Hipofibrinogenemia inducida por prednisona en un paciente con leucemia linfoblástica aguda / Hypofrinogenemia induced by prednisone in a case of acute lymphoblastic leukemia

La hipofibrinogenemia es una complicación infrecuente pero bien conocida en el contexto de las leucemias agudas. Generalmente, se asocia a la presencia de una coagulación intravascular diseminada, aunque pueden existir otras causas, como el daño hepático o el empleo de L-asparraginasa. La literatura recoge pocos casos en que esta alteración de la coagulación esté inducida por corticoides, como el aquí presentado. Se expone el caso de un niño diagnosticado de leucemia linfoblástica aguda que desarrolló hipofibrinogenemia tras iniciar tratamiento con prednisona, y se ha realizado una revisión bibliográfica a propósito del caso (AU)

Hypofibrinogenemia is an uncommon but well-recognized complication of acute leukaemia. In most instances, it has been associated with disseminated intravascular coagulation, although other causes as liver injury or treatment with L-asparraginase could exist. The literature includes rare cases in which this coagulation disorder is induced by prednisone as our report. We report the case of a child diagnosed of acute lymphoblastic leukemia with hypofibrinogenemia during the course of treatment with prednisone and we have reviewed the literature concerning the case (AU)

Osteoartropatía hipertrófica y enfermedad de Hodgkin / Hypertrophic osteoarthropathy and Hodgkin's disease

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[Osteosarcoma and ATR-16 syndrome: association or coincidence?]. / Osteosarcoma y síndrome ATR-16, ¿asociación o coincidencia?

ATR-16 syndrome is due to alterations on chromosome 16p13.3, and is usually accompanied by alpha-thalassemia, mild-moderate mental retardation, dysmorphic facial features, skeletal and genitourinary malformations. There are no references of the combination of ATR-16 syndrome and osteosarcoma in the literature. Osteosarcoma usually has a complex karyotype, characterized by a high degree of heterogeneity of chromosomal aberrations, among which is the involvement of chromosome 16. We report a case of a patient with ATR-16 syndrome diagnosed with femoral osteosarcoma.

Fallo hepático agudo neonatal: presentación de 2 casos y revisión de la bibliografía / Acute neonatal liver failure: two case studies and literature review

El fallo hepático agudo neonatal es una entidad poco frecuente, con una mortalidad muy elevada. La sospecha de fallo hepático ante situaciones de mal estado general y disfunción hepática, hipoglucemia recurrente o persistente y clínica de sepsis es fundamental para establecer un tratamiento precoz y efectivo. Existen pruebas de primera y segunda línea para poder orientar un diagnóstico etiológico, a la vez que se instauran medidas generales que permitan su estabilización, antes de considerar otras medidas terapéuticas, como el trasplante hepático. Presentamos el inicio y la evolución de 2 casos de fallo hepático neonatal diagnosticados de hemocromatosis y linfohistiocitosis hemofagocítica familiar (AU)

Acute neonatal liver failure is a rare entity with a high mortality rate. In order to establish an early and effective treatment, a high index of suspicion in newborns with a poor general condition and hepatic dysfunction, recurrent or persistent hypoglycaemia and signs and symptoms of sepsis, is critical. There are first- and second-line test to establish the aetiology, parallel to the implementation of general stabilization measures, and prior to the consideration of other therapeutic options such as liver transplantation. We present the onset and the outcome of two patients affected from acute neonatal liver failure that were diagnosed of hemochromatosis and hemophagocytic lymphohistiocytosis, respectively (AU)

Supraglotitis atípica en la era postepiglotitis / Atypical supraglottitis in postepiglottitis era

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Evaluación sistemática de la actuación de los pediatras ante un caso simulado de taquicardia supraventricular / Systematic assessment of the paediatrician’s performance during simulated supraventricular tachycardia

