CO72 357. Reconstrucción del cuerpo fibroso mitroaórtico: Resultados perioperatorios y supervivencia a largo plazo

ObjetivosDescribir las características clínicas, resultados perioperatorios y supervivencia a largo plazo de pacientes sometidos a sustitución valvular mitral y aórtica con reconstrucción de la fbrosa mitroaórtica según técnica de David.MétodosRevisión retrospectiva de pacientes sometidos a sustitución valvular y reconstrucción de la fibrosa mitroaórtica intervenidos en los últimos 14 años. Se incluyen 27 pacientes, 63% varones, edad 55,3±16,1 años, EuroSCORE 34,4±25,9. El 70,4% de los pacientes presentaban al menos una cirugía previa. La indicación para el procedimiento fue endocarditis infecciosa complicada con absceso de la unión mitroaórtica en el 74,1%; en el restante 25,9% la principal indicación fue la necesidad de ampliación del anillo aórtico en el seno de una valvulopatía mitroaórtica.ResultadosLa supervivencia perioperatoria (30 días) fue del 85,2%. No se observó mortalidad entre los pacientes sin endocarditis. Estancia media: 42,8±42,8 días. Complicaciones perioperatorias: accidente cerebrovascular agudo (ACVA) 3,1% (1), balón de contrapulsación intraaórtica (BCIA) 11,1% (3), insuficiencia renal aguda (IRA) 7,4% (2), infarto agudo de miocardio (IAM) 3,7% (1), reintervención por sangrado 14,8% (4), ventilación mecánica prolongada 11,1% (3), necesidad de marcapasos (MP) definitivo 22,2%. La supervivencia acumulada para los supervivientes a 1, 3 y 5 años fue de 85,9±7,6%, 75,8 ± 9,5% y 45,5 ± 11,1%, respectivamente. No se observó diferencia significativa al comparar la super-vivencia a 1, 3 y 5 años entre los grupos intervenidos por endocarditis y los no endocarditis (log rank 0,146).ConclusionesLa reconstrucción de la fibrosa mitroaórtica mediante técnica de David representa una alternativa terapéutica para el manejo de patología compleja que involucra esta estructura. Hemos observado buenos resultados perioperatorios y a largo plazo teniendo en cuenta el perfil de alto riesgo de los pacientes que requieren este tipo de intervención

Congenital long QT syndrome

Artículo especialThe long QT syndrome (LQTS) is an hereditary disease that produces a malfunction on the sodium and potassium channels of the heart and lengthens the duration of the cardiac repolarization stage. It is characterized by the appearance of syncopes, arrhythmias and even sudden death. There are two variants of the congenital LQTS: the autosomal dominant Romano-Ward syndrome and the autosomal recessive Jervell-Lange-Nielsen syndrome, that is associated with sensorineural deafness and is less frequent than the other type. The diagnosis is made from the clinical criteria, the electrocardiogram and the family history. In the last few years, molecular studies have been developed, opening new possibilities not only for its diagnosis but also for the treatment of these patients.N

Inversion charge study in TMO hole-selective contact-based solar cells

© 2023 IEEE. Personal use of this material is permitted. Permission from IEEE must be obtained for all other uses, in any current or future media, including reprinting/republishing this material for advertising or promotional purposes,creating new collective works, for resale or redistribution to servers or lists, or reuse of any copyrighted component of this work in other works.In this article, we study the effect of the inversion charge ( Q inv ) in a solar cell based on the hole-selective characteristic of substoichiometric molybdenum oxide (MoO x ) and vanadium oxide (VO x ) deposited directly on n-type silicon. We measure the capacitance–voltage ( C – V ) curves of the solar cells at different frequencies and explain the results taking into account the variation of the space charge and the existence of Q inv in the c-Si inverted region. The high-frequency capacitance measurements follow the Schottky metal–semiconductor theory, pointing to a low inversion charge influence in these measurements. However, for frequencies lower than 20 kHz, an increase in the capacitance is observed, which we relate to the contribution of the inversion charge. In addition, applying the metal–semiconductor theory to the high-frequency measurements, we have obtained the built-in voltage potential and show new evidence about the nature of the conduction process in this structure. This article provides a better understanding of the transition metal oxide/n-type crystalline silicon heterocontact.The authors would like to acknowledge the CAI de Técnicas Físicas of the Universidad Complutense de Madrid. The authors would also like to thank the Mexican grants program CONACyT for its financial collaboration.Peer ReviewedPostprint (author's final draft

