Slide Session : OS-CAD-03 ; Cardiology : Spanish Registry of Autoimmune Congenital Heart Block in Babies of Mothers Carrying Anti-Ro/la Antibodies (Rebacc-Geas-Semi)

( Pilar Brito Zeron ),( Marta Perez De Lis Novo ),( Pilar Rosich ),( Carles Tolosa ),( Joaquim Oristrell ),( Luis Saez Comet ),( Mercedes Perez Conesa ),( Jose Antonio Vargas Hitos ),( Jose Mario Sabi 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyze the outcomes and therapeutic management of affected pregnancies with autoimmune congenital heart block (aCHB). Methods: Retrospective multicenter study in Internal Medicine Departments; inclusion criteria consisted of: aCHB of any type (I, II or III), fetal EFE and/or cardiomyopathy, cardiac block diagnosed in utero or in the first postpartum month, and mothers carrying anti-Ro52, Ro60 and/or La autoantibodies. Results: A total of 25 pregnancies with aCHB were retrospectively analyzed in 21 anti-Ro/La+ mothers. The mean maternal age at the time of pregnancy with aCHB was 33,25 years. Only 2 mothers received treatment prior to the first affected pregnancy (hydroxychloroquine and hydroxychloroquine+prednisone). Cardiac block consisted of type I (n=1), type II (n=6) and type III (n=18). At diagnosis of aCHB, 15/22 women were treated with dexamethasone (one of them, along with IVIG) and 2/22 with ritodrine. Preventive treatment with IVIG was administered in 2 pregnancies in which a recurrence was observed. Pregnancy was interrupted in 7/25 pregnancies at a mean week of 23.43 (18.2-37), while 18/25 of pregnancies gave a live birth baby with a mean age of 35.71 weeks of birth (30-40). 11 babies required pacemaker implantation, 9 immediately after birth and 2 in the neonatal period (1 at 5 years of age and another at 12). Of the 15 pregnancies with aCHB treated with dexamethasone, 12 achieved pregnancy to term (1 type II disappeared, with no changes in the remaining cases) and there were 3 fetal deaths despite treatment. Of the 7 pregnancies not treated with dexamethasone, 3 babies were born alive (no reversal of the blockade) and there were 4 fetal deaths. Conclusions: aCHB is a serious problem with a fetal mortality of 28% and a high requirement for neonatal pacemaker placement (61%).

Slide Session : OS-HEM-03 ; Hematology : Hemophagocytic Syndrome in Internal Medicine: Iden-tification of Infectious Triggers, Therapeutic Manage-ment and Mortality in 88 Patients (REGHEM-GEAS-SEMI)

( Pilar BRITO ZERÓN ),( Marta PEREZ DE LIS NOVO ),( Roberto PÉREZ ALVAREZ ),( Pedro MORAL MORAL ),( Aleida MARTÍNEZ ZAPICO ),( Guadalupe FRAILE ),( Eva FONSECA ),( María VAQUERO HERRERO ),( Angela RUI 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyze the therapeutic management and the main epidemiological and clinical characteristics related to survival in a large series of patients with hemophagocytic syndrome (HS) diagnosed in Departments of Internal Medicine. Methods: In June 2013, the Study Group of Autoimmune Diseases (GEAS-SEMI) creates a national registry of patients with HS. Patients were diagnosed according to the fulfillment of the criteria of the Histiocytosis Society in 1991 and updated in 2004. Results: At June 15, 2014, the REGHEM registry included 88 patients with HS, 35 (40%) men and 55 (60%) women, with a mean age at diagnosis of 49.16 years (range 12- 84 years). During the admission that led to the diagnosis of SH, acute infections were identified in 50 (57%) patients, including viruses (n=20), bacteria (n=13), mycobacteria (n=8) and parasites/fungi (n=13). Patients were treated with corticosteroids (n=40), etoposide (n=12), cyclosporin A (n=12), methotrexate (n=4), tacrolimus (n=2) rituximab (n=3), intravenous immunoglobulins (n=2) and chemotherapy (n=5). A total of 44 (50%) patients died. The main factors associated with mortality were analytical parameters at diagnosis such as the presence of severe anemia (Hb <9 g/L, 89% vs 66%, p=0.01), platelet count <100,000/mm3 (96% vs 64%, p<0.001), neutropenia <1000/mm3 (59% vs 39%, p=0.044), and bacterial infections as precipitating agent (77% vs 45%, p=0.034); a clinical presentation including lymphadenopathy was associated with a lower mortality (59% vs 39%, p=0.044). Conclusions: Hemophagocytic syndrome is a severe multisystemic disease associated in nearly 50% of cases with an acute infection, and usually requires vital support in intensive care units. Despite this and the use of a complex therapeutic approach, half of the patients died. The main prognostic factor identified is the presence at diagnosis of severe cytopenias and the coexistence of bacterial infections.