Objetivo: El presente estudio persigue dos objetivos: a) analizar mediante una lista estructurada de tareas la calidad del manejo clínico en un escenario simulado de la taquicardia supraventricular (TSV) aguda y b) identificar posibles errores y áreas de mejora sobre las que incidir en el entrenamiento. Material y métodos: Se han revisado y valorado de forma sistemática los escenarios simulados de TSV realizados en los cursos de simulación avanzada pediátrica llevados a cabo entre junio2008 y abril 2010. Se programaron en el sistema SimBaby(R) tres escenarios de dificultad creciente: TSV estable (TSV-E), TSV inicialmente estable que progresa a inestable (TSV-EI) y TSV inestable (TSV-I). La evaluación del escenario se basó en una lista de 18 tareas derivadas de las recomendaciones del ILCOR. Resultados: Se analizaron 45 escenarios (15 escenarios de TSV-E, 25 de TSV e-I y 5 de TSVI),en los que participaron 167 pediatras. Se completaron correctamente 328 de 551 (59,5%) tareas posibles. El porcentaje medio (desviación estándar) de tareas correctas por escenario varió en función del caso: 63,4 (16,7) en la TSV-E, 47,8 (20,3) en la TSV-EI y 38,6 (31) en la TSV-I (p = 0,028). No se observaron diferencias significativas entre los pediatras de atención primaria y los de atención hospitalaria. La mayoría de los participantes diagnosticaron correctamente la TSV; sin embargo, se cometieron errores importantes como no identificar la inestabilidad hemodinámica en 20 de 43 (48%) escenarios, dosis incorrectas de adenosina en 18 de 39 (48%) escenarios, la administración inadecuada de adenosina en 23 de 39 (59%) escenarios y no reconocer la indicación de cardioversión inmediata en 15 de 31 (48%) escenarios. Conclusiones: Los pediatras saben identificar una TSV pero precisan mejorar su capacitación para tratarla de forma adecuada. El análisis sistemático de la actuación de los profesionales ante un caso simulado permite detectar tanto sus puntos fuertes como las áreas en las que es preciso reforzar la enseñanza (AU)

Introduction: The aims of this study are to: a) assess the quality in clinical management during a simulated scenario of acute supraventricular tachycardia (SVT) by means of a structured task-based checklist and to b) detect pitfalls and grey areas where reinforcement in training maybe needed. Material and methods: We systematically reviewed SVT simulated scenarios during simulation courses between June 2008 and April 2010. Three scenarios were programmed using SimBaby(R) simulation system, and included stable SVT (S-SVT), stable progressing to unstable SVT (SU-SVT) and unstable SVT (U-SVT). Scenarios were evaluated by means of an 18-task checklist based on ILCOR international recommendations. Results: A total of 45 scenarios were assessed with the participation of 167 paediatricians, including 15 S-SVT, 25 SU-SVT and 5 U-SVT scenarios. Out of a total of 551 possible tasks, 328 (59.5%) were completed correctly. The mean percentage of correct tasks per scenario was 63.4 (16.7) for S-SVT, 47.8 (20.3) for SU-ST and 38.6 (31) for U-SVT (p = 0.028). There were no significant differences between primary care paediatricians and hospital paediatricians. Most of the participants correctly identified non-sinus rhythm as SVT. However, important pitfalls wereobserved, including failure to identify haemodynamic instability in 20 out of 43 (48%) cases, an incorrect dose of adenosine in 18 out of 39 (48%), incorrect adenosine administration in 23 out of 39 (59%), and non-recognition of indication to emergent cardio version in 15 out of 31 (48%).Conclusions: Paediatricians are able to diagnose SVT correctly, but need to improve their skills in treatment. Systematic analysis of clinical performance in a simulated scenario allows the identification of strengths, as well as weak points, where reinforcement is needed(AU)

[Systematic assessment of the paediatrician's performance during simulated supraventricular tachycardia]. / Evaluación sistemática de la actuación de los pediatras ante un caso simulado de taquicardia supraventricular.

