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espanolIntroduccion La arteritis de celulas gigantes (ACG) es una vasculitis que afecta a arterias de mediano y gran calibre. La biopsia de la arteria temporal es la tecnica diagnostica de eleccion. Atendiendo a la demanda asistencial que supone, pretendemos evaluar la utilidad de la ecografia Doppler en los pacientes con sospecha de ACG, su sensibilidad y especificidad como prueba diagnostica y si puede suplir a la biopsia. Materiales y metodos Se ha realizado un estudio prospectivo de 57 pacientes entre febrero de 2015 y julio de 2016, que han sido diagnosticados con exploracion mediante ecografia Doppler y biopsia de arteria temporal. Resultados Fueron incluidos 57 pacientes, de los cuales 3 fallecieron durante el seguimiento y una paciente fue excluida del estudio al negarse a la biopsia. Otros 21 pacientes fueron diagnosticados de ACG por un especialista reumatologo tras un minimo de 6 meses de seguimiento. Presentaron ecografia positiva 22 pacientes, de los cuales 8 fueron diagnosticados de ACG y 4 de polimialgia reumatica. En nuestro estudio, la sensibilidad de la ecografia es del 42,6% y la especificidad es del 65,7%. Otros 19 pacientes presentaron biopsia positiva: todos ellos fueron diagnosticados de ACG. La biopsia presento una sensibilidad de un 73,7% y una especificidad de un 100%. Conclusiones Segun nuestro estudio, la utilidad de la ecografia es limitada y son necesarias nuevas investigaciones para determinar su papel en esta entidad. EnglishBackground Giant cell arteritis (GCA) is a vasculitis that affects medium- and large-sized arteries. Temporal artery biopsy is the gold standard for diagnosis. In view of the high demand for temporal biopsies, the purpose of this study is to evaluate the usefulness of Doppler ultrasonography in patients with suspected giant cell arteritis, to determine its sensitivity and specificity as a diagnostic test and to determine whether it would be possible to substitute biopsy for ultrasonography. Materials and methods A prospective study was undertaken including 57 patients from February 2015 to July 2016, who have undergone both ultrasonography and temporal biopsy. Results A total of 57 patients were included, 3 of whom died during the follow-up, and a patient was excluded from the study when she refused to have the biopsy. Another 21 patients were diagnosed with GCA by a rheumatologist after a minimum of 6 months of follow-up and 22 patients had positive ultrasonography, 8 of whom were diagnosed with GCA and 4 with polymyalgia rheumatica. In our study, the sensitivity of ultrasonography was 42.6%, and the specificity was 65.7%. A total of 19 patients had a positive biopsy, all of them were diagnosed with GCA. In our study, the sensitivity of the biopsy was 73.7% and the specificity was 100%. Conclusions In view of the data from our study, the usefulness of ultrasonography is questionable, and research about the role of ultrasonography in this disease should be further studied.

Resumen Introduccion y objetivos Los valores plasmaticos de galectina-3 (Gal-3) estan elevados y se correlacionan con la mortalidad total y cardiovascular en pacientes con insuficiencia cardiaca, pero su correlacion con el pronostico tras el trasplante cardiaco (TxC) es desconocida. El objetivo fue describir la tendencia evolutiva y el valor pronostico de este biomarcador tras el TxC. Metodos Mediante enzimoinmunoensayo, se midieron las concentraciones plasmaticas de Gal-3 en muestras de suero de 122 receptores de TxC, antes y 1, 3, 6 y 12 meses despues de este. Mediante regresion de Cox se analizo el valor pronostico del valor plasmatico de Gal-3 a los 12 meses del TxC. El objetivo primario del estudio fue la variable combinada muerte o disfuncion del injerto. Resultados Las concentraciones de Gal-3 disminuyeron progresivamente durante el primer ano tras el TxC (medianas: pretrasplante, 19,1 ng/ml; 1 ano postrasplante, 14,6 ng/ml; p Conclusiones Las concentraciones plasmaticas de Gal-3 disminuyeron progresivamente durante el primer ano tras el TxC. Un valor plasmatico elevado de Gal-3 1 ano tras el TxC se correlaciono con un pronostico adverso.

