Hyaluronic acid-mediated epithelial-mesenchymal transition of human lens epithelial cells via CD44 and TGF-β2

ABSTRACT Purpose: The epithelial-mesenchymal transition of human lens epithelial cells plays a role in posterior capsule opacification, a fibrotic process that leads to a common type of cataract. Hyaluronic acid has been implicated in this fibrosis. Studies have investigated the role of transforming growth factor (TGF)-β2 in epithelial-mesenchymal transition. However, the role of TGF-β2 in hyaluronic acid-mediated fibrosis of lens epithelial cell remains unknown. We here examined the role of TGF-β2 in the hyaluronic acid-mediated epithelial-mesenchymal transition of lens epithelial cells. Methods: Cultured human lens epithelial cells (HLEB3) were infected with CD44-siRNA by using the Lipofectamine 3000 transfection reagent. The CCK-8 kit was used to measure cell viability, and the scratch assay was used to determine cell migration. Cell oxidative stress was analyzed in a dichloro-dihydro-fluorescein diacetate assay and by using a flow cytometer. The TGF-β2 level in HLEB3 cells was examined through immunohistochemical staining. The TGF-β2 protein level was determined through western blotting. mRNA expression levels were determined through quantitative real-time polymerase chain reaction. Results: Treatment with hyaluronic acid (1.0 μM, 24 h) increased the epithelial-mesenchymal transition of HLEB3 cells. The increase in TGF-β2 levels corresponded to an increase in CD44 levels in the culture medium. However, blocking the CD44 function significantly reduced the TGF-β2-mediated epithelial-mesenchymal transition response of HLEB3 cells. Conclusions: Our study showed that both CD44 and TGF-β2 are critical contributors to the hyaluronic acid-mediated epithelial-mesenchymal transition of lens epithelial cells, and that TGF-β2 in epithelial-mesenchymal transition is regulated by CD44. These results suggest that CD44 could be used as a target for preventing hyaluronic acid-induced posterior capsule opacification. Our findings suggest that CD44/TGF-β2 is crucial for the hyaluronic acid-induced epithelial-mesenchymal transition of lens epithelial cells.

The Role of Marein During Endothelial-Mesenchymal Transition in Diabetic Kidney Disease / Rol de la Mareína Durante la Transición Endotelial-Mesenquimatosa en la Enfermedad Renal Diabética

SUMMARY: Marein is a flavonoid compound that reduces blood glucose and lipids and has a protective effect in diabetes. However, the effect and mechanism(s) of marein on renal endothelial-mesenchymal transition in diabetic kidney disease (DKD) have not been elucidated. In this study, single-cell sequencing data on DKD were analyzed using a bioinformation method, and the data underwent reduced dimension clustering. It was found that endothelial cells could be divided into five subclusters. The developmental sequence of the subclusters was 0, 1, 4, 2, and 3, of which subcluster 3 had the most interstitial phenotype.The expression of mesenchymal marker protein:Vimentin(VIM), Fibronectin(FN1), and fibroblast growth factor receptor 1 (FGFR1) increased with the conversion of subclusters. In db/db mice aged 13-14 weeks, which develop DKD complications after 8-12 weeks of age, marein reduced blood levels of glucose, creatinine, and urea nitrogen, improved structural damage in kidney tissue, and reduced collagen deposition and the expression of FN1 and VIM. Marein also up-regulated autophagy marker:Light chain 3II/I(LC3II/I) and decreased FGFR1 expression in renal tissue. In an endothelial-mesenchymal transition model, a high glucose level induced a phenotypic change in human umbilical vein endothelial cells. Marein decreased endothelial cell migration, improved endothelial cell morphology, and decreased the expression of VIM and FN1. The use of the FGFR1 inhibitor, AZD4547, and autophagy inhibitor, 3-Methyladenine(3-MA), further demonstrated the inhibitory effect of marein on high glucose-induced endothelial-mesenchymal transition by reducing FGFR1 expression and up-regulating the autophagy marker protein, LC3II/I. In conclusion, this study suggests that marein has a protective effect on renal endothelial- mesenchymal transition in DKD, which may be mediated by inducing autophagy and down-regulating FGFR1 expression.

La mareína es un compuesto flavonoide que reduce la glucosa y los lípidos en sangre y tiene un efecto protector en la diabetes. Sin embargo, no se han dilucidado el efecto y los mecanismos de la mareína sobre la transición endotelial- mesenquimatosa renal en la enfermedad renal diabética (ERD). En este estudio, los datos de secuenciación unicelular sobre DKD se analizaron utilizando un método de bioinformación y los datos se sometieron a una agrupación de dimensiones reducidas. Se descubrió que las células endoteliales podían dividirse en cinco subgrupos. La secuencia de desarrollo de los subgrupos fue 0, 1, 4, 2 y 3, de los cuales el subgrupo 3 tenía el fenotipo más intersticial. La expresión de la proteína marcadora mesenquimatosa: vimentina (VIM), fibronectina (FN1) y receptor del factor de crecimiento de fibroblastos. 1 (FGFR1) aumentó con la conversión de subgrupos. En ratones db/db de 13 a 14 semanas de edad, que desarrollan complicaciones de DKD después de las 8 a 12 semanas de edad, la mareína redujo los niveles sanguíneos de glucosa, creatinina y nitrógeno ureico, mejoró el daño estructural en el tejido renal y redujo la deposición y expresión de colágeno de FN1 y VIM. Marein también aumentó el marcador de autofagia: Cadena ligera 3II/I (LC3II/I) y disminuyó la expresión de FGFR1 en el tejido renal. En un modelo de transición endotelial-mesenquimal, un nivel alto de glucosa indujo un cambio fenotípico en las células endoteliales de la vena umbilical humana. Marein disminuyó la migración de células endoteliales, mejoró la morfología de las células endoteliales y disminuyó la expresión de VIM y FN1. El uso del inhibidor de FGFR1, AZD4547, y del inhibidor de la autofagia, 3-metiladenina (3-MA), demostró aún más el efecto inhibidor de la mareína en la transición endotelial-mesenquimal inducida por niveles altos de glucosa al reducir la expresión de FGFR1 y regular positivamente la proteína marcadora de autofagia. , LC3II/I. En conclusión, este estudio sugiere que la mareína tiene un efecto protector sobre la transición endotelial-mesenquimatosa renal en la ERC, que puede estar mediada por la inducción de autofagia y la regulación negativa de la expresión de FGFR1.

