Search

Rib internal anatomy and its cross-sectional morphology inform about important biomechanical or even evolutionary aspects. Classic histological studies require destructive techniques that are reprehensible depending on the case (e.g., fossils). In the last years, non-destructive CT-based methods are contributing to complementing previous knowledge without damaging the bone. Even though these methods have been proved to be useful to understand adult variation, we do not know whether these methods are useful to cover ontogenetic variation. This work compares classical histological methods with medical- and micro-CT to quantify the amount of mineral area at the rib midshaft (% Min. Ar.), a proxy for bone density. We compared cross-sections from an ontogenetic sample of 14 human first ribs ranging from perinates to adults using a) classical histology, b) HD (9–17 microns) and SD micro-CT (90 microns), and c) standard medical-CT (0.66 mm). We found that all the CT-based methods provide a larger % Min. Ar. compared to the histological techniques, but the HD micro-CT resolution is the only capable of producing results comparable to classical histology (p > 0.01), with the SD micro-CT and the medical-CT producing statistically larger results compared to classical histology (p < 0.01). In addition, it is important to state that the resolution of a standard medical-CT is not high enough to differentiate between mineral and non-mineral areas of the cross-sections for perinates and infants. These results could have important implications to avoid (when necessary) destructive techniques that are not appropriate in the case of highly valuable specimens such as fossils.

Introduction: Tyrosine kinase inhibitors have been a breakthrough in the treatment of advanced medullary thyroid cancer (MTC), and they can prolong progression-free survival (PFS). Case Presentation: A patient with MTC and metastatic spread to the lymph nodes, lungs, bones, breast, and cerebellum started treatment with vandetanib. During treatment, she developed secondary adrenal insufficiency and hypogonadotropic hypogonadism. After 9 years of vandetanib therapy, the disease has not progressed and the patient maintains a complete response of the breast metastases and a partial response of the other metastatic lesions. Conclusion: To our knowledge, this is the first reported case of secondary adrenal insufficiency and hypogonadotropic hypogonadism related to therapy with vandetanib. Moreover, the prolonged PFS and the complete disappearance of some of the metastatic lesions in this patient are truly unusual.

Background and Aims Due to COVID-19 pandemic we must continue attending in our hospital consultations and, at the same time, avoid transfers and visit that involve risks to our patients. For this reason, we implemented new forms of care. Mainly electronic consultation (eConsult) using institutional email as way of contact between Primary Care Providers (PCPs) and nephrologist, trying to decrease in-person visit. Objective To analyze the results of the creation in June 2020 of a Nephrology electronic consultation in our hospital. Method Retrospective observational study of eConsults made to our department, which serves a population of 200.000 people, with total of 9 primary care centers. The study period was June 10, 2020 to December 31, 2020. We have studied the number of eConsults, response time, type of consult made, problem resolution and subsequent follow up. Results Of 141 workdays, in 61 days (43%) there was eConsult, total 80 eConsults, mean 1.3 and median 1 daily (range 1 to 3). All primary care centers used this communication way, as well as 50 PCPs. Median response time for eConsult was 1 day (range 1-4). 55% patient was male, mean age 70.8 years (SD 13.9) (range 16-95 years). 64% it was his first assessment for Nephrology. The most frequent causes of eConsult: increase of creatinine (41.3%), uncontrolled blood pressure (12.5%), sodium and potassium disturbances (11.3%), treatment adjustment (11.3%) and changes in previous appointment (8.8%). After assessment eConsult, to 55% of patients was treatment adjustment, 22.5% were converted to an in-person visit, 11% they didn´t need any action but only 1 patient was referred to the emergency room. 44% patient is subsequent follow up by PCPs, 39% by nephrologist, 15% joint assessment PCP and nephrologist and 2% by other specialists. Only 2 patients (2.5%) made second eConsult. Conclusion The eConsult is an important help to PCPs to solve doubts quickly, avoid unnecessary the travels to hospital the patients, treatment adjustment above all in control of hypertension an ionic disturbance, and guide in handling of chronic kidney disease. The diffusion and empowerment of this type of consultation in a next future may decrease, partly, the usual saturation of face-to-face visit and optimize the patient population being seen by nephrologist.

