Plasma from patients undergoing allogeneic hematopoietic stem cell transplantation promotes NETOSIS in vitro and correlates with inflammatory parameters and clinical severity.

Introduction: NETosis, the mechanism by which neutrophils release extracellular traps (NETs), is closely related to inflammation. During the allogeneic hematopoietic stem cell transplantation (allo-HSCT), different stimuli can induce NETs formation. Inflammation and endothelial injury have been associated with acute graft-versus-host disease (aGVHD) and complications after allo-HSCT. We focus on the study of NETosis and its relation with cytokines, hematological and biochemical parameters and clinical outcomes before, during and after allo-HSCT. Methods: We evaluate the capacity of plasma samples from allo-HSCT patients to induce NETosis, in a cell culture model. Plasma samples from patients undergoing allo-HSCT had a stronger higher NETs induction capacity (NETsIC) than plasma from healthy donors throughout the transplantation process. An optimal cut-off value by ROC analysis was established to discriminate between patients whose plasma triggered NETosis (NETs+IC group) and those who did not (NETs-IC group). Results: Prior to conditioning treatment, the capacity of plasma samples to trigger NETosis was significantly correlated with the Endothelial Activation and Stress Index (EASIX) score. At day 5 after transplant, patients with a positive NETsIC had higher interleukin (IL)-6 and C-reactive protein (CRP) levels and also a higher Modified EASIX score (M-EASIX) than patients with a negative NETsIC. EASIX and M-EASIX scores seek to determine inflammation and endothelium damage, therefore it could indicate a heightened immune response and inflammation in the group of patients with a positive NETsIC. Cytokine levels, specifically IL-8 and IL-6, significantly increased after allo-HSCT with peak levels reached on day 10 after graft infusion. Only, IL-10 and IL-6 levels were significantly higher in patients with a positive NETsIC. In our small cohort, higher IL-6 and IL-8 levels were related to early severe complications (before day 15 after transplant). Discussion: Although early complications were not related to NETosis by itself, NETosis could predict overall non-specific but clinically significant complications during the full patient admission. In summary, NETosis can be directly induced by plasma from allo-HSCT patients and NETsIC was associated with clinical indicators of disease severity, cytokines levels and inflammatory markers.

Effort-based decision-making as a determinant of supported employment outcomes in psychotic disorders.

Schizophrenia is associated with a heavy economic burden in the United States that is partly due to the high rates of chronic unemployment. Individual Placement and Support (IPS) is an evidenced-based type of supported employment that can improve job obtainment and work outcomes in psychotic disorders. Outcomes vary widely and a persistent challenge for IPS is low levels of engagement in the initial job search phase. Past studies have focused on interview-based motivation deficits as a key determinant of poor treatment engagement and work outcomes in schizophrenia. New validated performance-based measures of motivation, including effort-based decision-making (EBDM) tasks, may explain supported employment outcomes and provide insights into individual differences in IPS outcomes. This study investigated the degree to which IPS engagement (i.e., number of sessions attended during the first four months of service delivery) was related to baseline interview-based motivation deficits and performance on three EBDM tasks - two tasks of physical effort and one of cognitive effort (i.e., Balloon Task, Effort Expenditure for Rewards Task, Deck Choice Effort Task) - in a sample (N = 47) of people with a psychotic disorder. Results indicated that the level of EBDM performance, specifically on the Balloon Task, predicted IPS engagement, accounting for an additional 17 % of the variance above and beyond interview-based motivation deficits (total R2 = 24 %). Overall, these findings suggest that addressing motivational deficits in effort-based decision-making may be beneficial to IPS engagement, which in turn may improve the trajectory of work outcomes.

t(10;12)(q24;q15): A new cytogenetic marker in hematological malignancies.

