A Randomized, Double-Blind, Placebo-Controlled Trial (TAURCAT Study) of Citrate Lock Solution for Prevention of Endoluminal Central Venous Catheter Infection in Neutropenic Hematological Patients.

Infection of long-term central venous catheters (CVCs) remains a challenge in the clinical management of cancer patients. We aimed to determine whether a lock solution with taurolidine-citrate-heparin would be more effective than placebo for preventing nontunneled CVC infection in high-risk neutropenic hematologic patients. We performed a prospective, multicenter, randomized (1:1), double-blind, parallel, superiority, placebo-controlled trial involving 150 hematological patients with neutropenia carrying nontunneled CVCs who were assigned to receive CVC lock solution with taurolidine-citrate-heparin or heparin alone. The primary endpoint was bacterial colonization of the CVC hubs. Secondary endpoints were the incidence of catheter-related bloodstream infection (CRBSI), CVC removal, adverse events related to the lock solution, and the 30-day case fatality rate. CVC lock solution with taurolidine-citrate-heparin was associated with less colonization of the CVC hubs than that with placebo, with no statistically significant differences: 4.1%, versus 10.1% (relative risk [RR] = 0.41, 95% confidence interval [CI] = 0.11 to 1.52), with a cumulative incidence of 4.17 (95% CI = 0.87 to 11.70) and 10.14 (95% CI = 4.18 to 19.79), respectively. There were no significant differences regarding the secondary endpoints. Only three episodes of CRBSI occurred during the study period. No adverse events related to the administration of the lock solution occurred. In this trial involving high-risk patients carrying nontunneled CVCs, the use of taurolidine-citrate-heparin did not show a benefit over the use of placebo. Nevertheless, the safety of this prevention strategy and the trend toward less hub colonization in the taurolidine-citrate-heparin group raise the interest in assessing its efficacy in centers with higher rates of CRBSI. (This study has been registered in ISRCTN under identifier ISRCTN47102251.).

Immediate Effects of Dasatinib on the Migration and Redistribution of Naïve and Memory Lymphocytes Associated With Lymphocytosis in Chronic Myeloid Leukemia Patients.

Introduction: Dasatinib is a dual SRC/ABL tyrosine kinase inhibitor used to treat chronic myeloid leukemia (CML) that is known to have unique immunomodulatory effects. In particular, dasatinib intake typically causes lymphocytosis, which has been linked to better clinical response. Since the underlying mechanisms are unknown and SRC family kinases are involved in many cell motility processes, we hypothesized that the movement and migration of lymphocytes is modulated by dasatinib. Patients, Materials and Methods: Peripheral blood samples from CML patients treated with second-line dasatinib were collected before and 2 h after the first dasatinib intake, and follow-up samples from the same patients 3 and 6 months after the start of therapy. The migratory capacity and phenotype of lymphocytes and differential blood counts before and after drug intake were compared for all study time-points. Results: We report here for the first time that dasatinib intake is associated with inhibition of peripheral blood T-cell migration toward the homeostatic chemokines CCL19 and CCL21, which control the trafficking toward secondary lymphoid organs, mainly the lymph nodes. Accordingly, the proportion of lymphocytes in blood expressing CCR7, the chemokine receptor for both CCL19 and CCL21, decreased after the intake including both naïve CD45RA+ and central memory CD45RO+ T-cells. Similarly, naïve B-cells diminished with dasatinib. Finally, such changes in the migratory patterns did not occur in those patients whose lymphocyte counts remained unchanged after taking the drug. Discussion: We, therefore, conclude that lymphocytosis induced by dasatinib reflects a pronounced redistribution of naïve and memory populations of all lymphocyte subsets including CD4+ and CD8+ T-cells and B-cells.

Variables motivacionales y cognitivas predictivas del rendimiento en estudiantes universitarios de ciencias de la salud / Cognitive and Motivational Predictive Variables Associated with Performance in University Students of Health Sciences