Aim: To assess the relationship between air pollution and the mortality of IPF. Methods: The probability of death was analyzed from 32 IPF patients diagnosed in our ILD Unit from 2011 to 2019 and it was related to the values of (CO, NO2, PM2.5, PM10, O3 and SO2) in Madrid during that period. Results: Thirty-two patients were reviewed with a mean age of 66±10 years at the time of diagnosis, 7 (21.9%) of them were women. The average of the pollutants from the three months prior to the date of death of the 11 patients was (34.4%) patients who died was SO2=9.518±4.397μg/m2, NO2=58.910±8.819μg/m2, CO=0.390±0.104mg/m2, O3=38.778±15.309 μg/m2, PM2,5=12.371±2.138 μg/m2 PM10= 23.583±5.196 μg/m2 and the average air in the previous three months to the last clinic visit in 21 (65,6%) patients who are still alive was SO2 =8.736±1.719 μg/m2, NO2=56.216±9.586μg/m2, CO=0.237±0.106 mg/m2 O3=48.294±14,059 μg/m2 PM2,5=11.029±2,280 μg/m2 PM10=22.376±4,533 μg/m2 . Firstly, we observed that those 28 (87.5%) patients who were treated had no association with the probability of death [OR=7,500 (0.676-83.264)] (p=0.101) and after that we saw that pollution levels in all patients were not associated with the likelihood of dying: OR SO2= 1.154 (0.521-2.557) (p=0.723), OR CO=1.247 (0.970-1.602) (p=0.085), OR NO2 = 0.829 (0.468-1.468) (p=0.520), OR O3 = 0.944 (0.578-1.544) (p=0.820), OR PM2,5 = 0.646 (0.152-2.744) (p=0.554), OR PM10 = 1.302 (0.668-2.537) (p=0.439). Conclusion: There is no evidence to affirm that air pollution in Madrid is associated with the probability of death in IPF patients.

The tall and narrow body shape of anatomically modern humans (Homo sapiens) evolved via changes in the thorax, pelvis and limbs. It is debated, however, whether these modifications first evolved together in African Homo erectus, or whether H. erectus had a more primitive body shape that was distinct from both the more ape-like Australopithecus species and H. sapiens. Here we present the first quantitative three-dimensional reconstruction of the thorax of the juvenile H. erectus skeleton, KNM-WT 15000, from Nariokotome, Kenya, along with its estimated adult rib cage, for comparison with H. sapiens and the Kebara 2 Neanderthal. Our three-dimensional reconstruction demonstrates a short, mediolaterally wide and anteroposteriorly deep thorax in KNM-WT 15000 that differs considerably from the much shallower thorax of H. sapiens, pointing to a recent evolutionary origin of fully modern human body shape. The large respiratory capacity of KNM-WT 15000 is compatible with the relatively stocky, more primitive, body shape of H. erectus., SCOPUS: ar.j, info:eu-repo/semantics/published

Graphical abstract, Objetivo Evaluar la capacidad diagnóstica de la angiografía pulmonar con tomografía computarizada multidetector (TCMD) y mapa de yodo en el diagnóstico del tromboembolismo pulmonar (TEP), en pacientes con enfermedad por coronavirus COVID-19. Métodos Estudio observacional retrospectivo sobre 81 pacientes consecutivos ingresados con infección respiratoria por COVID-19, a los que se realizó TCMD ante la sospecha clínica de TEP [disnea súbita, dolor torácico, hemoptisis, insuficiencia respiratoria grave (IRG) que no corrige con flujo elevado de O2] y/o elevación del D-dímero. Resultados De los 81 pacientes estudiados, 64 (79,01%) varones, identificamos TEP agudo en 22 (27,16%), en 13 (59,09%) bilateral y en 13 (59,09%) con áreas de hipoperfusión. De los 59 (72,83%) pacientes sin TEP en 41 (69,49%) se observó hipoperfusión (en un caso atribuible a enfisema pulmonar). En 18 (22,2%) del total de pacientes no se vio ni TEP ni hipoperfusión. El patrón «crazy paving» es un factor de riesgo para desarrollar TEP (OR1,94; IC95% 0,28-13,57), así como los patrones «consolidaciones» (OR 1,44; IC95% 0,24-8,48) y «engrosamiento septos/bronquiectasias» (OR 1,47; IC95% 0,12-17,81). Los pacientes con IRG refractaria al O2 mostraron un riesgo 6,36 veces superior para el hallazgo de hipoperfusión en el mapa de yodo. Conclusión La angiografía pulmonar con TCMD y mapa de yodo, al añadir la imagen funcional a la anatómica, es capaz de demostrar, además del TEP en arterias principales, lobares y segmentarias, la presencia de hipoperfusión en vasos distales, lo cual la convierte en una herramienta de gran utilidad para la adecuada orientación diagnóstica y terapéutica de los pacientes con afectación pulmonar por COVID-19.