Experiencia en la transición de adolescentes con enfermedad neuromuscular en el Hospital Garrahan / Experience in the transition of adolescents with neuromuscular disease at Garrahan Hospital

El desarrollo de nuevos abordajes terapéuticos ha generado un aumento en la esperanza de vida de los pacientes con enfermedades neuromusculares (ENM). Se trata de un grupo de enfermedades heterogéneas desde la clínica y los posibles tratamientos. La transición en los pacientes con ENM, implica un gran desafío por presentar niveles intelectuales dentro de rangos promedio, compromisos motor, respiratorio y cardiológico progresivos que resulta en aumento de la dependencia física conforme aumenta la necesidad de autonomía emocional del adolescente. La descripción de transiciones exitosas en ENM incluye intervenciones psicosociales individuales o grupales con un enfoque multidimensional e interdisciplinario, que contemple la participación de la familia para reducir la ansiedad y la preocupación sobre sus hijos. En el Hospital Garrahan los pacientes con ENM son atendidos dentro del Programa de Atención, Docencia e Investigación de Pacientes con Enfermedad Neuromuscular desde 2008. En este trabajo nos proponemos describir la experiencia en transición pre y post pandemia, de los adolescentes con ENM en seguimiento en el Hospital de Pediatría Garrahan (AU)

The development of new care and therapeutic approaches has generated an increase in the life expectancy of patients with neuromuscular diseases (NMD), a group of heterogeneous diseases from a clinical point of view. The transition in patients with MND involves a great challenge due to progressive motor, respiratory and cardiological compromises that result in an increase in physical dependence as the adolescent needs emotional autonomy. The description of successful transitions in patients with MND includes individual and psychosocial interventions with a multidimensional and interdisciplinary approach with family participation. Since 2008, we developed a Care, Teaching and Research Program for Patients with NMD Disease at the Garrahan Hospital. The objective of this work is to describe the pre- and post-pandemic transition experience of adolescents with NMD follow-up in our hospital (AU)

Transición de adolescentes en el Hospital Garrahan: 24 años de recorrido / Transition of adolescents at Garrahan Hospital: 24 years of experience

Este escrito presenta un recorrido retrospectivo de la transición de pacientes del Hospital de Pediatría "Dr. Juan P. Garrahan", centro de referencia nacional, a centros asistenciales de adultos. Se describen sus antecedentes y se destacan dos hitos que consideramos centrales como inicio de una trayectoria hospitalaria en transición de ya 24 años: la conformación de la Unidad de Adolescencia y Transición y la creación del Comité de Transición. A continuación se describen acciones que se desarrollaron en forma transversal en toda la institución, entre ellas programas de investigación, becas de formación, realización de jornadas, publicaciones institucionales y producciones artísticas. Finalmente planteamos un horizonte para este recorrido y los desafíos para la proyección futura de la transición de pacientes (AU)

This paper presents a retrospective review of the transition of patients from the Hospital de Pediatría "Dr. Juan P. Garrahan," a national reference center, to adult care centers. It describes the background and highlights two milestones that we consider central to the beginning of a 24-year hospital transition trajectory: the creation of the Adolescence and Transition Unit and the establishment of the Transition Committee. We then describe actions developed transversally throughout the institution, including research programs, training fellowships, conferences, institutional publications, and artistic productions. Finally, we propose a vision for this journey and discuss the challenges for the future projection of patient transition (AU)

Transición y transferencia del adolescente con cardiopatía congénita / Transition and transfer of the adolescent with congenital heart disease

En las últimas décadas los avances médicos han permitido la sobrevida de los niños con cardiopatías congénitas hacia la adolescencia y adultez. Un número sustancial de pacientes tienen lesiones persistentes o residuales que requerirán asistencia durante toda la vida. El manejo exitoso durante el proceso de transición en pacientes con cardiopatía congénita (CC) requiere un enfoque integral y colaborativo. Es fundamental desarrollar un programa de transición planificado que incorpore educación y autocontrol. La educación continua y la participación activa de pacientes y familias son esenciales. La formación de especialistas y la creación de unidades de atención de Adolescentes y Adultos con cardiopatía congénita (AACC) garantizarán una mayor supervivencia y calidad de vida en esta creciente población de pacientes en Argentina. La investigación continua y la implementación de mejores prácticas, con el apoyo de políticas sanitarias, son clave para abordar los desafíos y controversias en la gestión de la transición y transferencia (AU)

In recent decades, medical advances have allowed children with congenital heart disease (CHD) to survive into adolescence and adulthood. A substantial number of these patients have persistent or residual lesions that require lifelong care. Successful management during the transition process for patients with CHD requires a comprehensive and collaborative approach. It is critical to develop a planned transition program that incorporates education and self-management. Continuing education and the active participation of patients and families are essential. The training of specialists and the creation of Adolescent and Adult Congenital Heart Disease (AACHD) care units will ensure greater survival and quality of life for this growing patient population in Argentina. Continued research and implementation of best practices, supported by health policies, are key to addressing the challenges and controversies in transition and transfer management (AU)

Transición de jóvenes con trastornos del neurodesarrollo / The transition of youth with neurodevelopmental disorders

La transición de la atención pediátrica a la de adultos, en adolescentes y jóvenes con condiciones crónicas de salud, es un proceso necesario y difícil. El proceso de transición de estos pacientes contará con diferentes matices según la condición de salud, las características del sistema sanitario, los equipos de salud involucrados y los factores contextuales del joven y su familia. El objetivo central es que a través de una autonomía progresiva el joven llegue a ser un adulto competente en cuanto a asumir y manejar sus necesidades de salud, relaciones sociales y laborales que su enfermedad crónica le permita. Pero estos objetivos se ven interferidos cuando hablamos de jóvenes con condiciones crónicas que involucran el neurodesarrollo y es más complejo aún cuando nos referimos a jóvenes que no serán autónomos. Para las familias y cuidadores de estos jóvenes los centros pediátricos son un entorno más protegido que cuesta dejar. Por todo esto es que, si bien hay lineamientos generales a tener en cuenta, cada equipo debe revisar sus resultados para poder avanzar. Aún no se logró consenso acerca de cuál es el momento óptimo, la edad y las mejores estrategias, aunque es reconocida la dificultad para implementar un programa adecuado y más aún evaluar los resultados de los programas que se utilizan en la transición; se llevan adelante programas "genéricos" utilizados para diferentes enfermedades crónicas, o específicos para una enfermedad, otros programas combinan ambas estrategias. Basados en lo anterior hay jóvenes y familias que requieren un armado "artesanal y a medida" (AU)