Cytogenetic studies have played a crucial role in the discovery of genes involved in several diseases. In the field of oncohematology, cytogenetics is still necessary for the classification and prognosis of many diseases. Here we report a new recurrent chromosome translocation, t(10;12)(q24;q15), in two patients with different hematological malignancies: myelodysplastic syndrome with excess blasts (MDS-EB), and myelofibrosis (MF) secondary to essential thrombocythemia (ET). The chromosome alteration was observed as a sole karyotype change in the patient with MDS-EB, both at the initial diagnosis and following progression to MDS-EB2. A putative HMGA2-KLLN rearrangement by RNA-sequencing was detected in this patient. The patient with ET, had a normal karyotype at diagnosis and the t(10;12)(q24;q15) translocation emerged as a sole cytogenetic alteration after transformation, and when MF was evident. We reviewed the literature to determine whether this chromosome abnormality had previously been described in other hematological patients and found two cases: an aggressive T-cell lymphoblastic lymphoma (T-LBL) and a case of transformed chronic myeloproliferative syndrome (CMS), in both of which t(10;12)(q24;q15) was also the only karyotype change. The clinical evolution of all four cases suggested that t(10;12)(q24;q15) is associated with a poor outcome in oncohematological patients.

Cuerpo extraño en vía aérea inferior de dos años de evolución: reporte de caso / Foreign body in lower airway of two years of evolution: case repor

La aspiración de un cuerpo extraño es un acontecimiento fatal que puede conducir a la muerte, definido como el ingreso de manera accidental de un objeto, orgánico o inorgánico, a la vía aérea, alojándose ya sea en la laringe, tráquea o bronquios. Este evento es común en la población pediátrica especialmente entre niños de 18 meses a 3 años. El sitio de alojamiento más común es en el bronquio derecho. El diagnóstico tardío de cuerpo extraño en bronquio está relacionado a neumonías recurrentes, bronquiectasias y atelectasias. Presentamos el caso de un paciente de 12 años de edad con antecedentes de sospecha de aspiración de cuerpo extraño en vía aérea desde hace 2 años. Presentando en ese intervalo de tiempo tos productiva de color verde amarillento y persistente, halitosis, neumonías a repetición, y con hipocratismo digital simétrico. Con ayuda de Imágenes por tomografía axial computarizada y broncoscopia virtual se evidencia el cuerpo extraño alojado en bronquio principal izquierdo, revelando el pulmón izquierdo colapsado, hepatizado, con bronquiectasias inferiores, desplazamiento de corazón hacia la izquierda y pulmón derecho sobre distendido. * Médico residente de Tercer año pediatría, UNHA-VS...(AU)

Bone marrow characterization in COPD: a multi-level network analysis.

BACKGROUND: Bone marrow (BM) produces hematopoietic and progenitor cells that contribute to distant organ inflammation and repair. Chronic obstructive pulmonary disease (COPD) is characterized by defective lung repair. Yet, BM composition has not been previously characterized in COPD patients. METHODS: In this prospective and controlled study, BM was obtained by sternum fine-needle aspiration in 35 COPD patients and 25 healthy controls (10 smokers and 15 never-smokers). BM cell count and immunophenotype were determined by microscopy and flow cytometry, respectively. Circulating inflammatory (C-reactive protein, IL-6, IL-8) and repair markers (HGF, IGF, TGF-ß, VEGF) were quantified by ELISA. Results were integrated by multi-level network correlation analysis. RESULTS: We found that: (1) there were no major significant pair wise differences between COPD patients and controls in the BM structural characteristics; (2) multi-level network analysis including patients and controls identifies a relation between immunity, repair and lung function not previously described, that remains in the COPD network but is absent in controls; and (3) this novel network identifies eosinophils as a potential mediator relating immunity and repair, particularly in patients with emphysema. CONCLUSIONS: Overall, these results suggest that BM is activated in COPD with impaired repair capacity in patients with more emphysema and/or higher circulating eosinophils.

