Cancer Prevalence in Children with Inborn Errors of Immunity: Report from a Single Institution.

BACKGROUND: Inborn Errors of Immunity (IEI) comprise several genetic anomalies that affect different components of the innate and adaptive responses, predisposing to infectious diseases, autoimmunity and malignancy. Different studies, mostly in adults, have reported a higher prevalence of cancer in IEI patients. However, in part due to the rarity of most of these IEI subtypes (classified in ten categories by the Primary Immunodeficiency Committee of the International Union of Immunological Societies), it is difficult to assess the risk in a large number of patients, especially during childhood. OBJECTIVE: To document the cancer prevalence in a pediatric cohort from a single referral institution, assessing their risk, together with the type of neoplasia within each IEI subgroup. METHOD: An extensive review of clinical records from 1989 to 2022 of IEI patients who at some point developed cancer before the age of sixteen. RESULTS: Of a total of 1642 patients with IEI diagnosis, 34 developed cancer before 16 years of age, showing a prevalence (2.1%) significantly higher than that of the general age matched population (0.22). Hematologic neoplasms (mostly lymphomas) were the most frequent malignancies. CONCLUSION: This study represents one of the few reports focused exclusively in pediatric IEI cases, describing not only the increased risk of developing malignancy compared with the age matched general population (a fact that must be taken into account by immunologists during follow-up) but also the association of the different neoplasms with particular IEI subtypes, thus disclosing the possible mechanisms involved.

Impact of IKZF1 Deletions in the Prognosis of Childhood Acute Lymphoblastic Leukemia in Argentina.

An association of deletions in the IKZF1 gene (IKZF1del) with poor prognosis in acute lymphoblastic leukemia (ALL) has been demonstrated. Additional deletions in other genes (IKZF1plus) define different IKZF1del subsets. We analyzed the influence of IKZF1del and/or IKZF1plus in the survival of children with ALL. From October 2009 to July 2021, 1055 bone marrow samples from patients with ALL were processed by Multiplex ligation-dependent probe amplification (MLPA). Of them, 28 patients died during induction and 4 were lost-in-follow-up, resulting in an eligible 1023 cases. All patients were treated according to ALLIC-BFM-2009-protocol. Patients were classified into three subsets: IKZF1not-deleted (IKZFF1not-del), IKZF1deleted (IKZF1del) and IKZF1del plus deletion of PAX5, CDKN2A, CDKN2B and/or alterations in CRLF2 with ERG-not-deleted (IKZF1plus). The LFSp and SE were calculated with the Kaplan−Meier calculation and compared with a log-rank test. From the 1023 eligible patients, 835 (81.6%) were defined as IKZF1not-del, 94 (9.2%) as IKZF1del and 94 (9.2%) as IKZF1plus. Of them, 100 (9.8%) corresponded to Standard-Risk (SRG), 629 (61.5%) to Intermediate-Risk (IRG) and 294 (28.7%) to High-Risk (HRG) groups. LFSp(SE) was 7 5(2)% for IKZF1not-del, 51 (6)% for IKZF1del and 48 (6)% for IKZF1plus (p-value < 0.00001). LFSp(SE) according to the risk groups was: in SRG, 91 (4)% for IKZF1not-del, 50 (35)% IKZF1del and 100% IKZF1plus (p-value = ns); in IRG, 77 (2)% IKZF1not-del, 61 (10)% IKZF1del and 54 (7)% IKZF1plus (p-value = 0.0005) and in HRG, 61 (4)% IKZF1not-del, 38 (8)% IKZF1del and 35 (9)% IKZF1plus (p-value = 0.0102). The IKZF1 status defines a population of patients with a poor outcome, mainly in IRG. No differences were observed between IKZF1del versus IKZF1plus. MLPA studies should be incorporated into the risk-group stratification of pediatric ALL.

Progressive nodular histiocytosis in a 9-year-old boy treated with cobimetinib.

