Resumen Introducción. El trasplante de células madre hematopoyéticas es la opción curativa para algunas enfermedades y está aumentando el tiempo de supervivencia de los pacientes. La calidad de vida relacionada con la salud en estos pacientes no se evalúa de manera sistemática. Objetivos. Describir la calidad de vida relacionada con la salud y las complicaciones en niños con trasplante de células madre hematopoyéticas. Materiales y métodos. Es un estudio transversal en pacientes pediátricos sobrevivientes al trasplante. Se midió la calidad de vida relacionada con la salud, utilizando el cuestionario KIDSCREEN-27 en pacientes entre 8 y 14 años y la SF-12™ (Short Form-12) en pacientes mayores de 14 años. El análisis estadístico se realizó en el software Stata 12. Utilizamos el modelo de Rasch, trasladando estimación de parámetros a valores t para obtener el resultado de los cuestionarios. Resultados. En total, 42 pacientes respondieron alguno de los cuestionarios. Los eventos adversos más frecuentes fueron “enfermedad crónica de injerto Vs. Contra huésped” y “complicaciones endocrinas”. De acuerdo con la normalidad de datos del KIDSCREEN-27, los puntajes de las dimensiones “ambiente escolar” y “soporte social y pares” fueron inferiores al percentil 50. En el cuestionario SF-12™, el grupo que utilizaba inmunosupresores tuvo un menor puntaje en el componente físico. Conclusiones. En general, los resultados del KIDSCREEN-27 sugieren un cierto déficit de calidad de vida en pacientes entre 8 y 14 años. Los cuestionarios mostraron confiabilidad en la muestra.

Abstract Introduction: Hematopoietic stem cell transplantation is the curative option for some diseases and is increasing patient survival. The health-related quality of life in these patients is not systematically evaluated. Objectives: The present study sought to describe the health-related quality of life and complications in children who underwent hematopoietic stem cell transplantation. Materials and methods: A cross-sectional study was conducted on pediatric transplanted survivors. Health-related quality of life was measured using the KIDSCREEN-27 scale and Short Form-12 (SF-12) in patients between 8 and 14 years of age and those over 14 years, respectively. Statistical analysis was performed using STATA 12 software. We used the Rasch model person parameter estimates translated into T-values to score the questionnaire. Results: A total of 42 children answered the questionnaires. The most frequent adverse events were chronic graft Vs. host disease and endocrine complications. According to European norm data in the KIDSCREEN-12 scale, scores for the school dimension and social and peer support were below the 50th. percentile. The group administered immunosuppressants had lower scores on the physical component of the SF-12™ scale. Conclusions: In general, the KIDSCREEN-27 does appear to suggest some quality-of-life deficit in younger children. The scales showed reliability in this population.

Resumen En la población hipertensa se detecta una causa subyacente en 5% a 10%. Identificar etiología y establecer un tratamiento específico reduce el riesgo de daño de órgano blanco y, en algunos casos, puede curar la enfermedad primaria y la hipertensión. Dada su baja prevalencia, no se recomienda realizar una búsqueda exhaustiva de causas secundarias en todos los pacientes hipertensos. En este artículo se analiza cuándo y cómo debe estudiarse una hipertensión secundaria.

Abstract In the hypertensive population, between 5-10%, an underlying cause is detected. Identifying the etiology and giving specific treatment reduces the risk of hypertension-mediated organ damage and, in some cases, can cure the disease and high blood pressure. Due to its low prevalence, an exhaustive search for secondary causes is not recommended in all patients with hypertension. This article will discuss when and how to study secondary hypertension.

