Type and location of bronchiectasis in a cohort of adults with common variable immunodeficiency / Tipo y localización de bronquiectasias en una cohorte de adultos con inmunodeficiencia común variable

Background: Bronchiectasis are permanent dilatations of the bronchi. Its prevalence in patients with variable common immunodeficiency (CVID) is high, however there is little information regarding the type and location of the same; therefore the objective of this study is to know the type and location of bronchiectasis in a cohort of adult patients with CVID. Methods: It has been made a transversal, observational and descriptive study that included 32 adult patients with diagnosis of CVID according to the criteria of the European Society of Immunodeficiencies (ESID). All patients underwent high resolution pulmonary computed tomography (HRCT), which were interpreted by an expert radiologist. The frequency, type and location of bronchiectasis were reported using descriptive statistics. Results: Thirty-two adult patients, ten men (31.25%) and 22 women (68.7%), were included. 40.6% had bronchiectasis. 23% had a lobe involvement, 15.3% two lobes, 46.1% 3 lobes and 15.3% complete involvement of the parenchyma. The types of bronchiectasis were distributed as follows: tubular 38.4%, varicose 23% and cystic and tubular combinations 15.3%, cystic and varicose 15.3% and cystic, tubular and varicose 7.6%. Conclusions: Our results show that 40% of adult patients with CVID have BQs, usually affecting three pulmonary lobes, located mainly in the right and middle lower lobe; The tubular type, is the most common. Their timely diagnosis and treatment can improve survival and reduce costs for patients and health care.

Introducción: Las bronquiectasias (BQs) son dilataciones permanentes de los bronquios. Su prevalencia en pacientes con inmunodeficiencia común variable (IDCV) es alta, sin embargo existe escasa información respecto al tipo y localización de las mismas. El objetivo de este estudio es conocer el tipo y localización de las bronquiectasias en una cohorte de pacientes adultos portadores de IDCV. Métodos: Estudio transversal, observacional y descriptivo que incluyó a 32 pacientes adultos con diagnóstico de IDCV de acuerdo con los criterios de la Sociedad Europea de Inmunodeficiencias. A todos se les realizó tomografía de alta resolución pulmonar, las cuales fueron interpretadas por un médico radiólogo experto. Se reportó la frecuencia, tipo y localización de las bronquiectasias mediante estadística descriptiva.Resultados: se incluyeron 32 pacientes adultos, diez hombres y 22 mujeres. El 40.6% presentaron bronquiectasias. El 23% tenía afección en un lóbulo, el 15.3% dos lóbulos, 46.1% 3 lóbulos y el 15.3% afectación completa del parénquima; distribuidos de la siguiente manera: tubulares 38.4%, varicosas 23% y las combinaciones quísticas y tubulares 15.3%, quísticas y varicosas 15.3% y quísticas, tubulares y varicosas 7.6%. Conclusión: Nuestros resultados muestran que el 40% de los pacientes adultos con IDCV tienen BQs, suelen afectar tres lóbulos pulmonares, el tipo más común fue el tubular. Su diagnóstico y tratamiento oportuno puede mejorar la supervivencia y reducir los costos para el paciente y las instituciones de salud.

[Chronic myeloid leukemia in an adult with common variable immunodeficiency]. / Leucemia mieloide crónica en un adulto con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency is a primary immunodeficiency, in which from 70 to 80 % of patients have tumors and 25 % of cases are associated with autoimmune diseases. Common variable immunodeficiency patients have a higher incidence of neoplasms, with a risk 12-18 times higher than the general population. There are few cases of common variable immunodeficiency patients with leukemia. CLINICAL CASE: Female of 36 years old, with left upper quadrant pain, early satiety, weight loss of 8 kg in three months and splenomegaly. The complete blood count showed: leukocytosis 206 000/mL, with 8 % blasts, platelets 530 000/mL and hemoglobin 8 mg/dL. Abdominal ultrasound: 19??12 cm splenomegaly. Karyotype BCR/ABL IS 64.20 %, 100 % Philadelphia chromosome. The diagnosis was of chronic myeloid leukemia. Given the presence of recurrent respiratory tract infection, frequent diarrheas and reduced concentrations of IgG, IgM and IgA, common variable immunodeficiency was diagnosed and human immunoglobulin was used successfully. CONCLUSIONS: The association between chronic myeloid leukemia and common variable immunodefficiency is unusual. Given the high frequency of hematological neoplasm in common variable immunodeficiency patients, we suggest that hematological patients with repeated infections and decreased concentrations of immunoglobulin be referred to an immunological evaluation.