The transition from pediatric to adult care for adolescents and young people with chronic health conditions is a necessary yet challenging process. This transition will vary depending on the health condition, the characteristics of the health system, the health teams involved, and the contextual factors of the youth and their family. The central objective is for the youth to achieve progressive autonomy, becoming a competent adult capable of managing their health needs, social, and work relationships, as allowed by their chronic disease. However, these objectives become more complicated when dealing with young people with chronic conditions involving neurodevelopment, and even more so for those who will never achieve autonomy. For the families and caregivers of these youths, pediatric centers offer a more protected environment that is difficult to leave. For all these reasons, while general guidelines should be considered, each team must review its results to move forward. Consensus has not yet been reached on the optimal time, age, and best strategies for transition. It is recognized that implementing an adequate program is challenging, and evaluating the results of these programs is even more difficult. There are "generic" programs used for various chronic diseases, as well as disease-specific programs, and some programs combine both strategies. Given these complexities, some young people and their families require a "handcrafted and tailor-made" approach (AU)

El proceso de transición desde la perspectiva del aprendizaje / The transition process from a learning perspective

El avance científico y tecnológico ha incrementado la supervivencia de niños, niñas y adolescentes (NNyA) con Enfermedad Crónica (EC). Ello ha generado un aumento significativo de la población adolescente con cuidados especiales de salud dentro del ámbito pediátrico. Esta situación da relevancia a la necesidad de institucionalizar líneas de acción específicas para acompañar el proceso de transición de estos pacientes hacia la atención de adultos. Dentro del abordaje interdisciplinario, cada especialidad define sus intervenciones. Partiendo de la perspectiva multidimensional del aprendizaje y del concepto de Alfabetización en Salud (AS), el equipo de psicopedagogía participa de la transición de grupos de pacientes a los que asiste en clínicas específicas. El objetivo central de esta participación es favorecer en cada consultante el mayor nivel de autonomía posible, considerando sus recursos cognitivos, educativos, familiares y contextuales. Las líneas de intervención, se dirigen a promover el manejo de información sobre la propia condición de salud y el aprendizaje de habilidades relacionadas con la autogestión de su enfermedad. Este escenario busca promover una transición efectiva involucrando no sólo al paciente y su familia sino que además al equipo de salud en dicho proceso (AU)

Scientific and technological progress has increased the survival of children and adolescents with Chronic Diseases (CD). This has led to a significant increase in the adolescent population with special healthcare needs in the pediatric setting. Consequently, there is a pressing need to institutionalize specific lines of action to facilitate the transition of these patients to adult care. Within an interdisciplinary approach, each specialty defines its interventions. The educational therapy team participates in the transition of patient groups in specific clinics, based on the multidimensional perspective of learning and the concept of health literacy (HL). The main objective of this participation is to promote the highest possible level of autonomy in each patient, considering their cognitive, educational, family, and contextual resources. The interventions aim to enhance the management of information about their health condition and the learning of skills related to self-management of their disease. This approach seeks to promote an effective transition involving not only the patient and their family but also the health team in this process (AU)

Red de formación y de cuidados para la transición de adolescentes con inmunodeficiencias primarias / Care and training network for the transition of adolescents with primary immunodeficiencies

En este escrito nos proponemos relatar y sistematizar los aspectos centrales de la estrategia de gestión, formación y atención diseñada por el Servicio de Inmunología y Psicología Institucional del Hospital Garrahan junto a los Hospitales Durand y Posadas para la transición de adolescentes con inmunodeficiencias primarias a la atención de adultos. La formalización de las primeras transiciones comenzó en 2007 con el Hospital Durand y progresivamente se fueron complejizando y expandiendo los procesos y actores participantes, sumándose el Hospital Posadas y otros centros de atención que reciben un porcentaje menor de pacientes. El eje central de la estrategia fue la implementación de un sistema de rotaciones para los residentes del último año de la especialidad de adultos por el servicio de pediatría. La formalización de la estrategia también readecuó de manera gradual aspectos internos de la atención de los adolescentes en el hospital pediátrico. En una etapa posterior se implementaron encuentros por videoconferencia con centros de adultos para redefinir acuerdos entre los servicios. Además, la asociación civil de pacientes (Asociación de Ayuda al Paciente con Inmunodeficiencias Primarias - AAPIDP) cumplió un rol relevante desde los primeros años de la estrategia. Estas acciones propiciaron la creación de una red de formación y cuidados en inmunodeficiencias primarias para la transición (AU)

In this article, we aim to describe and systematize the central aspects of the management, training, and care strategy designed by the Departments of Immunology and Institutional Psychology of the Garrahan Hospital, in collaboration with the Durand and Posadas Hospitals, for the transition of adolescents with primary immunodeficiencies to adult care. The first transitions were formalized in 2007 with the Durand Hospital. Over time, the processes and actors involved have become more complex and expanded, incorporating the Posadas Hospital and other care centers that receive a smaller percentage of patients. The central axis of the strategy was the implementation of a rotation system for residents in their final year of the adult specialty in the Department of Pediatrics. The formalization of the strategy also led to gradual readjustments in the internal aspects of adolescent care within the pediatric hospital. In a later stage, videoconference meetings with adult centers were implemented to redefine agreements between departments. Additionally, the patient association (Asociación de Ayuda al Paciente con Inmunodeficiencias Primarias - AAPIDP) has played a significant role since the early years of the strategy. These actions have led to the creation of a network for training and care in primary immunodeficiencies for the transition (AU)

Adolescencia, adherencia y transición: Experiencia de un centro de trasplante renal pediátrico / Adolescence, adherence, and transition: Experience from a pediatric renal transplant center

Con el advenimiento de nuevas técnicas quirúrgicas y medicaciones inmunosupresoras la sobrevida de los niños trasplantados mejoró, llegando a la adultez. La continuidad de su tratamiento requiere un proceso planificado que permita su tránsito a un sistema de salud orientado al adulto. El objeto de este trabajo es mostrar la transición a centros de adultos en una cohorte de pacientes trasplantados renales en el Hospital Garrahan, describir sus características clínicas y demográficas, su evolución, y oportunidades de mejora implementadas. Debido a cambios médicos y su abordaje desde la interdisciplina, se dividió a la población en tres periodos: era 1 (1988-1999), era 2 (2000-2009), y era 3 (2010- 2023). En la era 1, 179 adolescentes continuaron su atención médica en un centro de adultos, 212 en la era 2 y 201 en la era 3. En la era 1 el seguimiento estaba coordinado por el nefrólogo de cabecera y eran consultados los servicios de Urología, Servicio Social y Salud Mental. En la era 2, se fortaleció el trabajo en interdisciplina y aún más a partir del 2011. Surgieron centros de trasplante de adultos que recibían adolescentes y médicos dedicados a ellos en forma preferencial. En la actualidad la transición comienza a los 12 años y progresa hasta los 18. El modelo implementado es la transición directa, entre el nefrólogo pediatra y el de adultos, con varias consultas secuenciales en ambos centros. Si bien la sobrevida del paciente e injerto mejoraron, el rechazo, asociado a no adherencia, es una asignatura por mejorar (AU)