Progressive nodular histiocytosis is a rare variant of non-Langerhans cell histiocytosis that affects the skin and mucous membranes and displays a progressive clinical course and poor response to treatment. We describe a case of severe progressive nodular histiocytosis harboring a KRAS p.G12S mutation in a 9-year-old boy, refractory to chemotherapy, who was successfully treated with the MEK inhibitor cobimetinib. This is the first report of the use of MEK inhibition for this histiocytosis subtype in a pediatric patient.

Smart Tree: An Architectural, Greening and ICT Multidisciplinary Approach to Smart Campus Environments.

At present, climate change, pollution, and uncontrolled urbanism threaten not only natural ecosystems, but also the urban environment. Approaches to mitigate these challenges and able to provide an alternative for the use of the space are deemed to be multidisciplinary, combining architecture, vegetation integration, circular economy and information and communications technologies (ICT). University campuses are a key scenario to evaluate such solutions as their student and research community is intrinsically willing to support these experiences and provide a wide knowledge on the fields necessary for their design and implementation. However, the creation of areas combining usability and sustainability is commonly lacking a multidisciplinary approach combining all these different perspectives. Hence, the present work aims to overcome this limitation by the development of a novel integrated approach for campus spaces for co-working and leisure, namely a "Smart Tree", where novel architecture, furniture design, flora integration, environmental sensoring and communications join together. To this end, a survey of the literature is provided, covering related approaches as well as general principles behind them. From this, the general requirements and constraints for the development of the Smart Tree area are identified, establishing the main interactions between the architecture, greening and ICT perspectives. Such requirements guide the proposed system design and implementation, whose impact on the environment is analyzed. Finally, the research challenges and lessons learned for their development are identified in order to support future works.

Leucemia linfoblástica aguda en niños con síndrome de Down: análisis comparativo con pacientes sin síndrome de Down / Acute lymphoblastic leukemia in children with Down syndrome: Comparative analysis versus patients without Down syndrome

Introducción: Los niños con síndrome de Down (SD) tienen mayor frecuencia de leucemia linfoblástica aguda (LLA) y menor supervivencia que pacientes sin síndrome de Down (NSD). Analizamos las características clínicas, demográficas-biológicas y respuestas al tratamiento en SD-LLA versus NSD-LLA. Pacientes y métodos: Pacientes (0-19 años) con LLA desde enero de 1990 a noviembre de 2016. Se compararon características demográficas biológicas y respuestas al tratamiento con chi cuadrado y Wilcoxon rank sum. La supervivencia global y el intervalo libre de eventos (ILE) se analizaron con Kaplan-Meier y el test log-rank. Resultados: Se incluyeron 1795 pacientes, 54 con SD. Los SD-LLA presentaron edad mayor (p= 0,0189). T odos inmuno fenotipo precursor-B, con menor incidencia de anomalías recurrentes (p < 0,0001). Demostraron mejor tasa de respuesta a prednisona (p= 0,09) y mayor mortalidad en inducción y remisión completa (p < 0,0001). Todas las muertes de los SD-LLA fueron relacionadas con el tratamiento. La sobrevida libre de eventos en niños SD-LLA vs.NSD-LLA fue 47 (± 8)% vs. 73 (± 1)% (p= 0,006) y el ILE de los SD-LLA vs. NSD-LLA fue 54 (± 9)% vs. 75 (± 1)% (p= 0,0297). La tasa de recaídas fue similar en ambos grupos (p= 0,6894). El ILE de los SD-LLA fue menor en el grupo de 6-9 años: 39 (± 19)% (p= 0,7885). Conclusiones: Los niños de 6-9 años con SD-LLA años presentó menor sobrevida. Aunque estos niños presentaron una mejor respuesta temprana, la sobrevida libre de eventos e ILE fueron menores debido a la mortalidad relacionada con el tratamiento.