Caracterización del lactante sibilante recurrente hospitalizado y utilidad del estudio internacional de sibilancias / Characterization of hospitalized Infants with recurrent wheezing and evaluation of the International Study of Wheezing

Antecedentes: La sibilancia es un trastorno muy frecuente en la infancia que contabiliza alta demanda de consulta médica y atención en los servicios de emergencia. Tiene relativa- mente altas tasas de hospitalización y está relacionado con la mortalidad infantil inducida por infecciones respiratorias. Objetivo: Esta- blecer las características clínicas y epidemioló- gicas de los lactantes hospitalizados en el Mario Catarino Rivas durante el período 15 de junio a 16 de septiembre del 2015 y determinar la utilidad del cuestionario Estudio Internacio- nal de las Sibilancias en el Lactante (EISL) modi- cado aplicado a padres/responsables. Pacien- tes y Métodos: Estudio descriptivo transversal no experimental. Los padres de 100 niños con edades comprendidas entre 6 y 18 meses hospitalizados en la sala lactantes respondie- ron a través de una entrevista directa el cues- tionario EISL modi cado. Para las característi- cas de los lactantes, se utilizaron frecuencias, porcentajes y medidas de tendencia central, para valorar la utilidad del test, se calculó con abilidad y validez total. Los datos se anali- zaron con SPSS versión 21.0 y Excel. Resulta- dos: la prevalencia de sibilancias recurrentes encontrada fue del 31% (n=31); con abilidad del cuestionario se valoró con el α cronba- ch=0.841 y KR-20=0.8333; validando el instru- mento con validez de contenido: juicio de expertos 0.88, validez de criterio: rxy≥0.83, validez de constructo: KMO=0.68; la validez total fue de 0.79 Conclusiones: la prevalencia de sibilancias es más alta que lo reportado en la literatura para América Latina; el cuestionario acortado EISL es válido estadísticamente para ser aplicado en el Hospital Mario Catarino Rivas en Lactantes...(AU)

Caracterización de los pacientes con fibrosis quistica diagnosticados por cloruros en sudor / Characterization of patients with Cystic Fibrosis diagnosed through sweat chloride test

Antecedentes y Objetivo: La Fibrosis Quística (FQ) es una enfermedad genética de herencia autosómica recesiva, caracterizada por disfun- ción de las glándulas de secreción exocrina. En Honduras no hay ningún tipo de revisión sobre este tema, por lo que se realizó este estudio con el objetivo de caracterizar los pacientes diagnosticados con FQ mediante prueba de Cloruros en Sudor. Pacientes y Métodos: Estu- dio descriptivo con enfoque cuantitativo, se revisaron expedientes clínicos y se aplicó un instrumento de 20 preguntas a pacientes en control médico, durante enero 2010 a junio 2015. Resultados: Se presentan cinco pacien- tes con diagnóstico de brosis quística. La edad al momento del diagnóstico varió de Recién nacido a 5 años; 2 de ellos con antecedentes familiares para dicha enfermedad. Al inicio de la enfermedad 3 tenían sintomatología respira- toria y digestiva. En relación a talla/edad al momento del diagnóstico, 4 se encontraban por debajo del percentil Z-3. La totalidad repor- tó cultivos positivos para Pseudomona auregi- nosa. Respecto al manejo terapéutico de los pacientes en la totalidad de ellos se indicó nebulizaciones con Salino hipertónico al 7%, Beta-2-agonistas, antibióticos sistémicos y suplementación enzimática y de vitaminas liposoluble. En 2 pacientes, se encontró la mutación ΔF508 en el gen CFTR. Conclusio- nes: La Fibrosis Quística todavía es una patolo- gía en gran medida desconocida. Se recomien- da implementar el tamizaje neonatal para ayudar al diagnóstico temprano y la prueba de cloruros en sudor en pacientes de mayor edad y con alta sospecha clínica de esta enfermedad...(AU)

Acute lymphoblastic leukemia with e1a3 BCR/ABL fusion protein. A report of two cases.

B Acute Lymphoblastic leukemia (B-ALL) with Philadelphia chromosome (Ph') is a neoplasm of lymphoblast committed to the B cell lineage. The clinical presentation of B-ALL Ph'+ is similar to B-ALL, but is more common in adults than in children. The e1a3 rare variant is produced by the fusion of BCR exon 1 to ABL exon 3. The presence of this translocation has been associated with good disease outcome for chronic myeloid leukemia in a very small series of only 5 cases; there is no such evidence for B-ALL. We report two new cases of B-ALL Ph+ with the rare e1a3 fusion transcript. The e1a3 and e1a2 (p190) transcripts have been reported to have a similar molecular weight and probably a similar clinical profile, thus in these cases the presence of e1a3 was associated with extramedullary infiltration and disease acceleration.