Abstract: Introduction: Bone marrow failure (BMF) syndromes are rare disorders with an annual incidence of 2-4 cases per million. Treatment options include immunosuppressive therapy (IST) and hematopoietic stem cell transplantation (HSCT). Objective: To analyze the outcomes of pediatric patients diagnosed with BMF treated in a tertiary care center. Patients and Method: Retrospective study of pediatric patients diagnosed with BMF who consulted at Fundación Valle de Lili, Cali. Descriptive statistical analysis was performed according to Acquired BMF (ABMF) and Inherited BMF (IBMF). The outcomes include treatment, complications, overall survival (OS) in transplant patients, calculated using the Kaplan Meier method. Results: We included 24 patients with BMF, average age 6.5 ± 4 years, and 50% were women. 58% presented IBMF, 9 with Fanconi anemia (FA), 2 dyskeratosis congenita, 2 congenital amegakaryocytic thrombocytopenia, and 1 presented Diamond-Blackfan anemia. 12 patients treated with HSCT had a 5-year OS of 83%. ABMF represented 42%. 6 patients received IST-HSCT, 3 received IST, and 1 received HSCT. The OS of the IST-HSCT group was 86%. Six patients died, four of them related to infection. Conclusions: In this series, there was a higher number of cases with IBMF. The OS of patients treated with HSCT is similar to that reported in recent studies. The most frequent cause of death was of infectious origin which has also been previously reported. The treatment esta blished in the patients showed favorable results in a Latin American tertiary care center.

Resumen: Introducción: Los síndromes de falla medular (SFM) son trastornos infrecuentes, con una incidencia anual de 2-4 casos por millón. Las opciones de tratamiento incluyen terapia de inmunosupresión (TIS) y restaura ción de la hematopoyesis con trasplante de progenitores hematopoyéticas (TPH). Objetivo: Analizar los desenlaces de pacientes pediátricos diagnosticados con SFM tratados en una institución de alta complejidad. Pacientes y Método: Estudio retrospectivo de pacientes pediátricos con diagnóstico de SFM que consultaron a la Fundación Valle del Lili, Cali. Se realizo análisis estadístico descriptivo según SFM adquirida (SFMA) y SFM congénita (SFMC). Los desenlaces incluyeron: tratamiento, complicaciones, supervivencia global (SG) en los trasplantados, calculada con el método Kaplan Meier. Resultados: Se incluyeron 24 pacientes con SFM, edad 6,5 ± 4 años, 50% mujeres. El 58% fue ron SFMC, 9 con anemia de Fanconi, 2 disqueratosis congénita, 2 trombocitopenia amegacariocítica congénita, uno anemia Diamond-Blackfan. Doce pacientes con TPH tuvieron SG a 5 años de 83%. SFMA correspondió al 42%, 6 recibieron TIS-TPH, 3 TIS y 1 TPH, la SG del grupo con TIS-TPH fue 86%. Seis pacientes fallecieron, 4/6 relacionadas con infección. Conclusiones: En esta serie fue mayor el número de casos con SFMC. La SG de los pacientes llevados a TPH es comparable con la reportada en estudios recientes. La causa de muerte predominante fue infecciosa que también se ha reportado previamente. El tratamiento instaurado en los pacientes de esta serie mostró resultados favorables en un centro de alta complejidad en un país latinoamericano.

Abstract Objective Cytomegalovirus infection and disease are significant causes of morbidity and mortality among patients with hematopoietic stem cell transplantation. The aim of this study was to assess the frequency of cytomegalovirus infection and characterize the patients who developed the disease. Methods A retrospective cohort study was performed among adult patients, recipients of allogeneic HSTC between 2008 and 2015. Taking into account the institutional protocol of prophylaxis infections in hematopoietic stem cell transplantation, patients received either preemptive therapy or prophylaxis with valganciclovir. Infection was defined as a positive pp65 antigenemia assay or PCR higher than 500 copies/mL. Disease was defined as viremia with evidence of end organ damage. Results Seventy patients were included, the median age was 36 years old (IQR 17–62). A total of 93% of the recipients had a positive serology. The Cytomegalovirus infection occurred in 59% of the patients. Eleven patients developed disease (16%), the most frequent manifestation being colitis, followed by pneumonitis and a single case of retinitis. There were no differences between the preemptive therapy or prophylaxis groups. The mean time of onset of the disease was day 94 post-transplant. Three patients developed disease with a viral load lower than 1000 copies/mL. Conclusion The incidence of cytomegalovirus infection after transplantation at our institution is high. It was found that the disease can occur with any level of viral load and is associated with high mortality.