Introducción: La motivación, el aprendizaje y la ejecución, presentan una relación recíproca según las últimas investigaciones en aprendizaje autorregulado. La motivación influye en el aprendizaje, los estudiantes organizan y elaboran mejor los materiales, se esfuerzan más, piden ayuda con más frecuencia y en definitiva están más comprometidos con la tarea académica. Por otro lado, la ejecución y lo que los estudiantes aprenden y realizan, puede, a su vez, aumentar o disminuir la motivación, a través de variables como la autoeficacia. Objetivo: Identificar qué variables motivacionales y cognitivas predicen el rendimiento académico y como lo hacen. Métodos: Se realizó un estudio descriptivo e inferencial con una muestra de 508 estudiantes de ciencias de la salud pertenecientes a dos universidades privadas españolas, a la que se evalúa a través del Motivated Strategies For Learning Questionnaire, la utilización de estrategias cognitivas, metacognitivas, de gestión de recursos y motivacionales; después, se realizó un modelo de regresión, tomando estas estrategias como variables independientes y el rendimiento medido a través de las notas de los estudiantes como variable dependiente. Resultados: La autoeficacia para el rendimiento fue la variable que mejor predijo el rendimiento académico en los estudiantes universitarios de la muestra. Otras variables que también se relacionaron con el rendimiento fueron la gestión del tiempo y el lugar, la búsqueda de ayuda y la meta de autoensalzamiento. Conclusiones: Los resultados académicos dependen de los métodos de evaluación, del uso de estrategias cognitivas, metacognitivas, y motivacionales adecuadas al contexto instruccional, y de una mejor gestión de los recursos de aprendizaje. Estos hallazgos nos orientan a los docentes a proponer intervenciones educativas dirigidas a aumentar el uso de estrategias motivacionales y de aprendizaje en estudiantes universitarios(AU)

Introduction: Motivation, learning and practice have a reciprocal relationship, according to latest research in self-regulated learning. Motivation influences learning, students organize and elaborate materials better, make greater effort, ask for help more frequently, and ultimately are more committed to the academic task. On the other hand, the execution and what students learn and do in practice, can in turn increase or decrease motivation, through variables such as self-efficacy. Objective: To establish what motivational and cognitive variables predict academic performance and how they do it. Methods: A descriptive and inferential study was carried out with a sample of 508 students of health sciences belonging to two Spanish private universities, which are evaluated, through an adapted Motivated Strategies for Learning Questionnaire, their usage of cognitive and metacognitive strategies, and others for resource management and associated with motivation. Later, we elaborated a regression model, taking these strategies as independent variables and the performance measured through the student's grades as a dependent variable. Results: Self-efficacy for performance was the variable that best predicted academic performance in the university students that made up the sample. Other variables that were also related to performance were the management of time and place, the search for help, and the goal of self-praise. Conclusions: The academic results depend on the evaluation methods, the use of cognitive, metacognitive, and motivational strategies appropriate to the instructional context, and on better management of learning resources. These findings guide teachers to propose educational interventions aimed at increasing the use of motivational and learning strategies in university students(AU)

Afrontamiento de la muerte en estudiantes de Ciencias de la Salud / Coping with death by students of Health Sciences

Introducción: La muerte es un proceso que está presente en la vida. Los estudiantes de Ciencias de la Salud están especialmente expuestos al mismo, así como al hecho de estar presentes y acompañar en el proceso de morir a los pacientes. Objetivos: Conocer los niveles de afrontamiento ante la muerte en estudiantes de Ciencias de la Salud. Conocer la influencia de otras variables tales como el género, edad, año académico, religión y experiencias previas ante la muerte en relación al afrontamiento de la muerte. Métodos: Se llevó a cabo un estudio descriptivo de corte transversal. La muestra estuvo compuesta por 227 estudiantes de la Universidad Alfonso X el Sabio (Madrid-España). Para medir el afrontamiento de la muerte se utilizó la Escala Bugen de Afrontamiento de la Muerte-EBAM. Resultados: Los estudiantes de enfermería presentaron mayores niveles de afrontamiento de la muerte que los estudiantes de farmacia y Actividad Física y Deporte. La prueba de Chi Cuadrado mostró que existía relación entre la titulación cursada y el nivel de afrontamiento de la muerte (p< 0,01). La t de student también constató que no existían diferencias estadísticamente significativas al considerar el Afrontamiento de la muerte y haber tenido experiencias previas con moribundos (p< 0,01). No se constató influencia del género, edad, año académico y religión con el nivel de afrontamiento. Conclusiones: De este estudio se deduce la necesidad de incluir en los planes formativos asignaturas que traten el proceso de la muerte de manera que se puedan manejar de forma adecuada aspectos psicológico-emocionales del profesional, cuestión que repercutirá en el aumento de la calidad asistencial(AU)