Freemartinism in replacement ewe-lambs of the Ripollesa sheep breed

Pilar Marí,Joaquim Casellas 대한수의학회 2018 Journal of Veterinary Science Vol.19 No.6

The freemartinism syndrome affects almost all female calves born as co-twins to male calves, whereas little is known about this phenomenon in female sheep. Within this context, 1,185 ewe-lambs from the Ripollesa sheep breed were genotyped for the presence of oY1 polymorphism (a non-autosomal region of the Y chromosome). Neither ewe-lambs from single births (856) nor ewe-lambs from all-female multiple births (170) were revealed as freemartins, whereas five of 159 ewe-lambs from multiple births with male co-twins were freemartins (3.15 ± 1.38%). All freemartin ewe-lambs were confirmed by physical examination of external genitalia. The results confirm a low incidence of freemartinism from heterosexual twin pregnancies in Ripollesa sheep.

Slide Session : OS-RHEU-08 ; Rheumatology : Minimally-Invasive Biopsy of Minor Salivary Glands in Internal Medicine: Searching for Systemic Infi ltrative Diseases

( Pilar Brito Zeron ),( Marta Perez De Lis Novo ),( Soledad Retamozo ),( Lluisa Alos ),( Albert Bove ),( Hoda Gheitasi ),( Belchin Kostov ),( Antoni Siso Almirall ),( Roberto Perez Alvarez ),( Manuel 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyze the safety and utility of minor lip biopsy by using the minimally invasive technique, performed in the Department of internal medicine, in patients presenting with immunonegative sicca syndrome. Methods: Prospective analysis of 122 patients (100 women, mean age 60 years) with sicca syndrome and negative anti-Ro/La antibodies in which minimally invasive biopsy of minor salivary glands was performed. Results: The most common histopathological feature consisted of lymphoplasmacytic infi ltration in 57 (47%) patients, followed by fi brosis in 46 (38%) and acinar atrophy in 32 (26%); the type of lymphoplasmacytic infi ltrate was detailed in 53 patients (21 lymphocytic, 20 plasmacytic, and 12 lymphoplasmacytic). According to the lymphocytic classifi cation of Chisholm and Mason, 53 (44%) were classifi ed as normal, 42 (35%) grade I, 11 (9%) grade II, 8 (7%) grade III and 7 (6 %) grade IV. Histopathologic diagnosis were: normal in 46 patients, non-specifi c sialadenitis in 42, primary Sjogren`s syndrome in 26 (15 as the fi nal diagnosis, 11 and probable), and other diagnoses in 6 (fat infi ltration in 5, systemic amyloidosis in 1); no cases of infi ltration by granulomas or IgG4+ were found. The presence of fi brosis was detected more frequently in women (43% vs 14%, p=0.014); the existence of fi brosis (67.70 vs 55.76 years) and acinar atrophy (69.50 vs 56.71 years) correlated with increasing age. No correlation between the degree of infi ltration and diagnostic or immunological features was found. Only 8% patients reported transient adverse events. Conclusions: In 21% of patients with an immunonegative sicca syndrome, a diagnosis of primary SS was confi rmed by minimally invasive biopsy, whereas 35% of patients disclosed a non-specifi c chronic sialadenitis, which could refl ect a “mild” SS that have no place in the current classifi cation criteria of this disease.

Slide Session : OS-RHEU-07 ; Rheumatology : Predicting Death in Patients with Primary Sjogren Syndrome: Prognostic Factors and Standardized Mortality Ratio in Comparison to the General Population (RESSPGEAS- SEMI)