Sexual dimorphism in the human respiratory system has been previously reported at the skeletal (cranial and thoracic) level, but also at the pulmonary level. Regarding lungs, foregoing studies have yielded sex‐related differences in pulmonary size as well as lung shape details, but different methodological approaches have led to discrepant results on differences in respiratory patterns between males and females. The purpose of this study is to analyse sexual dimorphism in human lungs during forced respiration using 3D geometric morphometrics. Eighty computed tomographies (19 males and 21 females) were taken in maximal forced inspiration (FI) and expiration (FE), and 415 (semi)landmarks were digitized on 80 virtual lung models for the 3D quantification of pulmonary size, shape and kinematic differences. We found that males showed larger lungs than females (P, This research is funded by CGL‐2012‐37279, CGL‐2015‐63648‐P (Ministry of Economy, Industry and Competitiveness, Spain) and PI10/02089 (Fondo de Investigación Sanitaria) Ministry of Health, Social Services and Equality, Spain.

Graphical abstract, Highlights • Chest deformities in Osteogenesis Imperfecta patients affect pulmonary function. • We present the rib cage deformities related to pulmonary function. • There are significant relations between ribs shape and spirometric parameters. • There is no relationship between thoracic spine shape and spirometric parameters. • Correction of rib cage deformities will serve for better patients’ management., The aim of the present study was to test the hypothesis that ribs shape changes in patients with OI are more relevant for respiratory function than thoracic spine shape. We used 3D geometric morphometrics to quantify rib cage morphology in OI patients and controls, and to investigate its relationship with forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1), expressed as absolute value and as percentage of predicted value (% pred). Regression analyses on the full sample showed a significant relation between rib shape and FEV1, FVC and FVC % pred whereas thoracic spine shape was not related to any parameter. Subsequent regression analyses on OI patients confirmed significant relations between dynamic lung volumes and rib shape changes. Lower FVC and FEV1 values are identified in OI patients that present more horizontally aligned ribs, a greater antero-posterior depth due to extreme transverse curve at rib angles and a strong spine invagination, greater asymmetry, and a vertically short, thoraco-lumbar spine, which is relatively straight in at levels 1–8 and shows a marked kyphosis in the thoraco-lumbar transition. Our research seems to support that ribs shape is more relevant for ventilator mechanics in OI patients than the spine shape.

Objective We aimed to assess the relationship between major air pollutants and the natural history and mortality of idiopathic pulmonary fibrosis (IPF). Methods We conducted a retrospective cohort study from 2013 to 2019 among 52 patients with IPF from the pneumology department of a tertiary hospital. According to their geocoded residential address, each patient was assigned a mean concentration of carbon monoxide (CO), nitrogen dioxide, particulate matter 2.5 and 10, ozone, and sulfur dioxide, as measured at a single surveillance station in central Madrid, Spain. We analyzed forced vital capacity (FVC), CO diffusing capacity, 6-minute walking test, degree of dyspnea, radiologic pattern, and signs of pulmonary hypertension in all patients. Results Patients’ mean age was 66 ± 10 years, and 79% were men. The mean predicted FVC was 78.9 ± 0.5%. Forty-two patients met the criteria for severe disease, and 18 patients died. Mortality was significantly associated with increased CO exposure (for each 0.1 mg/m2 increase: odds ratio 2.45, 95% confidence interval 1.39–4.56). We observed no association between any of the other investigated contaminants and IPF mortality or severity. Conclusions Air pollution, specifically that caused by carbon monoxide, can increase mortality in patients with IPF.

The human ribcage expands and contracts during respiration as a result of the interaction between the morphology of the ribs, the costo-vertebral articulations and respiratory muscles. Variations in these factors are said to produce differences in the kinematics of the upper thorax and the lower thorax, but the extent and nature of any such differences and their functional implications have not yet been quantified. Applying geometric morphometrics we measured 402 three-dimensional (3D) landmarks and semilandmarks of 3D models built from computed tomographic scans of thoraces of 20 healthy adult subjects in maximal forced inspiration (FI) and expiration (FE). We addressed the hypothesis that upper and lower parts of the ribcage differ in kinematics and compared different models of functional compartmentalization. During inspiration the thorax superior to the level of the sixth ribs undergoes antero-posterior expansion that differs significantly from the medio-lateral expansion characteristic of the thorax below this level. This supports previous suggestions for dividing the thorax into a pulmonary and diaphragmatic part. While both compartments differed significantly in mean size and shape during FE and FI the size changes in the lower compartment were significantly larger. Additionally, for the same degree of kinematic shape change, the pulmonary thorax changes less in size than the diaphragmatic thorax. Therefore, variations in the form and function of the diaphragmatic thorax will have a strong impact on respiratory function. This has important implications for interpreting differences in thorax shape in terms of respiratory functional differences within and among recent humans and fossil hominins. Anat Rec, 300:255-264, 2017. © 2016 Wiley Periodicals, Inc.

