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BACKGROUND: Games have been a part of human life since ancient times and are taught to children and adults who want to simultaneously have fun and learn. Nevertheless, in the third decade of this century, technology invites us to consider using video games to learn topics such as entrepreneurship. However, developing a serious game (SG) is difficult because everyone who forms part of the game development team requires adequate learning resources to acquire the necessary information and improve their game development skills. OBJECTIVE: This work aimed to detail the experience gained in developing ATIC (Aprende, Trabaja, Innova, Conquista [learn, work, innovate, conquer]), an SG proposed for teaching and learning entrepreneurship. METHODS: To develop a videogame, first, we established a game development team formed by professors, professionals, and students who have different roles in this project. Scrum was adopted as a project management method. To create concept art for the video game, designers collected ideas from various games, known as "getting references." In contrast, narratology considers the life of a recent university graduate immersed in real life, considering locations, characteristics, and representative characters from an essential city of Ecuador. RESULTS: In a Unity 3D video game in ATIC, the life of a university student who graduates and ventures into a world full of opportunities, barriers, and risks, where the player needs to make decisions, is simulated. The art of this video game, including sounds and music, is based on the landscape and characteristics of and characters from Cuenca, Ecuador. The game aims to teach entrepreneurs the mechanisms and processes to form their businesses. Thus, we developed the following elements of an SG: (1) world, (2) objects, (3) agents, and (4) events. CONCLUSIONS: The narrative, mechanics, and art of video games are relevant. However, project management tools such as leaderboards and appointments are crucial to influencing individuals' decision to continue to play, or not play, an SG. Developing a serious video game is not an easy task. It was essential to consider many factors, such as the video game audience, needs of learning, context, similarities with the real world, narrative, game mechanics, game art, and game sounds. However, overall, the primary purpose of a serious video game is to transmit knowledge in a fun way and to give adequate and timely feedback to the gamer. Finally, nothing is possible if the members of game development team are not satisfied with the project and not clear about their roles.

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OBJECTIVES: To determine weight, height and body mass index (BMI) of schoolchildren from the La Mancha-Centro health area and compare them with those determined by the Spanish Cross-sectional Growth Study 2010 (SCGS-2010). METHODS: Cross-sectional study of 954 schoolchildren aged 6-12 years. Mean, standard deviation, and percentile distributions of weight, height, and BMI by gender and age were obtained. Differences in each 6-month age group were analyzed. RESULTS: There was a progressive increase in BMI with age, which was significant in girls from nine years of age on and in boys from 8.5 years on. From age 10, average BMI was 2.3 kg/m2 higher than at younger ages (p < 0.001). The biggest difference between genders occurred at age 12: 2 ± 0.98 kg/m2 higher in boys (p = 0.042). Overall, no significant differences were found in weight, height and BMI vs. SCGS-2010, although mean weight of male children from La Mancha-Centro aged between 8.5 and 11.5 years was 3.9 kg higher than that of the rest of Spanish male children. CONCLUSIONS: Anthropometric parameters of schoolchildren from La Mancha-Centro do not significantly differ from national standards; however, preadolescent males from La Mancha-Centro weigh almost 4 kg more.

OBJETIVOS: Determinar peso, talla e índice de masa corporal (IMC) de escolares del área de salud La Mancha-Centro y compararlos con los definidos en el Estudio Transversal Español de Crecimiento 2010 (ETEC-2010). MÉTODOS: Estudio transversal de 954 escolares entre 6 y 12 años. Se obtuvieron media, desviación estándar y distribuciones percentilares de peso, talla e IMC por sexo y edad. Se analizaron las diferencias en cada grupo semestral de edad. RESULTADOS: Existió incremento progresivo del IMC con la edad, significativo en las niñas a partir de los nueve años y en los niños desde los 8.5 años. Desde los 10 años, el IMC promedio resultó 2.3 kg/m2 superior al de edades menores (p < 0.001); la mayor diferencia entre los sexos ocurrió a los 12 años: 2 ± 0.98 kg/m2 más en los varones (p = 0.042). Globalmente no se hallaron diferencias significativas de peso, talla e IMC con el ETEC-2010, aunque el peso medio de los niños manchegos de 8.5 a 11.5 años fue 3.9 kg mayor que el del resto de los niños españoles. CONCLUSIONES: Los parámetros antropométricos de los escolares manchegos no difieren de los estándares nacionales; sin embargo, los varones preadolescentes manchegos pesan casi 4 kg más.

