Sarcoma folicular de células dendríticas mediastinal: primer reporte de caso en Costa Rica / Mediastinal Follicular Dendritic Cell Sarcoma: First Case Report in Costa Rica

El sarcoma folicular de células dendríticas (SFCD) es una neoplasia maligna rara derivada de las células dendríticas foliculares. Ha sido clasificado, dadas sus características inmunohistoquímicas, como parte del grupo de los sarcomas, donde representa un porcentaje menor al 1%. Actualmente, existen menos de 1.000 reportes en la literatura a nivel mundial, lo cual plantea una dificultad no sólo diagnóstica, siendo confundido frecuentemente con neoplasias de tipo linfoide; sino también terapéutica al no existir un claro consenso sobre su manejo definitivo. Esta revisión de caso clínico describe el primer caso reportado de SFCD en Costa Rica.

Follicular dendritic cell sarcoma (SFCD) is a rare malignant neoplasm derived from follicular dendritic cells, which has been classified, given its immunohistochemical characteristics, as part of the group of sarcomas, where it represents less than 1%. Currently, there are less than 1000 reports in the literature worldwide, which generates a difficulty not only in diagnosis, being frequently confused with lymphoid type neoplasms; but also, as therapeutic as there is no clear consensus on its definitive management. This clinical case review describes the first reported case of SFCD in Costa Rica.

Paciente varón de tres años con sibilancias y tos crónica / Three-Year-Old Male with Wheezing and Chronic Cough

Se presenta el caso de un niño de 3 años con diagnóstico de asma, rinitis alérgica, características craneofaciales dismórficas e infecciones respiratorias altas y bajas recurrentes, manejado como asma desde un inicio. Como parte del estudio de comorbilidades, se decide realizar una prueba del sudor que sale en rango intermedio y más tarde se encuentra una mutación, donde se obtiene un resultado positivo para una copia que se asocia a fibrosis quística. Se revisará el caso, así como el diagnóstico, clínica y tratamiento del síndrome metabólico relacionado con el regulador de conductancia transmembrana de fibrosis quística (CRMS).

We present the case of a 3-year-old boy with a diagnosis of asthma, allergic rhinitis, dysmorphic craniofacial characteristics and recurrent upper and lower respiratory infections, managed as asthma from the beginning. As part of the study of comorbidi-ties, it was decided to carry out a sweat test that came out in the intermediate range and later one mutation was found, where a positive result was obtained for a copy that is associated with cystic fibrosis. The case will be reviewed, as well as the diagnosis, symptoms and treatment of the metabolic syndrome related to the cystic fibrosis trans-membrane conductance regulator (CRMS).

Tos crónica por hipersensibilidad refleja: un nuevo paradigma con implicancias diagnósticas y terapéuticas / Cough hypersensitivity syndrome: a new paradigm with diagnostic and therapeutic implications

La tos es un reflejo protector del aparato respiratorio que si se prolonga en el tiempo puede afectar seriamente la calidad de vida. Recientemente un nuevo paradigma califica a la tos por hipersensibilidad refleja consecuente a una exagerada sensibilidad de las terminaciones nerviosas periféricas a diversos estímulos y a alteraciones en los núcleos centrales integradores del reflejo. Este nuevo paradigma tiene implicancias terapéuticas, más allá del tratamiento de la causa de la tos. En este artículo se revisarán los nuevos mecanismos implicados en la tos por hipersensibilidad refleja y las nuevas drogas en investigación que modulan el reflejo tusígeno. A pesar de las nuevas investigaciones farmacológicas se necesitan evidencias más seguras para aconsejar un tratamiento "sintomático" de estos pacientes que contribuya al control de la hipersensibilidad central y periférica del reflejo de la tos.

