

Global attitudes in the management of acute appendicitis during COVID-19 pandemic: ACIE Appy Study

Background: Surgical strategies are being adapted to face the COVID-19 pandemic. Recommendations on the management of acute appendicitis have been based on expert opinion, but very little evidence is available. This study addressed that dearth with a snapshot of worldwide approaches to appendicitis. Methods: The Association of Italian Surgeons in Europe designed an online survey to assess the current attitude of surgeons globally regarding the management of patients with acute appendicitis during the pandemic. Questions were divided into baseline information, hospital organization and screening, personal protective equipment, management and surgical approach, and patient presentation before versus during the pandemic. Results: Of 744 answers, 709 (from 66 countries) were complete and were included in the analysis. Most hospitals were treating both patients with and those without COVID. There was variation in screening indications and modality used, with chest X-ray plus molecular testing (PCR) being the commonest (19\ub78 per cent). Conservative management of complicated and uncomplicated appendicitis was used by 6\ub76 and 2\ub74 per cent respectively before, but 23\ub77 and 5\ub73 per cent, during the pandemic (both P < 0\ub7001). One-third changed their approach from laparoscopic to open surgery owing to the popular (but evidence-lacking) advice from expert groups during the initial phase of the pandemic. No agreement on how to filter surgical smoke plume during laparoscopy was identified. There was an overall reduction in the number of patients admitted with appendicitis and one-third felt that patients who did present had more severe appendicitis than they usually observe. Conclusion: Conservative management of mild appendicitis has been possible during the pandemic. The fact that some surgeons switched to open appendicectomy may reflect the poor guidelines that emanated in the early phase of SARS-CoV-2

Effects of pre-operative isolation on postoperative pulmonary complications after elective surgery: an international prospective cohort study an international prospective cohort study

We aimed to determine the impact of pre-operative isolation on postoperative pulmonary complications after elective surgery during the global SARS-CoV-2 pandemic. We performed an international prospective cohort study including patients undergoing elective surgery in October 2020. Isolation was defined as the period before surgery during which patients did not leave their house or receive visitors from outside their household. The primary outcome was postoperative pulmonary complications, adjusted in multivariable models for measured confounders. Pre-defined sub-group analyses were performed for the primary outcome. A total of 96,454 patients from 114 countries were included and overall, 26,948 (27.9%) patients isolated before surgery. Postoperative pulmonary complications were recorded in 1947 (2.0%) patients of which 227 (11.7%) were associated with SARS-CoV-2 infection. Patients who isolated pre-operatively were older, had more respiratory comorbidities and were more commonly from areas of high SARS-CoV-2 incidence and high-income countries. Although the overall rates of postoperative pulmonary complications were similar in those that isolated and those that did not (2.1% vs 2.0%, respectively), isolation was associated with higher rates of postoperative pulmonary complications after adjustment (adjusted OR 1.20, 95%CI 1.05–1.36, p = 0.005). Sensitivity analyses revealed no further differences when patients were categorised by: pre-operative testing; use of COVID-19-free pathways; or community SARS-CoV-2 prevalence. The rate of postoperative pulmonary complications increased with periods of isolation longer than 3 days, with an OR (95%CI) at 4–7 days or ≥ 8 days of 1.25 (1.04–1.48), p = 0.015 and 1.31 (1.11–1.55), p = 0.001, respectively. Isolation before elective surgery might be associated with a small but clinically important increased risk of postoperative pulmonary complications. Longer periods of isolation showed no reduction in the risk of postoperative pulmonary complications. These findings have significant implications for global provision of elective surgical care. We aimed to determine the impact of pre-operative isolation on postoperative pulmonary complications after elective surgery during the global SARS-CoV-2 pandemic. We performed an international prospective cohort study including patients undergoing elective surgery in October 2020. Isolation was defined as the period before surgery during which patients did not leave their house or receive visitors from outside their household. The primary outcome was postoperative pulmonary complications, adjusted in multivariable models for measured confounders. Pre-defined sub-group analyses were performed for the primary outcome. A total of 96,454 patients from 114 countries were included and overall, 26,948 (27.9%) patients isolated before surgery. Postoperative pulmonary complications were recorded in 1947 (2.0%) patients of which 227 (11.7%) were associated with SARS-CoV-2 infection. Patients who isolated pre-operatively were older, had more respiratory comorbidities and were more commonly from areas of high SARS-CoV-2 incidence and high-income countries. Although the overall rates of postoperative pulmonary complications were similar in those that isolated and those that did not (2.1% vs 2.0%, respectively), isolation was associated with higher rates of postoperative pulmonary complications after adjustment (adjusted OR 1.20, 95%CI 1.05–1.36, p = 0.005). Sensitivity analyses revealed no further differences when patients were categorised by: pre-operative testing; use of COVID-19-free pathways; or community SARS-CoV-2 prevalence. The rate of postoperative pulmonary complications increased with periods of isolation longer than 3 days, with an OR (95%CI) at 4–7 days or ≥ 8 days of 1.25 (1.04–1.48), p = 0.015 and 1.31 (1.11–1.55), p = 0.001, respectively. Isolation before elective surgery might be associated with a small but clinically important increased risk of postoperative pulmonary complications. Longer periods of isolation showed no reduction in the risk of postoperative pulmonary complications. These findings have significant implications for global provision of elective surgical care