INTRODUCCIÓN: la inmunodeficiencia común variable es la inmunodeficiencia primaria más sintomática, 70 a 80 % de los pacientes presentan neoplasias. Existen escasos informes de pacientes portadores de leucemia mieloide crónica e inmunodeficiencia común variable. CASO CLÍNICO: mujer de 36 años, quien inició su padecimiento con dolor en hipocondrio izquierdo, pérdida de peso de 8 kg en tres meses y esplenomegalia. Los resultados de la biometría hemática indicaron cuenta leucocitaria de 206 000/mL, 8 % de blastos, cuenta plaquetaria de 530 000/mL y hemoglobina de 8 g/dL. Con la ultrasonografía abdominal se identificó esplenomegalia de 19 ´ 12 cm. El cariotipo mostró el gen BCR/ABL 64.20 % IS y cromosoma Filadelfia 100 %. Se diagnosticó leucemia mieloide crónica. Por infecciones gastrointestinales y respiratorias frecuentes, así como por concentraciones reducidas de IgG, IgM e IgA, la paciente fue valorada por el servicio de alergia e inmunología clínica, donde se diagnosticó inmunodeficiencia común variable. CONCLUSIONES: la relación de la leucemia mieloide crónica con la inmunodeficiencia común variable es poco frecuente. Es importante que los pacientes portadores de leucemia mieloide crónica que cursen con infecciones de repetición, se realicen cuantificación de inmunoglobulinas séricas y, en caso de disminución de las mismas, sean referidos para su valoración y tratamiento.

Preparation of a three-dimensional extracellular matrix by decellularization of rabbit livers.

INTRODUCTION: the availability of transplantable livers is not sufficient to fulfill the current demand for grafts, with the search for therapeutic alternatives having generated different lines of research, one of which is the use of decellularized three-dimensional biological matrices and subsequent cell seeding to obtain a functional organ. OBJECTIVE: to produce a decellularization protocol from rabbit liver to generate a three-dimensional matrix. METHODS: a combination of physical, chemical (Triton X-100 and SDS) and enzymatic agents to decellularize rabbit livers was used. After 68 h of retrograde perfusion, a decellularized translucent matrix was generated. To evaluate if the decellularization protocol was successful, with the extracellular matrix being preserved, we carried out histological (light microscopy and scanning electron microscopy) and biochemical (DNA quantification) studies. RESULTS: the decellularization process was verified by macroscopic observation of the organ using macroscopic staining, which revealed a correct conservation of bile and vascular trees. A microscopic observation corroborated these macroscopic results, with the hematoxylin-eosin staining showing no cells or nuclear material and the presence of a portal triad. Wilde´s staining demonstrated the conservationof reticulin fibers in the decellularized matrix. In addition, scanning electron microscopy revealed a preserved Glisson´s capsule and a decellularized matrix, with the DNA quantification being less than 10 % in the decellularized liver compared to control. Finally, the time taken to develop the decellularization protocol was less than 96 hours. CONCLUSIONS: the proposed decellularization protocol was correct, and was verified by an absence of cells. The hepatic matrix had preserved vascular and bile ducts with a suitable three-dimensional architecture permitting further cell seeding.

Preparación de una matriz extracelular tridimensional por descelularización de hígados de conejos / Preparación de una matriz extracelular tridimensional por descelularización de hígados de conejos / Preparation of a three-dimensional extracellular matrix by decellularization of rabbit livers

Introducción: el número de hígados trasplantables es insuficiente para satisfacer las necesidades actuales de la demanda de injerto. La búsqueda de alternativas terapéuticas ha generado diferentes líneas de investigación, una de ellas es la utilización de matrices biológicas tridimensionales descelularizadas y la posterior siembra celular para obtener un órgano funcional. Objetivo: obtención de un protocolo de descelularización de hígado de conejo que genere una matriz hepática tridimensional. Métodos: una combinación de detergentes (Triton X-100 y SDS), agentes físicos y enzimáticos se utilizaron para descelularizar hígados de conejo. Los órganos se pefundieron en forma retrógrada con distintos agentes químicos durante 68 horas. Luego los hígados se examinaron por técnicas morfológicas (microscopía óptica y electrónicade barrido) y bioquímicas (cuantificación de ADN) para evaluar una correcta descelularización así como la obtención de una matriz extracelular preservada. Resultados: la observación macroscópica del órgano permitió inferir la descelularización del mismo. Las tinciones macroscópicas utilizadas mostraron una correcta conservación de los árboles biliar y vascular. Por otro lado, la observación microscópica del hígado permitió corroborar los resultados macroscópicos observados, la tinción de hematoxilina-eosina mostró ausencia de células y de material nuclear así como la presencia de la tríada portal. La tinción de Wilde evidenció la conservación de las fibras de reticulina en la matriz descelularizada. Asimismo, la microscopía electrónica de barrido reveló una cápsula de Glisson conservada y la descelularización de la cuantificación de ADN fue inferior al 10 % en el hígado descelularizado con respecto al hígado control. Finalmente, el tiempo utilizado para la descelularización fue inferior a las 96 horas. Conclusiones: el protocolo de descelularización propuesto fue apropiado ya que se verificó una ausencia de células y una matriz hepática con conductos vasculobiliares conservados y con una arquitectura tridimensional adecuada para una futura siembra celular(AU)

