RESUMEN
The purpose of the present study was to assess the early stages of development of mouse first molar roots in the osteopetrotic context of RANKL invalidation in order to demonstrate that the radicular phenotype observed resulted not only from defective osteoclasts, but also from loss of cell-to-cell communication among dental, periodontium and alveolar bone cells involving RANKL signaling. Two experimental models were used in this study: Rankl mutants with permanent RANKL invalidation, and C57BL/6J mice injected during the first postnatal week with a RANKL neutralizing antibody corresponding to a transient RANKL invalidation. The dento-alveolar complex was systematically analyzed using micro-CT, and histological and immunohistochemical approaches. These experiments showed that the root elongation alterations observed in the Rankl-/- mice were associated with reduced proliferation of the RANK-expressing HERS cells with a significant decrease in proliferating cell nuclear antigen (PCNA) expression and a significant increase in P21 expression. The phenotypic comparison of the adult first molar root at 35 days between permanent and transitory invalidations of RANKL made it possible to demonstrate that alterations in dental root development have at least two origins, one intrinsic and linked to proliferation/differentiation perturbations in dental-root-forming cells, the other extrinsic and corresponding to disturbances of bone cell differentiation/function.
Asunto(s)
Homocigoto , Mutación , Odontogénesis/genética , Ligando RANK/genética , Raíz del Diente/crecimiento & desarrollo , Raíz del Diente/metabolismo , Animales , Biomarcadores , Expresión Génica , Genotipo , Inmunohistoquímica , Ratones , Fenotipo , Raíz del Diente/diagnóstico por imagenRESUMEN
Introducción: El almidón de maíz (AM) está indicado en el manejo de niños con glucogenosis hepática (GH).Objetivo: Describir la respuesta clínica en siete niños con GH I y III, del Hospital Pablo Tobón Uribe de Medellín, Colombia, al cambiar el AM de uso alimenticio (UA), por AM de pureza farmacológica (PF). Metodología: Estudio observacional, retrospectivo, descriptivo realizado en 51 meses en el que se comparan pruebas bioquímicas y evolución clínica de siete niños ambulatorios con GH, quienes recibían AMUA y se les cambió a AMPF, por recaída en sus controles bioquímicos, relacionados con el cambio en la composición del AM tradicional, al que se le adicionó una mezcla de vitaminas y minerales. Resultados: Se incluyeron 3 niñas y 4 niños entre 13 y 148 meses, 3 con GH I y 4 con GH III, que recibían AMUA, y luego de cambiar a AMPF, presentaron mejoría clínica y bioquímica así: glucemia en ayunas de 77 mg/dL (48-90) a 85 (68-119) mg/dL, ALT de 390 U/L (47-1410) a 159 (47-345) U/L y triglicéridos de 487 (186-1797) mg/dL a 240 (112-614) mg/dL.Conclusiones: El AMPF en niños con GH I y III, demostró resultados mejores, que los obtenidos con el AMUA. La evidencia bioquímica y clínica, permite recomendar su utilización en el tratamiento actual de los pacientes con estos tipos de GH.
Introduction: Corn starch (CS) is indicated for the management of children with liver glucogenosis (LG). Objective: To describe the clinic outcome in 7 children with LG I and III, from Hospital Pablo Tobón Uribe from Medellín, Colombia, to change food (F) CS by the pharmacological purity (PP) CS. Methods: Observational, retrospective, descriptive study performed in 51 months comparing biochemical and clinical course of 7 children outpatient with LG, those receiving FCS and were switched to PPCS by relapse in biochemical controls related to the change in the composition of the traditional commercial CS, which was added a mixture of vitamins and minerals. Results: Were included 3 girls and 4 boys between 13 and 148 months, 3 with LG I and 4 with LG III, receiving FCS, and after switching to PPCS, they improved the biochemical tests and clinical status. Conclusions: PPCS in children with LG I and III, showed better results than those obtained with FCS. The biochemical and clinical evidence both to recommend its use in the current treatment of patients with these types of LG.