Objectives The human respiratory apparatus is characterized by sexual dimorphism, the cranial airways of males being larger (both absolutely and relatively) than those of females. These differences have been linked to sex-specific differences in body composition, bioenergetics, and respiratory function. However, whether morpho-functional variation in the thorax is also related to these features is less clear. We apply 3D geometric morphometrics to study these issues and their implications for respiratory function. Material and methods Four hundred two landmarks and semilandmarks were measured in CT-reconstructions of rib cages from adult healthy subjects (Nmale = 18; Nfemale = 24) in maximal inspiration (MI) and maximal expiration (ME). After Procrustes registration, size and shape data were analyzed by mean comparisons and regression analysis. Respiratory function was quantified through functional size, which is defined as the difference of rib cage size between MI and ME. Results Males showed significantly larger thorax size (p

[Objectives]: Sexual dimorphism is an important biological factor underlying morphological variation in the human skeleton. Previous research found sex-related differences in the static ribcage, with males having more horizontally oriented ribs and a wider lower ribcage than females. Furthermore, a recent study found sex-related differences in the kinematics of the human lungs, with cranio-caudal movements of the caudal part of the lungs accounting for most of the differences between sexes. However, these movements cannot be quantified in the skeletal ribcage, so we do not know if the differences observed in the lungs are also reflected in sex differences in the motion of the skeletal thorax., [Materials and methods]: To address this issue, we quantified the morphological variation of 42 contemporary human ribcages (sex-balanced) in both maximal inspiration and expiration using 526 landmarks and semilandmarks. Thoracic centroid size differences between sexes were assessed using a t test, and shape differences were assessed using Procrustes shape coordinates, through mean comparisons and dummy regressions of shape on kinematic status. A principal components analysis was used to explore the full range of morphological variation., [Results]: Our results show significant size differences between males and females both in inspiration and expiration (p, This work was partially funded by the IdEx University of Bordeaux Investments for the Future program (ANR-10-IDEX-03-02), the projects CGL2012-37279 and CGL2015-63648P (Ministry of Economy, Industry and Competitiveness, Spain) and PI10/02089 (Fondo de Investigación Sanitaria, Ministry of Health, Social Services and Equality Spain).

© The Author(s) 2018., Our most recent fossil relatives, the Neanderthals, had a large brain and a very heavy body compared to modern humans. This type of body requires high levels of energetic intake. While food (meat and fat consumption) is a source of energy, oxygen via respiration is also necessary for metabolism. We would therefore expect Neanderthals to have large respiratory capacities. Here we estimate the pulmonary capacities of Neanderthals, based on costal measurements and physiological data from a modern human comparative sample. The Kebara 2 male had a lung volume of about 9.04 l; Tabun C1, a female individual, a lung volume of 5.85 l; and a Neanderthal from the El Sidrón site, a lung volume of 9.03 l. These volumes are approximately 20% greater than the corresponding volumes of modern humans of the same body size and sex. These results show that the Neanderthal body was highly sensitive to energy supply., This research is funded by CGL-2012–37279, CGL-2015–63648-P, CGL2016–75109-P (Ministry of Economy, Industry and Competitiveness, Spain), PI10/02089 (Fondo de Investigación Sanitaria, Ministry of Health, Social Services and Equality Spain) and the Leakey Foundation.

Resumen Objetivos Evaluar la validez diagnostica de la puncionaspiracion con aguja fina (PAAF) percutanea en lesiones mediastinicas considerando la biopsia o el seguimiento clinico como patron de referencia. Pacientes y metodos Se realizo PAAF percutanea guiada por TC a 131 pacientes con lesiones mediastinicas. Se uso un TC helicoidal con cortes de 3–10 mm y baja dosis de radiacion (40 mAs, 120 kV). Las muestras fueron examinadas in situ por un citologo para determinar su validez. Se obtuvo comprobacion histologica mediante biopsia o estudio de pieza quirurgica en 73 pacientes y seguimiento clinico en 50, comparandose los resultados globales y en subgrupos. Resultados En 126 pacientes (96,2%) el material fue valido para diagnostico. Ciento tres lesiones (78,6%) fueron consideradas malignas (62 tumores primarios y 41 metastasis) y 23 (17,6%) benignas. En los 123 pacientes de los que se dispuso de seguimiento clinico o patologico, la PAAF permitio identificar malignidad con una sensibilidad del 95,2% (IC95%: 89,2–97,9%), especificidad 84,2% (IC95%: 62,4–94,5%), valor predictivo positivo 97,1% (IC95%: 91,7–99,0%), valor predictivo negativo 76,2% (IC95%: 54,9–89,4%), razon de verosimilitud positiva 6,03 (IC95%: 2,13–17,05) y exactitud 93,5% (IC95%: 87,7–96,7%). La complicacion mas frecuente fue el neumotorax (3 casos). La correlacion citohistologica fue elevada tanto en las lesiones malignas (kappa 0,641) como en las benignas (kappa 0,607). Conclusiones La PAAF percutanea guiada por TC es una tecnica segura y eficaz para el diagnostico de masas mediastinicas con alta rentabilidad para la deteccion de malignidad.