Introduction. Children with Down syndrome (DS) more commonly have acute lymphoblastic leukemia (ALL) and a lower survival rate than those without Down syndrome (WDS). We analyzed the clinical, demographic, and biological characteristics and treatment response of children with DS-ALL versus those WDS-ALL. Patients and methods: Patients with ALL between January 1990 and November 2016. The demographic and biologic characteristics and treatment response were compared using the χ² and Wilcoxon rank-sum tests. The overall survival and event-free interval (EFI) were analyzed using the Kaplan-Meier and log-rank tests. Results. 1795 patients were included; 54 had DS. Patients with DS-ALL were older (p= 0.0189). All had B-cell precursor immunophenotype and a lower incidence of recurrent abnormalities (p < 0.0001). They showed a better response rate to prednisone (p= 0.09) and a higher mortality in induction and complete remission (p < 0.0001). All deaths of patients with DS-ALL were treatment-related. The event-free survival (EFS) was 47% (± 8%) versus 73% (± 1%) (p= 0.006) and the EFI was 54% (± 9%) versus 75% (± 1%) (p= 0.0297) among patients with DS-ALL versus those WDS-ALL, respectively. The rate of relapse was similar in both groups (p= 0.6894). The EFI of patients with DS-ALL was lower in the group aged 6-9 years: 39% (± 19%) (p= 0.7885). Conclusions. A lower survival was observed among children aged 6-9 years with DS-ALL. Although these children showed a better early response, their EFS and EFI were lower due to treatment-related mortality.

Acute lymphoblastic leukemia in children with Down syndrome: Comparative analysis versus patients without Down syndrome. / Leucemia linfoblástica aguda en niños con síndrome de Down: análisis comparativo con pacientes sin síndrome de Down.

INTRODUCTION: Children with Down syndrome (DS) more commonly have acute lymphoblastic leukemia (ALL) and a lower survival rate than those without Down syndrome (WDS). We analyzed the clinical, demographic, and biological characteristics and treatment response of children with DS-ALL versus those WDS-ALL. Patients and methods: Patients with ALL between January 1990 and November 2016. The demographic and biologic characteristics and treatment response were compared using the χ² and Wilcoxon rank-sum tests. The overall survival and event-free interval (EFI) were analyzed using the Kaplan-Meier and log-rank tests. RESULTS: 1795 patients were included; 54 had DS. Patients with DS-ALL were older (p= 0.0189). All had B-cell precursor immunophenotype and a lower incidence of recurrent abnormalities (p < 0.0001). They showed a better response rate to prednisone (p= 0.09) and a higher mortality in induction and complete remission (p < 0.0001). All deaths of patients with DS-ALL were treatment-related. The event-free survival (EFS) was 47% (± 8%) versus 73% (± 1%) (p= 0.006) and the EFI was 54% (± 9%) versus 75% (± 1%) (p= 0.0297) among patients with DS-ALL versus those WDS-ALL, respectively. The rate of relapse was similar in both groups (p= 0.6894). The EFI of patients with DS-ALL was lower in the group aged 6-9 years: 39% (± 19%) (p= 0.7885). CONCLUSIONS: A lower survival was observed among children aged 6-9 years with DS-ALL. Although these children showed a better early response, their EFS and EFI were lower due to treatment-related mortality.

Introducción: Los niños con síndrome de Down (SD) tienen mayor frecuencia de leucemia linfoblástica aguda (LLA) y menor supervivencia que pacientes sin síndrome de Down (NSD). Analizamos las características clínicas, demográficas-biológicas y respuestas al tratamiento en SD-LLA versus NSD-LLA. Pacientes y métodos: Pacientes (0-19 años) con LLA desde enero de 1990 a noviembre de 2016. Se compararon características demográficas biológicas y respuestas al tratamiento con chi cuadrado y Wilcoxon rank sum. La supervivencia global y el intervalo libre de eventos (ILE) se analizaron con Kaplan-Meier y el test log-rank. Resultados: Se incluyeron 1795 pacientes, 54 con SD. Los SD-LLA presentaron edad mayor (p= 0,0189). Todos inmuno fenotipo precursor-B, con menor incidencia de anomalías recurrentes (p < 0,0001). Demostraron mejor tasa de respuesta a prednisona (p= 0,09) y mayor mortalidad en inducción y remisión completa (p < 0,0001). Todas las muertes de los SD-LLA fueron relacionadas con el tratamiento. La sobrevida libre de eventos en niños SD-LLA vs.NSD-LLA fue 47 (± 8)% vs. 73 (± 1)% (p= 0,006) y el ILE de los SD-LLA vs. NSD-LLA fue 54 (± 9)% vs. 75 (± 1)% (p= 0,0297). La tasa de recaídas fue similar en ambos grupos (p= 0,6894). El ILE de los SD-LLA fue menor en el grupo de 6-9 años: 39 (± 19)% (p= 0,7885). Conclusiones: Los niños de 6-9 años con SD-LLA años presentó menor sobrevida. Aunque estos niños presentaron una mejor respuesta temprana, la sobrevida libre de eventos e ILE fueron menores debido a la mortalidad relacionada con el tratamiento.

