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BACKGROUND: Pulmonary complications are common in primary immunodeficiency diseases (PID) and contribute to morbidity and mortality in these patients. However, their varied presentation and a general lack of awareness of PID in this setting make early diagnosis and treatment difficult. The aim of this study was to define the warning signs of PID in patients with respiratory manifestations, the necessary diagnostic tests, and the therapeutic management of both children and adults. METHODS: A review of the literature was performed, and 43 PID interdisciplinary specialists were consulted. RESULTS: This document identifies the pulmonary and extrapulmonary manifestations that should prompt a suspicion of PID, the immunological and respiratory tests that should be included in the diagnostic process according to the level of care, recommendations regarding the use of immunoglobulin replacement therapy according to the specific immunodeficiency, and the minimum recommended immunological and pulmonary monitoring in these patients. CONCLUSIONS: This document is the first to combine scientific evidence with the opinion of a broad panel of experts specializing in the treatment of patients with immunodeficiencies. It aims to provide a useful tool for all practitioners who are regularly involved in the management of these patients.

The first Spanish multi-centre study on the microbiology of cystic fibrosis (CF) was conducted from 2013 to 2014. The study involved 24 CF units from 17 hospitals, and recruited 341 patients. The aim of this study was to characterise Pseudomonas aeruginosa isolates, 79 of which were recovered from 75 (22%) patients. The study determined the population structure, antibiotic susceptibility profile and genetic background of the strains. Fifty-five percent of the isolates were multi-drug-resistant, and 16% were extensively-drug-resistant. Defective mutS and mutL genes were observed in mutator isolates (15.2%). Considerable genetic diversity was observed by pulsed-field gel electrophoresis (70 patterns) and multi-locus sequence typing (72 sequence types). International epidemic clones were not detected. Fifty-one new and 14 previously described array tube (AT) genotypes were detected by AT technology. This study found a genetically unrelated and highly diverse CF P. aeruginosa population in Spain, not represented by the epidemic clones widely distributed across Europe, with multiple combinations of virulence factors and high antimicrobial resistance rates (except for colistin).

Martinez-Garcia MA,1,2 Athanazio RA,3 Girón R,4 Máiz-Carro L,5 de la Rosa D,6 Olveira C,7 de Gracia J,2,8 Vendrell M,9 Prados-Sánchez C,10 Gramblicka G,11 Corso Pereira M,12 Lundgren FL,13 Fernandes De Figueiredo M,14 Arancibia F,15 Rached SZ3 1Pulmonary Service, Polytechnic and University La Fe Hospital, Valencia, Spain; 2CIBERes, CIBER de Enfermedades Respiratorias. Madrid. Spain; 3Pulmonary Division, Heart Institute (Incor), Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo; 4Pneumology Service, Hospital La Princesa, 5Pneumology Service, Hospital Ramón y Cajal, Madrid, 6Pneumology Unit, Hospital Plató, Barcelona, 7Pneumology, Málaga Regional University Hospital, Instituto de Biomedicina de Málaga (IBIMA), Málaga University, Spain; 8Pneumology Service, Hospital Vall d’Hebron, Barcelona, 9Bronchiectasis Group IDIBGI, Dr. Trueta University Hospital. UdG. Ciberes CB06/06/0030, 10Unidad de Fibrosis Quística y Bronquiectasias. Hospital Universitario La Paz. Madrid. Spain; 11Pneumology Service, Hospital del Tórax Dr A Cetrángolo, Buenos Aires, Argentina; 12Pneumology Service, Universidade Estadual de Campinas UNICAMP, Sao Paulo, 13Pneumology Service, Hospital Octávio de Freitas, Recife, 14Pneumology Service, Hospital de Messejana, Fortaleza, Brazil; 15Pneumology Service, Instituto Nacional del Tórax, Santiago de Chile, Chile Background: Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients.Objective: Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality.Methods: The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three countries (Brazil, Argentina, and Chile). The main outcome was the number of annual exacerbations/hospitalizations, with all-cause and respiratory-related deaths as the secondary outcomes. A statistical evaluation comprised the relative weight and ideal cut-off point for the number or severity of the exacerbations and was incorporated into the FACED score (E-FACED). The results obtained after the application of FACED and E-FACED were compared in both the cohorts.Results: A total of 1,470 patients with bronchiectasis (819 from the construction cohorts and 651 from the external validation cohorts) were followed up for 5 years after diagnosis. The best cut-off point was at least two exacerbations in the previous year (two additional points), meaning that the E-FACED has nine points of growing severity. E-FACED presented an excellent prognostic capacity for exacerbations (areas under the receiver operating characteristic curve: 0.82 for at least two exacerbations in 1 year and 0.87 for at least one hospitalization in 1 year) that was statistically better than that of the FACED score (0.72 and 0.78, P