Background: Our aim was to analyze the longitudinal changes in cardiac biomarker levels in hemodialysis patients with high comorbidity treated in our special hospital unit. We hypothesize that strict volume control strategy (salt-restricted diet, extended dialysis sessions and dry weight clinical assessment and reassessment in every session) could prevent progression of left-ventricular damage and, therefore, progressive increment in cardiac biomarker levels over time. Methods: This is a longitudinal cohort study including 46 dialysis patients in which a strategy of strict volume control has been adopted. N-terminal pro-B-type natriuretic peptide (NT-proBNP), troponin T and C-reactive protein (CRP) levels were measured at baseline and prospectively at 4, 8 and 12 months. The possible association between volume control and cardiac biomarker levels was analyzed. Results: Dry weight could be reduced (p < 0.01) over time. A reduction in systolic BP (p < 0.05) and in CRP levels (p < 0.05) was observed, whereas NT-proBNP and troponin T values remained stable. However, patients in the high quartile of NT-proBNP at baseline showed a reduction (p = 0.02) in troponin T over time with no significant trend (p = 0.08) to progressive reduction in NT-proBNP values. Conclusions: Strict volume control in dialysis patients may prevent progressive increment in cardiac biomarker levels over time. The impact seems to be higher among patients with higher levels at baseline in whom strict volume control can even reduce cardiac biomarker levels on follow-up.

The human ribcage expands and contracts during respiration as a result of the interaction between the morphology of the ribs, the costo-vertebral articulations and respiratory muscles. Variations in these factors are said to produce differences in the kinematics of the upper thorax and the lower thorax, but the extent and nature of any such differences and their functional implications have not yet been quantified. Applying geometric morphometrics we measured 402 three-dimensional (3D) landmarks and semilandmarks of 3D models built from computed tomographic scans of thoraces of 20 healthy adult subjects in maximal forced inspiration (FI) and expiration (FE). We addressed the hypothesis that upper and lower parts of the ribcage differ in kinematics and compared different models of functional compartmentalization. During inspiration the thorax superior to the level of the sixth ribs undergoes antero-posterior expansion that differs significantly from the medio-lateral expansion characteristic of the thorax below this level. This supports previous suggestions for dividing the thorax into a pulmonary and diaphragmatic part. While both compartments differed significantly in mean size and shape during FE and FI the size changes in the lower compartment were significantly larger. Additionally, for the same degree of kinematic shape change, the pulmonary thorax changes less in size than the diaphragmatic thorax. Therefore, variations in the form and function of the diaphragmatic thorax will have a strong impact on respiratory function. This has important implications for interpreting differences in thorax shape in terms of respiratory functional differences within and among recent humans and fossil hominins. Anat Rec, 300:255-264, 2017. © 2016 Wiley Periodicals, Inc.

La neumonia organizada es una enfermedad pulmonar poco frecuente, con gran variabilidad en los hallazgos radiologicos, de la que hasta el momento existe un escaso numero de casos en la mayoria de los estudios publicados en la bibliografia. Hemos estudiado mediante tomografia computarizada de alta resolucion 34 casos con diagnostico histologico establecido de neumonia organizada valorando los diferentes patrones de presentacion. De estos casos, 25 fueron idiopaticos y 9 secundarios. Los hallazgos observados fueron: consolidaciones parenquimatosas (76%), vidrio deslustrado (59%), dilataciones bronquiales (53%), nodulos centrolobulillares (35%), engrosamientos septales (23%), signo del halo (15%) y signo del halo invertido (12%). Se observo una mayor presencia de engrosamientos septales y un menor numero de remisiones completas en los casos secundarios.

Organizing pneumonia is an uncommon lung disease with a wide variety of radiologic findings, few of which have been discussed in the literature. We performed high resolution computed tomography on 34 patients with a histological diagnosis of organizing pneumonia and studied the images they presented. Twenty-five of the cases were idiopathic and 9 secondary. The findings observed were parenchymal consolidation (76%), ground glass opacity (59%), bronchial dilatation (53%), centrilobular nodules (35%), septal thickening (23%), halo sign (15%), and reversed halo sign (12%). Secondary cases presented more findings of septal thickening and fewer complete remissions.

