Leucemias agudas pediátricas de inmunofenotipo inusual / Childhood acute leukemias of unusual phenotype

La mayoría de las Leucemias Agudas (LA) pediátricas pueden clasificarse como Linfoblásticas (principalmente de fenotipo B o T) o Mieloblásticas dependiendo del linaje celular de los blastos, recibiendo tratamiento específico de acuerdo a esta caracterización. La inmunotipificación de las LA se basa en la evaluación de la expresión de antígenos de superficie y/o intracitoplasmáticos de diferenciación linfoide (B o T) o mieloide (My) en los blastos, lo cual permite definir la estirpe celular y clasificar la LA de acuerdo al grado de maduración. Sin embargo, existen grupos particulares poco frecuentes de LA cuya clasificación resulta dificultosa y por eso se las denomina LA de linaje ambiguo (fenotipo mixto/indiferenciadas) y LA de linaje dendrítico. Las de fenotipo mixto son aquellas en las que los blastos expresan marcadores de más de un linaje, y las indiferenciadas aquellas que no expresan antígenos específicos para ningún linaje. Diferentes convenciones se han ido desarrollando para definir y clasificar estos fenotipos inusuales, siendo la más actualizada la propuesta por la Organización Mundial de la Salud (2008). De acuerdo a estas pautas, de 1301 casos de LA diagnosticados entre abril de 1994 y abril de 2009, 28 fueron re-clasificados como LA de linaje Ambiguo, 3 como leucemia mieloide aguda minimamente diferenciadas y 3 como LA de células dendríticas. Debido a lo infrecuente de estos casos, su caracterización resulta relevante, ya que la bibliografía presenta, en general, sólo comunicaciones esporádicas de estos fenotipos particulares. Dada la importante casuística del Hospital Garrahan y el amplio seguimiento de los pacientes, el relevamiento de estos casos inusuales permite caracterizarlos desde el inmunofenotipo, la morfología/citoquímica, la citogenética/biología molecular y evaluar su presentación clínica, evolución, respuesta al tratamiento y sobrevida libre de eventos con la finalidad de colaborar con la definición de su pronóstico y eventualmente con la elaboración de protocolos de tratamiento diferenciados para estos subgrupos de LA (AU)

The majority of childhood acute leukemias (AL) can be classified as lymphoblastic (mainly phenotype B or T) or myeloblastic, depending on the cell lineage of the blasts, requiring specific treatment according to this characterization. Immunotypification of AL is based on surface and/or intracytoplasmic antigen expression with lymhoid (B or T) or myeloide (My) blast differentiation, allowing definition of cell lineage and classification of the AL according to the grade of maturation. Nevertheless, there are rare cases of AL that are difficult to classify, denominated AL of ambiguous lineage (mixed/undifferentiated lineage) and acute dendritic cell leukemia. In AL of the mixed phenotype, the blasts express markers of more than one lineage and in undifferentiated AL, the blasts lack antigen expression of any specific lineage. Different conventions have tried to define and classify these unusual phenotypes, among which the most recent proposal of the World Health Organization (2008). According to the criteria of the latter, of 1301 cases of AL diagnosed between April 1994 and April 2009, 28 were re-classified as AL of ambiguous lineage, 3 as minimally differentiated acute myeloid leukemia, and 3 as acute dendritic cell leukemia. Characterization of these cases is important, as in the literature only sporadic reports of these rare phenotypes are found. Given the large number of patients with a long follow-up of the Garrahan Hospital, a review of these unusual cases allowed characterization from the point of view of the immunophenotype, morphology/cytochemistry, cytogenetics/molecular biology and to evaluate clinical presentation, evolution, response to treatment, and event-free survival to help define the prognosis and develop protocols for the treatment of these subgroups of AL (AU)

[Assessment of microleakage of adhesive restorative materials in deciduous teeth]. / Avaliação da infiltração marginal de materiais restauradores adesivos em dentes decíduos.

The microleakage of Chelon-Fil, Vitremer, Compoglass-F and Z100 was evaluated in deciduous teeth. Class V restorations were placed in the buccal aspect of 40 extracted primary canines; they were thermocycled, and immersed in 0.5% basic fuchsin. Three slices of each tooth were obtained in order to analyze the penetration of dye in the gingival and occlusal walls, under magnification of 40 X. The results showed that Chelon-Fil had the highest degree of marginal leakage. Vitremer, Compoglass-F and Z100 presented similar penetration of dye. The microleakage of Chelon-Fil and Vitremer was greater in the gingival wall than in the occlusal wall. Microleakage was the same in both gingival and occlusal walls when cavities were filled, either with Z100 or with Compoglass-F.

Prevalence of intranasal ectopic teeth in children with complete unilateral and bilateral cleft lip and palate.

OBJECTIVE: The objective of this study was to determine the prevalence of ectopic eruption of intranasal teeth. DESIGN: This was a retrospective study, where records of children with repaired cleft lip and palate were analyzed. SETTING: The study was conducted at a large craniofacial center, the Hospital for Rehabilitation of Craniofacial Anomalies, USP, Bauru, São Paulo, Brazil. SUBJECTS: The sample consisted of 815 records from patients with bilateral cleft lip and palate (BCLP) and 1,495 records from patients with unilateral cleft lip and palate (UCLP). The age of the subjects was 5 to 10 years old and the groups included both males and females. RESULTS: The results showed that 0.61% of the children with BCLP and 0.40% of those with UCLP had an intranasal tooth. The prevalence of an intranasal tooth for the whole group was 0.48%, and it appeared to be more common in females.

Multidisciplinary research carried out in the sample area of southern latium aimed at defining the seismotectonic model of the area

The main geological, geochemical, historical and geophysical researches carried out for many years by ENEA, in collaboration with researches of other organizations, aimed at the definition of a possible neotectonic and seismotectonic model of the area, are listed. The research are of primary importance for the study of seismic hazard and of late Quaternary climatic variations.(AU)