Síndromes muy poco frecuentes

Dismorfología, Citogenética y Clínica: Resultados de estudios sobre los datos del ECEMCIn an attempt to facilitate the knowledge of the malformation syndromes that have very few frequencies to pediatricians and first health care physicians, particularly to those of rural areas, we have selected six new syndromes. As in previous years, the syndromes are selected from the ECEMC database registry. In this Boletín we include the following syndromes: Townes-Bröcks, MMT, Smith-Lemli-Opitz, Coffin-Siris, Espleno-gonadal fusion, and Silver-Russell syndromes. For each syndrome, we described the most important clinical characteristics, and the present knowledge of their causal factors.N

A more sustainable and efficient definition of Regulated Deficit Irrigation phases in olive (Olea europaea L.)

Resumen del trabajo presentado en 20th EGU General Assembly, clelebrada en Viena (Austria) del 04 al 13 de abril de 2018.Water is a limited but highly essential resource, with large quantities required for agriculture. Regulated Deficit Irrigation (RDI) is an agricultural technique with great relevance for water savings worldwide, in which water stress is imposed by irrigation withholding based on fruit growth phases. The objective of this method is to identify phases where water stress has little or no effect on yield. RDI in olive has been demonstrated as an efficient tool to save water without negatively affecting yield. In olive trees, the mid-summer “pit hardening” is recognized as the most drought-resistant phenological stage, and has been used successfully for RDI water savings even though neither the description, boundaries, nor length of the period have been cleared reported. Many studies merely utilize a constant reference date for pit hardening, providing no explanation regarding how it was estimated or measured, while a few cases report the resistant to a knife-cut as the proper method to identify hardening, but leave unclear whether it represents its onset or completion. Recent studies have addressed these uncertainties, better showing the nature and duration of olive pit hardening, to which RDI can now be fitted. The objective of this current work was to determine if a RDI strategy more precisely fitted to pit hardening influences yield. In Ciudad Real (Spain) in 2016 and 2017, four irrigation treatments were applied in an ‘Arbequina’ olive orchard planted at 7 x 4.75 m in 1999. Treatment T1 consisted in water stress during pit hardening, aiming to maintain stem water potential (SWP) of -2 MPa during this phase. Treatment T2 was severely water stressed, aimed at maintaining -3 MPa during the same phase. In the rest of the season, before and after pit hardening, both treatments were irrigated to prevent water stress. Additionally, a highly deficit treatment was established (T3), irrigated only after pit hardening was completed. A control treatment (T0), irrigated following FAO methodology, was established to determine potential yield. Irrigation water savings with respect to T0 were approximately 45%, 57%, and 77% for T1, T2, and T3, respectively. There were no significant yield differences among treatments, although 2017 was nearly significant (P = 0.06), as when both years were considered together, due to the low yield of T3. Average yields from the two years were 25, 24, 23 and 21 kg tree-1 in T0, T1, T2 and T3, respectively. In conclusion, basing RDI on a new, more precise definition of the pit hardening phase produces similar yields with higher water savings, with the consequent environmental, economic and energetic benefits.Peer reviewe

Deficit irrigation and emerging fruit crops as a strategy to save water in Mediterranean semiarid agrosystems