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Resumen Objetivos: Determinar peso, talla e índice de masa corporal (IMC) de escolares del área de salud La Mancha-Centro y compararlos con los definidos en el Estudio Transversal Español de Crecimiento 2010 (ETEC-2010). Métodos: Estudio transversal de 954 escolares entre 6 y 12 años. Se obtuvieron media, desviación estándar y distribuciones percentilares de peso, talla e IMC por sexo y edad. Se analizaron las diferencias en cada grupo semestral de edad. Resultados: Existió incremento progresivo del IMC con la edad, significativo en las niñas a partir de los nueve años y en los niños desde los 8.5 años. Desde los 10 años, el IMC promedio resultó 2.3 kg/m2 superior al de edades menores (p < 0.001); la mayor diferencia entre los sexos ocurrió a los 12 años: 2 ± 0.98 kg/m2 más en los varones (p = 0.042). Globalmente no se hallaron diferencias significativas de peso, talla e IMC con el ETEC-2010, aunque el peso medio de los niños manchegos de 8.5 a 11.5 años fue 3.9 kg mayor que el del resto de los niños españoles. Conclusiones: Los parámetros antropométricos de los escolares manchegos no difieren de los estándares nacionales; sin embargo, los varones preadolescentes manchegos pesan casi 4 kg más.

Abstract Objectives: To determine weight, height and body mass index (BMI) of schoolchildren from the La Mancha-Centro health area and compare them with those determined by the Spanish Cross-sectional Growth Study 2010 (SCGS-2010). Methods: Cross-sectional study of 954 schoolchildren aged 6-12 years. Mean, standard deviation, and percentile distributions of weight, height, and BMI by gender and age were obtained. Differences in each 6-month age group were analyzed. Results: There was a progressive increase in BMI with age, which was significant in girls from nine years of age on and in boys from 8.5 years on. From age 10, average BMI was 2.3 kg/m2 higher than at younger ages (p < 0.001). The biggest difference between genders occurred at age 12: 2 ± 0.98 kg/m2 higher in boys (p = 0.042). Overall, no significant differences were found in weight, height and BMI vs. SCGS-2010, although mean weight of male children from La Mancha-Centro aged between 8.5 and 11.5 years was 3.9 kg higher than that of the rest of Spanish male children. Conclusions: Anthropometric parameters of schoolchildren from La Mancha-Centro do not significantly differ from national standards; however, preadolescent males from La Mancha-Centro weigh almost 4 kg more.

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This study is focused on the massively multiplayer online games' acceptance. In general, while specialized literature reveals that the online gaming industry has grown strongly in recent years, little evidence is identified on its user acceptance. In this manner, the present study is an attempt to fill this gap. Concretely, two aims are defined: (1) proposing an acceptance model to predict the continuance usage of massively multiplayer online games, and (2) knowing how this continuance usage encourages social well-being. The model proposed employing the structural equation modeling with partial least squares (PLS-SEM) methodology. This PLS-SEM model has been defined using a combination of the technology acceptance model (TAM) and the self-determination theory (SDT), comprising eight constructs: (1) autonomy and competence, (2) relatedness, (3) achievement and challenge, (4) flow experience, (5) perceived enjoyment, (6) social well-being, (7) perceived ease of use, and (8) continuance intention. The findings reveal that continuance intention impacts on social well-being. Moreover, the pivotal role of flow experience for continuance intention has been demonstrated. Additionally, continuance intention has been impacted by the perceived enjoyment, showing that it is a key construct for the acceptance of massively multiplayer online games. Thus, two contributions are highlighted. First, these results provide the gaming industry and software developer companies with considerations on gamers' motivations in the online game design, in order to stimulate and incentivize its use. Second, the present study can be useful for academicians and practitioners to understand the online gamers' emotions and well-being, showing some light over their psychology and mental health. Finally, limitations and future directions are exposed.