Introduction: the availability of transplantable livers is not sufficient to fulfill the current demand for grafts, with the search for therapeutic alternatives having generated different lines of research, one of which is the use of decellularized three-dimensional biological matrices and subsequent cell seeding to obtain a functional organ. Objective: to produce a decellularization protocol from rabbit liver to generate a three-dimensional matrix. Methods: a combination of physical, chemical (Triton X-100 and SDS) and enzymatic agents to decellularize rabbit livers was used. After 68 h of retrograde perfusion, a decellularized translucent matrix was generated. To evaluate if the decellularization protocol was successful, with the extracellular matrix being preserved, we carried out histological (light microscopy and scanning electron microscopy) and biochemical (DNA quantification) studies. Results: the decellularization process was verified by macroscopic observation of the organ using macroscopic staining, which revealed a correct conservation of bile and vascular trees. A microscopic observation corroborated these macroscopic results, with the hematoxylin- eosin staining showing no cells or nuclear material and the presence of a portal triad. Wilde’s staining demonstrated the conservation of reticulin fibers in the decellularized matrix. In addition, scanning electron microscopy revealed a preserved Glisson’s capsule and a decellularized matrix, with the DNA quantification being less than 10 % in the decellularized liver compared to control. Finally, the time taken to develop the decellularization protocol was less than 96 hours. Conclusions: the proposed decellularization protocol was correct, and was verified by an absence of cells. The hepatic matrix had preserved vascular and bile ducts with a suitable three-dimensional architecture permitting further cell seeding(AU)

The overdamped van Hove function of atomic liquids.

Using the generalized Langevin equation formalism and the process of contraction of the description we derive a general memory function equation for the thermal fluctuations of the local density of a simple atomic liquid. From the analysis of the long-time limit of this equation, a striking equivalence is suggested between the long-time dynamics of the atomic liquid and the dynamics of the corresponding Brownian liquid. This dynamic equivalence is confirmed here by comparing molecular and Brownian dynamics simulations of the self-intermediate scattering function and the long-time self-diffusion coefficient for the hard-sphere liquid.

[Latex Allergy in Patients Who Underwent Surgical Treatment. Case series]. / Alergia a látex en pacientes operados. Serie de casos.

Latex allergy is an underdiagnosed condition, and it is the second cause of perioperative anaphylaxis. This condition represents a clinical challenge due to the severity of reactions caused by the exposure to latex in allergic individuals. It is necessary to know the risk factors in order to suspect the diagnosis of latex allergy, and to apply the preventive measures in the medical care of patients allergic to latex. Other safety measures and pre-medication, as avoiding objects and medical equipment made of latex, should be taken since they can cause anaphylaxis. We present six cases of patients with latex allergy, who underwent surgical procedures. In five of them with positive skin test with latex, we used preventive measures and pre-medication 72 hours before and 48 hours after surgery. Only one patient, a sixty year old woman, with urticaria related to kiwi, avocado and potatoes, who was surgically intervened without pre-medication, presented grade IV anaphylaxis.

[Autoimmune hematologic diseases in adult patients with common variable immunodeficiency]. / Enfermedades hematológicas autoinmunes en adultos con inmunodeficiencia com˙n variable.

BACKGROUND: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by defective immunoglobulin production. Twenty percent of patients may also have associated autoimmune diseases, especially hematologic, whose prevalence is estimated at 5 to 8%. OBJECTIVE: To report the occurrence of autoimmune haematological disorders in CVID adults. METHODS: We included 18 patients with CVID, who underwent hematologic evaluation, which included: CBC, serum folate or vitamin B complex and bone marrow aspirate. RESULTS: Of a total of 18 patients, 11 women (61%), mean age 38 ± 13.5, six patients (33.3%) had autoimmune diseases, five of them had blood diseases (4 with PTI and one with Evans syndrome). Three patients had more than one autoimmune disease and one presented three autoimmune diseases (Evans syndrome, hypoparathyroidism and celiac disease with a history of autoimmune hemolytic anemia). CONCLUSIONS: The prevalence of autoimmune diseases in this group of patients with CVID was higher than the 20% reported in the literature. Hematological diseases should be suspected in CVID patients, since half of our cases presented them.

Self-consistent theory of collective Brownian dynamics: theory versus simulation.

A recently developed theory of collective diffusion in colloidal suspensions is tested regarding the quantitative accuracy of its description of the dynamics of monodisperse model colloidal systems without hydrodynamic interactions. The idea is to exhibit the isolated effects of the direct interactions, which constitute the main microscopic relaxation mechanism, in the absence of other effects, such as hydrodynamic interactions. Here we compare the numerical solution of the fully self-consistent theory with the results of Brownian dynamics simulation of the van Hove function G(r,t) and/or the intermediate scattering function F(k,t) of four simple model systems. Two of them are representative of short-ranged soft-core repulsive interactions [(sigma/r)(mu), with mu>>1], in two and in three dimensions. The other two involve long-ranged repulsive forces in two (dipolar, r(-3) potential) and in three (screened Coulomb, or repulsive Yukawa interactions) dimensions. We find that the theory, without any sort of adjustable parameters or rescaling prescriptions, provides an excellent approximate description of the collective dynamics of these model systems, particularly in the short- and intermediate-time regimes. We also compare our results with those of the single exponential approximation and with the competing mode-mode coupling theory.