Introduction: Death is a process that is present in life. Health science students are particularly exposed to it, as well as to the fact of being present and accompanying dying patients in such process. Objectives: To know the levels of coping with death in students of health sciences. To know the influence of other variables such as gender, age, academic year, religion and previous experiences with death in association to coping with death. Methods: A descriptive, cross-sectional study was carried out. The sample consisted of 227 students from Alfonso X el Sabio University in Madrid, Spain. For measuring coping of death, Bugen's Coping with Death Scale was used. Results: Nursing students showed higher levels of coping with death than Pharmacy or Physical Activity and Sport students. The chi-square test showed a relation between the degree completed and the level of coping with death (p< 0,01). The Student's t-test also showed no statistically significant differences when considering coping with death together with having previous experiences with dying (p< 0,01). No influence of gender, age, academic year and religion was found in association with the level of coping. Conclusions: This study shows the need to include, in the syllabuses, subjects that treat the process of death, so that psychological-emotional aspects of the professional can be handled appropriately, as it is an issue that will have an impact on the increase in the quality of healthcare(AU)

Vitamin D: Effect on Haematopoiesis and Immune System and Clinical Applications.

Vitamin D is a steroid-like hormone which acts by binding to vitamin D receptor (VDR). It plays a main role in the calcium homeostasis and metabolism. In addition, vitamin D display other important effects called "non-classical actions." Among them, vitamin D regulates immune cells function and hematopoietic cells differentiation and proliferation. Based on these effects, it is currently being evaluated for the treatment of hematologic malignancies. In addition, vitamin D levels have been correlated with patients' outcome after allogeneic stem cell transplantation, where it might regulate immune response and, accordingly, might influence the risk of graft-versus-host disease. Here, we present recent advances regarding its clinical applications both in the treatment of hematologic malignancies and in the transplant setting.

Estrés en estudiantes de enfermería / Stress in Nursing students

Introducción: los profesionales de la salud están expuestos a situaciones estresantes, las cuales generan importantes consecuencias negativas para la salud; incluso durante el periodo de formación. El adecuado afrontamiento de las situaciones que generan estrés durante las prácticas puede dificultar el aprendizaje y el futuro desarrollo profesional.Objetivos: evaluar el nivel de estrés en estudiantes de enfermería durante el período de formación práctica, su significación y su posible relación con el año académico cursado. Métodos: los instrumentos de medida utilizados fueron la escala de estrés percibido (PSS-14) y el cuestionario KEZKAK que mide los estresores en las prácticas clínicas. De los 744 estudiantes matriculados, se recogió una muestra compuesta por 289 estudiantes (39 porciento) pertenecientes al área de enfermería de la Universidad Alfonso X el Sabio (Madrid-España) en el periodo de los 3 cursos de formación de dicha disciplina. La media de edad de los alumnos evaluados fue de 21 años. Resultados: a lo largo de todos los cursos las mujeres sufren más estrés que los hombres. El segundo curso en el que se produce una mayor percepción de estrés. En cuanto a las situaciones que producen mayor nivel de estrés, se describen algunos componentes significativos: "Estrés por competencia profesional"," Estrés por interacción con el paciente"," Estrés por confrontación con el sufrimiento" y" Estrés por relación profesional". Conclusión: el nivel de estrés se relaciona con el año académico cursado(AU)

Introduction: Health professionals are exposed to stressful situations, which produce important negative consequences for their health, even during the training period. The appropriate way they cope with the situations that produce stress during the practices can make learning and future professional development difficult. Objective: To evaluate the level of stress in Nursing students during the period of their practical training, its significance and its possible relation with the academic year in course. Methods: The measurement instruments used were the perceived stress scale (PSS-14) and the KEZKAK questionnaire that measures stressors in clinical practice. Out of the 744 students enrolled, a sample of 289 students (39 percent) belonging to the Nursing area of ​​the Alfonso X el Sabio University (Madrid-Spain) was chosen during the three training courses of this discipline. The mean age of the students evaluated was 21 years. Results: Throughout the academic years, women suffer more stress than men. The second year is the one in which there is a greater perception of stress. As for the situations that produce a greater level of stress, some significant components are described: stress by professional competence, stress by interaction with the patient, stress by confrontation with suffering, and stress by professional relation. Conclusion: the level of stress is related to the academic year in course(AU)

Mapas conceptuales para aumentar el rendimiento académico en los estudiantes de Enfermería / Conceptual maps for increasing the academic performance of Nursing students