Cough is a protective reflex of the respiratory system that if persist over time can seriously affect the quality of life. Recently, a new paradigm qualifies cough as a consequence of a reflex hypersensitivity resulting from an exaggerated sensitivity of the peripheral nerve endings to various stimuli and central neural alterations in cough processing. This new paradigm has therapeutic implications, beyond the causal treatment of cough. In this article we will review the new mechanisms involved in cough due to reflex hypersensitivity and new therapeutic strategies under development that modulate the cough reflex. Despite the new pharmacological investigations, safer evidence is needed to advise a "symptomatic" treatment of these patients that contributes to the control of central and peripheral hypersensitivity of the cough reflex

Diagnóstico y tratamiento de la tos crónica en pediatría / Diagnostic and treatment approach of chronic cough in childhood

La tos crónica es un síntoma de diversos procesos patológicos de origen respiratorio o extrarrespiratorio, con impacto negativo en la calidad de vida del niño y su familia. El pediatra debe guiar su acción en busca del diagnóstico etiológico. Una cuidadosa historia clínica es clave para el diagnóstico y una guía para solicitar los estudios complementarios necesarios para la detección de las causas definitivas. La exacta identificación etiológica de la tos crónica debe ser seguida de un tratamiento específico. El abordaje sintomático con antitusivos y mucolíticos es excepcionalmente necesario y debería reservarse para algunas situaciones de tos inespecífica sin causa aparente.(AU)

Chronic cough is a symptom of various respiratory and non-respiratory conditions with negative impact on quality of life of children and their families. The pediatricians should focus their efforts in search for etiological diagnosis. A careful medical history and physical examination are the mainstays of diagnosis and guidance to further studies that may contribute to detection of final causes. The etiological identification of chronic cough must be followed by specific treatment. The symptomatic approach with antitussives and mucolytic drugs is exceptionally necessary and should be considered for some situations of nonspecific cough without any specific disease association.(AU)

Diagnóstico y tratamiento de la tos crónica en pediatría. / [Diagnostic and treatment approach of chronic cough in childhood].

Chronic cough is a symptom of various respiratory and non-respiratory conditions with negative impact on quality of life of children and their families. The pediatricians should focus their efforts in search for etiological diagnosis. A careful medical history and physical examination are the mainstays of diagnosis and guidance to further studies that may contribute to detection of final causes. The etiological identification of chronic cough must be followed by specific treatment. The symptomatic approach with antitussives and mucolytic drugs is exceptionally necessary and should be considered for some situations of nonspecific cough without any specific disease association.

Tos crónica pediatría / Chronic cough in pediatrics

The objective has been to identify the different etiologies and elaborate a diagnostic and therapeutical methodology for patients with chronic cough. During one year we studied prospectively 83 patients with persistent cough of daily appearance with an evolution of four or more weeks and no previous etiologic diagnosis. We worked on three diagnostic (D) levels. Dl: Based on the anamnesis and physical examination. D2: Sequential incorporation of complementary exams. D3: Evaluation of the response to the specific treatment. We divided the population into 2 groups: Gl healthy children, G2 children followed in our hospital for different conditions. The mean age was 4.7 years (range, 3 months to 15 years), and the average duration of cough was 4.9 months (range, 1 to 36 months). In Gl the following causes were identified in 78 children: cough variant asthma 41 (52 percent), asthma + upper respiratory tract infections 8 (10 percent), asthma + lower respiratory tract infections 6 (7 percent), postnasal drip syndrome (sinusitis, adenoiditis) 5 (6 percent), psychogenic 6 (7 percent), undetermined 4 (5 percent), gastroesophageal reflux 2, asthma + cigarette 2, AIDS 1, Sjogren syndrome 1, vascular ring 1, cricopharyngeal foreign body 1. In G2 out of 5 children we have found: 2 children with chronic encephalopathies who had swallowing disorders and gastroesophageal reflux, 1 patient with Down syndrome presenting hypogammaglobulinemia and bronchiectasis, 1 tracheaesophageal fistula in H in a child with recurrent pneumonia, 1 lymphocytic pneumonia in an AIDS patient. The Dl was correct in 92 percent of the cases. The specific therapy has proved useful for achieving the remission of the symptoms. Although asthma is the most frequent cause of chronic cough, other etiologies exist and must be ruled out.(AU)

Predictores de coqueluche al ingreso en lactantes hospitalizados con infección respiratoria aguda baja. / [Pertussis predictors in hospitalized infants with acute lower respiratory tract infection].