Background: Clinical and demographical knowledge on Spanish cystic fibrosis (CF) patients is incomplete as no national registry exists. CF-microbiology has not been studied at national level. The results of the first Spanish multicenter study on CF microbiology are presented. Methods: 24 CF-Units for adult (n = 12) and pediatric (n = 12) patients from 17 hospitals provided sputa and clinical data from 15 consecutive patients. Cultures and susceptibility testing were performed. Colonization impact on pulmonary function was assessed. Results: 341 patients [mean (SD) age 21 (11) years, 180 >= 18 years, mean (SD) FEV1 = 68 (25)%] were included. Pseudomonas aeruginosa was reported as chronic, intermittent or absent in 46%, 22% and 32% of patients, respectively. The annual prevalence was 62%. Positive P. aeruginosa and methicillin-resistant Staphylococcus aureus cultures were significantly associated with lower FEV1 (p < 0.001 and p = 0.003, respectively). Conclusions: The representative subset of the Spanish CF-population which has been clinically, demographically and microbiologically. characterized will serve as a reference for future CF studies in Spain. (C) 2015 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

RATIONALE: The clinical presentation and prognosis of non-cystic fibrosis bronchiectasis are both very heterogeneous. OBJECTIVES: To identify different clinical phenotypes for non-cystic fibrosis bronchiectasis and their impact on prognosis. METHODS: Using a standardized protocol, we conducted a multicenter observational cohort study at six Spanish centers with patients diagnosed with non-cystic fibrosis bronchiectasis before December 31, 2005, with a 5-year follow-up from the bronchiectasis diagnosis. A cluster analysis was used to classify the patients into homogeneous groups by means of significant variables corresponding to different aspects of bronchiectasis (clinical phenotypes): age, sex, body mass index, smoking habit, dyspnea, macroscopic appearance of sputum, number of exacerbations, chronic colonization with Pseudomonas aeruginosa, FEV1, number of pulmonary lobes affected, idiopathic bronchiectasis, and associated chronic obstructive pulmonary disease. Survival analysis (Kaplan-Meier method and log-rank test) was used to evaluate the comparative survival of the different subgroups. MEASUREMENTS AND MAIN RESULTS: A total of 468 patients with a mean age of 63 (15.9) years were analyzed. Of these, 58% were females, 39.7% had idiopathic bronchiectasis, and 29.3% presented with chronic Pseudomonas aeruginosa colonization. Cluster analysis showed four clinical phenotypes: (1) younger women with mild disease, (2) older women with mild disease, (3) older patients with severe disease who had frequent exacerbations, and (4) older patients with severe disease who did not have frequent exacerbations. The follow-up period was 54 months, during which there were 95 deaths. Mortality was low in the first and second groups (3.9% and 7.6%, respectively) and high for the third (37%) and fourth (40.8%) groups. The third cluster had a higher proportion of respiratory deaths than the fourth (77.8% vs. 34.4%; P < 0.001). CONCLUSIONS: Using cluster analysis, it is possible to separate patients with bronchiectasis into distinct clinical phenotypes with different prognoses.

Pseudomonas aeruginosa is the main pathogen in bronchopulmonary infections in cystic fibrosis (CF) patients. It can only be eradicated at early infection stages while reduction of its bacterial load is the therapeutic goal during chronic infection or exacerbations. Neonatal screening and pharmacokinetic/pharmacodynamic knowledge has modified the management of CF-patients. A culture based microbiological follow-up should be performed in patients with no infection with P. aeruginosa. At initial infection, inhaled colistin (0,5-2 MU/tid), tobramycin (300 mg/bid) or aztreonam (75 mg/tid) with or without oral ciprofloxacin (15-20 mg/kg/bid, 2-3 weeks) are recommended. In chronic infections, treatment is based on continuous administration of colistin or with a 28-day on-off regimen with tobramycin or aztreonam. During mild-moderate exacerbations oral ciprofloxacin (2-3 weeks) can be administered while serious exacerbations must be treated with intravenous combination therapy (beta-lactam with an aminoglycoside or a fluoroquinolone). Future studies will support antibiotic rotation and/or new combination therapies. Epidemiological measures are also recommended to avoid new P. aeruginosa infections and "patient-to-patient transmission" of this pathogen. (C) 2014 SEPAR. Published by Elsevier Espana, S.L.U. All rights reserved.