[Abstract] Introduction and objectives: Circulating galectin-3 (Gal-3) is elevated and significantly correlates with all-cause and cardiovascular mortality in patients with heart failure. However, the relationship between serum Gal-3 and heart transplant (HT) outcomes is unclear. The aim of this study was to describe the longitudinal trend and prognostic value of Gal-3 levels after HT. Methods: Banked serum samples were available from 122 HT recipients, collected before transplant and at 1, 3, 6, and 12 months posttransplant. Gal-3 levels in these serum samples were measured by enzyme immune assay. Multivariable Cox regression was performed to determine the prognostic value of 12-month posttransplant Gal-3 serum levels. The primary endpoint was the composite variable all-cause death or graft failure over long-term posttransplant follow-up. Results: Circulating Gal-3 concentration steadily decreased during the first year after HT (median values: pretransplant, 19.1 ng/mL; 1-year posttransplant, 14.6 ng/mL; P

[Abstract] Introduction and objectives. Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases. Methods. We conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied. Results. We identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index

[Abstract] Background. Giant cell arteritis (GCA) is a vasculitis that affects medium- and large-sized arteries. Temporal artery biopsy is the gold standard for diagnosis. In view of the high demand for temporal biopsies, the purpose of this study is to evaluate the usefulness of Doppler ultrasonography in patients with suspected giant cell arteritis, to determine its sensitivity and specificity as a diagnostic test and to determine whether it would be possible to substitute biopsy for ultrasonography. Materials and methods. A prospective study was undertaken including 57 patients from February 2015 to July 2016, who have undergone both ultrasonography and temporal biopsy. Results. A total of 57 patients were included, 3 of whom died during the follow-up, and a patient was excluded from the study when she refused to have the biopsy. Another 21 patients were diagnosed with GCA by a rheumatologist after a minimum of 6 months of follow-up and 22 patients had positive ultrasonography, 8 of whom were diagnosed with GCA and 4 with polymyalgia rheumatica. In our study, the sensitivity of ultrasonography was 42.6%, and the specificity was 65.7%. A total of 19 patients had a positive biopsy, all of them were diagnosed with GCA. In our study, the sensitivity of the biopsy was 73.7% and the specificity was 100%. Conclusions. In view of the data from our study, the usefulness of ultrasonography is questionable, and research about the role of ultrasonography in this disease should be further studied. [Resumen] Introducción. La arteritis de células gigantes (ACG) es una vasculitis que afecta a arterias de mediano y gran calibre. La biopsia de la arteria temporal es la técnica diagnóstica de elección. Atendiendo a la demanda asistencial que supone, pretendemos evaluar la utilidad de la ecografía Doppler en los pacientes con sospecha de ACG, su sensibilidad y especificidad como prueba diagnóstica y si puede suplir a la biopsia. Materiales y métodos. Se ha realizado un estudio prospectivo de 57 pacientes entre febrero de 2015 y julio de 2016, que han sido diagnosticados con exploración mediante ecografía Doppler y biopsia de arteria temporal. Resultados. Fueron incluidos 57 pacientes, de los cuales 3 fallecieron durante el seguimiento y una paciente fue excluida del estudio al negarse a la biopsia. Otros 21 pacientes fueron diagnosticados de ACG por un especialista reumatólogo tras un mínimo de 6 meses de seguimiento. Presentaron ecografía positiva 22 pacientes, de los cuales 8 fueron diagnosticados de ACG y 4 de polimialgia reumática. En nuestro estudio, la sensibilidad de la ecografía es del 42.6% y la especificidad es del 65.7%. Otros 19 pacientes presentaron biopsia positiva: todos ellos fueron diagnosticados de ACG. La biopsia presentóuna sensibilidad de un 73.7% y una especificidad de un 100%. Conclusiones. Según nuestro estudio, la utilidad de la ecografía es limitada y son necesarias nuevas investigaciones para determinar su papel en esta entidad.

