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PURPOSE: To characterize the pediatric population with inborn errors of immunity (IEI) that was treated with hematopoietic stem cell transplantation (HSCT) in three reference centers in Colombia. What have been the characteristics and outcomes of hematopoietic stem cell transplantation in pediatric patients with inborn errors of immunity in three reference care centers in Colombia between 2007 and 2018? METHODS: We conducted an observational, retrospective cohort study in children with a diagnosis of IEI who underwent HSCT between 2007 and 2018. RESULTS: Forty-seven patients were identified, and 5 were re-transplanted. Sixty-eight percent were male. The median age at diagnosis was 0.6 years, and for HSCT was 1.4 years. The most common diseases were chronic granulomatous disease (38%) followed by severe combined immune deficiencies (19%) and hemophagocytic lymphohistiocytosis (15%). Cord blood donors were the most used source of HSCT (44%). T cell-replete grafts from haploidentical donors using post-transplantation cyclophosphamide represent 37% of the cohort. All patients received conditioning, 62% with a non-myeloablative regimen. Calcineurin inhibitors were the main graft-versus-host disease prophylaxis (63.8%). Acute graft-versus-host disease developed in 35% of the total patients. The most frequent post-transplant infections were viral and fungal infections. The 1-year overall survival rates for the patients who received HSCT from identical, haploidentical, and cord sources were 80%, 72%, and 63%, respectively. The 5-year overall survival was 63%. CONCLUSIONS: HSCT is a curative treatment option for some IEI and can be performed with any donor type. Early and timely treatment in referral centers can improve survival.
Asunto(s)
Enfermedades Genéticas Congénitas/genética , Enfermedades Genéticas Congénitas/terapia , Predisposición Genética a la Enfermedad , Trasplante de Células Madre Hematopoyéticas , Enfermedades de Inmunodeficiencia Primaria/etiología , Enfermedades de Inmunodeficiencia Primaria/terapia , Preescolar , Colombia , Terapia Combinada , Diagnóstico Diferencial , Femenino , Estudios de Asociación Genética , Enfermedades Genéticas Congénitas/diagnóstico , Enfermedades Genéticas Congénitas/mortalidad , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Trasplante de Células Madre Hematopoyéticas/métodos , Humanos , Lactante , Depleción Linfocítica , Masculino , Fenotipo , Enfermedades de Inmunodeficiencia Primaria/diagnóstico , Enfermedades de Inmunodeficiencia Primaria/mortalidad , Donantes de Tejidos , Resultado del TratamientoRESUMEN
Resumen Introducción y objetivo: La reabsorción radicular apical externa (RRAE) asociada al movimiento dental ha sido tema de investigación en el campo de la ortodoncia y la endodoncia. La detección temprana traería un beneficio para el paciente y el profesional; ha sido descrita como una complicación o secuela del tratamiento de ortodoncia que resulta en la pérdida permanente de estructura radicular El dignóstico eficiente es un aspecto esencial para definir la terapéutica adecuada y el éxito del tratamiento. El propósito de este estudio fue evaluar los cambios en la longitud radicular y del conducto como consecuencia del tratamiento ortodóncico.< Materiales y métodos: Se evaluaron 42 dientes , incisivos centrales superiores de 21 pacientes (12 mujeres, 9 hombres). Los cambios en la longitud radicular y amplitud del conducto fueron determinadas por medio de mediciones radiográficas obtenidas antes de iniciar el tratamiento (T1) y posteriormente a los 6 (T2), 12 (T3) y 18 meses (T4) utilizando un posicionador de técnica paralela RINN® XCP® (DENTSPLY) y el Sistema de radiovisiografía CDR de SCHICK ®. las imágenes obtenidas se importaron y procesaron con el sistema DBS WIN®, aquí se llevaron a cabo las mediciones correspondientes. Resultados: Todos los Incisivos evaluados mostraron disminución de la longitud y de la amplitud del conducto radicular durante los 18 meses de tratamiento. Conclusión: La RRAE se puede detectar en etapas tempranas del tratamiento de Ortodoncia, se presenta más significativamente en los primeros 6 meses, parece estar relacionado con las fuerzas de la fase de alineación y nivelación.
Abstract Introduction and objective: External apical root resorption ( RRAE ) associated with tooth movement has been the subject of research in the field of orthodontics and endodontics. Early detection would bring a benefit to the patient and the professional; It has been described as a complication of orthodontic treatment resulting in permanent loss of root structure. Efficient diagnosis is an essential aspect in order to define appropriate therapeutic and treatment success. The purpose of this study was to assess changes in root length and canal as a result of orthodontic treatment. Materials and methods: 42 teeth, maxillary central incisors of 21 patients (12 women, 9 men ) were evaluated. Changes in root length and width of the canal were determined with radiographic measurements obtained before starting treatment (T1 ) and at 6 ( T2 ), 12 ( T3 ) and 18 months ( T4 ) using a RINN® XCP® ( DENTSPLY) positioner technique and CDR System radiovisiography SCHICK®. Images obtained were imported and processed with the WIN® DBS system with which the corresponding measurements were made. Results: All tested incisors showed decreased length and breadth of the root canal during the 18 months of treatment. Conclusion: RRAE can be detected at early stages of orthodontic treatment , it appears most significantly during the first 6 monthsof treatment and seems to be related to the forces during alignment and leveling phases.
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En la actualidad, se han venido desarrollando y utilizando, una serie de herramientas interactivasa nivel odontológico, que mejoran y facilitan el proceso de enseñanza y aprendizaje, proporcionando bases para el desarrollo profesional del alumno y el odontólogo.