Chronic myeloid leukemia with an e1a3 BCR-ABL fusion protein: transformation to lymphoid blast crisis.

Chronic myelogenous leukemia (CML) results from the neoplastic transformation of a hematopoietic stem cell. CML is cytogenetically characterized by the presence of the Philadelphia chromosome (Ph'). Most patients with CML express e13a2 or e14a2 mRNAs that result from a rearrangement of the major breakpoint cluster regions (M-BCR) generating the 210-kDa (p210BCR-ABL) fusion proteins b2a2 or b3a2 respectively. The e1a3 CML-related atypical translocation has been reported with an indolent clinical course, low leukocyte count, long chronic phase even without treatment and good response to therapy. We report the case of a patient initially diagnosed as CML in chronic phase whose cells expressed the e1a3 variant. The patient readily responded to imatinib 400 mg with the achievement of a rapid complete cytogenetic response and the normalization of the blood count values, but after 5 months transformed into lymphoid blast crisis.

Enfermedad plauropulmonar complicada en el paciente pediátrico / Complicated pleuropulmonary disease in pediatric patients

Antecedentes: La neumonía tiene gran connotación en pediatría por su incidencia, gravedad potencial y elevada mortalidad. Las complicaciones se presentan hasta en 40% de los casos, principalmente la Enfermedad Pleuropulmonar Complicada (EPPC). Objetivo: Caracterizar clínica, radiológica y microbilógica- mente la EPPC en niños ingresados en sala de pediatría del Hospital Nacional Dr. Mario Catari- no Rivas (HNMCR) en un período comprendido entre Octubre del 2013 y Octubre del 2014. Pacientes y métodos: Estudio descriptivo transversal, realizado en niños con edad com- prendida entre 28 días y 18 años con diagnósti- co EPPC ingresados al Servicio de Pediatría. Fueron 21 niños hospitalizados con estos crite- rios. Los datos se recolectaron de cada expe- diente a través de un formulario que reunía información desde la hospitalización hasta el egreso del paciente. Resultados: El 62% eran mayores de 5 años. La relación hombre-mujer fue de 1:1. El 24% de los pacientes estaban desnutridos. Se diagnosticaron 5 tipos de EPPC, los más comunes fueron el empiema (n=6, 29%) y el pioneumotórax (n=5, 24%). Se cultivó el líquido pleural encontrando S. aureus y S. pneu- moniae en 44% y 22% respectivamente. El 36% de los cultivos fueron negativos. Conclusiones: Los pacientes con EPPC fueron en su mayoría escolares y adolescentes eutróficos. No se encontró relación que indique la desnutrición como factor de riesgo para la EPPC. Existe una relación directamente proporcional entre el tiempo de inicio de los síntomas, y las formas más avanzadas de EPPC.

Genetics of lymphocytes influences the emergence of second cancer in chronic lymphocytic leukemia.

BACKGROUND: Patients affected by chronic lymphocytic leukemia (CLL) have an increased risk of developing a second cancer. There is not a definitive explanation for this phenomenon, although some hypotheses have been postulated. The aim of the present work was to assess the presence of second cancer in untreated patients with CLL who were cytogenetically characterized, and secondly to investigate if there is a correlation between the genetics of CLL and the emergence of second cancer. PATIENTS AND METHODS: We performed conventional cytogenetics and Fluorescent in situ hybridization analyses in a series of 106 patients. RESULTS: We observed that nearly 8% of cases developed second cancer, mostly epithelial tumors. The majority of them presented two common features, del(13)(q14.3) and the presence of at least two genetic alterations. CONCLUSION: We suggest that the genetic background of CLL, particularly the presence of several genetic alterations, influences the emergence of second cancer in patients affected by CLL.