Introducción: una de las herramientas que aumenta tanto el interés como el valor de la tarea es el uso del mapa conceptual, que utiliza la representación visual de conceptos relacionados entre sí a través de interconectores. Objetivo: comparar si el uso de estos mapas conceptuales mejora el rendimiento académico y si existen diferencias en el rendimiento en relación al turno académico asignado. Métodos: para ello se seleccionaron de manera aleatoria a 200 alumnos de Grado de Enfermería (Centro Universitario de San Rafael Nebrija-Madrid-España). Resultados: los análisis realizados indican que las medias en cuanto al rendimiento académico son más elevadas en el grupo de alumnos que usaron mapas conceptuales, encontrándose diferencias significativas respecto a los que no lo usaron (t= -2,43; gl: 198; p> ,05). Por otro lado, se rechazó la influencia del turno asignado sobre el rendimiento académico. Conclusiones: el uso de los mapas conceptuales produce un efecto positivo en el rendimiento académico, lo que nos plantea la necesidad de realizar una docencia teórico-práctica más centrada en este método de aprendizaje(AU)

Introduction: One of the tools that increases both interest and value of the task is the use of conceptual maps, which uses the visual representation of concepts related to each other through interconnectors. Objective: To compare whether the use of these conceptual maps improves the academic performance and if there are differences in the performance in relation to the assigned academic shift. Methods: 200 students for the Nursing Degree (University Center of San Rafael Nebrija, Madrid, Spain) were randomly chosen. Results: The analyses carried out indicate that the means in terms of academic performance are higher in the group of students who used conceptual maps, finding significant differences in relationship to those who did not use them (t= -2.43; gl: 198; p> .05). On the other hand, the influence of the assigned shift on academic performance was rejected. Conclusions: The use of conceptual maps produces a positive effect on the academic performance, which raises the need for a theoretical-practical teaching centered on this method of learning(AU)

The incorporation of comorbidities in the prognostication of patients with lower-risk myelodysplastic syndrome.

Chronic medical diseases, evaluated by several comorbidities indexes have been reported to influence on overall survival in patients with myelodysplastic syndrome (MDS). However, these studies included patients with lower and higher-risk disease by IPSS. This study retrospectively evaluates the role of comorbidities (evaluated by the MDS comorbidity index; MDS-CI) together with clinical parameters in a series of 232 patients with LR-MDS (defined as either an IPSS score of low/intermediate-1 and favorable cytogenetic categories by IPSS-R). In multivariate analysis, together with age >75 years, diabetes requiring therapy and hemoglobin <10 g/dL; the incorporation of comorbidities by the MDS-CI (HR = 2.5; p< 0.0001) were independently associated to the probability of nonleukemic death (NLD). The combination of these variables allowed development of a model, which categorizes patients in three different groups with significantly different probability of NLD overtime (p< 0.001). This integrated score confirms the importance of comorbidities at diagnosis of patients with LR-MDS.

Immunomodulatory Effect of Vitamin D after Allogeneic Stem Cell Transplantation: Results of a Prospective Multicenter Clinical Trial.

PURPOSE: We describe the results of a prospective multicenter phase I/II trial evaluating the impact of the use of vitamin D (VitD) from day -5 to +100 on the outcome of patients undergoing allogeneic transplantation (EudraCT: 2010-023279-25; ClinicalTrials.gov: NCT02600988). EXPERIMENTAL DESIGN: A total of 150 patients were included in three consecutive cohorts of 50 patients each group: control group (CG, not receive VitD); low-dose group (LdD, received 1,000 IU VitD daily); and high-dose group (HdD, 5,000 IU VitD daily). We measured levels of VitD, cytokines, and immune subpopulations after transplantation. RESULTS: No significant differences were observed in terms of cumulative incidence of overall and grades 2-4 acute GVHD in terms of relapse, nonrelapse mortality, and overall survival. However, a significantly lower cumulative incidence of both overall and moderate plus severe chronic GVHD (cGVHD) at 1 year was observed in LdD (37.5% and 19.5%, respectively) and HdD (42.4% and 27%, respectively) as compared with CG (67.5% and 44.7%, respectively; P < 0.05). In multivariable analysis, treatment with VitD significantly decreased the risk of both overall (for LdD: HR = 0.31, P = 0.002; for HdD: HR = 0.36, P = 0.006) and moderate plus severe cGVHD (for LdD: HR = 0.22, P = 0.001; for HdD: HR = 0.33, P = 0.01). VitD modified the immune response, decreasing the number of B cells and naïve CD8 T cells, with a lower expression of CD40L. CONCLUSIONS: This is the first prospective trial that analyzes the effect of VitD postransplant. We observed a significantly lower incidence of cGVHD among patients receiving VitD. Interestingly, VitD modified the immune response after allo-SCT. Clin Cancer Res; 22(23); 5673-81. ©2016 AACR.