A short and efficient route to enantiomerically pure hexahydroxy- and pentahydroxy-perhydroazaazulenes, ring-homologues of castanospermine, starting from the sole isoxazolidine derivative obtained in the 1,3-dipolar cycloaddition of a d -galactose-derived nitrone and methyl acrylate, is established. The procedure allows both backbone and stereochemical modulation of the products by choice of the starting monosaccharide. Structural assignment was based on crystallographic analysis of the starting isoxazolidine and NMR techniques. The products were tested for inhibitory activity against several glycosidases.

The development of a glycoconjugate vaccine against N. meningitidis type A bacterium is greatly hampered by the chemical lability of the phosphodiester bridges joining the N-acetyl mannosamine repeating units of its capsular polysaccharide. We describe the first synthesis of the phosphonodisaccharide α-D-Man-pNAc-[1 →CH 2 -P(O)(O - )→6]-β-D-ManpNAc-(I →O) (CH 2 ) 3 NH 2 as a stable analogue of the corresponding phosphate-bridged disaccharide. The key phosphonoester linkage is obtained by condensation of monosaccharide building blocks under Mitsunobu conditions. Moreover, the protected precursor of the target compound is suitably designed to allow further elongation and synthesis of higher oligomers.

Tras la presentacion de un caso de enfermedad de Niemann-Pick (N-P) tipo B se realiza una revision de la literatura en la que se describen los hallazgos radiologicos, con especial hincapie en la afectacion pulmonar. Se trata de un paciente de 40 anos diagnosticado a los 6 anos de posible enfermedad de N-P sospechada por presentar hepatoesplenomegalia y patron reticular en radiografia de torax, con pruebas de funcion respiratoria inicialmente normales. Dicho diagnostico se confirmo mediante medicion de la actividad de la enzima esfingomielinasa acida (ASM) en cultivo de fibroblastos de piel y demostracion de la presencia de mutaciones en el gen de la ASM. Ante una enfermedad de deposito habria que tener siempre en cuenta la afectacion pulmonar, y la tecnica radiologica mas fiable es la tomografia computarizada de alta resolucion. Aunque el patron encrazy-paving no es el mas especifico de esta patologia, la enfermedad de N-P deberia estar en el diagnostico diferencial.

We have carried out a review of the literature after the presentation of a case of Niemann-Pick (N-P) disease type B reporting the radiological findings with special emphasis on lung affectation. The patient is 40 years old and was diagnosed at the age of 6 with possible N-P disease suspected due to the presentation of hepatosplenomegaly and reticular pattern on chest radiography, with initially normal lung function tests. Said diagnosis was confirmed by measuring the activity of acid sphingomyelinase (ASM) enzyme in skin fibroblast cultures and by demonstrating the presence of mutations in the ASM gene. In cases of storage disease, lung affectation should always be considered, and the most reliable radiological technique is high-resolution computed tomography. Although the “crazy-paving” pattern is not the most specific for this pathology, N-P disease should be included in the differential diagnosis.

Describimos el caso de una paciente de 18 anos con sindrome de Cowden, en quien una radiografia simple objetivo un nodulo pulmonar solitario que resulto ser un hemangioma esclerosante. El hemangioma esclerosante de pulmon es una neoplasia pulmonar poco frecuente, que suele presentarse como un nodulo solitario periferico en mujeres asinto-maticas. En la actualidad, aunque la histologia del hemangioma esclerosante esta bien definida, son objeto de discusion la histiogenesis de esta entidad y su tratamiento. Uno de los principales problemas diagnosticos es diferenciar histologicamente el hemangioma esclerosante del adenocar-cinoma pulmonar con patron papilar.

This research was funded by the Spanish Ministry of Economy and Competitiveness (CGL2012-37279, MINECO), the Leakey Foundation, and PI10/02089 ( Fondo de Investigación Sanitaria) Ministry of Health, Spain.

Figura 1. A) Radiografia PA de torax. B y C) TC de torax con contraste intravenoso, corte axial a nivel de la ventana aortopulmonar en ventana de mediastino y de pulmon, respectivamente. D) TCMD reconstruccion coronal en maxima intensidad de proyeccion (MIP) ventana de mediastino. E y F) RM de torax, cortes axiales a nivel de la ventana aortopulmonar. Cortes axiales potenciados en T2 y T1 con supresion grasa.

We have carried out a review of the literature after the presentation of a case of Niemann-Pick (N-P) disease type B reporting the radiological findings with special emphasis on lung affectation. The patient is 40 years old and was diagnosed at the age of 6 with possible N-P disease suspected due to the presentation of hepatosplenomegaly and reticular pattern on chest radiography, with initially normal lung function tests. Said diagnosis was confirmed by measuring the activity of acid sphingomyelinase (ASM) enzyme in skin fibroblast cultures and by demonstrating the presence of mutations in the ASM gene. In cases of storage disease, lung affectation should always be considered, and the most reliable radiological technique is high-resolution computed tomography. Although the "crazy-paving" pattern is not the most specific for this pathology, N-P disease should be included in the differential diagnosis.