INTRODUCTION: The aims of this study are to: a) assess the quality in clinical management during a simulated scenario of acute supraventricular tachycardia (SVT) by means of a structured task-based checklist and to b) detect pitfalls and grey areas where reinforcement in training may be needed. MATERIAL AND METHODS: We systematically reviewed SVT simulated scenarios during simulation courses between June 2008 and April 2010. Three scenarios were programmed using SimBaby® simulation system, and included stable SVT (S-SVT), stable progressing to unstable SVT (SU-SVT) and unstable SVT (U-SVT). Scenarios were evaluated by means of an 18-task checklist based on ILCOR international recommendations. RESULTS: A total of 45 scenarios were assessed with the participation of 167 paediatricians, including 15 S-SVT, 25 SU-SVT and 5 U-SVT scenarios. Out of a total of 551 possible tasks, 328 (59.5%) were completed correctly. The mean percentage of correct tasks per scenario was 63.4 (16.7) for S-SVT, 47.8 (20.3) for SU-ST and 38.6 (31) for U-SVT (p=0.028). There were no significant differences between primary care paediatricians and hospital paediatricians. Most of the participants correctly identified non-sinus rhythm as SVT. However, important pitfalls were observed, including failure to identify haemodynamic instability in 20 out of 43 (48%) cases, an incorrect dose of adenosine in 18 out of 39 (48%), incorrect adenosine administration in 23 out of 39 (59%), and non-recognition of indication to emergent cardioversion in 15 out of 31 (48%). CONCLUSIONS: Paediatricians are able to diagnose SVT correctly, but need to improve their skills in treatment. Systematic analysis of clinical performance in a simulated scenario allows the identification of strengths, as well as weak points, where reinforcement is needed.

Incidence of complications and mortality in a type 2 diabetes patient cohort study followed up from diagnosis in a primary healthcare centre.

AIMS: To determine the microvascular and macrovascular complications and mortality incidence rates and to identify the related factors in patients recently diagnosed with type 2 diabetes between 1991 and 2000 and followed until 2006. METHODS: Retrospective longitudinal study in a primary healthcare center. Patients without any measure of glycaemia in the 3 years previous to diabetes diagnosis were excluded. Annual incidence rates for microvascular and macrovascular complications and mortality were estimated. Analysis of KaplanMeier survival curves and Cox proportional risk models by gender were done. RESULTS: Of 469 patients [mean age: 60.4 (SD 10.7) years, 53.9% women], 80 died principally of tumoral (38.7%) and cardiovascular (30%) causes. The mean follow-up period was 8.81 years. (SD 3.21). The complication rates per 1000 patients/year (95% CI) were: microvascular complications 29.11 (22.97-36.38), macrovascular complications 24.10 (19.05-30.08) and mortality 19.23 (15.25-23.93), all of those being significantly greater in males except for cerebrovascular disease. Complications and mortality were associated with age, HbA1c, HDL-cholesterol, blood pressure and smoking with a different significance for each gender. HbA1c was related to microvascular complications in both sexes and to macrovascular complications only in women. CONCLUSION: The annual rates for death and complications in a Mediterranean type 2 diabetic patient cohort followed from diagnosis were lower than those published in Anglo-Saxon countries. Males showed higher death and complication rates except in terms of cerebrovascular disease. Predictors of complication and death were different depending on gender. In terms of mortality, unlike in other studies, only one-third of the deaths were for cardiovascular causes.

Simulación avanzada para pediatras de atención primaria. Desarrollo de un programa itinerante y opinión de los participantes / Advanced simulation for primary care paediatricians. Development of an itinerant program and opinions of participants