( Pilar BRITO ZERÓN ),( Marta PEREZ DE LIS NOVO ),( Belchin KOSTOV ),( Roser SOLANS ),( Guadalupe FRAILE ),( Carlos SUÁREZ CUERVO ),( Arnau CASANOVAS ),( Francisco Javier RASCÓN ),( Rami QANNETA ),( R 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyze prognostic factors and standardized mortality ratio (SMR) with respect to the general population in a cohort of Spanish patients with primary Sjogren`s syndrome (SS). Methods: In October 2013, the RESSP-GEAS-SEMI database included 1045 consecutive patients who met the 2002 SS-criteria. Hazard ratios (HR) and confi dence intervals (95% CI) obtained in the adjusted regression model were calculated. The SMR was estimated using the life tables of the general population of Spain in 2012. Results: The cohort included 982 (94%) women with a mean age at diagnosis of 54 years and a mean disease evolution of 118 months; 115 (11%) patients died due to systemic disease (n=18), infection (n=21), cardiovascular disease (n=35), hematologic malignancy (n=10) and other causes (n=31). The SMR for the total cohort of patients (adjusted for age and sex with the general Spanish population) was 4.66. Survival rates at 5, 10, 20 and 30 years were 96.0%, 90.5%, 80.9% and 60.4%, respectively. The Cox-regression analysis identifi ed the following baseline variables at diagnosis associated with death: male gender (HR 2.98, p<0.001), altered parotid scintigraphy (HR 2.81, p=0.043), lymphopenia (HR 1.63, p=0.034), anti-La antibodies (HR 1.51, p=0.034), low C3 (HR 1.93, p=0.034), low C4 (HR 2.06, p=0.016), monoclonal gammopathy (HR 1.81, p=0.047) and cryoglobulins (HR 2.58, p<0.001). The main baseline factors associated with mortality caused by systemic disease were systemic-activity at diagnosis, cytopenias, monoclonal gammopathy, cryoglobulins, and hypocomplementemia. Conclusions: Primary SS should not be considered a mild disease, since mortality is almost 5 times greater with respect to general population, with an overall survival at 20 years of 81%. Patients with a lower survival are those who present with active disease at diagnosis and associated immunological markers of B-cell hyperactivity.

A Review of International Risk Sharing for Policy Analysis

Pilar Poncela,Michela Nardo,Filippo M. Pericoli 대외경제정책연구원 2019 East Asian Economic Review Vol.23 No.3

issues from the perspective of the European Union. The traditional analyses contemplate three risk-sharing channels: the capital markets channel (through cross border portfolio investments), international transfers and the credit markets channel (via savings). Comparative analyses reveal that, on average, about 80% of the shock remains unsmoothed in Europe while only about 18% of the shock is transmitted to consumers within the US. From aggregated figures, there is space for improving, particularly, the cross-border investments channel in Europe. In this sense, the completion of the Banking and Capital Markets Union are expected to boost risk sharing across European member states. We also review new additional issues usually not contemplated by the traditional literature as depreciation, migration and the role of sovereigns and two new additional channels recently considered in the literature: government consumption and the real exchange rate. Finally, we also examine recent analysis related to the geographic distribution of risk sharing.

Poster Session : PS 0640 ; Respiratory Medicine ; Infl uence of Epidemiological Profi le in the Clinical Expression of Sarcoidosis: Study of 160 Patients with Systemic Disease

( Pilar Brito Zeron ),( Marta Perez De Lis Novo ),( Roberto Perez Alvarez ),( Jacobo Sellares ),( Soledad Retamozo ),( Xavier Bosch ),( Fernanda Hernandez ),( Josep Manel Santos ),( Antoni Siso Almira 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyse the infi uence of the epidemiological profi le in the disease expression of a large series of patients with sarcoidosis. Methods: Study cohort including 160 patients diagnosed with sarcoidosis in the last 10 years. The diagnosis of sarcoidosis was based on clinical criteria and imaging studies, together with the histopathological demonstration of non-necrotizing granulomas, and excluding other granulomatous diseases, mainly infectious (tuberculosis). Results: There were 106 women and 54 men, with a mean age at diagnosis of 46.87 years. The main clinical manifestations present at diagnosis were respiratory symptoms (40%), followed by general symptoms (31%), skin lesions (29%) and the incidental fi nding of imaging fi ndings in asymptomatic patients (18%). 37% of patients had adisease onset <40 years. This early onset of disease was reported mainly in males (44% vs 28%, p=0.027) and was associated with a higher frequency of general symptoms (42% vs 25%, p=0.023), erythema nodosum (29% vs 16%, p=0.041), uveitis (14% vs. 5%, p=0.05) and joint involvement (17% vs 5%, p=0.014), but with a lower frequency of interstitial lung disease (25% vs 42%, p=0.028) and splenic involvement (0% vs 7%, p=0.037). With respect to the differential presentation of the disease according to gender, women diagnosed with sarcoidosis had a higher mean age at diagnosis (48.8 vs 42.8 years, p=0.017), a higher frequency of skin involvement (35% vs 13%, p=0.003) and a lower frequency of pulmonary (34% vs 52%, p=0.022), hepatosplenic (2% vs 9%, p=0.044) and nervous system (3% vs 13%, p=0.032) involvements in comparison with males. Conclusions: An early disease onset (<40 years) was mainly associated with general and cutaneous involvement, while sarcoidosis in men affects more frequently vital organs.