Children and Adolescent Hodgkin Lymphoma in Argentina: Long-term Results After Combined ABVD and Restricted Radiotherapy.

OBJECTIVE: Prospective analysis of clinical characteristics and long-term treatment results of a pediatric cohort with Hodgkin lymphoma (HL) treated in a single institution with ABVD and restricted radiotherapy (RT). PATIENTS AND METHODS: Between September 2000 and December 2015, 165 new consecutive assessable patients with HL were registered at our institution. Lymphocyte predominant nodular HL was excluded. Low risk (LR) patients were stage I and IIA (no bulky disease, <4 involved ganglionar areas and no lung hilar nodes), high risk (HR) was assigned to stage IV and any other stage with bulky mediastinum. The rest of the cohort was treated as intermediate risk (IR). Chemotherapy for LR and IR patients was 4 and 6 courses of ABVD regimen, respectively. These subsets received Low-dose involved field radiotherapy only in case of partial remission at the end of chemotherapy (21 Gy in initially involved areas, plus 14 Gy boost on residual disease). The HR group was treated with 6 courses of ABVD followed always with 21 Gy involved field radiotherapy if complete remission (CR) was achieved. A boost of 14 Gy was added to residual disease in case of partial remission. RESULTS: Median age was 10.6 years (range, 2.7 to 17 y). Males: 117 (71%); females: 48 (29%). Eighteen (11%) patients were stage I, 76 (46%) stage II, 35 (21%) stage III, and 35 (21%) stage IV. Forty-nine (30%) patients were assigned to LR, 49 (30%) to IR, and 67 (40%) to HR. Forty-three patients (26%) had "bulky" mediastinum involvement. One hundred thirty (79%) patients achieved CR after chemotherapy and 161 (98%) after RT. Four patients (all HR), did not respond to initial therapy and died of disease. One patient died in first CR due to adenovirus infection on previously therapy-related damaged lungs. Seventeen (10%) patients relapsed and 13 of them remained in second CR after further therapy. Seventy-six (46%) patients could be spared from RT and cured of disease (88% of LR patients and 67% of IR patients). With a median follow-up of 5 years, event free and overall survival were 0.84 (SE: 0.03) and 0.95 (SE: 0.02), respectively. Overall survival according to risk group was 1 for LR, 0.93 for IR, and 0.85 for HR. Acute toxicity and late effects due to therapy were not significant. CONCLUSIONS: The strategy of avoiding RT for LR and IR patients that responded completely to ABVD chemotherapy achieved very good results. For the HR group, the combination of 6 cycles of ABVD and Low-dose involved field radiotherapy was efficacious with similar good results. Nearly half of the patients could be cured without RT.

Second Neoplasms in Children Following a Treatment for Acute Leukemia and/or Lymphoma: 29 Years of Experience in a Single Institution in Argentina.