BACKGROUND: Pertussis, or whooping cough, caused by Bordetella pertussis (BP) is a re-emerging problem in our environment. Although generally considered that the disease is relatively easy to identify infections respiratory syncytial virus (RSV) can present with similar symptoms in infants remains difficult discrimination. OBJECTIVE: Compare clinical symptoms at admission and complementary studies in infants hospitalized with acute respiratory infection (ARI) and RSV by BP to establish markers that enable their early clinical prediction. MATERIALS AND METHODS: Observational, analytical, case-crossover cross comparing younger than 6 months hospitalized with suspected IRA and pertussis (2007-2012) in which BP identified (PCR and culture) and / or VRS (immunofluorescence in nasal secretions). Coinfections were excluded. Bivariate analysis was performed by calculating OR with 95

CI. Were considered significant at p <0.05. The variables studied were age, sex, hits cough, cyanosis, vomiting, apnea, wheezing and CBC with differential RESULTS: We included 174 infants, 72 (41

) BP and 102 (59

) VRS. Age 2 ± 1 months (range :1-6). In both groups was documented cough and wheeze (OR: 1.2 (0.9 to 1.5) p: 0.1 and OR = 0.9 (0.8 to 1.06) p: 0.2, respectively ). Cyanosis (87

vs. 6

, OR: 14.8 p <0.01), apnea (38

vs. 3

, OR: 13.4 p <0.01) and vomiting (26

vs. 5

, OR: 3.4 p <0.01) were more frequent in infants with BP. The absolute lymphocyte count was significantly higher in children with BP (9387 ± 6317 vs. 5127 ± 2766, p <0.01). By ROC curve was identified at 9000 cells / ml as the best point to differentiate VSR BP (AUC = 0.73, 95

CI :0,64-0, 81). CONCLUSIONS: In infants under 6 months with IRA income presence of apnea, cyanosis and lymphocytosis allowing predict significantly differentiate between pertussis those with RSV infections.

Predictores de coqueluche al ingreso en lactantes hospitalizados con infección respiratoria aguda baja / [Pertussis predictors in hospitalized infants with acute lower respiratory tract infection].

BACKGROUND: Pertussis, or whooping cough, caused by Bordetella pertussis (BP) is a re-emerging problem in our environment. Although generally considered that the disease is relatively easy to identify infections respiratory syncytial virus (RSV) can present with similar symptoms in infants remains difficult discrimination. OBJECTIVE: Compare clinical symptoms at admission and complementary studies in infants hospitalized with acute respiratory infection (ARI) and RSV by BP to establish markers that enable their early clinical prediction. MATERIALS AND METHODS: Observational, analytical, case-crossover cross comparing younger than 6 months hospitalized with suspected IRA and pertussis (2007-2012) in which BP identified (PCR and culture) and / or VRS (immunofluorescence in nasal secretions). Coinfections were excluded. Bivariate analysis was performed by calculating OR with 95

CI. Were considered significant at p <0.05. The variables studied were age, sex, hits cough, cyanosis, vomiting, apnea, wheezing and CBC with differential RESULTS: We included 174 infants, 72 (41

) BP and 102 (59

) VRS. Age 2 ± 1 months (range :1-6). In both groups was documented cough and wheeze (OR: 1.2 (0.9 to 1.5) p: 0.1 and OR = 0.9 (0.8 to 1.06) p: 0.2, respectively ). Cyanosis (87

, OR: 13.4 p <0.01) and vomiting (26

, OR: 3.4 p <0.01) were more frequent in infants with BP. The absolute lymphocyte count was significantly higher in children with BP (9387 ± 6317 vs. 5127 ± 2766, p <0.01). By ROC curve was identified at 9000 cells / ml as the best point to differentiate VSR BP (AUC = 0.73, 95

CI :0,64-0, 81). CONCLUSIONS: In infants under 6 months with IRA income presence of apnea, cyanosis and lymphocytosis allowing predict significantly differentiate between pertussis those with RSV infections.