La influencia de la pareja en el ajuste a la enfermedad cardiaca / The influence of the couple in coping with heart disease

Resumen El objetivo del presente estudio fue analizar la influencia del apoyo social de la pareja en el ajuste a la enfermedad cardiaca, así como su influencia en la adhesión a las pautas clínicas. La muestra estuvo compuesta por 109 pacientes con sus parejas. Se llevaron a cabo las mediciones mientras realizaban el Programa de Rehabilitación Cardiaca del Hospital Ramón y Cajal de Madrid. Los resultados indican que la pareja no es una fuente de apoyo relevante para el ajuste a la enfermedad del paciente (apoyo recibido [p = 0.46] y satisfacción con el apoyo recibido [p = 0.41]. Sin embargo, el apoyo de la pareja sí resulta especialmente importante de cara al cumplimiento de hábitos cardiosaludables, sobre todo en el seguimiento realizado a los 8 y a los 12 meses (p < 0.001), lo que conduce a analizar la posibilidad de que la pareja no sea importante para el ajuste a la enfermedad debido posiblemente a que la muestra está pasando por una intervención clínica y, por tanto, es posible que otras fuentes como profesionales de la salud u otros pacientes estén ejerciendo un papel predominante de apoyo. En el momento en que esta supervisión clínica desaparece, el apoyo de la pareja es de vital importancia para la adhesión a las pautas recomendadas, las cuales tienen relación con cambios en el estilo de vida y, por tanto, en la prevención de recaídas.

Abstract The main aim of our study is to analyze the influence of social support on cardiac illness adjustment and on health recommendations adherence. The sample was 109 patients and their partners, all of them included in the Cardiac Rehabilitation Program of the Ramon y Cajal Hospital in Madrid. Our results show that partner support has not a significant impact on patients¿ adjustment (received support [P = .46] and satisfaction with received support [P = .041]). However, partner support has a significant effect on adherence to health recommendations (food, physical exercise, smoking, etc), most of all in the 8 and 12 months' follow-up (P < .001). Perhaps, in the first stages of illness, support provided for other sources (health professionals, other patients, etc.) could be more important than partner support, most of all taking into account that patients were included in a clinical intervention. As supervision decreases, the effect of partner support on adherence increases. The implications of our results for future interventions and research are discussed.

Emerging BCR/ABL1 Mutations Under Treatment with Tyrosine Kinase Inhibitors in Paediatric Acute Lymphoblastic Leukaemia.

We report on the emergence and clinical relevance of an unusual BCR-ABL1 kinase domain mutational status in a 2-year-old female with p210-BCR-ABL Philadelphia chromosome-positive acute lymphoblastic leukaemia. We detected three BCR-ABL1 clones determined by the presence of the E255V, D276G and F317L mutations. We point out the usefulness of searching for mutated populations that survive tyrosine-kinase inhibitor therapy and the role of their clonal selection over time in relation to therapeutic intervention.

Overexpression of GYS1, MIF, and MYC is associated with adverse outcome and poor response to azacitidine in myelodysplastic syndromes and acute myeloid leukemia.

BACKGROUND: The prognosis of myelodysplastic syndromes (MDS) and acute myeloid leukaemia (AML) is very heterogeneous. PATIENTS AND METHODS: We analyzed the prognostic value of several genes in a cohort of 85 MDS and AML patients. RESULTS: Overexpression of glycogen synthase 1 and macrophage migration inhibitory factor genes had an adverse outcome in multivariate analysis (P = .003 and P < .001, respectively). Furthermore, the higher expression of myelocytomatosis oncogene was associated with a lower response to azacitidine (P = .03). CONCLUSION: In the current study we identified a specific gene expression profile as prognostic factors for response to azacitidine and survival in MDS and AML.

Patterns of infection in patients with myelodysplastic syndromes and acute myeloid leukemia receiving azacitidine as salvage therapy. Implications for primary antifungal prophylaxis.