Water scarcity in Mediterranean climate areas will be progressively aggravated by climate change, population increase and urban, tourism and industrial activities. To protect water resources and their integrity for future use and to improve biodiversity, besides following advanced deficit irrigation strategies in fruit cultivation, attention could well be directed towards what are at present underused plant materials able to withstand deficit irrigation with minimum impact on yield and fruit quality. To this end, the state of the art as regards deficit irrigation strategies and the response of some very interesting emerging fruit crops [jujube (Zizyphus jujuba Mill.), loquat (Eriobotrya japonica Lindl.), pistachio (Pistacia vera L.) and pomegranate (Punica granatum L.)] are reviewed. The strengths and weaknesses of deficit irrigation strategies and the mechanisms developed by these emerging fruit crops in the face of water stress are discussed. The response of these crops to deficit irrigation, with special attention paid to the effect on yield but also on fruit quality and health-related chemical compounds, was analysed in order to assess their suitability for saving water in Mediterranean semiarid agrosystems and to analyze their potential role as alternatives to currently cultivated fruit crops with higher water requirements. Finally, the factors involved in establishing an identity brand (hydroSOS) to protect fruits obtained under specific DI conditions are discussed

HLA-DRB1 association with Henoch-Schonlein purpura

Objective: Henoch-Schönlein purpura (HSP) is the most common vasculitis in children but it is not exceptional in adults. Increased familial occurrence supports a genetic predisposition for HSP. In this context, an association with the human leukocyte antigen-HLA-DRB1*01 phenotype has been suggested in Caucasian individuals with HSP. However, data on the potential association of HSP with HLA-DRB1*01 were based on small case series. To further investigate this issue, we performed HLA-DRB1 genotyping of the largest series of HSP patients ever assessed for genetic studies in Caucasians. Methods: 342 Spanish patients diagnosed with HSP fulfilling the American College of Rheumatology and the Michel et al classification criteria, and 303 sex and ethnically matched controls were assessed. HLA-DRB1 alleles were determined using a PCR-Sequence-Specific-Oligonucleotide Probe (PCR-SSOP) method. Results: A statistically significant increase of HLA-DRB1*01 in HSP patients when compared with controls was found (43% vs 7%, respectively; p<0.001; odds ratio-OR=2.03 [1.43-2.87]). It was due to the increased frequency of HLA-DRB1*0103 phenotype in HSP (14% vs 2%; p<0.001; OR=8.27 [3.46-23.9]). These results remained statistically significant after adjusting for Bonferroni correction. In contrast, a statistically significant decreased frequency of the HLA-DRB1*0301 phenotype was observed in patients compared to controls (5.6% vs 18.1%, respectively; p<0.001, OR=0.26 [0.14-0.47]), even after adjustment for Bonferroni correction. No HLA-DRB1 association with specific features of the disease was found. Conclusion: Our study confirms an association of HSP with HLA-DRB1*01 in Caucasians. Also, a protective effect against the development of HSP appears to exist in Caucasians carrying the HLA-DRB1*03 phenotype

Epidermolysis bullosa (EB): Pathogenesis, clinical, diagnostic and genetic aspects, molecular basis, epidemiological aspects, management of patients with EB and translational implications of mutation analysis

Dismorfología, Citogenética y Clínica: Resultados de estudios sobre los datos del ECEMCUnder the term "Epidermolysis Bullosa" (EB), there is a heterogeneous group of vesicular disorders that are generally congenital and of genetic origin, and affect skin and often mucosas. It is remarkable the extreme fragility of these epithelia; the vesicles arise spontaneously as well as induced by even slight trauma or the influence of high temperatures. Their content is sero-hemorrhagic and in the scarring can be very difficult. As a consecuence of the scarring processes, joint contractures and fusion of digits (pseudo-syndactyly) can occur. Pathogenetically, EB is caused by abnormal blistering at the basement membrane zone in the dermal-epidermal attachment zone and its surroundings. This is due to alterations in the attachment complexes, and some mutations have been identified in a total of 10 genes expressed in such level. Four main types of EB can be distinguished (simple, junctional, dystrophic and hemidesmosomal), depending on the level at which the cleavage that forms the bulla takes place, although about 30 subtypes have been described. The clinical characteristics or optic microscopy are not adequate for diagnosis, and it is mandatory to perform electron microscopy, immunofluorescent and immunohistochemical studies, as well as mutation analysis if available. Regarding the epidemiological aspects, in the Spanish Collaborative Study of Congenital Malformations (ECEMC), since 1976 up to December 2004, a total of 2,204,264 liveborn infants were controlled and, among them 27 cases have been identified, for a minimum frequency of 0.12 per 10,000 (95% confidence interval:0.08-0.18). It seems generally accepted that the determination of the frequency of EB is subject to multiple biases and registration of cases is always incomplete. We have also reviewed the issues related to management of EB patients by clinicians.N