INTRODUCTION: Childhood acute leukemias (AL) and lymphomas achieve good survival rates. However, second neoplasms (SN) are a devastating event. METHODS: From August 1987 to December 2016, 34 of 3321 (1%) patients with diagnosis of AL or lymphoma developed SN. SN were AL (n=16), CNS tumors (n=5), endocrinal tumors (n=3), lymphomas (n=2), schwannoma (n=2) assorted sarcomas (n=4), retinal melanoma (n=1), and Vanek tumor (n=1). Median latency was 51 (range, 10 to 110) months for hematological malignancies and 119 (range, 25 to 236) months for solid tumors (P=0.001). RESULTS: A total of 33 patients with SN were treated taking into account cumulative doses of anthracyclines and radiotherapy. Twenty-three (67.6%) patients achieved complete remission (CR), 5 died early during therapy and 5 were refractory or partial responders. Six patients presented relapses of the SN and 1 died in CR. Seventeen patients remain alive in CR, with a median follow-up of 110 (range, 4 to 276) months. CONCLUSIONS: (1) The latency period was significantly longer for patients developing solid tumors than for those developing AL. (2) AL was the most frequent SN. (3) Our results strongly encourage giving standard therapy to SN, considering cumulative doses of previous treatment, since similar probabilities of surviving as "de novo" counterparts can be achieved.

Micosis fungoide. Experiencia en un hospital pediátrico / Mycosis Fungoides: Experience in a Pediatric Hospital

La micosis fungoide (MF) es el linfoma cutáneo primario de células T más frecuente. Su aparición en la infancia es excepcional. Objetivos: Describir las características epidemiológicas, clínicas, histopatológicas e inmunofenotípicas de los pacientes con MF. Describir los tratamientos utilizados y la evolución. Material y método: Se incluyeron todos los pacientes admitidos en el Hospital de Pediatría Dr. J. P. Garrahan (Argentina) en el período comprendido entre agosto de 1988 y julio de 2014 con diagnóstico clínico e histopatológico de MF. Resultados: Se diagnosticaron 14 pacientes con MF. La distribución por sexo fue M/F: 1:1,33. La edad media al diagnóstico fue de 11,23 años (rango: 8 a 15 años). El tiempo promedio de evolución hasta el momento del diagnóstico fue de 3 años y 6 meses (rango: 4 meses a 7 años). Todos los pacientes presentaron la forma clínica hipopigmentada y en el 42% se asoció la forma clásica. El 50% (n = 7) exhibió un inmunofenotipo CD8 positivo de forma exclusiva. El 78% presentó estadio IB. La fototerapia fue el tratamiento de elección. Cuatro pacientes tuvieron por lo menos una recaída y 3 demostraron progresión de su enfermedad a nivel cutáneo. La evolución fue favorable en todos los casos. Conclusiones: La MF es una entidad infrecuente en la infancia, siendo la forma hipopigmentada la más frecuente. Su diagnóstico es tardío debido a la similitud con otras enfermedades hipopigmentadas frecuentes en la niñez. A pesar de tener un buen pronóstico, presenta alta tasa de recidivas y requiere un seguimiento a largo plazo (AU)

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. Objectives: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. Material and method: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. Results: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. Conclusions: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary (AU)

Mycosis Fungoides: Experience in a Pediatric Hospital. / Micosis fungoide. Experiencia en un hospital pediátrico.

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. OBJECTIVES: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. MATERIAL AND METHOD: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. RESULTS: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. CONCLUSIONS: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary.

Series de Pediatría Garrahan. El niño con cáncer / Garrahan Pediatrics Series. The child with cancer