[Abstract] Background: Acute cellular rejection (ACR) is a major complication in heart transplantation (HTx). Endomyocardial biopsy is the reference method for early detection of ACR, but a new non-invasive approach is needed. Tentative candidates could be circulating microRNAs. This study aimed to discover and validate microRNAs in serum for ACR detection after HTx. Methods: This prospective, observational, single-center study included 121 HTx patients. ACR was graded according to International Society of Heart and Lung Transplantation classification (0R−3R). First, in the discovery phase, microRNA expression profile was carried out in serum samples from patients at pre-rejection, during, and post-rejection time (0RS1 → 2RS2 → 0RS3). Relative expression (2-ΔCq) of 179 microRNAs per sample was analyzed by reverse transcription quantitative polymerase chain reaction. Second, a microRNA with a significant rise and fall pattern during ACR was selected for the next validation phase, where it was analyzed (reverse transcription quantitative polymerase chain reaction) in serum samples from 2 groups of patients: the no-ACR group (0R grade) and the ACR group (≥2R grade). Finally, a sensitivity analysis (receiver operating characteristic curve) was done to assess microRNA accuracy for ACR detection in HTx. Results: A total of 21 ACR episodes (0RS1 → 2RS2 → 0RS3) with their respective serum samples (n = 63) were included in the discovery phase. Among the 179 microRNAs analyzed, only miR-181a-5p met the rise and fall criteria. In the validation phase, miR-181a-5p relative expression (2-ΔCq) in the ACR group (n = 45) was significantly overexpressed (p < 0.0001) vs the no-ACR group (n = 45). miR-181a-5p showed an area under the curve of 0.804 (95% confidence interval: 0.707-0.880); sensitivity and specificity of 78% and 76%, respectively; and a negative predicted value of 98%. This study received financial support from Instituto de Salud Carlos III (PI15/02224), is part of the research activities of the Centro de investigación Biomédica en Red Enfermedades Cardiovasculares (CIBERCV), and was cofinanced with FEDER Funds

[Abstract] Introduction and objectives. Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases. Methods. We conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied. Results. We identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index

Only five cases of multifocal medulloblastoma in the adult have been reported to date. We present a case in a male patient in his 50th decade of life who presented with three extra-axial lesions associated with a parenchymatous lesion of the right middle cerebellar peduncle. Sputum sample examination revealed larvae compatible with strongyloides stercoralis, which was our main differential diagnosis. Histological and immunohistochemical studies revealed the existence of a desmoplastic medulloblastoma.

Background Acute Cellular Rejection (ACR) remains a major complication in heart transplantation (HT). Since 1970, endomyocardial biopsy (EMB) has been the gold standard for ACR early detection. However, this invasive procedure has several limitations such as risk of complications, interobserver variability, false negative results and high cost. Therefore, a new non-invasive ACR biomarker is needed. Potential candidates could be microRNAs (miRs), small regulatory RNAs whose serum expression profiling could be modified in ACR patients. The aim of this study is the identification of differentially serum miRs expression patterns in ACR after HT. Methods Among HT performed in our Hospital (2013–2018), we selected patients with following criteria: i) “0R → 2R → 0R” EMB pattern for ACR (0R=no ACR, 2R=moderate ACR); ii) no antibody mediated rejection; iii) serum collected the same day as the EMBs. The 3 serum samples per patient matched in time with EMB were called “0Rs1 → 2Rs2 → 0Rs3”. In each of these serum samples, 179 miRs expression profile according manufacturer's instructions were analysed. Global Mean (GM) method was used to normalize our results expressed as miRs relative expression [2(–ΔCq)]. Expression pattern was defined as a 2 steps process: a significative rise (0Rs1 → 2Rs2) followed by a significative fall (2Rs2 → 0Rs3), or vice versa, in miR expression. Analysis of differences between 3 groups were calculated using one-way ANOVA with matched data and post-hoc Tukey test. ROC curve was generated for selected miRNA. A two-sided p Results 21 patients and their serum “0Rs1 → 2Rs2 → 0Rs3” samples were included in the study. Among 179 miRs analysed, 5 miRs showed significative difference between 0Rs1 and 2Rs2, 3 miRs were over-expressed (miR-181a-5p, miR-339-3p, let-7f-5p) and 2 under-expressed (miR-505-3p, miR-2110). Only miR-181a-5p met significative difference between 2Rs2 and 0Rs3 showing a statistical significative rise/fall pattern. Serum miR-181a-5p ROC analysis demonstrated significant discrimination between biopsy proven ACR from no-ACR biopsy, AUC=0.692 (p=0.0335). Differentially expressed miRs Mean relative expression 2(–ΔCq) (95% CI) Adjusted p-value (ANOVA) AUC (95% CI) 0Rs1 2Rs2 0Rs3 0Rs1 vs 2Rs2 2Rs2 vs 0Rs3 0Rs1 vs 0Rs3 miR-181–5p 0.80 (0.60–1.02) 1.11 (0.90–1.31) 0.86 (0.71–1.01) 0.0194 0.0225 NS 0.692 (0.529–0,855) Relative Expression 2(–ΔCq) and AUC expressed as mean (95% Confident Interval). NS: not significative. Conclusion Our findings suggest miR-181a-5p as a novel serological biomarker for detecting ACR after HT. After this preliminary discovery phase, miR-181a-5p is currently in a new validation phase to demonstrate its diagnostic performance. Acknowledgement/Funding This work was supported by a grant from isciii (PI15/02224) and it is part of the of the “CIBERCV”. Co-financed with FEDER Funds