Incidence, etiology, and outcome of infectious episodes in patients with myeloid neoplasms receiving azacitidine are uncertain, with no prospective data available in this group of patients. The aim of the current study was to analyze the incidence and factors related to the probability of infection in a cohort of patients with myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) treated with azacitidine who did not receive any type of antimicrobial prophylaxis. Significantly, the group of patients who received prior intensive chemotherapy had more infectious episodes (P = 10(-4)), and particularly, invasive aspergillosis (P = .015), than patients who received frontline azacitidine. Primary antifungal prophylaxis might be recommended in MDS and AML patients receiving azacitidine as salvage therapy after intensive regimens.

Clinical prognostic factors for survival and risk of progression to acute myeloid leukemia in patients with myelodysplastic syndromes with < 10% marrow blasts and non-unfavorable cytogenetic categories.

UNLABELLED: Prognosis of myelodysplastic syndromes (MDS) is an area of ongoing interest. Identification of patients with poor outcome in the categories of lower risk disease is critical. In this study, we classify a cohort of 332 lower risk MDS into 3 groups with differences in survival and risk for leukemic progression that could drive treatment approaches to improve prognosis in a fraction of these patients. BACKGROUND: Prognosis of MDS and particularly in patients categorized as lower risk (< 10% blasts or low and intermediate-1 International Prognostic Scoring System [IPSS]) is very heterogeneous and includes patients with very different outcomes with current scoring systems. Recently, a new cytogenetic classification has been proposed for the revised IPSS in predicting the outcome for MDS. PATIENTS AND METHODS: To evaluate the prognostic significance of multiple variables for survival and risk of progression to acute myeloid leukemia, we analyzed baseline characteristics of 332 lower risk MDS patients within the lower risk cytogenetic categories by IPSS and the recent proposal for the new cytogenetic classification. RESULTS: In multivariate analysis, severity of cytopenias, age > 60 years, bone marrow blasts (5%-9%) and transfusion dependency significantly influenced outcome. The combination of these variables allowed development of a model which categorizes patients in 3 different groups with median survival of 95, 44, and 13 months for groups 1, 2, and 3, respectively (P < .001). In addition, this score also stratified patients for their risk for leukemic progression, estimated at 2 years in 3.1%, 7.6%, and 21.3% for each group (P = .024). CONCLUSION: Although karyotype remains the main prognostic factor in MDS, the current study identifies clinical parameters predicting outcome among patients with the better cytogenetic profile. Degree of cytopenias, blasts 5%-9% and transfusion dependence might identify a subset of patients within the nonadverse karyotype, in which early or more aggressive approaches could possibly be required to improve survival or prevent disease progression.

Aging of porous silicon in physiological conditions: cell adhesion modes on scaled 1D micropatterns.

The surface properties of porous silicon (PSi) evolve rapidly in phosphate-buffered saline. X-ray photoelectron spectra indicate the formation of a Si-OH and C-O enriched surface, which becomes increasingly hydrophilic with aging time. Multiscale stripe micropatterns of Si and PSi have been fabricated by means of a high-energy ion-beam irradiation process. These micropatterns have been aged in physiological conditions and used to analyze human mesenchymal stem cell (hMSC) adhesion. The actin cytoskeleton of hMSCs orients following the uniaxial micropatterns. In the wider Si stripes, hMSCs are dominantly located on Si areas. However, for reduced Si widths, adhesion is avoided on PSi by a split assembly of the actin cytoskeleton on two parallel Si areas. These results confirm that nanostructured Si-OH/C-O-rich surfaces with hydrophilic character are specially adapted for the creation of cell adhesion surface contrasts.

[Intraductal breast papillomas in patients with Costello syndrome]. / Papilomas intraductales de mama en paciente afectada de síndrome de Costello.

Costello syndrome is a multisystemic congenital disorder with a very low prevalence. The pathogenesis remains unclear and predisposes to the development of tumors of ectodermal origin. Diagnosis is clinical, based on findings of mental and growth retardation and a characteristic phenotype. We report the case of a patient with Costello syndrome who was referred to our unit with a suspected diagnosis of intraductal papilloma based on the presence of various episodes of nipple discharge. Postoperative histopathological study confirmed the diagnosis of multiple intraductal papilloma. We review the literature on the topic and discuss the advisability of aggressive surgical therapy, given the predisposition of these patients to develop both benign and malignant tumors.