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Mutations in NPM1, FLT3 and CEBPA genes are found in 25-35 % of adult acute myeloblastic leukemia (AML) cases and correlate with prognosis. To date, there have been no reports about these mutations in pediatric AML from Argentina. The aims of the present study were to describe the incidence of NPM1, FLT3 and CEBPA mutations and to analyze their prognostic impact in this population. The incidences of these mutations within a population of 216 pediatric AML cases were: NPM1-mutated 4.2 %, CEBPA-mutated 1.9 %, FLT3-ITD 10.2 % and FLT3-TKD 7.9 %. Among 33 patients with normal karyotype, we found significantly higher frequencies for NPM1-mutated 24.2 % and CEBPA-mutated 12.1 %. Overall survival (pOS) for the 163 eligible non-acute promyelocytic leukemia cases was 46.2 ± 4.3 %, while leukemia-free survival probability was 51.0 ± 4.4 % (n = 135). The NPM1-mutated/FLT3-ITD-negative genotype showed better outcome than any other combined NPM1/FLT3 genotype; this difference was statistically significant within the group of high-risk patients (pOS ± SE 83.3 ± 15.2 % versus 33.1 ± 4.7 %; p = 0.0251). This is the first report of the frequencies of these mutations in Argentina. Despite the limited number of patients, a favorable prognosis of AML with genotype NPM1-mutated/FLT3-ITD-negative was confirmed. This is especially relevant within the high-risk group of patients, as it may contribute to the detection of patients with better prognosis, and thus avoid unnecessary treatment intensification.

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In South America, cutaneous leishmaniasis is the most frequent clinical form of leishmaniasis. Bolivia is one of the countries with higher incidence, with 33 cases per 100,000 individuals, and the disease is endemic in 70% of the territory. In the last decade, the number of cases has increased, the age range has expanded, affecting children under 5 years old, and a similar frequency between men and women is found. An entomological study with CDC light traps was conducted in three localities (Chipiriri, Santa Elena and Pedro Domingo Murillo) of the municipality of Villa Tunari, one of the main towns in the Chapare province (Department of Cochabamba, Bolivia). A total of 16 specimens belonging to 6 species of the genus Lutzomyia were captured: Lu. aragaoi, Lu. andersoni, Lu. antunesi, Lu. shawi, Lu. yuilli yuilli and Lu. auraensis. Our results showed the presence of two incriminated vectors of leishmaniasis in an urbanized area and in the intradomicile. More entomological studies are required in the Chapare province to confirm the role of vector sand flies, the intradomiciliary transmission of the disease and the presence of autochthonous cases of cutaneous leishmaniasis.

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BACKGROUND: Onychomycosis is one of the commonest dermatological diseases worldwide. The antifungal activity of current medications varies, and treatment failure occurs in 25-40% of treated patients. AIMS: To evaluate the in vitro antifungal activity of itraconazole, fluconazole, terbinafine and voriconazole against isolates taken from patients with onychomycosis. METHODS: Nail isolates were evaluated according to methods described in the protocols of the Antifungal Susceptibility Testing Subcommittee of the European Committee on Antimicrobial Susceptibility Testing (AFST-EUCAST) and the Clinical and Laboratory Standards Institute (CLS M38-A), and a CLSI M38-A modified technique for dermatophytes. Antifungal agents tested included terbinafine, itraconazole, voriconazole and fluconazole. RESULTS: In total, 103 isolates of Candida species (n = 58), Fusarium species (n = 10), Fusicoccum dimidiatum (n = 4), Scytalidium hyalinum (n = 1) and dermatophytes (n = 30) were evaluated. Itraconazole and voriconazole were the most active agents against Candida species, whereas terbinafine and voriconazole were most potent against dermatophytes. Fusarium species had the highest minimum inhibitory concentration (MIC) values with all antifungal agents. CONCLUSIONS: The aetiological agents of onychomycosis that we found differ from those found in other countries, suggesting that the heat and humidity of the Colombian climate could favour yeast nail infections. The lowest MICs for Candida species (obtained with voriconazole, followed by itraconazole) may be explained by emerging resistant strains. Against dermatophytes, the lowest MICs were obtained with terbinafine, followed by voriconazole. MIC values for the evaluated agents were higher for non-dermatophyte filamentous fungi than for other fungi. As MIC breakpoints have not yet been established for onychomycosis therapies, it remains unclear if in vitro activities of antifungal drugs are predictive of clinical outcome. Well-designed clinical studies are necessary to assist clinicians in choosing the best antifungal agents.