Introducción y objetivos: La simulación avanzada (SA) es una metodología docente útil para la capacitación de profesionales en el ámbito hospitalario, con aplicación limitada en atención primaria (AP). Nuestro objetivo ha sido desarrollar un proyecto de SA orientado a los pediatras de AP y conocer las opiniones de los alumnos como uno de los elementos para valorar su idoneidad. Material y métodos: Fase 1: Se organizó un grupo de trabajo multidisciplinario, auspiciado por la Sociedad Española de Pediatría de Atención Primaria, para diseñar y poner en práctica el curso. Fase 2: Se pusieron en marcha cursos itinerantes por distintas ciudades españolas. Al finalizar cada curso se realizó una encuesta anónima de opinión, centrada en los aspectos motivacionales y la adecuación del programa y la metodología. Cada ítem se puntuó de 0 (muy mal) a 10 (muy bien). Período de estudio: mayo de 2008 a mayo de 2009. Resultados: Tras analizar las necesidades formativas de la población diana, el tiempo disponible, el material docente existente y la metodología de simulación y análisis de casos, se diseñó un modelo de curso y se programaron los casos. Se realizaron 12 cursos en 12 ciudades. Asistieron 186 profesionales y 177 (95,2%) contestaron la encuesta. La puntuación media de los ítems principales fue la siguiente: organización general (9,23±0,50), objetivos en relación con las expectativas (9,29±0,43), curso útil para la situación laboral (9,42±0,43), casos cercanos a la realidad laboral (9,18±0,42) y buena relación docentes-alumnos (9,68±0,20). Conclusiones: El curso de SA para pediatría de AP, con el formato propuesto, es factible y se adapta a las necesidades de la población diana. Los pediatras de AP consideran a esta enseñanza una herramienta útil para su formación continuada y la mejora de su capacitación (AU)

Introduction and objectives: Advanced simulation (AS) is a teaching methodology that has shown to be useful for training health staff at hospital level; however, its application in primary care paediatrics is very limited. Our objective was the development of an AS project focused on the learning needs of the primary care paediatricians, as well as to know the participants’ opinions as one of the elements to assess its appropriateness. Material and methods: Phase 1: A multidisciplinary working group was organized and sponsored by the SEPEAP to design and put the course into practice. Phase 2: Itinerant courses were carried out in several cities in Spain. At the end of each course, a survey was carried out that was focused on motivation aspects, pertinence of contents and methodology. Each item was scored on a numerical scale from 0 (very bad) to 10 (very good). Study period: May 2008 to May 2009. Results: After analysing the potential learning needs of target population, available time, teaching material available and methodology of simulation and debriefing, a course model was designed and the cases were programmed. Twelve courses were carried out in 12 cities. The total number of participants was 186; of them, 177 (95.2%) answered the survey. Mean±SD scores for main items were: organization (9.23±0.50), objectives related to prior expectation (9.29±0.43), usefulness of course program to work activity (9.42±0.43), cases that resemble reality (9.18±0.42) and good instructors-participants relationship (9.68±0.20). Conclusions: The AS course for primary care paediatrics, with the proposed format, is feasible and well adapted to the needs of the target population. Primary care paediatricians consider this type of teaching and learning activity as a useful tool for their continuing education and for improving their professional abilities (AU)

[Advanced simulation for primary care paediatricians. Development of an itinerant program and opinions of participants]. / Simulación avanzada para pediatras de atención primaria. Desarrollo de un programa itinerante y opinión de los participantes.

INTRODUCTION AND OBJECTIVES: Advanced simulation (AS) is a teaching methodology that has shown to be useful for training health staff at hospital level; however, its application in primary care paediatrics is very limited. Our objective was the development of an AS project focused on the learning needs of the primary care paediatricians, as well as to know the participants' opinions as one of the elements to assess its appropriateness. MATERIAL AND METHODS: Phase 1: A multidisciplinary working group was organized and sponsored by the SEPEAP to design and put the course into practice. Phase 2: Itinerant courses were carried out in several cities in Spain. At the end of each course, a survey was carried out that was focused on motivation aspects, pertinence of contents and methodology. Each item was scored on a numerical scale from 0 (very bad) to 10 (very good). STUDY PERIOD: May 2008 to May 2009. RESULTS: After analysing the potential learning needs of target population, available time, teaching material available and methodology of simulation and debriefing, a course model was designed and the cases were programmed. Twelve courses were carried out in 12 cities. The total number of participants was 186; of them, 177 (95.2%) answered the survey. Mean+/-SD scores for main items were: organization (9.23+/-0.50), objectives related to prior expectation (9.29+/-0.43), usefulness of course program to work activity (9.42+/-0.43), cases that resemble reality (9.18+/-0.42) and good instructors-participants relationship (9.68+/-0.20). CONCLUSIONS: The AS course for primary care paediatrics, with the proposed format, is feasible and well adapted to the needs of the target population. Primary care paediatricians consider this type of teaching and learning activity as a useful tool for their continuing education and for improving their professional abilities.