Vitamin D deficiency has been linked to cardiovascular disease and mortality in hemodialysis (HD) patients. The purpose of the present cross-sectional study was to analyze the Vitamin D status of dialysis patients from a single center, study determinants of Vitamin D deficiency, and assess its implications on outcome.A prospective observational study of 115 prevalent dialysis patients was carried out, in which clinical and dialysis-related characteristics including routine biochemistry were studied in relation to levels of 25-hydroxyvitamin-D (25[OH]D, chemiluminescence). Survival was assessed after a median follow-up period of 413 days.25(OH)D deficiency and insufficiency was present in 51% and 42% of the patients, respectively. Only 7% of the patients showed normal 25(OH)D levels. Peritoneal dialysis patients presented the lowest 25(OH)D levels. Also, a significant difference was found between on-line hemodiafiltration (OL-HDF) and conventional HD (11 [6 to 16] versus 19 [13 to 27] ng/mL; P0.001; 25th to 75th percentiles, conventional HD versus OL-HDF respectively). In multinomial logistic regression analysis, patients on conventional HD had 8.35 greater odds (95% CI [2.04 to 34.20]) of 25(OH)D deficiency than OL-HDF even after adjustment for sex, parathyroid hormone, pH, and Charlson comorbidity index. During the follow-up period, 18 patients died. Both crude and adjusted (hazard ratio, 6.96; 95% CI [1.44 to 33.64]) Cox analysis identified 25(OH)D deficiency as a mortality risk factor.This observational study underlines the high prevalence of hypovitaminosis D in dialysis patients and its strong implications on outcome. Furthermore, our results suggest that OL-HDF was associated with a better preservation of the vitamin D status as compared with conventional HD.

Objective To evaluate the diagnostic accuracy of the percutaneous fine needle aspiration cytology (FNAC) for mediastinal lesions by using histology or follow-up clinical diagnosis as gold standard. Patients and Methods CT-guided percutaneous FNAC was performed on 131 patients with mediastinal lesions. Helical CT was used with 3–10mm image thickness range and low radiation dose (40mAs, 120kV). Samples were immediately examined by a cytologist to determine if they were representative. Histological samples were obtained by means of biopsy or resection specimens in 73 patients and clinical follow-up in 50. Results The material was satisfactory for diagnosis in 126 patients (95.2 %), in whom 103 lesions (78.6%) were considered malignant (62 primary tumours and 41 metastases) and 23 (17.6%) benign. In the 123 patients with clinical monitoring or pathological diagnosis, using FNAC led to the identification of malignancy with a sensitivity of 95.2 % (95%CI: 89.2–97.9%), specificity 84.2% (95%CI: 62.4–94.5%), positive predictive value 97.1% (95%-CI: 91.7–99.0%), negative predictive value 76.2% (95%CI: 54.9–89.4%), likelihoodratio positive 6.03 (95%CI: 2.13–17.05) and accuracy 93.5% (95%CI: 87.7–96.7%). Pneumothorax was the most frequent complication (3 cases). There was good agreement between the cytological findings and the histological findings, not only for malignant lesions (kappa coefficient: 0.641) but also for benign (kappa 0.607). Conclusions CT-guided percutaneous FNAC is a safe and effective technique for the diagnosis of the mediastinal masses, with a high diagnostic yield for malignancy depicting.

19 páginas, 4 figuras, 2 tablas, 4 esquemas., The dipolar cycloaddition of (Z)-N-benzyl-(3-O-benzyl-1,2-O-isopropylidene-α-d-ribofuranos-5-ylidene)amine N-oxide to methyl acrylate gives a 53:16:26:5 diastereomeric mixture of isoxazolidine derivatives. The dipolar cycloaddition of the xylo analogue to methyl acrylate is more diastereoselective, producing a 44:13:43 mixture of only three diastereomers. The ribo-configured adducts have been converted (4 steps only) into the new (2R,6S,7S,8R,8aR)-, (2S,6S,7S,8R,8aR)-, (2S,6S,7S,8R,8aS)- and (2R,6S,7S,8R,8aS)-2,6,7,8-tetrahydroxyindolizidines. Similarly, the two xylo-configured major isoxazolidine derivatives were converted into the known derivatives (2R,6S,7R,8R,8aS)- and (2S,6S,7R,8R,8aR)-2,6,7,8-tetrahydroxyindolizidines. The six isomeric indolizidine derivatives obtained have been evaluated for their inhibiting activities towards 15 glycosidases. Only the (2R,6S,7S,8R,8aR)-configured isomer is a selective inhibitor of amyloglucosidases from Aspergillus niger (IC50 = 350 μM) and from Rhizopus mold (IC50 = 90 μM, Ki = 195 μM, non-competitive), the other indolizidines show very little inhibitory activity at 1 mM concentration., We thank the ‘Dirección General de Investigación, Ministerio de Ciencia y Tecnología’ of Spain (Grant No. BQU2000-1155), the ‘Dirección General de Universidades e Investigación, Consejería de Educación y Ciencia’ of Andalusia (FQM 142) and the Swiss Federal Science Foundation for financial support. We also thank Miss Catherine Schuetz for her technical assistance.