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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)

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We report on a novel case of pure partial tandem duplication 1q42q43 confirmed by fluorescence in situ hybridization (FISH). We compare the manifestations of our patient with similar cases previously reported. We conclude that the most common clinical manifestations of trisomy 1q42qter are prenatal and postnatal growth retardation, relative macrocephaly, triangular face, prominent forehead, broad nasal bridge, abnormal philtrum, micro/retrognathia, cardiac defects and mental retardation. We would like to emphasize the importance of the FISH technique in the identification of the duplicated segment.

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Muchos avances se han logrado en los últimos 30 años en el tratamiento de la leucemia linfoblástica aguda (LLA) pediática, a nivel mundial y tamibén en nuestro Hospital. Después de su apertura en agosto de 1987 hasta noviembre de 2002 fueron ingresados 989 pacientes con diagnóstico de LLA de los cuales 896 fueron evaluables. Los mismos fueron tratados con 3 protocolos sucesivos: 92 (7 LLa 87), 374 (1 LLA 90) y 430 (1 LLA 96). Las tasas de remisión completa (RC) fueron de 95,6 en el primer protocolo, 94,4 en el segundo y 96,9 en el tercero y un 2,1, 2,9 y 1,8 de los pacientes fallecieron durante la inducción en los respectivos estudios. El principal evento observado fue la recaída de la enfermedad y fallecieron en RC 6 de los pacientes del 7 LLA 87, 4,8 del 1 LLA 90 y 4,3 del 1 LLA 96. La pSLE (EE) fue de 61 (5), 63 (5) y 72 (6), respectivamente, siendo esta diferencia estadísticamente significativa (p=0,0237). El Hospital ha incorporado los métodos diagnósticos necesarios para una mejor estratificicón de los pacientes, y fueron mejoradas las medidas de soporte ofrecidas a los pacientes. Como consecuencia de los logros mencionados se observó una disminución en las tasas de muerte durante la inducción y en RC, con un aumento gradual y significativo de la pSLE. Estos resultados muestran una mejora a lo largo del tiempo y nuestros esfuerzos deben orientarse a una aún mejor optimización de las herramientas diagnósticas, terpéuticas y de soporte para lograr alcanzar los estándares internacionales pra esta población de pacientes.

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El tratamiento de elección para las leucemias agudas pediátricas es la quimioterapia convencional, que ha permitido obtener tasas de sobrevida que actualmente parecen difíciles de superar. En los últimos años se han intensificado las investigaciones dirigidas a descubrir nuevos blancos terapéuticos, entre los que se encuentra el receptor FLT3. Los blastos leucémicos puede presentar formas mutadas de dicho receptor, siendo las más frecuentes mutaciones internas en tándem (FLT3 ITD) y mutaciones puntuales en la zona de activación (FLT3 ALM). Objetivos: Poner a punto la detección de mutacoines de FLT3, analizar su prevalencia en nuestra población de pacientes con diagnóstico de Leucemia Mieloblástica Aguda (LMA) o de Leucemia Linfoblática Aguda en infantes (LLA I), y evaluar su asociación con parámetros clínicos y de laboratorio. Pacientes y Método: El estudio de las mutaciones se realizó por RT PCR, en un total de 122 pacientes (92 LMA y 30 LLA 1). Resultados: Se detectaron mutaciones en el 15,2 de las LMA y en 10 de las LLA -1. La prevalencia de las FLT3 ITD mostró un aumento gradual con la edad de los pacientes, y la media de edad fue significativamente mayor. Con respecto a asociaciones con recuentos leucocitarios, alteraciones genéticas, subtipos FAB y valor pronóstico, si bien hubo difrencias éstas no furon significtivas. Conclusiones: Este es el primer estuido de mutaciones en FLT3 realizado en población pediátrica en nuestro país. La detección de estas mutaciones permitirá individualizar, en el futuro, a los niños candidatos a recibir drogas inhibidoras de FLT3, actualmente en desarrollo.