[Burnout syndrome among health workers in pediatrics]. / Síndrome de desgaste profesional en el personal asistencial pediátrico.

BACKGROUND: Burnout syndrome (BS) is a chronic adaptation disorder that provokes serious problems in occupational behavior. OBJECTIVE: In the present study we assessed the prevalence of burnout syndrome in pediatric healthcare workers. DESIGN: Cross-sectional, descriptive study. MATERIAL AND METHODS: In November 2002, the Maslach Burnout Inventory was given to 127 staff members (doctors, nurses and nurse assistants) working in a pediatrics department. The questionnaire includes 22 items that explore three aspects of BS: a) emotional exhaustion; b) depersonalization, and c) personal achievement. RESULTS: The questionnaire was completed by 93 staff members (73.2 % of the whole sample; 83 % women and 17 % men). The mean age was 38.6 +/- 10.2 years and the median number of years worked was 10 (range: 1-37 years). Twenty-nine percent were pediatricians, 50.5 % were nurses and 20.5 % were nurse assistants. A total of 20.8 % had a high level of BS, 19.8 % had a moderate level and 59.4 % showed a low level. When sub-scales were applied, the results showed that 67.7 % of respondents presented a low level of personal achievement, 14.5 % had high scores of emotional exhaustion and 23.9 % obtained high scores in the depersonalization scale. CONCLUSIONS: BS is present in a significant percentage of hospital workers attending pediatric patients. In our sample, the most notable component of BS was the lack of personal achievement. This finding alerted us to the eventual presence of negative attitudes toward self and professional activity, as well as to the loss of interest in pediatric care, low productivity and diminished self-esteem. We believe that specific strategies should be implemented to attenuate the factors influencing the development of BS in pediatric health staff.

Síndrome de desgaste profesional en el personal asistencial pediátrico / Burnout syndrome among health workers in pediatrics

Antecedentes: El síndrome de desgaste profesional es un trastorno adaptativo crónico que condiciona alteraciones importantes de la conducta laboral. Objetivo: En este trabajo se ha pretendido conocer su prevalencia entre el personal asistencial pediátrico, para la cual se diseñó un estudio transversal y descriptivo. Material y métodos En noviembre de 2002 se presentó el cuestionario Maslach Burnout Inventory (MBI) a 127 personas (médicos, enfermeras y auxiliares) que trabajan en un Departamento de Pediatría. El cuestionario consta de 22 ítems que exploran tres aspectos del síndrome de desgaste profesional: a) cansancio emocional; b) despersonalización, y c) realización personal. Resultados: El cuestionario fue contestado por 93 personas (tasa de respuesta: 73,2 %). El 83 % de las encuestas fueron cumplimentadas por mujeres y el 17 % por varones. La media de edad fue de 38,6 ± 10,2 años y la mediana de antigüedad en el puesto de trabajo de 10 años (límites, 1-37 años). El 29,0 % eran pediatras, el 50,5 %, personal de enfermería, y el 20,5 %, auxiliares de enfermería. Según la escala utilizada, el 20,8 % de los entrevistados presentaban un alto grado de síndrome de desgaste profesional, el 19,8 % un nivel moderado y el 59,4 % un nivel bajo de síndrome de desgaste profesional. En relación con las subescalas del cuestionario, el 67,7 % de los encuestados presenta un bajo grado de realización personal, el 14,5 % obtiene puntuaciones altas de cansancio emocional y el 23,9% tiene valores altos en la escala de despersonalización. Conclusiones: El síndrome de desgaste profesional es un trastorno presente en un porcentaje considerable de las personas que atienden a pacientes pediátricos a nivel hospitalario. En la muestra estudiada, la falta de realización personal se destaca sobre los demás componentes del síndrome, lo que alerta sobre la posible existencia de actitudes negativas hacia uno mismo y la actividad laboral, pérdida de interés por la atención a los niños, baja productividad y disminución de la autoestima. Por lo tanto, deberían tomarse medidas para atenuar los factores que influyen en el desarrollo del síndrome de desgaste profesional entre el personal asistencial pediátrico