Informe del Taller Ibérico de Histocompatibilidad 2013. Componente de análisis de situación de procedimiento de pruebas cruzadas en guardias de trasplante de órganos / Report of the 2013 Iberian Histocompatibility Workshop of 2013. Component analysis of the proceedings of cross-matching procedures in emergency organ transplantation

No disponible

"Aunque usted no lo crea": doble arco aórtico descubierto en búsqueda de un cuerpo extraño / "Believe it or not": Double aortic arch discovered in search of a foreign body

Se presenta caso de paciente de dos años de edad, quien ingresó al hospital con sospecha fundada de cuerpo extraño en vía aérea, luego de haber presentado un evento asfíctico. Sin embargo, al realizar la broncoscopía, se encontró una compresión extrínseca pulsátil diagnóstica de anillo vascular. Se confirmó el diagnóstico con la angiotomografía que reveló doble arco aórtico completo, además de mos- trar el cuerpo extraño vegetal (semilla de licha o rambután, Naphelium lappaceum ) ubicado en el tercio distal del esófago. A pesar de las limitantes propias de la institución logramos realizar el diagnóstico clínico, e imagenológico de la paciente además de resolver la patología de urgencia extrayendo el cuerpo extraño en vía digestiva en 24 horas tras su ingreso...(AU)

B cells from common variable immunodeficiency patients fail to differentiate to antibody secreting cells in response to TLR9 ligand (CpG-ODN) or anti-CD40+IL21.

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterised by hypogammaglobulinaemia and antibody deficiency to T dependent and independent antigens. Patients suffer from recurrent respiratory infections and poor response to vaccination. Although the underlying molecular defect is unknown, most CVID patients show impaired late B cell differentiation. We investigated B cell differentiation and immunoglobulin secretion induced by two different stimuli: TLR9 specific ligand (CpG-ODN) and anti-CD40 combined with IL21. The contribution of BCR signalling (anti-IgM stimulation) was also evaluated. B cells from CVID patients produced low levels of IgG and IgA in response to both kinds of stimuli that was not restored by anti-IgM. Production of IgM was conserved when cells were stimulated with anti-CD40 and IL21. These results point to a wide signalling defect in B lymphocytes from CVID patients that may be related to their hypogammaglobulinaemia and poor response to vaccination.

Aspiración de cuerpos extraños en pediatría. experiencia de 15 años en el Hospital Nacional Dr. Mario Catarino Rivas / Foreign bodies Aspiration in a Pediatric Population. A 15 years experience in the National Hospital "Dr. Mario Catarino Rivas

La aspiración en vía respiratoria de un cuerpo extraño en la edad pediátrica es un evento relativamente frecuente y potencialmente peligroso, que exige el máximo conocimiento de la patologíay su sospecha diagnóstica fundada permitiéndonos así el inicio de un tratamiento inmediato para evitar temidas complicaciones incluyendo la muerte del niño. Se presenta la experiencia de 145 casos en el Hospital Nacional "Mario Catarino Rivas" de la Ciudad de San Pedro Sula, Honduras en el período 1996 - 2010. Predominaron en esta serie: el sexo masculino, tiempo de aspiración menor de una semana de evolución, la radiografía de tórax normal como hallazgo radiológico predominante, las semillas como el cuerpo extraño más frecuentemente extraído, siendo la broncoscopía rígida el procedimiento más utilizado para su extracción...(AU)

Defective B cell response to TLR9 ligand (CpG-ODN), Streptococcus pneumoniae and Haemophilus influenzae extracts in common variable immunodeficiency patients.

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinaemia and antibody deficiency to both T dependent and independent antigens. Patients suffer from recurrent sinopulmonary infections mostly caused by Streptococcus pneumoniae and Haemophilus influenzae, but also gastrointestinal or autoimmune symptoms. Their response to vaccination is poor or absent. In this study we investigated B cell activation induced by the TLR9 specific ligand (CpG-ODN) and bacterial extracts from S. pneumoniae and H. influenzae known to stimulate several TLR. We found that B cells from CVID patients express lower levels of CD86 after stimulation with CpG-ODN, S. pneumoniae and H. influenzae extracts in combination with anti-IgM antibody and also display a lower proliferative index when stimulated with bacterial extracts. Our results point to a broad TLR signalling defect in B lymphocytes from CVID patients that may be related to the hypogammaglobulinaemia and poor response to vaccination characteristic of these patients.