Bronchoscopist's perception of the quality of the single-use bronchoscope (Ambu aScope4™) in selected bronchoscopies: a multicenter study in 21 Spanish pulmonology services

Background: The disposable bronchoscope is an excellent alternative to face the problem of SARS-CoV-2 and other cross infections, but the bronchoscopist's perception of its quality has not been evaluated. Methods: To evaluate the quality of the Ambu-aScope4 disposable bronchoscope, we carried out a cross-sectional study in 21 Spanish pulmonology services. We use a standardized questionnaire completed by the bronchoscopists at the end of each bronchoscopy. The variables were described with absolute and relative frequencies, measures of central tendency and dispersion depending on their nature. The existence of learning curves was evaluated by CUSUM analysis. Results: The most frequent indications in 300 included bronchoscopies was bronchial aspiration in 69.3% and the median duration of these was 9.1 min. The route of entry was nasal in 47.2% and oral in 34.1%. The average score for ease of use, image, and aspiration quality was 80/100. All the planned techniques were performed in 94.9% and the bronchoscopist was satisfied in 96.6% of the bronchoscopies. They highlighted the portability and immediacy of the aScope4TM to start the procedure in 99.3%, the possibility of taking and storing images in 99.3%. The CUSUM analysis showed average scores > 70/100 from the first procedure and from the 9th procedure more than 80% of the scores exceeded the 80/100 score. Conclusions: The aScope4™ scored well for ease of use, imaging, and aspiration. We found a learning curve with excellent scores from the 9th procedure. Bronchoscopists highlighted its portability, immediacy of use and the possibility of taking and storing images

Role of MUC1 rs4072037 polymorphism and serum KL-6 levels in patients with antisynthetase syndrome

Mucin 1/Krebs von den Lungen-6 (KL-6) is proposed as a serum biomarker of several interstitial lung diseases (ILDs), including connective tissue disorders associated with ILD. However, it has not been studied in a large cohort of Caucasian antisynthetase syndrome (ASSD) patients. Consequently, we assessed the role of MUC1 rs4072037 and serum KL-6 levels as a potential biomarker of ASSD susceptibility and for the differential diagnosis between patients with ILD associated with ASSD (ASSD-ILD +) and idiopathic pulmonary fibrosis (IPF). 168 ASSD patients (149 ASSD-ILD +), 174 IPF patients and 523 healthy controls were genotyped for MUC1 rs4072037 T > C. Serum KL-6 levels were determined in a subgroup of individuals. A significant increase of MUC1 rs4072037 CC genotype and C allele frequencies was observed in ASSD patients compared to healthy controls. Likewise, MUC1 rs4072037 TC and CC genotypes and C allele frequencies were significantly different between ASSD-ILD+ and IPF patients. Additionally, serum KL-6 levels were significantly higher in ASSD patients compared to healthy controls. Nevertheless, no differences in serum KL-6 levels were found between ASSD-ILD+ and IPF patients. Our results suggest that the presence of MUC1 rs4072037 C allele increases the risk of ASSD and it could be a useful genetic biomarker for the differential diagnosis between ASSD-ILD+ and IPF patients