Background: Burnout syndrome (BS) is a chronic adaptation disorder that provokes serious problems in occupational behavior. Objective: In the present study we assessed the prevalence of burnout syndrome in pediatric healthcare workers. Design: Cross-sectional, descriptive study. Material and methods In November 2002, the Maslach Burnout Inventory was given to 127 staff members (doctors, nurses and nurse assistants) working in a pediatrics department. The questionnaire includes 22 items that explore three aspects of BS: a) emotional exhaustion; b) depersonalization, and c) personal achievement. Results: The questionnaire was completed by 93 staff members (73.2 % of the whole sample; 83% women and 17% men). The mean age was 38.6 +/- 10.2 years and the median number of years worked was 10 (range: 1-37 years). Twenty-nine percent were pediatricians, 50.5% were nurses and 20.5% were nurse assistants. A total of 20.8 % had a high level of BS, 19.8 % had a moderate level and 59.4 % showed a low level. When sub-scales were applied, the results showed that 67.7 % of respondents presented a low level of personal achievement, 14.5 % had high scores of emotional exhaustion and 23.9 % obtained high scores in the depersonalization scale. Conclusions: BS is present in a significant percentage of hospital workers attending pediatric patients. In our sample, the most notable component of BS was the lack of personal achievement. This finding alerted us to the eventual presence of negative attitudes toward self and professional activity, as well as to the loss of interest in pediatric care, low productivity and diminished self-esteem. We believe that specific strategies should be implemented to attenuate the factors influencing the development of BS in pediatric health staff

[Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans]. / Proteína C activada humana recombinante en el tratamiento de niños con púrpura fulminante meningocócica.

Meningococcal purpura fulminans (MPF) produces high mortality and morbidity, despite appropriate standard therapy. Administration of recombinant human activated protein C (rhAPC) has been successfully applied in adults with MPF and pediatric studies are under way. We report three pediatric patients with MPF treated with rhAPC as compassionate therapy. In two of these patients, positive clinical and laboratory effects were observed and both children achieved full recovery. The remaining patient died after 36 hours from refractory multiorgan failure. No rhAPC-related adverse effects were detected. The reported cases highlight the usefulness of rhAPC in children with MPF at least as a rescue compassionate treatment. Further clinical trials are needed to better delineate its efficacy and administration schedule in children.

Proteína C activada humana recombinante en el tratamiento de niños con púrpura fulminante meningocócica / Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans

La púrpura fulminante meningocócica (PFM) es una enfermedad con una elevada morbimortalidad a pesar de un tratamiento antibiótico y soporte vital adecuados. La proteína C activada humana recombinante (PCAhr) se ha utilizado con éxito en el tratamiento de pacientes adultos con PFM y actualmente están en marcha estudios para evaluar su utilidad en pacientes pediátricos. Se presentan 3 niños con PFM que recibieron PCAhr como tratamiento compasivo. En dos de ellos, se observaron efectos beneficiosos clínicos y analíticos, en los cuales se logró su recuperación sin secuelas. El tercer paciente falleció transcurridas 36 h, debido a un fracaso multiorgánico refractario. En ninguno de los casos se detectaron efectos adversos relacionables con la PCAhr. Los casos descritos señalan la utilidad de la PCAhr en niños con PFM, al menos como tratamiento de rescate y compasivo. Son necesarios ensayos clínicos que evalúen su eficacia y definan las pautas de tratamiento en niños (AU)