Riñón único funcionante y su seguimiento en Pediatría

Contexto: el riñón único funcionante (RUF) es una entidad que puede ser de tipo congénito y es causada por una agenesia renal unilateral o un riñón displásico multiquístico, o adquirido de forma secundaria a una nefrectomía unilateral, condicionando al paciente a un seguimiento estricto rutinario, así como en la implementación de algunas pautas de prevención en los pacientes pediátricos. Objetivo: analizar la importancia del seguimiento en los pacientes pediátricos con RUF. Metodologí:a se hace una revisión de la literatura mostrando desde la embriología, fisiología, etiología, aspectos clínicos y estudios diagnósticos en pacientes con RUF para así, posteriormente, ofrecer unas pautas de seguimiento y manejo en pacientes pediátricos. Resultados: los pacientes con RUF pueden presentar hiperfiltración glomerular compensatoria, seguida de lesión glomerular con hipertensión, albuminuria y reducción de la tasa de filtración glomerular, sin embargo, para prevenir el daño renal se deben realizar seguimientos clínico, paraclínico e imagenológico cuidadosos de todo paciente con RUF, según la condición lo amerite. Conclusiones: se hace necesaria la unificación de pautas de seguimiento en los pacientes pediátricos con RUF y hacer énfasis en aquellos factores de riesgo que predisponen a compromiso renal.

Background: The Solitary functioning kidney (SFK) is an entity that can be congenital type caused by unilateral renal agenesis or multicystic dysplastic kidney, or acquired secondary to unilateral nephrectomy conditioning to a strict routine follow-up, as well as the implementation of some prevention guidelines in pediatric patients. Purpose: To analyze the importance of follow-up in pediatric patients with a single functioning kidney. Methodology: A review of the literature is made showing embryology, physiology, etiology, clinical aspects and diagnostic studies in patients with SFK in order to subsequently offer guidelines for follow-up and management in pediatric patients. Results: Patients with SFK may present compensatory glomerular hyperfiltration, followed by glomerular injury with hypertension, albuminuria and reduced glomerular filtration rate, however, to prevent renal damage a careful clinical, paraclinical and imaging follow-up of every patient with SFK should be performed as the condition warrants. Conclusions: It is necessary to unify follow-up guidelines in pediatric patients with SFK and to emphasize those risk factors that predispose to renal compromise.

Síndrome de opsoclonia mioclonia idiopático: Reporte de caso en una paciente de 13 meses / Idiopathic opsoclonus myoclonus syndrome: Case report in a 13-month-old female patient

Resumen El síndrome de opsoclonia mioclonía es una entidad neurológica poco frecuente que afecta a los niños en la etapa preescolar. Clínicamente se caracteriza por una triada clásica de opsoclonía, mioclonía y ataxia aguda, con una evolución progresiva o incluso de manera incompleta. Su etiología puede ser paraneoplásica, en la mayoría de los casos en asociación con neuroblastomas, así como postinfecciosa o parainfecciosa, autoinmune o idiopática. En objetivo del tratamiento es la inmunomodulación con terapia de primera línea con esteroides endovenosos aunque pudiendo asociarse a recaídas y secuelas a largo plazo en el ámbito neurológico y conductual. El síndrome de opsoclonia mioclonía representa un reto diagnóstico en los pacientes con ataxia aguda dada la variedad de presentación clínica, por tanto es importante tener una alta sospecha diagnostica para garantizar un tratamiento oportuno y evitar secuelas futuras.

Abstract Opsoclonus myoclonus syndrome is a rare neurological entity affecting preschool children. Clinically it is characterized by a classic triad of opsoclonus, myoclonus, and acute ataxia, with a progressive or even incomplete course. Its etiology can be paraneoplastic, in most cases in association with neuroblastomas, as well as postinfectious or parainfectious, autoimmune or idiopathic. The goal of treatment is immunomodulation with first-line therapy with intravenous steroids, although it can be associated with relapses and long-term neurological and behavioral sequelae. The opsoclonus myoclonus syndrome represents a diagnostic challenge in patients with acute ataxia given the variety of clinical presentations, therefore it is important to have a high diagnostic suspicion to ensure timely treatment and aoid future sequelae.

Actualización de resultados demográficos y terapéuticos de pacientes con Leucemia Mieloide Aguda no promielocítica en el Hospital Clínico de la Pontificia Universidad Católica de Chile / Acute myeloid leukemia. Analysis of 114 patients

BACKGROUND: Acute myeloid leukemia (AML) is the most common leukemia in adults. Aim: To Describe our population of patients with AML and report the outcomes of our treatments. MATERIAL AND METHODS: Review of electronic clinical records of 114 patients with AML with a median age of 57 years (59% men). Results: Seventeen percent of patients were classified as low risk, 38% as intermediate risk and 33% as high risk. Seventy-six percent of patients were treated with intensive chemotherapy. Five years overall survival according to cytogenetic risk was 59, 41, and 12% in low, intermediate, and high-risk patients, respectively. The outcomes were better in patients under 60 years. The median survival of patients treated with intensive chemotherapy aged less than 60 years and 60 years and above was 3.4 and 1 year, respectively. CONCLUSIONS: Our results are comparable to those reported in developed countries. Improving the survival of patients 60 years and older is our main challenge.