With the advent of new surgical techniques and immunosuppressive medications, the survival of transplanted children has improved, allowing them to reach adulthood. The continuity of their treatment requires a planned process that facilitates their transition to an adult-oriented healthcare system. The aim of this study was to examine the transition to adult centers in a cohort of renal transplant patients at Garrahan Hospital, describing their clinical and demographic characteristics, their evolution, and the improvement opportunities implemented. Based on medical changes and the interdisciplinary approach, the population was divided into three periods: era 1 (1988- 1999), era 2 (2000-2009), and era 3 (2010-2023). In era 1, 179 adolescents continued their medical care in an adult center, 212 in era 2, and 201 in era 3. In era 1, follow-up was coordinated by the attending nephrologist with consultations from Urology, Social Services, and Mental Health Services. In era 2, interdisciplinary work was strengthened, and even more so since 2011. Adult transplant centers were created to receive adolescents with physicians dedicated to their care on a preferential basis. Currently, the transition begins at 12 years of age and progresses up to 18. The implemented model involves direct transition between the pediatric nephrologist and the adult nephrologist, with several sequential consultations in both centers. Although patient and graft survival have improved, rejection associated with non-adherence remains an area for improvement

Características clínicas, virológicas, inmunológicas y psicosociales de los adolescentes con infección por HIV: 15 años después de la implementación del programa de transición / Clinical, virological, immunological, and psychosocial characteristics of adolescents with HIV infection: 15 years after the implementation of the transition program

Desde 2007, el Servicio de Epidemiología e Infectología, ha implementado un programa de transición que busca optimizar la atención de los adolescentes con infección por el HIV durante el paso de la atención pediátrica a la de adultos. Objetivo: Describir las características clínicas, epidemiológicas, virológicas y psicosociales de los adolescentes con infección HIV atendidos en el Programa y analizar el proceso de transición. Materiales y Métodos: Estudio de cohorte retrospectivo. Se incluyeron a los adolescentes, atendidos en el Programa de Transición entre enero de 2019 y diciembre de 2023, en el Hospital Garrahan, con al menos un resultado de CV y CD4+ en ese período. Se obtuvo la información de la historia clínica electrónica y se analizaron variables clínicas, epidemiológicas, virológicas, terapéuticas y psicosociales. Resultados: Se incluyeron 124 pacientes. La vía de transmisión fue vertical en el 92,74% y el estadio clínico e inmunológico era avanzado. En el momento de la transición 77,4% se encontraban con supresión virológica y con recuperación inmunológica. El 55,6% ya realizó la transición a un centro de adultos, 31,4% continúan en el programa, 11,3% se perdieron en el seguimiento y 1,7% fallecieron. Se recopilaron los datos de 31 pacientes transferidos. La mediana de seguimiento fue de 2 años; 25 pacientes (80,6%) continúan en seguimiento. Conclusiones: A pesar de la pandemia de COVID-19, el programa logró la retención de los adolescentes con infección por HIV y una transferencia sostenida en el tiempo. Además de un programa de transición estructurado para garantizar una atención continua y de calidad, es necesario continuar evaluando la evolución postransición (AU)

Since 2007, the Epidemiology and Infectious Diseases Department has implemented a transition program to optimize the care of adolescents with HIV infection during their transition from pediatric to adult care. Objective: To describe the clinical, epidemiological, virological, and psychosocial characteristics of adolescents with HIV infection treated in the program and to analyze the transition process. Materials and Methods: A retrospective cohort study was conducted. Adolescents followed in the Transition Program at Garrahan Hospital between January 2019 and December 2023, with at least one viral load and CD4+ result during that period, were included. Information was obtained from electronic medical records, and clinical, epidemiological, virological, therapeutic, and psychosocial variables were analyzed. Results: A total of 124 patients were included. The route of transmission was vertical in 92.74%, and the clinical and immunologic stage was advanced. At the time of transition, 77.4% were virologically suppressed and had achieved immunologic recovery. Of the patients, 55.6% had already transitioned to an adult center, 31.4% were still in the program, 11.3% were lost to follow-up, and 1.7% died. Data were collected from 31 transferred patients, with a median follow-up of 2 years; 25 patients (80.6%) remain in follow-up. Conclusions: Despite the COVID-19 pandemic, the program successfully retained HIVinfected adolescents and ensured sustained transition over time. In addition to a structured transition program to ensure continuous and quality care, it is necessary to continue evaluating post-transition outcomes (AU)

Transición en enfermedades inflamatorias intestinales: de la pediatría a la adultez / Transition in inflammatory bowel diseases: from childhood to adulthood

El 25% de los pacientes con Enfermedades Inflamatorias Intestinales (EII) se diagnostican antes de los 20 años. En la mayor parte de los centros del país se lleva a cabo la "transferencia" del paciente desde un centro de atención pediátrico a uno de adultos. La "transición" es un criterio de calidad con beneficios en el control de la EII reduciendo el número de recaídas, de hospitalizaciones y de cirugías. Por tal motivo hemos desarrollado un Programa Interdisciplinario de Transición entre dos hospitales de referencia nacional e internacional en EII. Materiales y métodos: Entre 1/2021 y 12/ 2022 se incorporaron 24 pacientes que ingresaron en 3 fases: Fase 1 Pacientes entre 14 y 16 años asistidos en el Hospital Garrahan (HG) con un abordaje interdisciplinario. Fase 2. A partir de los 17 años se realizaron 2 (dos) encuentros en el HG en conjunto con gastroenterólogos de adultos evaluando adherencia y autonomía y la Fase 3 llevada a cabo en el Hospital B. Udaondo (HBU) sólo con el equipo de adultos luego de 6 meses de realizada la transferencia evaluando adherencia al tratamiento, consultas a emergencias, internación y/o cirugías Resultados: Al inicio del Programa el 66% de los pacientes presentaban una actividad moderada a severa vs el 8% al finalizar la fase 3. Luego de la transferencia el 12,5% necesito ingreso a guardia e internación y un 8% tratamiento quirúrgico. El 83% de los pacientes continúan en seguimiento luego de 6 meses de haber sido transferidos (AU)