Abstract Background This study aimed to describe the incidence, microbiological profile, and risk factors associated with bacteremia in pediatric patients with hematopoietic stem cell transplantation (HSCT). Methods A retrospective cohort study was performed on patients under 18 years old who underwent HSCT due to any indication, between January 2012 and January 2017. The patient data were collected from the first 100 days post-HSCT. Bacteremia was defined as the isolation of bacteria from at least one blood culture. The relation between bacteremia and associated risk factors was evaluated using univariate and multivariate analysis. Results We enrolled a total of 111 pediatric patients who received HSCT during the period of study. The total number of patients who developed at least one episode of bacteremia was 46 (41.4%), and the total number of bacteremia episodes was 62. Rectal swabs were performed in 44 patients to detect prior colonization and this showed that 25% of them were positive, the main pathogen isolated being carbapenem-resistant enterobacteriaceae. The Gram-negative bacteria cases accounted for 60% of 62 events. The main Gram-negative bacteria isolated were Klebsiella pneumoniae and Escherichia coli and the majority were resistant strains. The mortality rate on day +100 for the whole cohort was 18% (n = 20). Regarding the patients with bacteremia, the mortality rate on day +100 was 30% (n = 14), and the cause of death was related to infection in 10 of them. We did not find any statistically significant risk factor for the development of bacteremia. Conclusion Bacteremia is common after HSCT, and there is a high frequency of resistant Gram-negative infections which is related to high mortality.

Resumen Introducción: En pacientes con leucemia mieloide aguda (LMA) el trasplante de progenitores hematopoyético (TPH) es el único tratamientoz curativo. El objetivo de este estudio es presentar la experiencia y resultados del trasplante haploidéntico en pacientes adultos con LMA en la Fundación Valle del Lili, Cali - Colombia. Materiales y métodos: Estudio de cohorte retrospectivo de pacientes que recibieron trasplante haploidéntico entre 2013 y 2017, con acondicionamiento mieloablativo y ciclofosfamida postrasplante, en Fundación Valle del Lili, Cali (Colombia). Resultados: Se realizaron 47 trasplantes en pacientes con leucemia mieloide aguda en la fecha de estudio, se incluyeron en el análisis 21 pacientes con donante haploidéntico, a 3 años tanto la supervivencia global y libre de eventos fue del 38%. La incidencia acumulada de mortalidad relacionada al trasplante fue del 26% a 100 días y del 38,3%, a 38 meses de seguimiento. La incidencia acumulada de recaída a 38 meses fue del 19%. Con respecto a la enfermedad injerto versus huésped (EICH) se encontró que la incidencia acumulada de EICH aguda grado II-IV, grado III-IV y EICH crónico fue del 19%, 5% y 19% respectivamente. Conclusión: Los resultados de este estudio sugieren que el trasplante haploidéntico es una alternativa factible como tratamiento para pacientes con diagnóstico de LMA en nuestro medio.

Abstract Introduction: In patients with acute myeloid leukemia (AML), hematopoietic progenitor transplantation (PHT) is the only curative treatment. The objective of this study is to present the experience and results of haploidentical transplantation in adult patients with AML at the Valle del Lili Foundation, Cali - Colombia. Materials and methods: Retrospective cohort study of patients who received haploidentical transplantation between 2013 and 2017, with myeloablative conditioning and post-transplant cyclophosphamide, in Fundación Valle del Lili, Cali (Colombia). Results: 47 transplants were performed in patients with acute myeloid leukemia on the study date, 21 patients with haploidentical donors were included in the analysis, at 3 years both overall and event-free survival was 38%. The cumulative incidence of transplant-related mortality was 26% at 100 days and 38.3% at 38 months of follow-up. The cumulative incidence of relapse at 38 months was 19%. Regarding graft versus host disease (GVHD), it was found that the cumulative incidence of acute GVHD grade II-IV, grade III-IV and chronic GVHD was 19%, 5% and 19% respectively. Conclusion: The results of this study suggest that haploidentical transplantation is a feasible alternative as a treatment for patients diagnosed with AML in our environment.