La sobrevida de los niños con enfermedades oncológicas ha aumentado de manera considerable en las últimas décadas y este logro se alcanzó, entre otros factores, gracias a la detección temprana de la patología, los avances en los métodos diagnósticos, la administración de terapéuticas específicas adaptadas al riesgo y la adecuada implementación de medidas de soporte. Sin embargo, el cuidado de estos pacientes sigue representando un difícil desafío y requiere la conformación de equipos en los que el pediatra cumple un rol fundamental en la atención conjunta con el oncólogo y en la coordinación de la intervención de los demás especialistas. Este nuevo volumen aborda esta interesante temática y entre sus características destacadas se encuentran: El estudio de importantes temas, como la prevención del cáncer en pediatría en el mundo y en la Argentina, y la necesidad de construir programas de integración, educación e investigación en el cáncer pediátrico; el niño con una masa abdominal, con sus estrategias diagnósticas y las eventuales urgencias metabólicas, infectológicas y nutricionales durante el período de inducción, y cómo anticiparlas y prevenirlas; las situaciones clínicas de riesgo, como la compresión medular, el síndrome de vena cava superior y las complicaciones asociadas con la utilización de irinotecán; los aspectos ginecológicos en las niñas con cáncer, como las conductas frente al riesgo de sangrado menstrual durante el período de inducción, la actividad sexual y el embarazo durante el tratamiento, y la preservación de la fertilidad; y la leucemia linfoblástica aguda en etapa de reinducción, período especialmente significativo por la elevada morbilidad y las dificultades en el soporte clínico que requieren estos pacientes. La inclusión en todos los capítulos de casos clínicos ejemplificadores con su evolución y desenlace, textos destacados con los conceptos más importantes y puntos clave para recordar. Una obra sólida y práctica, que transmite la experiencia de los profesionales de una institución del prestigio internacional del Hospital de Pediatría Prof. Dr. Juan P. Garrahan, dedicada a todos los pediatras dondequiera que trabajen al servicio de los niños.

Pharmacogenetic studies in children with acute lymphoblastic leukemia in Argentina.

The aim of this study was to evaluate the influence of the most common genetic variants in methylenetetrahydrofolate reductase (MTHFR), thiopurine methyltransferase (TPMT) and glutathione-S-transferases (GSTs) on the outcome of acute lymphoblastic leukemia (ALL) treatment in Argentinean children. Two hundred and eighty-six patients with ALL treated with two Berlin-Frankfurt-Münster (BFM)-based protocols were analyzed. Ten genetic variants were studied. Toxicity was evaluated during the consolidation phase. Children who received 2 g/m(2)/day of methotrexate and carried at least one 677T allele in MTHFR showed an increased risk of developing severe leukopenia (p = 0.004) and neutropenia (p = 0.003). Intermediate-risk (IR) patients with a heterozygous TPMT genotype had a higher probability of event-free survival than those with a wild-type genotype. Genotyping of MTHFR polymorphisms might be useful to optimize consolidation therapy, reducing the associated severe hematologic toxicity. Further studies are necessary to establish the usefulness of MTHFR and TPMT variants as additional markers to predict outcome in the IR group.

Cianosis diferencial inversa: ¿qué significa? / Reversed differential cyanosis: what does it mean?

La cianosis diferencial-término que indica una mejor oxigenación de la parte superior del cuerpo (preductal) respecto a la inferior (posductal)-es un hallazgo bien conocido en pediatría, y su causa más frecuente es la hipertensión pulmonar persistente del recién nacido. Por otro lado, hay casos excepcionales en los que se registra una cianosis diferencial inversa y la parte superior del cuerpo está peor oxigenada que la inferior. En este trabajo presentamos 2 casos clínicos con dicho hallazgo: un recién nacido con transposición de grandes vasos con hipertensión pulmonar y otro con drenaje venoso pulmonar anómalo total supracardiaco. Se revisa la bibliografía y se explica la fisiopatología de la cianosis diferencial inversa, demostrándose que es patognomónica de una cardiopatía congénita severa. Concluimos que la medición simultánea de la saturación en el territorio preductal y posductal mediante pulsioximetría debería ser siempre parte integrante de la valoración del recién nacido cianótico(AU)

Differential cyanosis -better oxygenation of the upper (preductal) part of the body with respect to the lower (postductal) part- is a well-known condition in pediatrics, being persistent pulmonary hypertension of the newborn its most common cause. On the other hand, reversed differential cyanosis (RDC) -upper body less oxygenated than the inferior- is a rare condition. This report describes two newborns presenting RDC: a case with transposition of the great arteries with pulmonary hypertension and a case with supracardiac total anomalous pulmonary vein connection. We review the existing literature and discuss its physiopathological bases, demonstrating that this condition is pathognomonic of severe congenital cardiopathy. Therefore, simultaneous preductal and postductal oxygen saturation should be always documented as part of the evaluation of the cyanotic newborn(AU)