Twenty-five percent of patients with inflammatory bowel diseases (IBD) are diagnosed before the age of 20 years. In most centers in the country, the "transfer" of the patient from a pediatric to an adult care center is done. However, "transition" is a quality criterion with benefits in the control of IBD by reducing the number of relapses, hospitalizations, and surgeries. For this reason, we developed an Interdisciplinary Transition Program between two national and international reference hospitals in IBD. Materials and Methods: Between January 2021 and December 2022, we incorporated 24 patients into a three-phase program. Phase 1 involved patients between 14 and 16 years of age seen at Garrahan Hospital (HG) with an interdisciplinary approach. Phase 2 began from 17 years of age, with two meetings held at HG involving adult gastroenterologists to evaluate adherence and autonomy. Phase 3 was conducted at Hospital B. Udaondo (HBU) only with the adult team, six months after the transfer, evaluating adherence to treatment, emergency consultations, hospitalizations, and/or surgeries. Results: At the beginning of the program, 66% of the patients presented with moderate to severe disease activity, compared to 8% at the end of Phase 3. After the transfer, 12.5% of the patients required emergency department visits and hospitalization, and 8% required surgical treatment. Eighty-three percent of the patients continue in the program and are still being followed up six months after the transfer (AU)

Rol de la figura de enlace en el modelo e transición en endocrinología. Experiencia en el ámbito público / Role of the liaison person in the transition model in Endocrinology. Experience in the public setting

Un proceso de transición planificado entre los sistemas de salud pediátricos y de adultos es necesario para poder garantizar una continuidad en la atención de los adolescentes. El objetivo del trabajo fue evaluar la población de pacientes del Servicio de Endocrinología del Hospital Garrahan en fase de transición y sus familias, desarrollar un protocolo para la transición de los adolescentes con patología endocrinológica crónica al Hospital de Clínicas José de San Martin y evaluar el rol de la "figura de enlace" en este proceso. Materiales y Métodos: Estudio observacional, transversal/prospectivo. Se obtuvieron datos sobre la consulta ambulatoria de 72 adolescentes mayores a 15 años con patología endocrinológica a los cuales se los acompañó en el proceso de transición. Se realizaron entrevistas y encuestas a los adolescentes, sus familias y a 16 endocrinólogos intervinientes en el seguimiento (9 pediátricos- 7 adultos). Resultados: La mayoría de los adolescentes evidenciaron falta de autonomía general, con mayor afectación en el área de "seguimiento de los problemas de salud". Esto, junto al paternalismo del pediatra y la sobreprotección familiar representaron inconvenientes para la transición. La mitad de los adultos entrevistados consideraron falta de autonomía o preparación en sus hijos considerando la edad ideal para la transición entre los 18-21 años. Las sensaciones referidas por los pacientes como sus acompañantes incluyen principalmente el miedo y ansiedad, y llamativamente en los pacientes la vergüenza. La creación de un consultorio de transición en el centro de adultos y el acompañamiento de la "figura de enlace", permitieron una mejor articulación y continuidad en el cuidado de la salud (AU)

TA planned transition process between pediatric and adult health systems is necessary to ensure continuity of care for adolescents. The aim of this study was to evaluate the patient population of the Endocrinology Service at Garrahan Hospital during the transition phase, along with their families, to develop a protocol for transitioning adolescents with chronic endocrinological disorders to Hospital de Clínicas José de San Martín, and to evaluate the role of the "liaison person" in this process. Materials and Methods: This observational, cross-sectional/ prospective study obtained data from outpatient consultations of 72 adolescents over 15 years of age with endocrinological disorders who were accompanied during the transition process. Interviews and surveys were conducted with the adolescents, their families, and 16 endocrinologists involved in the follow-up (9 pediatricians and 7 adult physicians). Results: Most of the adolescents showed a general lack of autonomy, with greater challenges in the area of "follow-up of health problems." This, combined with the paternalism of the pediatrician and the overprotection of the family, represented obstacles to the transition. Half of the parents interviewed perceived a lack of autonomy or preparation in their children, considering the ideal age for transition to be between 18-21 years old. The primary feelings reported by the patients and those who accompanied them included fear and anxiety, with patients also feeling embarrassment. The creation of a transition clinic in the adult center and the support of a "liaison person" allowed for better coordination and continuity in health care (AU)

Transición a la medicina del adulto en sobrevivientes de un trasplante alogénico de células progenitoras hematopoyéticas / Transition to adult medicine in survivors of allogeneic hematopoietic stem cell transplantation

Los sobrevivientes de un trasplante alogénico de células progenitoras hematopoyéticas (TACPH) pediátrico presentan alto riesgo de padecer problemas de salud. Debido a esta vulnerabilidad, la continuidad del cuidado impacta en su pronóstico y la transición a la medicina del adulto (TMA) es un proceso clave. Objetivo: Evaluar el proceso actual de TMA de los receptores de TACPH en nuestro hospital. Métodos: Diseño: observacional retrospectivo y prospectivo. Población: todos los pacientes (p) que realizaron su TMA desde enero/2022 a marzo/2023. Instrumentos: entrevista personal; material escrito; resumen de historia clínica; escalas TRAQ 5.0 (transición), PedsQL 4.0 (CVRS) y Lansky (funcionalidad); elección de estrategias de seguimiento según complejidad y requerimientos; contacto con profesionales de adultos; entrevista telefónica luego de 6 meses posTMA; red conformada. Resultados: 36p completaron la TAM (33 presencial, 3 virtual). Edad m19 años (m6 años de seguimiento), 70% del interior del país, 58% TACPH por enfermedad maligna, 64% TACPH familiar. A la TMA: antecedente EICHc 50%, segunda enfermedad maligna 2%, compromiso órganos 75% (m2/p, r0-8, mayormente endocrinológicas, oculares y neurológicas), 94% Lansky ≥80 (r50-100), PedsQL m82 (27% ≤75), TRAQ m3.4 (r1.7- 4.8). Derivación: todos los p cubrían sus necesidades (30% en centros de alta complejidad o expertos en THA) pero 3p debieron readecuar las estrategias, 5p presentaban complicaciones en actividad o necesidad de pronta resolución. Contacto posterior: 30/33p continuaban seguimiento, 3p pudieron retomarlo, 9p nuevas complicaciones/tratamientos. Red: 20 profesionales/instituciones. Conclusiones: Se refuerza la necesidad y utilidad de un proceso de TMA tanto formal como personalizado según necesidades individuales de los pacientes con TACPH (AU)