Resumen Objetivo: El mieloma múltiple representa la primera causa de trasplante autólogo de células madre hematopoyéticas (TCMH). El objetivo del presente trabajo es describir los resultados del TCMH en pacientes con diagnóstico de mieloma múltiple en la Fundación Valle del Lili. Materiales y métodos: Se realizó un estudio observacional, descriptivo de una cohorte retrospectiva, se incluyeron pacientes mayores de 18 años con TCMH autólogo tratados entre 2008 y 2016. Los desenlaces fueron: supervivencia global, supervivencia libre de progresión y la respuesta de la enfermedad postrasplante. Se realizó un análisis estadístico descriptivo y el análisis de supervivencia se hizo con el método Kaplan-Meier. Resultados: Durante el periodo de estudio se trasplantaron 103 pacientes. La mediana de la edad fue 57 años. El subtipo de inmunoglobulina secretada fue: 75% de IgG, 18% de IgA, 5% no secretor y 2% oligosecretor. El estadio Durie Salmon en la mayoría fue IIIA (43,7%). Previo al trasplante la mayoría de los pacientes estaba en muy buena respuesta parcial (31%), seguido por respuesta completa (25,2%) y respuesta parcial (19,4%). La supervivencia global y libre de progresión a 5 años fue de 71% y de 40%, respectivamente. Posterior al trasplante: el 33% de los pacientes estaba en muy buena respuesta parcial, el 25% en respuesta completa estricta, el 22% en respuesta completa, el 12% en respuesta parcial y el 8% en enfermedad progresiva o recaída. Conclusión: El TCMH autólogo es una estrategia que se asocia a buenas tasas de supervivencia, baja toxicidad y adecuada respuesta de la enfermedad postrasplante.

Abstract Objective: Multiple myeloma in Colombia is the one of the main reasons for autologous hematopoietic stem cell transplantation (HSCT). The aim of this study is to describe the results of the HSCT in adult patients diagnosed with multiple myeloma in the Fundación Valle del Lili. Materials and methods: An observational, descriptive study of a retrospective cohort was carried out. Patients older than 18 years with a diagnosis of multiple myeloma with autologous HSCT between 2008 and 2016 were included. The outcomes were overall survival, progression-free survival, and post-transplant disease response. A descriptive statistical analysis was carried out for all the variables considered in the analysis. The survival analysis was performed using the Kaplan-Meier method. Results: During the study period, transplants were performed on 103 patients with a diagnosis of multiple myeloma. The median age was 57 years. The subtype of secreted immunoglobulin was 75% IgG, 18% IgA, 5% non-secretor, and 2% oligo-secretor. The majority of patients were in Durie Salmon stage was IIIA (43.7%). Prior to transplantation 31% had a very good partial response, 25.2% complete response, 19.4% partial response, 10.7% progressive disease, 6.8% stable disease, 2.9% complete strict response, and in 3.9% of the patients the report of the disease status was not found. The 5-year overall survival was 71% (95% CI: 53-83) and progression-free survival was 40% (95% CI 25-54). After transplantation 33% of the patients were in very good partial response, 25% in strict complete response, 22% in complete response, 12% partial response, and 8% progressive disease or relapse. Conclusion: Autologous HSCT is a strategy that is associated with good survival rates, low toxicity, and an adequate post-transplant disease response.

Resumen Objetivo: Describir la experiencia en términos de supervivencia y complicaciones de los pacientes llevados a trasplante de células madre hematopoyéticos (TCMH) en los últimos 15 años. Materiales y métodos: Se realizó un estudio descriptivo, tipo cohorte retrospectiva en el que se incluyeron pacientes menores de 18 años con diagnóstico de leucemia mieloide aguda (LMA), leucemia mieloide crónica (LMC) y síndrome mielodisplásico (SMD) llevados a TCMH entre enero de 2001 y diciembre de 2015. Los desenlaces fueron supervivencia global, supervivencia libre de evento y las complicaciones relacionadas con el trasplante. Resultados: Durante el periodo de estudio se trasplantaron: 43 pacientes con diagnóstico de neoplasias mieloides; 31 con LMA (72%); 4 con LMC (9%) y 8 con SMD (19%). Se realizaron 11 trasplantes de donante idéntico, 11 haploidénticos, 11 autólogos y 10 de sangre de cordón umbilical. De los pacientes con LMA (31 casos), el 58% fueron sometidos a trasplante en primera remisión completa y el 39% en segunda o subsecuente remisión completa. La supervivencia global y libre de evento a 5 años fue 56% y 38% respectivamente. La mortalidad relacionada al trasplante en el día 100 fue del 15%, la incidencia acumulada de enfermedad injerto contra huésped 59%, la infección por citomegalovirus 39%, las infecciones bacterianas 54% y la cistitis hemorrágica 14%. Conclusión: La baja tasa de mortalidad y complicaciones relacionadas al trasplante sugiere que el trasplante de células madre hematopoyéticas es una alternativa factible como tratamiento para pacientes con neoplasias mieloides en nuestro medio.