Costes de una unidad de preparación de citostáticos en un hospital de México / No disponible

Objetivo: Cuantificar el costo de preparación de citostáticos en una unidad de mezclas intravenosas de un hospital en México.Método: Se estimó el costo anual de preparación de citostáticos con base en la información de 92 días de operación, considerando los costos por medicamentos, servicio de mezclado (incluye solución base, control de calidad, servicios y mermas) y salarios. Los costos se estimaron en pesos mexicanos de 2006.Resultados: El costo por citostático varía desde 82 hasta23.000 pesos mexicanos, dependiendo del tipo de fármaco utilizado.Se estimó el costo anual de preparación de quimioterapiasen 38.901.231,04 pesos mexicanos (2.839.505,92 €) distribuyéndose como sigue: un 96,8% por costo de medicamentos, un 1,21% por salarios del personal y un 1,99% por servicio de mezclado.Conclusiones: La cuantificación de los costos de preparación de citostáticos provee de una referencia para futuros estudios económicos en el área de farmacia hospitalaria en México

Method: The annual cost of preparing cytostatic drugs based on the information of 92 days, considering the costs of drugs, the mixing service (including standard solution, quality control, servicesand waste) and salaries were estimated. The costs are estimated in Mexican pesos in 2006.Results: The cost per cytostatic drug varies from 82 to 23,000 Mexican pesos, depending on the type of drug used. It is estimated that the annual cost of preparing drugs for chemotherapy is 38,901,231.04 Mexican pesos (2,839,505.02 €) distributed as follows:96.8% for drug costs, 1.21% for staff salaries and 1.99% for the preparation service. Conclusions: The estimation of the costs of preparing cytostatic drugs serves as a reference for future economic studies in the hospital pharmacy area in Mexico

Enfermedades emergentes y reemergentes. Una visión actual / Emerging and reemerging diseases - An uo-dated view

El concepto de Enfermedades Infecciosas Emergentes fue acuñado en 1992 por el Instituto de Medicina de los EEUU, para referirse a las enfermedades infecciosas descubiertas en los últimos años y a las ya conocidas consideradas controladas, en franco descenso o casi desaparecidas, que volvieron a emerger. En el pasado, las enfermedades transmisibles fueron una importante causa de morbimortalidad, pero el descubrimiento en el último tercio del siglo XIX de sus agentes causales y el conocimiento de los reservorios, fuentes de infección, mecanismos de transmisión y factores de susceptibilidad llevaron al establecimiento de medidas preventivas con base científica que permitieron, conjuntamente con el desarrollo socioeconómico, disminuir de manera importante su incidencia y sobre todo su mortalidad en los países desarrollados. En los años 70 y 80 disminuyó el interés y la atención prestada a las enfermedades transmisibles por los funcionarios de Salud Pública, médicos e investigadores, convirtiéndose las enfermedades crónicas degenerativas en el centro de su atención. Los resultados netos de estos cambios fueron una disminución en conjunto de los programas frente a las enfermedades transmisibles; el deterioro de los esfuerzos de vigilancia, olvidados en la priorización de las asignaciones presupuestarias y una disminución de la pericia técnica frente a las enfermedades infecciosas tradicionales(AU)

Estrategias de tratamiento de la rinoconjuntivitis y el asma durante el embarazo / Treatment strategies in rhinoconjunctivitis and asthma during pregnancy