Cardiac allograft vasculopathy (CAV) is a complex disease of the vasculature of the grafted heart. It is the major cause of graft loss and mortality 1 year posttransplantation. Imaging techniques are the main tools for detecting and classifying CAV. The disease typically involves the epicardial coronary arteries and spreads into the intramyocardial branches. Pathologic features are heterogeneous and include concentric proliferative intimal lesions and vascular inflammation, which can be associated with conventional atherosclerotic plaques. The involvement of the small arteries and the microcirculation, the microvasculopathy, is responsible for structural and functional changes. Among the multiple risk factors associated with CAV, antibody-mediated rejection is currently recognized as the principle.

Background The aim of the study was to analyze the mechanism of deterioration of implanted arteries. Methods Eleven patients were included. Samples of vascular segments obtained from multiorgan donors and samples of the same vascular segments after explantation in the recipient were analyzed. Blood group, time of cold and warm ischemia, cause of death, time spent in the intensive care unit, time of storage of the cryopreserved grafts, and anatomopathological and immunohistochemical studies were analyzed using the preimplant samples obtained from the multiorgan donor. For samples obtained from the recipient, blood group, duration for which the tissue from the donor has been implanted, reason for graft explantation, and anatomopathological and immunohistochemical studies were analyzed. Results Histopathologically, the main finding has been the substitution of the muscular cap of the arterial wall by an intense fibrosis, in most of the cases, of a symmetrical nature. Besides this degeneration of myocytes, there is marked perivascular fibrosis and fibrointimal thickening also exists. The T lymphocytes suggest the importance of the immunological mechanism in the distortion of the architecture of the arteries. The atherosclerosis plays a less relevant role. Conclusions Evidence of immune-mediated injury was found, and this mechanism seems to be responsible for the degenerative process in cryopreserved homografts.

Fetal rhabdomyoma is a benign neoplasm of rhabdomyoblasts that is found in both children and adults. Although there is often a predilection for the head and neck region, these tumors have been identified in several anatomic sites. A primary frontonasal presentation, however, has not been described yet. We report the first case of a fetal rhabdomyoma arising in the frontonasal region of a 3-year-old girl. This rare form of presentation is very interesting because of the need for a differential diagnosis with other entities. The clinician must keep in mind other possibilities typical of frontonasal region in childhood like nasal dermal sinus, anterior cephalocele and nasal glioma. For the pathologist, the most important reason to recognize and diagnose fetal rhabdomyoma in its various forms and locations is to avoid confusion with his malignant counterpart, the rhabdomyosarcoma.

Purpose: To assess the effects of myxomavirus-derived immunomodulatory proteins (Serp-1 and M-T7), on circulating mononuclear blood cells (PBMC) isolated from cardiac transplant patients. Methods and Materials: PBMC were isolated from 21 orthotopic heart transplant patients, 16 without and 5 with documented allograft vasculopathy, ages18 66 years old; 1 to 204 months post heart transplantation; all receiving standard immunosuppression medications. Cell activation was assessed by adhesion assay with calcein cell labeling. Cell isolates were segregated into treatment groups: (1)saline for baseline control, (2)Dimethyl sulfoxide (DMSO) for vehicle control, (3)cell activation with phorbol myristate acetate (PMA), (4)Serp-1 with PMA and (5) M-T7 with PMA. The inhibitory effect of Serp-1 and M-T7 on cell activation was measured based on changes in the adherent cell counts quantified by a spectrofluorometer. Statistical analysis by ANOVA was then performed. Results: PMA treatment was unable to further activate cells from patients with diagnosed allograft vasculopathy suggesting chronic elevated activation in circulating PBMC resistant to further activationan. These cells did not respond to Serp-1 or M-T7 treatment. Cells from patients without allograft vasculopathy conversely were activated by PMA (p 0.0001). M-T7 suppressed cell activity in the patients without vasculopathy despite standard immunosuppressive regimen (p 0.0001), thus indicating that patients on full anti-rejection therapy display further responses to antiinflammatory therapy despite full therapeutic regimens. Conclusions: Patients with known cardiac allograft vasculopathy have ongoing chronic activation of circulating peripheral mononuclear cells, unresponsive to further activation or anti-inflammatory treatment. M-T7, a unique virus derived chemokine modulator, significantly suppressed cell activation in patients without vasculopathy, suggesting potential for further treatment despite current optimal medical immunosuppression.