Pediatric allogeneic hematopoietic stem cell transplant (HSCT) survivors are at high risk for health problems. Because of this vulnerability, continuity of care impacts their prognosis and transition to adult medicine (TAM) is a key process. Objective: To evaluate the current process of TAM of HSCT recipients in our hospital. Methods: A retrospective and prospective observational study was conducted. The population included all patients (p) who underwent TAM from January 2022 to March 2023. Instruments used included personal interviews, written materials, medical history summaries, the TRAQ 5.0 (transition), PedsQL 4.0 (HRQoL), and Lansky (functionality) scales. Follow-up strategies were chosen according to complexity and requirements, with contact established with adult professionals and a telephone interview conducted six months post-TAM in an established network network. Results: 36p completed TAM (33 face-to-face, 3 online). Mean age was 19 years (with a mean of 6 years of follow-up); 70% were from the provinces of the country, 58% underwent HSCT due to malignant disease, 64% had familial HSCT. At TAM: 50% had a history of GVHD, 2% had a second malignant disease, and 75% had organ involvement (mean of 2 per patient, ranging from 0 to 8, mostly endocrinological, ocular, and neurological), 94% had Lansky ≥80 (range, 50-100), mean PedsQL was 82 (27% ≤75), mean TRAQ was 3.4 (range, 1.7-4.8). Referral needs were met for all patients (30% in tertiary-level centers or with experts in allogeneic HSCT), although 3 patients had to readjust strategies, and 5 had complications requiring prompt resolution. In subsequent contact, 30 out of 33 patients continued follow-up, 3 resumed it, and 9 experienced new complications or treatments. The network included 20 healthcare providers/institutions. Conclusions: This study reinforces the need for and usefulness of a formal and personalized TAM process according to the individual needs of patients with HSCT (AU)

Experiencia de la transición en un Servicio de Adolescencia / Transition experience in an Adolescence Unit

Se define transición como el proceso de preparación, adaptación e integración paulatina por el cual un paciente joven con una patología crónica desarrolla las habilidades y dispone de los recursos Se aborda la experiencia y estadística de la Unidad de Adolescencia y Transición durante el 2022. Materiales y métodos: estudio descriptivo, observacional, retrospectivo y de corte transversal. Se incluyeron en el estudio los adolescentes de entre 17 y 21 años atendidos en el servicio de adolescencia del Hospital J.P Garrahan durante el periodo de 1 enero 2022 al 31 de diciembre de 2022. Resultados: se incluyeron 179 pacientes (111 eran mujeres, 68 varones, incluido un trans varón). La mediana de edad de derivación al servicio de adolescencia fue de 17,26 años. Provenían del Gran Buenos Aires el 74% de los pacientes. Se logró la transición del 62.2% (112 pacientes). El principal centro de derivación fue el Hospital de Clínicas con el 21.3% (24 pacientes) Conclusión: Aunque los resultados mostrados son favorables, es necesaria una correcta valoración y evaluación inicial del paciente, con una planificación pertinente del proceso y con la incorporación de un equipo de transición o unidad transicional (AU)

Transition is defined as the process of preparation, adaptation, and gradual integration by which a young pa - tient with a chronic condition develops the skills and resources needed for adult care. Materials and Methods: This was a descriptive, observational, retrospective, and cross-sectional study. Adolescents between 17 and 21 years of age seen at the Adolescence Unit of the J.P Garrahan Hospital from January 1, 2022, to December 31, 2022, were included in the study. Results: A total of 179 patients were included (111 female, 68 male, including one trans male). The median age of referral to the Adolescence Unit was 17.26 years. Seventy-four percent of the patients came from the Greater Buenos Aires area. Transition was achieved in 62.2% (112 patients). The main referral center was the Hospital de Clínicas, with 21.3% (24 patients). Conclusion: Although the results are favorable, a correct initial evaluation and assessment of the patient is necessary, along with relevant planning of the process and the incorporation of a transition team or transitional unit (AU)

Transición del paciente adolescente con mielomeningocele en su paso al hospital de adultos / Transition of the adolescent patient with myelomeningocele to adult hospital care

El acceso a una salud integral de calidad a lo largo de la vida es un derecho de todos los adolescentes y jóvenes; pero sobre todo resulta relevante en aquellos pacientes con enfermedades complejas crónicas como el mielomeningocele pues de no realizarse tiene repercusión notoria sobre su estado de salud con mayor riesgo de morbimortalidad. En este artículo se comenta la experiencia sobre transición de pacientes con mielomeningocele que se realizó en forma organizada entre los servicios de Consultorio de Mielomeningocele y Hospital de día Polivalente del Hospital Garrahan y el Servicio de Adolescencia del Hospital Ramos Mejía. Se trata de un nuevo modelo asistencial de trabajo interdisciplinario y colaborativo teniendo como eje una fluida comunicación interinstitucional. Este acuerdo formal entre ambos hospitales contó con el recurso humano especializado y la estructura física adecuada para el abordaje integral de esta compleja enfermedad crónica. Este programa propició un entorno al paciente que aseguró el seguimiento por equipos interdisciplinarios. Esta es la mejor opción para brindar un cuidado integral, equitativo, coordinado y accesible mejorando la calidad de vida de los pacientes con mielomeningocele a largo plazo (AU)

Access to quality comprehensive health throughout life is a right of all adolescents and young people; but above all it is relevant in those patients with complex chronic diseases such as myelomeningocele because if it is not performed it has a notable impact on their health status with a greater risk of morbidity and mortality. This article discusses the transition experience of patients with myelomeningocele that was carried out in an organized manner between the Myelomeningocele Clinic and Multipurpose Day Hospital services of the Garrahan Hospital and the Adolescence Service of the Ramos Mejía Hospital. It is a new care model of interdisciplinary and collaborative work with fluid inter-institutional communication as its axis. This formal agreement between both hospitals had specialized human resources and the appropriate physical structure for the comprehensive approach to this complex chronic disease. This program provided an environment for the patient that ensured follow-up by interdisciplinary teams. This is the best option to provide comprehensive, equitable, coordinated and accessible care, improving the quality of life of patients with myelomeningocele in the long term (AU)