Introducción: El asma es una enfermedad con alta incidencia sobre todo en gente joven, grupo de población al que pertenecen las mujeres en edad fértil. Hemos revisado los últimos artículos sobre el tema para poder mantener el asma bien controlada y así evitar efectos no deseados tanto en la madre como en el feto. Igualmente se aborda el control de la rino-conjuntivitis, tan incapacitante para multitud de personas y tan olvidada e infratratada por muchos profesionales. Intentamos dar una perspectiva que nos parece razonable para el uso de medicación como β -adrenérgicos, corticoides (sistémicos/inhalados/nebulizados), epinefrina e inmunoterapia específica con alergenos inhalantes. Material y métodos: Hemos revisado los últimos artículos sobre el tema y los que nos han parecido más relevantes. Además no sólo nos hemos centrado en el aspecto farmacológico, sino que hemos tratado de buscar aquellos textos donde se da también una estrategia de tratamiento global, sin descuidar la educación en el manejo de su enfermedad. En esta revisión se comprueba que existe medicación suficiente para el control de estas enfermedades por lo que no hay motivo para infratratar. Conclusiones: El embarazo no es una enfermedad, sino un momento en la vida de una mujer en el que hay que tener mayor control del habitual sobre su patología de base. El objetivo del tratamiento del asma en el embarazo es que la paciente esté libre de síntomas y prevenir las posibles exacerbaciones, para así evitar un mayor daño fetal, tanto por la medicación que se necesita, como por los efectos de la crisis sobre el feto. Es importante destacar que casi todos los autores coinciden en que una crisis asmática en una mujer embarazada se trata igual que en una paciente no embarazada. No hay que dejar de tratar la rino-conjuntivitis por parecer una patología menor ya que se dispone de un importante arsenal terapéutico catalogado como de categoría B por la FDA. La inmunoterapia específica con alergenos no está contraindicada en el embarazo, ni hay que suspenderla, pero no está recomendado iniciarlo durante el mismo

Background: The incidence of asthma is high, especially in young people, a population group that includes women of reproductive age. We reviewed recent publications on asthma control during pregnancy to avoid undesired effects on both the mother and fetus. The prevalence of rhinoconjunctivitis is also high, although this disease is often under-treated by physicians. The use of β2-agonists, corticoids (systemic/inhaled/nebulized), epinephrine and specific allergen immunotherapy is discussed. Methods: We reviewed recent publications on asthma during pregnancy as well as other articles of interest. Articles providing data on drug therapy, overall strategies and patient education were selected. Sufficient drugs are available for the management of this disease and under-treatment cannot be justified. Conclusions: Pregnancy is not a disease, but constitutes a period when special care must be taken with underlying diseases. The aim of asthma treatment during pregnancy is to prevent fetal complications due to the effects of medication and asthma crises by keeping the mother symptom free and preventing possible exacerbations. Almost all authors agree that asthma crises in pregnant women should be treated no differently from those in non-pregnant women. Treatment of rhinoconjunctivitis should not be stopped during pregnancy since a wide variety of FDA category B drugs is available. Specific allergen immunotherapy should not be suspended during pregnancy as it is not contraindicated. However, this therapy should not be initiated during pregnancy

Neuroblastoma: primitivo oculto. Descripción de casos / Neuroblastoma with occult primary. Description of 2 cases

El neuroblastoma es el tumor extracraneal más frecuente en la infancia representando aproximadamente un 8 por ciento de las enfermedades malignas. Se analizó la forma de presentación clínica, evolución respuesta al tratamiento de dos pacientes que presentaron como diagnóstico neuroblastoma con tumor primitivo oculto. La forma de presentación de estos pacientes fue atípica. Caracterizada por la presencia de dolores óseos, radiografía de huesos largos patológicas y aspirado de médula ósea con infiltración neoplásica, sin evidencia de tumor tanto en examen clínico como en los estudios por imágenes. En niños, las neoplasias sólidas de origen desconocido correponden a menos del 1 por ciento del total, siendo esta incidencia algo mayor en el adulto. Un interrogatorio adecuado y dirigido, la evaluación radiológica correcta, la determinación de catecolaminas urinarias y el medulograma son herramientas útiles para arribar al diagnóstico en las presentaciones poco habituales de esta enfermedad.

Retención de un poste cilindrocónico. Estudio comparativo de seis cementos y dos formas de cementación distintas / No disponible

Propósito: La mayoría de los diferentes tipos de postes (..) (AU)

Purpose: Many different types post recommended for clinical (..) (AU)

Toxicidad pancreática de los anthimoniales en pacientes infectados por el VIH / Pancreatic toxicity of antimonials in patients with HIV infection

No disponible