Pulmonary exacerbation (PEx) is a hallmark of cystic fibrosis. Although several criteria have been proposed for the definition of PEx, no consensus has yet been reached. Very often, many PEx cases go unreported. A standardized and validated definition is needed to reduce variability in clinical practice. The pathophysiology of recurrent episodes remains unclear, and both onset and risk are multifactorial. PEx leads to increased healthcare costs, impaired quality of life and a cycle in which PEx causes loss of lung function, which predisposes to further episodes. The number of episodes affects survival. Although early diagnosis and aggressive treatment are highly recommended, measures to prevent the emergence of new PEx are even more important. In particular, inhaled antibiotics administered under new treatment schedules could play a key role in preventing exacerbations and thus delay decline in lung function and reduce mortality. The primary objective is zero exacerbations.

Changes in soil heavy metal extractability following the cessation of biosolids applications were studied in a long-term field experiment. Two anaerobically digested biosolids from wastewater treatment plants in Madrid (Sur and Viveros) were applied to cropland from 1983 to 1990. Soil samples were collected in the 1st, 5th and 9th year after the last biosolids application. Soil pH did not vary significantly after biosolids applications. Organic matter and total heavy metals (Zn, Pb, Cd, Ni, Cr and Cu) concentrations initially increased but then declined over time, mostly after the first 5 years following biosolids application. Metal extracted with DPTA increased in Sur treatments during the 1st year and diminished thereafter. However, in Viveros treatments, heavy metals extracted increased during the 1st year, declined in 1995, and showed a slight increase in 1999. These changes in heavy metal extractability were widely observed in the percentage of extractable metal recovery (EMR). The differences observed in the pattern of the two sources of biosolids applied could be due to the different rates of decomposition of their organic matter. © 2002 Elsevier Science Ltd. All rights reserved.

Recurrent endobronchial infection in cystic fibrosis requires treatment with intravenous antibiotics for several weeks, which is usually administered in hospital, affecting health costs and quality of life for patients and their families. It is not known whether patients receiving intravenous treatment at home have better or equivalent health outcomes, if costs are reduced or if it is preferred than in-hospital treatment. Home treatment requires training to patients and carers and usually needs a few previous days in hospital.To determine whether home intravenous antibiotic therapy in cystic fibrosis is as effective as in-patient intravenous antibiotic therapy and if it is preferred by patients and/or families.References to trials were obtained from the specialist cystic fibrosis trials register held by the editorial base of the Cochrane Cystic Fibrosis and Genetic Disorders Group. Handsearching of the abstracts books of all Spanish Conferences on cystic fibrosis and the last European Conference (Stockholm, 2000) was carried out by authors.Randomised controlled trials where home intravenous antibiotic treatment for patients with cystic fibrosis was compared with in-hospital intravenous antibiotic treatment, including adults and children with cystic fibrosis. All kinds of antibiotics and regimens administered intravenous were included.Three reviewers independently selected the trials to be included in the review, assessed methodological quality of each trial and extracted data using a standardised form. Because of several limitations, narrative synthesis was used at this stage.One study was included with 17 patients aged 10 to 41 years with an infective exacerbation by Pseudomonas aeruginosa. All their 31 admissions were analysed as independent events. Outcomes were measured at 21 days of follow-up after initiation of treatment. Home patients had fewer investigations performed than hospital patients (p0.002) and general activity was higher in the home group. No differences were found for clinical outcomes, adverse events, complications of intravenous lines or line changes or time to next admission. Home patients received less low-dose home maintenance antibiotic. Quality of life measures showed no differences for dyspnoea and emotional state, but fatigue and mastery were worse for home patients, possibly due to a higher general activity and need of support. Personal, family, sleeping and eating disruptions were less important for home than hospital admissions. Home therapy was cheaper for families and the hospital. Indirect costs were not determined.The current evidence is restricted to one small study. It suggests that in the short term home therapy does not harm patients and in general reduces social disruptions. The decision to attempt home treatment should be based on an individual basis and appropriate local resources. More research is urgently required.