PKMYT1 Promotes Epithelial-Mesenchymal Transition Process in Triple-Negative Breast Cancer by Activating Notch Signaling

ABSTRACT Background: Triple-negative breast cancer (TNBC) is a subtype of breast cancer (BC) that lacks receptors for targeted therapy. Deeper insight into the molecular mechanisms regulating TNBC metastasis is urgently needed. The epithelial-mesenchymal transition process facilitates the metastasis of neighboring epithelial tumor cells. Protein kinase, membrane-associated tyrosine/threonine 1 (PKMYT1), a member of the Wee family of protein kinases, is upregulated in BC, and its high expression predicts poor prognosis in BC patients. Notch signaling activation is a pathognomonic feature of TNBC. PKMYT1 has been found to induce EMT in non-small cell lung cancer by activating Notch signaling. However, whether PKMYT1 exerts effects on TNBC progression by regulating Notch signaling remains unknown. Objectives: The objective of this study was to investigate whether PKMYT1 exerts effects on TNBC progression by regulating Notch signaling. Methods: Fifty cases of surgically resected BC samples (tumor and adjacent non-tumor tissue samples) were collected from patients diagnosed with BC. We measured the expression of PKMYT1 in clinical samples with real-time quantitative polymerase chain reaction (RT-qPCR). For in vitro analysis, RT-qPCR and Western blotting were conducted to evaluate PKMYT1 expression in TNBC cells. Then, the viability, migration, and invasion of TNBC cells were detected by cell counting kit-8 assays, wound healing assays, and Transwell assays. The EMT event was examined by evaluating the levels of EMT-associated proteins. For in vivo analysis, xenograft models in nude mice were established to explore PKMYT1 roles. E-cadherin and Ki67 expression in xenograft models were estimated by immunohistochemistry staining. Hematoxylin and eosin staining was performed to assess tumor metastasis. The underlying mechanisms by which PKMYT1 affected the malignant phenotypes of TNBC cells were explored by Western blotting measuring the pathway-associated proteins. Results: PKMYT1 was upregulated in BC tissues and cells, and its knockdown prevented cell proliferation, migration, invasion, and EMT event in TNBC. Mechanistically, Notch signaling was inactivated by PKMYT1 depletion, and Notch activation abolished the PKMYT1 silencing-induced inhibition in the malignant phenotypes of TNBC cells. For in vivo analysis, PKMYT1 knockdown inhibited tumorigenesis and metastasis of TNBC. Conclusion: PKMYT1 promotes EMT, proliferation, migration, and invasion of TNBC cells and facilitates tumor growth and metastasis by activating Notch signaling.

GSK3B inhibition reduced cervical cancer cell proliferation and migration by modulating the PI3K/Akt signaling pathway and epithelial-to-mesenchymal transition

Previous studies show that glycogen synthase kinase 3β (GSK3B) plays an important role in tumorigenesis. However, its role in cervical cancer is unclear. The present study silenced GSK3B with siRNAs and/or chemical inhibitors to determine its role in HeLa cervical cancer cell proliferation and migration as well as in xenograft tumor growth. Cell Counting Kit (CCK)-8 and 5-ethynyl-2'-deoxyuridine (EdU) assays were used to determine cell survival and proliferation. Scratch and Transwell® assays were used to evaluate cell migration. Xenograft tumors were used to evaluate the effect of GSK3B on tumor growth. Transcriptomic sequencing was used to clarify the mechanisms underlying the foregoing processes. Public databases and clinical specimens showed that GSK3B was upregulated in cervical cancer tissues and correlated with poor prognosis. In vitro experiments indicated that GSK3B inhibition reduced cell viability, proliferation, and migration. In vivo experiments demonstrated that GSK3B inhibition slowed xenograft tumor growth. Transcriptomic sequencing revealed that GSK3B inhibition modulated the phosphatidylinositol 3-carboxykinase (PI3K)/protein kinase B (Akt) and extracellular matrix (ECM)-receptor interaction signaling pathways. GSK3B inhibition decreased the protein levels of phosphorylated PI3K and Akt and the levels of mesenchymal markers but increased those of epithelial markers. An activator of the PI3K/Akt signaling pathway counteracted the suppressive effects of GSK3B inhibition on HeLa cell viability and proliferation and on PI3K/Akt signaling. Our data suggested that GSK3B regulated cervical cancer cell proliferation and migration by modulating the PI3K/Akt signaling pathway and epithelial-to-mesenchymal transition (EMT).

Tecnologia educacional para cuidadores de pessoas idosas com limitações pós-acidente vascular cerebral / Educational technology for caregivers of older people with post-stroke limitations

Resumo: O Acidente Vascular Cerebral (AVC) é caracterizado como um déficit neurológico atribuído a uma lesão aguda do sistema nervoso central por uma causa vascular. Constitui-se uma das principais causas de mortes, acometendo principalmente a população idosa e, diante das incapacidades impostas pelo AVC, surge a necessidade de cuidados, que são frequentemente realizados pelo cuidador familiar. Este estudo teve como objetivo construir uma tecnologia educacional direcionada aos cuidadores familiares de pacientes idosos pós-Acidente Vascular Cerebral, dependentes de cuidados básicos para as atividades de vida diária, isto é, uma tecnologia em formato de vídeo, constituindo o produto desta pesquisa. Trata-se de estudo metodológico, realizado em uma unidade de internamento de pacientes neurológicos em um hospital universitário de grande porte localizado na região Sul do país. A amostra foi composta por 14 juizes especialistas em neurologia e/ou gerontologia do território nacional e 15 cuidadores familiares de idosos. A coleta de dados ocorreu no período de dezembro a fevereiro de 2024. O estudo foi composto por três etapas: 1) Pré-produção: construção da sinopse, argumento, roteiro e storyboard do vídeo educacional, para essa etapa foi realizada uma revisão integrativa, visando fundamentação teórica para o desenvolvimento do roteiro para tecnologia educacional com posterior validação do conteúdo do vídeo por juízes especialistas, por meio do Instrumento de Validação de Conteúdo Educacional em Saúde (IVCES). Para análise do instrumento foi utilizado o Índice de Validade de Conteúdo (IVC) e o valor considerado aceitável quando o resultado da proporção de concordância entre os juízes especialistas foi igual ou superior a 0,78; 2) Produção: construção do vídeo educacional e avaliação do vídeo pelos cuidadores familiares dos idosos internados na Neurologia, por meio do instrumento Suitability Assessment of Materials (SAM), com avaliação do conteúdo, exigência de alfabetização, ilustrações, layout e apresentação e adequação cultural; 3) Pós-produção: edição e versão final do vídeo educacional para posterior divulgação. Como resultado da pesquisa, identificado na literatura quais orientações os cuidadores e/ou familiares de pessoa idosas que sofreram AVC deveriam receber sobre os cuidados pós-AVC no preparo para a alta hospitalar. Realizada produção do roteiro do vídeo, que foi validado pela concordância dos juízes especialistas (IVC= 0,93). O vídeo foi composto por 51 cenas em resolução Full HD, com duração de 8 minutos. Houve 99% de concordância entre os cuidadores familiares de idosos participantes, tornando-o adequado e validado. Após finalização dessa etapa, foi preenchido o requerimento para emissão do certificado de produto brasileiro do vídeo educacional na Agência Nacional do Cinema, divulgado em plataforma digital YouTube. O vídeo demonstrou-se uma ferramenta que auxiliou na aquisição de conhecimentos fundamentais sobre o tema, uma vez que é uma forma de disseminar informações de forma atraente, simples, dinâmica e democrática. O produto desenvolvido tem a potencialidade de ser replicado em outras unidades da instituição que atendam ao mesmo perfil de pacientes, uma vez que a tecnologia proporciona orientações e esclarecimentos aos cuidadores familiares de idosos sobre o tema.