We describe the case of an 18-year-old female with Cowden syndrome in whom a simple x-ray detected a solitary pulmonary nodule that was identified as a sclerosing hemangioma. Pulmonary sclerosing hemangioma is an unusual lung neoplasm which typically presents as a solitary peripheral nodule in asymptomatic women. Although the histology of this entity is well defined, its origin and treatment is debated. One of the main diagnostic problems is to histologically differentiate a pulmonary sclerosing hemangioma from a papillary lung carcinoma.

considered. Of APS patients with thrombosis 79% had the 4 G allele of PAI-1 gene and 21% had no this mutation. Both arterial and more frequently venous thrombosis were associated with the 4 G/5 G polymorphism and high PAI-1 levels in APS patients. At the same time, patients with SLE+APS had higher PAI-1 levels than patients with PAPS. Comment: The thrombosis in patients with APS may be partially connected with decrease in Pg or increase in PAI-1 levels induced by aPL. The 4 G/5 G polymorphism PAI-1 gene is an predictor risk factor for the development of thrombosis in APS.

Background: Diabetes and other cardiovascular risk factors (CVRF) have been studied because their potential effect in venous thromboembolic disease (VTE). There are controversial results but recent metanalisis haven t found relation between traditional CVRF and risk of suffering deep vein thrombosis (DVT) or pulmonary embolism (EP). However, there is short information about the characteristics of DM patients suffering VTE. Our purpose was to study the basal characteristics, comorbidities, way of presentation and diagnostic procedures in patients suffering diabetes. Methods: Collected basal characteristics, comorbidities and predisposal factors of diabetic patients (DM) seen in our venous thromboembolism clinic since 2001. Results: Out of 1523 patients seen in our clinic, we detected 118 diabetic patients (7.09%), although we excluded data from 489 (32.18%). 30/118 patients (5.8%) suffered DVT being DM. 118/1002 patients (8.8%) suffered EP with or without DVT being DM. Basal characteristics: Age 73.2 DM, 63.19 non diabetics (no-DM). Weight: 75.25 kg DM, 74.02 no-DM. Comorbidities: 89.4% DM, 65.7% no-DM. Ischemic heart disease: 12.7% DM, 9.9% no-DM; Stroke: 6.77% DM, 4.8% no-DM; Peripheral Artery disease: 4.23% DM, 1.39% no-DM; Arterial Hypertension: 74.5% DM, 38.1% no-DM. Active tabaquism: 10.1% DM, 12.2% no-DM. Concomitant treatments: 92.1% DM, 62.4% no-DM. Use of antiagregants: 19.25%DM, 11.3% no-DM; Statins: 26.2% DM, 13.6% no-DM. Predispose factors of VTE: Cancer 22% DM, 17.1% no-DM; Surgical procedure 12.7% DM, 11.79% no-DM; previous VTE 19.4% DM, 16.7% no-DM; long distance travel 2.5% DM, 2.7% no-DM; Non-surgical immobilization 29.6% DM, 23.4% noDM; varicose disease 37.2% DM, 26.4% no-DM. Symptoms of EP were quite similar in both groups except: Haemoptysis 1.1% DM, 3.8% no-DM; Thoracic pain 35.2% DM and 44.3% no-DM; fever appeared in 3.4% DM and 7% no-DM. Diagnoses of VTE was made with Ecography for DVT patients in 96.7% DM, 99% no-DM; EP patients were diagnosed with CT-angiography in 96.5% DM, 89.5% no-DM. Of EP patients an ecography was performed in 56.8% DM and 63.7% no-DM to determine concomitant DVT. Basal blood test samples were similar except for renal insufficiency that accounts for 19.3% DM and 16.1 % no-DM. Comment: In our cohort, DM patients are older, have several comorbidities, mainly, ischemic heart disease, stroke, peripheral artery disease, renal insufficiency and arterial hypertension. Also there are more cancer patients and immobilization in DM patients. Diagnoses were made with similar methods. EP symptoms differ in DM patients. More studies are needed to evaluate influence of diabetes in VTE presentation and evolution.