Resultados a largo plazo de una cohorte chilena: la edad del paciente no incide en el resultado del trasplante alogénico de precursores hematopoyéticos para leucemia mieloide aguda / Age does not affect the outcome of allogeneic hematopoietic precursor transplantation for acute myeloid leukemia

BACKGROUND: In our country, transplantation centers differ in the age limit for allogeneic hematopoietic transplantation (ALOHT). In our program, transplants with age- adjusted conditioning are performed in patients until 70 years old. Currently more than 60% of ALOHT reported to the Center for International Bone Marrow Transplantation Research (CIBMTR) are performed in patients older than 40 years. AIM: To report our experience with ALOHT in acute myelogenous leukemia (AML), analyzing patient age at transplantation in different periods and transplant results in different age groups. MATERIAL AND METHODS: A retrospective analysis of the database of adult hematopoietic transplants in AML patients was performed. Demographic data, disease characteristics, transplant data, survival and relapse times, and mortality were collected. RESULTS: In our program, 1030 transplants were performed in adults and 119 ALOHT were performed in AML patients, between 1990 and 2020. The median age of patients in all periods was 41 years, (range 16-69). The median age was 33 and 45 years, in the periods 1990-2000 and 2000-2020 respectively (p < 0.01). Seventy-eight patients received myeloablative conditioning (median age 44 years) and 41 reduced intensity conditioning (median age 53 years). Five-year overall survival was 44.6% (confidence intervals (CI) 41-48). Non relapse mortality of all periods was 19% (CI 17 - 40%) and relapse rate was 17 % (CI 16-22). No difference in five years overall survival among patients younger than 40, 41 to 50 and over 51 years was observed. Conclusions: Overall Survival, non-relapse mortality and relapse rate were similar in younger and older patients in our program and similar to those previously reported in other centers.

El derecho a la alimentación y la justicia de género en Venezuela. Indicadores de Progreso / The Right to Food and Gender Justice in Venezuela. Progress Indicators

Se establecieron Indicadores estructurales, de procesos y de resultados, como indicadores de procesos basados en los establecidos en el Protocolo de San Salvador, para el monitoreo del derecho a la alimentación en Venezuela bajo el principio transversal de igualdad y no discriminación: la justicia de género. Al determinar Indicadores estructurales, se estableció que en Venezuela no se contempla el derecho a la alimentación adecuada, bajo una declaración expresa en la Constitución de la República Bolivariana de Venezuela y/o las Leyes establecidas para tal fin, debiendo establecerse su declaración expresa bajo el orden jurídico legal del país, así como su vinculación con poblaciones en situación de vulnerabilidad, atendiendo lo concerniente al derecho a la alimentación y la población con justicia de género. Los Indicadores de procesos establecidos determinaron una elevada opacidad estadística en instituciones oficiales y gubernamentales sobre los alcances del establecimiento de las denominadas misiones sociales y programas de ayuda alimentaria, así como el alcance desglosado de los programas por grupos tradicionalmente excluidos como el enfoque de justicia de género. Al determinar indicadores de resultados, no se evidenciaron en los portales oficiales de los entes del Estado, la existencia de estadísticas de tasa de desnutrición de forma general o para distintos sectores poblacionales (niños, niñas, jóvenes, mujeres, adultos mayores, personas con discapacidad, grupos étnicos) sobre la base de la Tasa de desnutrición global. La aplicación de estos indicadores determinaron el no cumplimiento por parte del estado, de la garantía de un derecho a la alimentación adecuada, requiriéndose una profunda transformación estructural y de procesos, bajo la premisa de los derechos humanos, a los fines de poder establecer una significativa y necesaria mejora en los indicadores de resultados, así como generar soluciones para transformar las desigualdades de género, ante la creciente tendencia del establecimiento de una feminización de la pobreza en Venezuela(AU)