Abstract Objective: To describe the experience, in terms of survival and complications, with patients that received a haematopoietic stem cell transplantation (HSCT) in the last 15 years. Materials and methods: A descriptive, retrospective, cohort study was conducted on patients less than 18 years-old with a diagnosis of acute myeloid leukaemia (AML), chronic myeloid leukaemia (CML), and myelodysplastic syndrome (MDS) and received HSCT between January 2001 and December 2015. The outcomes were, overall survival, event-free survival, and complications associated with the transplant. Results: During the study period, a total of 43 patients with myeloid neoplasms received a transplant, of which 31 (72%) had AML, 4 (9%) with CML, and 8 (19%) with MDS. A total of 11 identical donor transplants were performed, as well as 11 haploidentical, 11 autologous, and 10 with umbilical cord blood. Of the patients with AML (31 cases), 58% were subjected to a transplant in the first full remission, and 39% in the second or subsequent full remission. The overall and event-free survival at 5 years was 56% and 38%, respectively. The transplant-related mortality at day 100 was 15%, with an accumulated incidence of graft versus host disease of 59%, cytomegalovirus infection of 39%, with 54% bacterial infections, and 14% haemorrhagic cystitis. Conclusion: The low mortality and complications rate associated with the transplant suggests that haematopoietic stem cell transplantation is a viable alternative as a treatment for patients with myeloid neoplasms in our country.

Resumen Objetivo: describir los perfiles clínicos y hemodinámicos de pacientes hospitalizados por falla cardíaca aguda. Métodos: estudio transversal de pacientes de 18 años o más, con diagnóstico de falla cardíaca aguda admitidos a un centro hospitalario de alta complejidad en Cali, Colombia, en un período de 7 meses. Se recolectó información sociodemográfica, clínica, perfiles clínicos y hemodinámicos de la falla cardiaca al ingreso, estancia hospitalaria y mortalidad. Se describieron las variables cuantitativas como promedios o medianas, y las categóricas como valor absoluto y porcentajes. Se realizaron tablas de frecuencia, y se graficaron los porcentajes de pacientes que ingresaron según el perfil clínico y hemodinámico de falla cardiaca aguda. Resultados: se incluyeron 99 pacientes con 143 episodios de falla cardíaca aguda. La mitad eran hombres y la mediana de edad 66 años (rango 21 a 97 años). 60% fueron afrodescendientes y 14,14% mestizos. Los perfiles clínicos más frecuentes fueron falla cardíaca aguda descompensada (72,46%), edema pulmonar agudo (10,14%) y falla cardiaca asociada a síndrome coronario agudo en 6,52%. Los perfiles hemodinámicos fueron ‘‘caliente y seco’’ 10,87%, ‘‘caliente y húmedo’’ 55,07%, ‘‘frío y húmedo’’ 28,26% y ‘‘frío y seco’’ en 5,8%. Conclusiones: la falla cardíaca aguda descompensada es el perfil de presentación clínica más común en nuestra población con dos tercios del total de casos. El perfil hemodinámico más frecuente fue el ‘‘caliente y húmedo’’ (Stevenson B), resultados que concuerdan con lo observado en estudios locales y registros internacionales.