Slide Session : OS-RHEU-03 ; Rheumatology : Genetic Variability of The CD5 Lymphocytic Immunoreceptor and Clinical Expression of Systemic Autoimmune Diseases Related to B-Cell Hyperactivity

( Pilar Brito Zeron ),( Hoda Gheitasi ),( Marta Perez De Lis Novo ),( Noelia Armiger ),( Maria Jose Soto Cardenas ),( Myriam Gandia ),( Albert Bove ),( Xavier Bosch ),( Soledad Retamozo ),( Miriam Aka 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To investigate the mechanisms involved in lymphocyte activation mediated by the CD5 immunoreceptor in patients with systemic autoimmune diseases related to B-cell hyperactivity. Methods: A total of 508 patients were analyzed (273 primary Sjogren syndrome patients, 135 SLE patients, 47 SS associated HCV patients and 53 controls). CD5 genotyping receptor was analyzed by PCR-SBT technique with the detection of the SNP7 (rs2241002) andSNP26 (rs2229177). The homozygous haplotype CC/CC was classifi ed as inductor of autoimmunity, whereas the heterozygous haplotype CC/TT was a protective factor. Results: The haplotype CC/CC was found in 7.5% of controls vs 19% of patients with primary SS fulfi lling the 2002 criteria (p=0.047), 12% of SS-1993 criteria (p=0.39), 18% of SLE (p=0.10) and 21% of SS-HCV patients (p=0.049); the protective haplotype CC/TT was observed in 28% of controls vs 19% of patients with primary SS-2002 criteria (p=0.15), 13% of SS-1993 criteria (p=0.01), 15% of SLE (p=0.04) and 15% of SS-HCV patients (p=0.10). A specifi c analysis was carried out in patients with primary SS-2002 criteria comparing the clinical and immunological features of patients carrying protective haplotypes (n=33) and those carrying inductor haplotypes (n=33). A higher frequency of anti-La/SS-B antibodies (67% vs 39%, p=0.048) and a trend for an increased frequency of extraglandular involvement (52% vs 36%), neutropenia (33% vs 18%), ANA (91% vs 82%), FR (53% vs 48%) and low levels of C3 (12% vs 3%) was found in carriers of inductor haplotypes with respect to those carrying protective haplotypes. Conclusions: We found a higher frequency of the CC/CC haplotype (associated with increased autoimmunity) of the gene that encodes the immunoreceptor lymphocytic CD5 in SS patients, that was related to an increased extraglandular systemic activity, especially a higher frequency of anti-La/SS-B antibodies.

The reversal of women’s earnings equalizing effect: When and where do changes in women’s earnings increase inequality?

Pilar Gonalons-Pons 한국사회학회 2018 한국사회학회 심포지움 논문집 Vol.2018 No.5

Comorbidity Between Anorexia Nervosa and Depressive Disorder: A Narrative Review

Maria Pilar Calvo-Rivera,Maria Isabel Navarrete-Páez,Isabel Bodoano,Luis Gutiérrez-Rojas 대한신경정신의학회 2022 PSYCHIATRY INVESTIGATION Vol.19 No.3

Objective Anorexia nervosa (AN) is a disorder with a significantly high prevalence. Affective disorders, including depressive disorder (DD), often coexist with this eating disorder (ED). In this review we will focus on its prevalence, associated vulnerability factors, clinical manifestations, possible etiological factors and its prognosis.Methods A bibliographic search was carried out in the PubMed database selecting those articles that approached the subject of comorbidity between AN and DD. The search was limited to articles published from January 1990 to December 2021.Results Of the 1891 abstracts reviewed, 33 studies met inclusion criteria. The prevalence of this comorbidity was extremely variable between studies, which exposed their heterogeneity. As to symptomatology this comorbidity presents itself with more severity, greater expression of psychological traits and greater cognitive impairment. Certain personality traits are postulated as vulnerability factors. Genetic factors such as neurochemicals seem to be involved in its pathogenesis.Conclusion The comorbidity between DD and ED have important influence in its symptomatic expression, severity and prognosis. Some of the analyzed studies provide consistent data, but there are others that are contradictory. It would be necessary to increase the number of studies and use a unified methodology.