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Outbreaks of foot-and-mouth disease (FMD) from January 1982 through December 2003 were used to examine variations in serotype- and species-specific risk for three control programmes in Colombia: (1982-1983) vaccination, using an aluminium hydroxide, saponin adjuvant, required but not enforced; (1984-1996) vaccination, using an oil double-emulsion adjuvant, required but not enforced; and (1997-2003) enforced vaccination, using an oil double-emulsion adjuvant, restricted animal movement enforced, and slaughter of infected animals. Hypotheses were tested for trend, cyclicity and seasonality in FMD occurrence, and for species- and serotype-specific differences in morbidity and case-fatality. The spatial density of outbreaks was estimated by kernel smoothing. The frequency of outbreaks decreased most between 1984 and 1996 (p < 0.01) for serotype A and between 1997 and 2003 (p < 0.01) for serotype O. Outbreaks occurred in cycles of 3-4 years for both serotypes (p < 0.05). Morbidity was not significantly different in pigs from that in cattle for serotype A-associated outbreaks (p = 0.314), but was higher in pigs than in cattle (p = 0.019) for serotype O-associated outbreaks. For both serotypes, case-fatality was higher for pigs than for cattle (p < 0.009). Temporal variation in FMD incidence provided insight into the expected evolution of FMD control for countries with similar conditions and where FMD is endemic.

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Chlamydophila abortus is the aetiological agent of enzootic abortion in small ruminants in which it infects the placenta to cause abortion during the last trimester of gestation. In a mouse model, a Th1 immune response involving IFN-gamma production and CD8+ T cells is necessary for the infection to be resolved. The authors previously demonstrated that infection with Nippostrongylus brasiliensis, a rodent gastrointestinal nematode extensively used in experimental models to induce Th2 responses, alters the specific immune response against C. abortus infection, increasing bacterial multiplication in liver and reducing specific IFN-gamma production. The aim of the present work was to clarify whether a Th2 immune response has any influence on the success of vaccination using both inactivated and attenuated vaccines. The results showed that the Th2 response established prior to vaccination did not influence the induction of protection offered by the vaccines. However, the effectiveness of this protective response can be altered, depending on the adjuvant employed in the inactivated vaccines, when the Th2 response is established after vaccination, just before challenge with C. abortus.

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BASIS: A significant proportion of our patients has described to have problems from tolerance to Dolquine, a new presentation of hydroxychloroquine recently marketed in Spain, compared to Plaquenil. The objective was to know the tolerability and the adverse effects of this new presentation. PATIENTS AND METHOD: A cross-sectional multicenter study on 133 patients treated with Dolquine was conducted. RESULTS: Of the 133 patients (87% women; average age [AA]: 32.9 [15.4] years) who received Dolquine during an average period of 6.7 (1.4) months, 32 patients (24%) described to have more problems with this drug in comparison with other antimalarial. The adverse effects experienced were: bitter taste (62.4%), difficulty in swallowing the tablet (13.5%), dyspepsia (9.8%), nausea (7.5%), vomiting (1.5%), pruritus (1.5%), diarrhea (0.7%), and instability feeling (0.7%). The presence of gastrointestinal adverse effects was not related to the consumption of gastroerosive drugs, gastric protectors, or a high number of drugs. The attrition rate was 9.8%. Conclusions. Dolquine induces lower tolerance and more gastrointestinal adverse effects than Plaquenil, pointing out its bitter taste and the difficulty in swallowing it. Despite this higher intolerance there was not an increase in the attition rate from the antimalarial treatment in comparison to other series.