Se presentan los resultados del comportamiento friccional de cinco diferentes combinaciones de cabezas protésicas femorales y polietileno acetabular, investigadas en un simulador de cadera. Las cabezas femorales de aleaciones de Cr-Co-Mo, Cr-Co-Mo con implantación iónica y de cerámica de zirconia, se articularon con cotilos de polietileno de ultra-alto-peso molecular y se sometieron a tres millones de ciclos de marcha cada uno, utilizando un simulador de cadera de cinco estaciones. El desgaste de los cotilos protésicos, evaluado por mediciones gravimétricas demostró que cuando el espesor de polietileno era de 10,9 mm el desgaste producido por cabezas de Cr-Co-Mo de 28 mm era de 0,14 mm/año y de 0,09 mm/año si la cabeza utilizada era de 28 mm de aleación de Cr-Co- Mo con implantación iónica. Cuando el espesor del acetábulo era de 7 mm el desgaste ocasionado por cabezas de cerámica de zirconia de 28 mm fue de 0,04 mm/año; sin embargo si tenía espesor 10,9 mm y las cabezas de cerámica de zirconia eran de 28 y 32 mm, no aparecía desgaste alguno. Estos resultados indican que el diámetro de la cabeza de cerámica de 28 y 32 mm, parece no tener importancia alguna, pero sí la tiene el que el espesor del acetábulo protésico sea menor de 10 mm. Ultra high molecular weight polyethylene (UHMWPE) metal backed acetabular cups were articulated against Cr-Co-Mo alloy, ion implanted Cr-Co-Mo and zirconia ceramic femoral heads in a five station hip joint simulator, running three tests of three millions walking cycles with each one of the five different head-cup combinations. The wear rate of the acetabular cups was measured gravimetrically. When the thickness of the cup was 10.9 mm, the mean wear rate against 28 mm diameter Cr-Co-Mo femoral heads was 0.14 mm/year, and 0.09 mm/year 'when the cup was articulated against 28 mm ion implanted Cr-Co-Mo femoral heads. When the thickness of the cup was 7 mm the mean wear rate was 0.04 mm/year against 28 mm zirconia heads. The results indicate that significant reduction in the wear of the UHMWPE cup can be achieved when zirconia femoral head is used and that the diameter of the zirconia femoral head, 28 vs 32 mm, seems unimportant as wear factor. A cup thickness below 10 mm may result disadvantageous.

There are no doubts that Hispania was, under the Roman Empire, the most profitable and abundant in resources of all the mining districts exploded by Rome -Hispania, Galia, Danubian Provinces, Dacia, Greece, Asia Minor, Syria, Egypt and North Africa-. Plinius gives the same data and hands many more in the passages dedicated to the mining and the working of the metals in his Natural History. Of all the many mining places that this author refers, the exploiting in copper at Cerro Muriano presents the main interest; of this he says that it was of an unbeatable quality, used, among other applications, to manufacture the very famous "Copper of Campania". The utilization of fusing agents, adapted to the process of copper obtainment, is fundamental for obtaining this high quality. The addition of these fusing agents was very important for the control of the work parameters and to reach the required temperatures. The high purity of the obtained copper shows a very close knowledge of the phenomena involved in the process. Durante el Imperio Romano, de todos los distritos mineros explotados por Roma -Hispania, Galia, las provincias danubianas, Dacia, Grecia, Asia Menor, Siria, Egipto y el Norte de África-, sin duda el más rentable y más abundante en recursos era Hispania. Plinio facilita muchos datos en los pasajes dedicados a la minería y el trabajo de los metales en su Historia Natural. Entre los muchos lugares mineros que este autor cita, nos interesa ahora la explotación de cobre en Cerro Muriano, del cual dice que era de una calidad inmejorable, utilizado, entre otras aplicaciones, para fabricar el famosísimo cobre de Campania. Para poder obtener esta elevada calidad, es fundamental la utilización de fundentes adecuados en el proceso de obtención de cobre. La adición de estos fundentes era vital para controlar los parámetros de trabajo y alcanzar las temperaturas requeridas. La elevada pureza del cobre obtenido demuestra un conocimiento muy ajustado de los fenómenos que tenían lugar.