Structural, process and result indicators were established, as process indicators based on those established in the Protocol of San Salvador, for monitoring the right to food in Venezuela, under the cross-cutting principle of equality and non-discrimination: gender justice . When determining structural indicators, it was established that in Venezuela the right to adequate food is not contemplated, under an express declaration in the Constitution of the Bolivarian Republic of Venezuela and / or the Laws established for that purpose, and its express declaration must be established under the legal order of the country, as well as its relationship with populations in vulnerable situations, taking into account the right to food and the population with gender justice. The indicators of established processes determined, a high statistical opacity in official and governmental institutions regarding the scope of the establishment of the so-called social missions and food aid programs, as well as the disaggregated scope of the programs by traditionally excluded groups, such as the gender justice approach. When determining results indicators, the existence of statistics on the malnutrition rate in general or for different population sectors (boys, girls, youth, women, older adults, people with disabilities, ethnic groups) based on the Global Malnutrition Rate, was not evidenced in the official portals of the State entities. The application of these indicators determined the non-compliance by the state with the guarantee of a right to adequate food, requiring a profound structural and process transformation, under the premise of human rights, in order to establish a significant and necessary improvement in the results indicators, as well as generating solutions to transform gender inequalities, given the growing trend of the establishment of a feminization of poverty in Venezuela(AU)

El derecho a la alimentación y la feminización de la pobreza en Venezuela / The right to food and feminization of poverty in Venezuela

El Estado no ha utilizado todos los recursos disponibles para garantizar la realización progresiva del derecho a la alimentación. Este incumplimiento sistemático, profundiza la vulnerabilidad del derecho a la alimentación de la nación, agrava la inseguridad alimentaria y la crisis social y económica del país, lo que deriva en la incidencia directa en las mujeres, de situaciones que fomentan la feminización de la pobreza. Este artículo presenta una revisión del subconsumo calórico crónico e inadecuado en mujeres, con énfasis en mujeres embarazadas, el fomento de la existencia de matricentralidad, el aumento en la proporción de hogares con jefaturas de hogar ejercida por mujeres, la desigualdad de ingresos por género, la afectación de la salud reproductiva y aumento sustancial del embarazo precoz y muerte materna, el aumento en la proporción de mujeres económicamente inactivas, el aumento de brecha generacional en la dedicación en exclusividad de los quehaceres del hogar, y la segregación y discriminación laboral. Se evidenció en el país, una tendencia general de mujeres sobrecargadas de trabajo, con una tendencia al aumento de jefaturas del hogar ejercida por mujeres y una tendencia creciente a una feminización de la pobreza. Las observaciones indican que existen más posibilidades de que los hogares encabezados por mujeres sean más pobres y que la feminización de la pobreza acarrea la feminización de las estrategias de supervivencia y la posible continuidad generacional de la pobreza(AU)

The government has not used all available resources to guarantee the progressive realization of the right to food. This systematic breach deepens the vulnerability of the nation's right to food, aggravates food insecurity and the country's social and economic crisis, resulting in the direct impact on women of situations that foster the feminization of poverty. This article presents a review of chronic and inadequate caloric under-consumption in women, with emphasis on pregnant women, the promotion of the existence of matricentrality, the increase in the proportion of households headed by women, and income inequality by gender, the affectation of reproductive health and substantial increase in early pregnancy and maternal death, the increase in the proportion of economically inactive women, the increase in the generation gap in the exclusive dedication of household chores, and segregation and discrimination labor. In the country, there was a general tendency of women overloaded with work, with a tendency to increase the number of heads of households exercised by women and a growing tendency towards the constitution of a feminization of poverty. The observations indicate that there are more possibilities for women-headed households to be poorer and that the feminization of poverty leads to the feminization of survival strategies and the possible generational continuity of poverty(AU)

Outcomes in relapsed Hodgkins lymphoma treated with autologous and allogeneic hematopoietic cell transplantation at the Pontificia Universidade Católica

Hodgkins lymphoma is a highly curable disease. Autologous and reduced intensity allogeneic hematopoietic cell transplantations are alternatives to treat relapsed patients. Here, we report on the results of one service using these procedures. Methods: All patients who underwent transplantations in our institution between 1996 and 2014 were retrospectively studied and demographics, toxicities and survival rate were analyzed. Results: This study evaluated 24 autologous and five reduced intensity allogeneic transplantations: the median ages of the patients were 29 and 32 years, respectively. At the time of autologous transplantation, ten patients were in complete remission, nine had chemosensitive disease but were not in complete remission, three had refractory disease and the status of two is unknown. In the allogeneic group, two were in complete remission and three had chemosensitive disease. The 5-year overall survival after autologous transplantation was 42% (66% patients were in complete remission, 37% had chemosensitive disease with incom- plete remission and 0% had refractory disease) and 1-year overall survival after allogeneic transplantation was 80%. Transplant-related mortality was 0% in patients conditioned with the ifosfamide/carboplatin/etoposide (ICE), carmustine/etoposide/cyclophosphamide (BEC) and carmustine/etoposide/cytarabine/melphalan (BEAM) regimens, 37% in patients condi- tioned with busulfan-based regimens and 20% in allogeneic transplantations. Conclusions: Hematopoietic cell transplantation for relapsed Hodgkin's lymphoma is a potentially curative procedure especially in patients in complete remission at the time of autologous transplantations, and possibly after allogeneic transplantations. Further studies are necessary to clarify the role of allogeneic transplantations in the treatment of relapsed Hodgkin's lymphoma.