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La técnica de hidratación in situ con fluorescencia (FISH) detecta aneuploidias cromosómicas en células interfásicas. En el presente trabajo se efectuó esta técnica, en células de la mucosa bucal, con el objetivo de detectar bajos niveles de mosaicismo en pacientes con diagnóstico presuntivo de Sindrome de Turner (ST) y cariotipo normal en sangre periférica. Un segundo objetivo fue el de comparar la frecuencia de mosaicismos en ambos tejidos. En el período 1988-2000 se seleccionaron 110 pacientes que ingresaron al laboratorio de citogenética con diagnóstico presuntivo de ST y cariotipo normal o mosaico. De estas solo 18 pudieron ingresar en el presente estudio (8 con cariotipo 46,XX, 7 con mosaicos involucrando al cromosoma X y 3 con mosaicos con material del cromosoma Y). Se estudió con grupo control constituido por 10 mujeres normales. Fueron analizados 200 o más núcleos interfásicos de la mucosa bucal por individuo. La sensibilidad analítica de la sonda fue del 96%. Se halló mosaicismo en 2 pacientes 46,XX (11,5% y 14,55%) el cual fue corroborado en un segundo estudio. En las pacientes mosaicos se observaron las mismas líneas celulares por ambas metodologías pero discordancia en su frecuencia. Este trabajo sugiere que FISH en células de la mucosa bucal podría ser una metodología alternativa para detectar mosaicismos tejido especificos. Es un buen complemento de la citogenética clásica al permitir acceder a un mayor conocimiento de la constitución cromosómica en pacientes con rasgos relevantes de ST y cariotipo normal.

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Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disease (8q21) from the family of the genetically determined chromosomal instability syndromes. The disorder is characterized by microcephaly, growth retardation, immunodeficiency, and high incidence of cancer. Several noninflammatory anomalies of the musculoskeletal system have been described in patients with this syndrome. We describe an Argentinian girl with all the clinical, immunological, and cytogenic characteristics described for NBS plus a juvenile rheumatoid arthritis-like syndrome. To our knowledge this is the first report of a patient with the NBS who presented with a symmetric chronic polyarthritis resembling JRA.

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BACKGROUND: The association between t(8;21) and granulocytic sarcoma (GS) is well known, but to the authors' knowledge the prognostic significance of GS in these patients has not been defined clearly. METHODS: Between January 1990 and July 1999 174 children with acute myeloid leukemia were admitted to the study institution. Translocation (8;21) was identified in 20 patients (11.5%). Eighteen patients were evaluable for the current study and 8 presented with GS at the time of diagnosis (GS+). RESULTS: The authors defined two groups of patients: those who were GS+ and those who were GS-. One patient in the GS+ group and two patients in the GS- group died during the induction phase of the study. Complete remission was achieved in seven patients in the GS+ group and eight patients in the GS- group. Two patients developed a recurrence in the GS+ group as did one patient in the GS- group. The event free-survival probability (the standard error) was 58% (18%) in the GS+ group and 70% (14%) in the GS- group. Localization of GS was in only one site in seven patients and at multiple sites in one patient. Patients with an epidural mass received local radiotherapy (one patient) or surgery (two patients). Two of these patients developed paraplegia as sequelae: one patient after surgery and one patient after radiotherapy. One patient with orbital GS received local radiotherapy because of progressive proptosis. The remaining four patients had a complete resolution of the GS with chemotherapy only. CONCLUSIONS: In the current study of patients with t(8;21)(q22;q22), the presence of granulocytic sarcoma was not found to be an adverse prognostic factor. However, careful attention should be paid, especially to patients with an epidural site, to avoid sequelae. Chemotherapy appears to be the optimum treatment for these children.

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We describe an 8 year old boy who had received chemotherapy for an acute promyelocytic leukemia and developed a secondary leukemia 27 months after the diagnosis of this first malignancy. Blasts cells were positive for cytoplasmic markers CD22, CD3 and myeloperoxidase. Cell surface T and myeloid-associated markers were also detected. Cytogenetic study disclosed monosomy 7. The patient achieved complete remission, but relapsed 15 months later with identical immunophenotypic and cytogenetic findings. Three-lineage commitment is proved by the expression of specific criteria for myeloid, and lymphoid T and B typing. A multipotent immature progenitor must be the target of leukemogenic agents. The prognosis is obviously ominous.

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