Abstract Objective: To describe the clinical and haemodynamic profiles of patients admitted to hospital due to acute heart failure. Methods: A cross-sectional study was conducted on patients aged 18 years or older with a diagnosis of acute heart failure admitted to a high complexity hospital in Cali, Colombia, during a 7-month period. Sociodemographic, clinical and haemodynamic information of the cardiac failure was recorded on admission, as well as hospital stay and mortality. The quantitative variables are described as means or medians, and the categorical variables as absolute value and percentages. Frequency tables were made, and a graph made of the percentages of patients admitted, according to the clinical and haemodynamic profile of the acute heart failure. Results: The study included as total of 99 patients with 143 episodes of acute heart failure. Half of the patients were male, and the median age was 66 years (range 21 to 97 years). The majority (60%) were of African descent and 14.14% were Mestizos. The most common clinical profiles were decompensated heart failure (72.46%), acute pulmonary edema (10.14%), and heart failure associated with acute coronary syndrome in 6.52%. The haemodynamic profiles were ‘‘warm and dry’’ in 10.87%, ‘‘warm and wet’’ in 55.07%, ‘‘cold and wet’’ in 28.26%, and ‘‘cold and dry’’ in 5.8%. Conclusions: Decompensated acute heart failure is the most common clinical presentation profile in the population studied, with two-thirds of all cases. The most frequent haemodynamic profile was ‘‘warm and wet’’, results that agree with those observed in local studies and in international registers.

Resumen Objetivo: Determinar el riesgo de presentar eventos cardiovasculares mayores en pacientes con síndrome coronario agudo e implante de stent, que suspendieron la doble terapia antiagregante antes de un año de tratamiento. Métodos: Estudio analítico de pacientes con síndrome coronario agudo e implante de stent que recibieron doble terapia antiagregante al egreso hospitalario. Se describieron las características sociodemográficas, clínicas y paraclínicas. Se determinó la prevalencia de suspensión de doble terapia antiagregante antes de un año de tratamiento y el riesgo de eventos cardiovasculares mayores mediante un modelo de riesgos proporcionales Cox. Resultados: Se incluyeron 873 pacientes. La prevalencia de suspensión de doble terapia antiagregante fue 39,18%. El grupo que continuó la terapia por un año tuvo mayor frecuencia de enfermedad coronaria previa (19,13% p = 0,03). La principal indicación del procedimiento en los pacientes que suspendieron la terapia fue infarto sin ST elevado (36,8%). La suspensión de la doble terapia antiagregante antes del año estuvo asociada con mayor incidencia de MACE a un año (HR 1,31 95% IC 0,65-2,62 p = 0,45). La presencia de enfermedad arterial periférica, diabetes mellitus y más de un vaso enfermo se relacionó con eventos adversos cardiovasculares a un año. Conclusiones: La suspensión antes de los doce meses de la doble terapia antiagregante en pacientes con implante de stent posterior a un síndrome coronario agudo, es frecuente y parece no estar asociada con mayor incidencia de desenlaces cardiovasculares mayores; sin embargo, la causa de la interrupción puede influir en los desenlaces clínicos y debe tenerse en cuenta en la práctica clínica.

Abstract Objective: To determine the risk of developing major adverse cardiovascular events (MACEs) in patients with acute coronary syndrome and stent implant who suspended dual antiplatelet therapy before one year of treatment. Methods: Analytical study of patients with acute coronary syndrome and stent implant who received dual antiplatelet therapy upon hospital admission. Sociodemographic, clinical and paraclinical features were described. The prevalence of dual antiplatelet therapy suspensión before one year of treatment was determined, and for assessing the risk of major adverse cardiovascular events a Cox proportional hazard regression model was used. Results: 873 patients were included. Prevalence of dual antiplatelet therapy suspension was 39.18%. The group who continued the therapy during a year had a higher frequency of previous coronary disease (19.13%, p = 0.03). The main indication for the procedure in patients who interrupted their treatment was infarction without ST elevation (36.8%). Dual therapy suspensión before one year was not related to a higher incidence of MACEs after one year (HR1.31, CI 95% 0.65-2.62 p = 0.45). The presence of peripheral arterial disease, diabetes mellitus and more than one abnormal vessel was related to adverse cardiovascular effects within one year. Conclusions: The suspension of dual antiplatelet therapy before twelve months in patients with a stent implant posterior to an acute coronary syndrome is frequent and does not seem to be associated to a higher incidence of major adverse cardiovascular events; nevertheless, the reason for the interruption could influence the clinical outcome and must be taken into account for clinical practice.