Abstract: Cerebral Vascular Accident (CVA) is characterized as a neurological deficit attributed to an acute injury of the central nervous system due to a vascular cause. It is one of the main causes of death, affecting mainly the elderly population and, given the disabilities imposed by stroke, the need for care arises, which is often carried out by the family caregiver. This study aimed to build an educational technology aimed at family caregivers of elderly post-stroke patients, dependent on basic care for activities of daily living, that is, a technology in video format, constituting the product of this research. This is a methodological study, carried out in an inpatient unit for neurological patients in a large university hospital located in the southern region of the country. The sample was made up of 14 judges specializing in neurology and/or gerontology from the national territory and 15 family caregivers of the elderly. Data collection took place from December to February 2024. The study consisted of three stages: 1) Preproduction: construction of the synopsis, argument, script and storyboard of the educational video, for this stage an integrative review was carried out, aiming at theoretical foundation for the development of the script for educational technology with subsequent validation of the video content by expert judges, through the Health Educational Content Validation Instrument (IVCES). To analyze the instrument, the Content Validity Index (CVI) was used and the value was considered acceptable when the result of the proportion of agreement between the expert judges was equal to or greater than 0.78; 2) Production: construction of the educational video and evaluation of the video by family caregivers of elderly people hospitalized in Neurology, using the Suitability Assessment of Materials (SAM) instrument, with content evaluation, literacy requirements, illustrations, layout and presentation and cultural suitability; 3) Postproduction: editing and final version of the educational video for subsequent dissemination. As a result of the research, the literature identified what guidance caregivers and/or family members of elderly people who suffered a stroke should receive regarding post-stroke care in preparation for hospital discharge. The video script was produced, which was validated by the agreement of the expert judges (IVC= 0.93). The video was composed of 51 scenes in Full HD resolution, lasting 8 minutes. There was 99% agreement among family caregivers of participating elderly people, making it appropriate and validated. After completing this stage, the application was completed to issue the Brazilian product certificate for the educational video at the National Cinema Agency, published on the YouTube digital platform. The video proved to be a tool that helped in the acquisition of fundamental knowledge on the topic, as it is a way of disseminating information in an attractive, simple, dynamic and democratic way. The developed product has the potential to be replicated in other units of the institution that serve the same patient profile, as the technology provides guidance and clarification to family caregivers of elderly people on the topic.

Research progress in hypoxia-induced endothelial-to-mesenchymal transition induced pulmonary vascular remodeling and drug interventions / 中国药理学与毒理学杂志

Pulmonary hypertension(PH)is the pulmonary vascular remodeling caused by endothe-lial dysfunction and other factors,which leads to the increase of pulmonary artery pressure and finally right heart failure and patient death.Medications can significantly improve the symptoms of patients with pulmonary hypertension,but drugs can't cure vascular remodeling and right heart failure once for all,so it is urgent to find new treatments.Endothelial cells can obtain mesenchymal phenotypes after hypoxia stimulation,that is,endothelial-to-mesenchymal transition(EndMT).Increasing studies have shown that EndMT plays an important role in pulmonary vascular remodeling of PH.In this review,three signaling pathways related to induction of endothelial-to-mesenchymal transition under hypoxic conditions and research progress in related drugs are elaborated:① Transforming growth factor-β/bone morphogenetic protein(TGF-β/BMP)signaling pathway.The TGF-β/BMP pathway is mediated by Smad,and hypoxia regulates the expressions of EndMT-related genes by inhibiting or inducing the phosphorylation level of Smad in the downstream medium.② Notch signaling pathway.Hypoxia can enhance Jagged/Notch signaling pathway and promote the EndMT process.③Wnt/β-catenin signaling pathway.Hypoxia increases the expression of β-catenin,activates the Wnt signaling pathway,and regulates the expressions of EndMT regulatory genes.The significance of hypoxia-induced EndMT as an impor-tant pathogenic factor in PH is clarified in order to provide new ideas for the improvement of pulmonary vascular remodeling and recommend new effective strategies for the prevention and treatment of PH.

Nursing care of an infant with severe bronchopulmonary dysplasia during the transition period from hospitalization to family / 中华护理杂志

To summarize the nursing care of a very low birth weight premature infant with severe type Ⅱbronchopulmonary dysplasia(BPD)during the transition period from hospitalization to home.The care of the infant was provided one-on-one by a BPD specialist nurse throughout the period.The key points of transitional care from hospitalization to home include:implementing tracheotomy and mechanical ventilation care to ensure stable blood oxygen saturation of the infant;providing nutritional support to improve the nutritional status of the infant;implementing step-by-step rehabilitation measures to improve the neuromotor development of the infant;implementing family integrated care to promote the primary caregivers of the infant to master nursing knowledge and skills;conducting personalized discharge follow-up with a multidisciplinary team to improve the quality of home care for this infant.After being hospitalized for 106 days,the infant was successfully discharged with a tracheotomy tube.At the age of 2 years and 6 months,a tracheotomy closure surgery was performed.After the surgery,the infant was able to breathe autonomously without symptoms of breathing difficulties and returned to normal family life.