Experiencia de 22 años de trasplante autólogo de células hematopoyéticas en pacientes con mieloma múltiple o amiloidosis sistémica. 1992-2014 / Autologous hematopoietic cell transplantation in patients with multiple myeloma. Experience in 53 patients

Background: Autologous hematopoietic cell transplantation (THA) in patients with multiple myeloma and amyloidosis is the standard of care to promote disease free survival and quality of life. Aim: To report our experience with THA in patients with multiple myeloma. Material and Methods: Retrospective review of the hematopoietic cell transplantation database of a hospital of a Medical School. Forty seven patients with multiple myeloma and six with amyloid light chain amyloidosis were identified. Clinical and demographic data were obtained from the records. Results: The overall five year survival of patients was 55%. Transplant-related or non-relapse mortality occurred in 7%. We found no differences in outcomes among patients younger or older than 50 years. Conclusions: Our data supports that THA can be done in our country with similar results to those obtained in international transplantation centers. Chronological age should not be a limitation to offer this therapy to patients with multiple myeloma and amyloidosis.

Ocorrência de mosca-branca em Annona squamosa no estado de São Paulo / Occurrence of whitefly on Annona squamosa in São Paulo State, Brazil

No Brasil, é restrito o conhecimento sobre distribuição geográfica e plantas hospedeiras de moscas-brancas (Hemiptera: Aleyrodidae), principalmente em plantas cultivadas. Nesta comunicação, é registrada a ocorrência de Aleurodicus mirabilis (Cockerell, 1898) em altas infestações em fruta-do-conde (Annona squamosa Linnaeus, 1753) no estado de São Paulo, Brasil.

In Brazil the knowledge about the geographical distribution and host plants of whiteflies (Hemiptera: Aleyrodidae) is limited, mainly on crops. In this communication, the occurrence of Aleurodicus mirabilis (Cockerell, 1898) in high infestations in custard apple (Annona squamosa Linnaeus, 1753) is recorded in the state of São Paulo, Brazil.

Cisplatino y 5-Fluorouracilo en perfusión continua de 96 horas para canceres recidivantes o metastasicos de cabeza y cuello / Cisplatine and 5-Fluorouracil in continuing perfusion during 96 hours to recurrents or metastasis in head and neck

A 36 pacientes con carcinoma epidermoide recurrente o metastásico de cabeza y cuello fueron tratados con cisplatino y 5-fluorouracilo, ambos en perfusión continua de 96 horas. Veintiocho presentaban recidiva local, seis metástasis y dos recidiva local y a distancia. Trece habían recibido previamente quimioterapia. Se consiguieron ocho respuestas completas (22 por ciento) y siete parciales (19 por ciento), con una respuesta objetiva del 41 por ciento. La supervivencia actuarial durante un periodo de seguimiento de 59 meses fue del 17 por ciento, con una supervivencia media de ocho meses. La supervivencia actuarial en los pacientes con respuesta completa fue del 50 por ciento a los 59 meses. Las manifestaciones de toxicidad más frecuentemente encontradas fueron estomatitis, leucopenia y vómitos. El esquema terapéutico resulta efectivo y beneficia principalmente a los pacientes con recidiva local, en los cuales es posible conseguir respuesta completa.

Sindrome del olor a pies sudados: acidemia isovalerica / The sweety feet odor syndrome: iso valeric acidemia

Se describe el primer caso de acidemia Isovalérica diagnosticado en Venezuela. El cuadro clínico apareció al cuarto día de vida. El hedor característico del "Síndrome del olor a pies sudados", proveniente de la superficie corporal y de la orina del paciente; junto con la acidosis, hipotonía, letargia, leucopenia y trombocitopenia; orientó la búsqueda de laboratorio hacia la detección de un posible error innato del metabolismo. Se determinaron aminoácidos y ácidos orgánicos en sangre y orina por cromatografía en capa fina y en papel, identificandolos y cuantificándolos por cromatografía líquida de alta presión y cromatografía de gases/espectrometría de masas. La presencia en plasma de ácido Isovalérico, 3-OH_Isovalérico y 4-OH-Isovalérico, Isovaleril Glicina y 3-Metilbutiril Lactona; así como la excreción urinaria de estos compuestos, acompañados por ácidos Levulínicos, Metilsuccínico, Mesacónico (Metilfumárico), se Isovaleril-3-D-glucurónido; estableció el diagnóstico de ésta metabolopatía congénita, cuya causa es un defecto en la enzima