Hughes syndrome: The discovery of the antiphospholipid syndrome.

In the 40 years since the original detailed description of antiphospholipid syndrome (APS), the condition has come to be regarded as one of the most common autoimmune diseases. The impact of the description has been enormous - for example, the recognition that some individuals with connective tissue diseases require anticoagulation rather than corticosteroids or anti-inflammatory treatment has bought about fundamental change in medical practice. In obstetrics, APS is now regarded as the most important prothrombotic cause of recurrent pregnancy loss - with pregnancy success improving from below 20% to current live birth rate over 80%. In neurology, APS may be associated with up to 20% of strokes in people under 40 - a striking figure not least in terms of medical economics, let alone in potentially preventable suffering. In vascular medicine, APS links immunology with thrombosis and vascular disease and may well provide insights into immunological factors in the pathogenesis of atherosclerosis.

Thrombotic antiphospholipid syndrome: From guidelines to clinical management.

Thrombotic manifestations, mainly venous thromboembolism (VTE) and stroke, are the most common and potentially life-threatening presentations of antiphospholipid syndrome (APS). The management of APS requires the assessment of the antiphospholipid antibodies (aPL) profile, of concurrent systemic lupus erythematosus or other systemic autoimmune diseases and the presence of risk factors for cardiovascular disease and bleeding. Anticoagulation with vitamin K antagonists (VKA) remains the cornerstone of therapy for thrombotic APS. As platelets play a central role in APS, low-dose aspirin is the first option for primary thromboprophylaxis in asymptomatic aPL carriers, and also plays a role as combination therapy with VKAs in arterial thrombosis. Treatment with direct oral anticoagulants (DOACs) could be considered in certain low-risk situations, although they are not recommended in patients with arterial thrombosis or triple positive aPL. Adjuvant therapies such as hydroxychloroquine and statins may be useful in complex settings such as thrombotic recurrences or high risk of bleeding. In this article, we review the evidence and the recommendations of the guidelines for the treatment of APS, and provide a critical and practical approach of its management from our clinical perspective.

Anti-Xa activity below range is related to thrombosis in patients with severe COVID-19.

OBJECTIVE: We aimed to anlayse the relationship between anti-Xa activity below range and thomboembolic events. DESIGN: Single center prospective observational longitudinal cohort study (February-November 2021). SETTING: Patients admitted to the ICU of a University Hospital. PARTICIPANTS: Patients with severe COVID-19 pneumoniae. INTERVENTIONS: Enoxaparin was used for prophylactic and therapeutic anticoagulation. Enoxaparin dosing and dose adjustment were based on anti-Xa activity according to the hospital protocol. MAIN VARIABLES OF INTEREST: Target: thomboembolic events. PREDICTORS: demographics, pharmacotherapy, anti-Xa measurements, clinical data, and laboratory results. Logistic regression was used to identify independent risk factors for thomboembolic events. RESULTS: Data were available for 896 serum anti-Xa measurements from 228 subjects. Overall, 71.9% were male, with a median age of 62. Most patients needed invasive mechanical ventilation (87.7%) and mortality was 24.1%. A total of 28.9% new thomboembolic events were diagnosed. There were 27.1% anti-Xa measesurements below range. When multivariable logistic regression analysis was performed anti-Xa activity below range (RR, 4.2; p = 0.000), C-reactive protein (25 mg/L increase) (RR, 1.14; p = 0.005) and D-dimer (1000 ng/L increase) (RR, 1.06; p = 0.002) were the independent factors related to new thomboembolic events in patients with severe COVID-19. CONCLUSIONS: Anti-Xa activity below range, C-reactive protein and D-dimer were the independent factors related to thomboembolic events in patients with severe COVID-19. Purposely designed clinical trials should be carried out to confirm the benefit of an anti-Xa monitoring.

Bariatric and general surgical procedures in obese patients with a history of venous thromboembolism and concurrent anticoagulation therapy.

OBJECTIVE: The objective of this study was to examine the use and outcomes of perioperative anticoagulation (AC) in obese patients with a known history of venous thromboembolism event (VTE). METHOD: A retrospective review of a prospective database for patients with a VTE history undergoing bariatric and general surgery at a single center (1/2008-12/2017) was performed. Factors assessed included demographics, surgical details, and outcomes. RESULTS: Sixty-five patients underwent 76 procedures: 46 females (71%); mean age 51 years (range 26-73), mean weight 284 pounds (range 110-558), mean body mass index 45 (range 19-87). Comorbidities include hypertension (60%), gastroesophageal reflux disease (54%), osteoarthritis (49%), obstructive sleep apnea (45%), and diabetes (37%). Operations: 22 general surgeries (29%), 20 sleeve gastrectomies (26%), 12 revisions/conversions (16%), 12 Roux-en-Y gastric bypasses (16%), and 10 gastric bands (13%). Modalities: 67% laparoscopic, 28% robotic, and 5% open. Twenty-two patients (34%) had a pre-operative inferior vena cava filter placed with no complications. The mean length of stay was 4.4 days (range 1-31). Complications: seven 30-day readmissions (9%), one 30-day reoperation (1%), and two 90-day VTEs (3%). Thirty-day readmissions: four for inability to tolerate PO, two for small bowel obstruction, and one for symptomatic anastomotic ulcer. CONCLUSIONS: In our patients, post-operative AC could be started without an increased risk of bleeding in patients with a history of VTE undergoing bariatric surgery.

OBJETIVO: Examinar el uso y los resultados de la anticoagulación perioperatoria en pacientes bariátricos con antecedentes de tromboembolia venosa (TEV). MÉTODO: Revisión retrospectiva (base de datos prospectiva) de pacientes sometidos a cirugía general y bariátrica (1/2008-12/2017). Se evaluaron datos demográficos, detalles quirúrgicos y resultados. RESULTADOS: Sesenta y cinco pacientes se sometieron a 76 procedimientos: 46 mujeres (71%), edad media 51 años (rango: 26-73), peso medio 284 libras (rango: 110-558), índice de masa corporal medio 45 (rango: 19-87). Comorbilidad: hipertensión (60%), enfermedad por reflujo gastroesofágico (54%), osteoartritis (49%), apnea obstructiva del sueño (45%), diabetes (37%). Operaciones: 22 cirugía general (29%), 20 gastrectomías en manga (26%), 12 revisiones/conversiones (16%), 12 Y-de-Roux (16%), 10 bandas gástricas (13%). Modalidades: 67% laparoscópica, 28% robótica, 5% abierta. A 22 pacientes (34%) se les colocó un filtro de vena cava inferior preoperatorio sin complicaciones. La estancia media fue de 4.4 días (rango: 1-31). Complicaciones: 7 reingresos a los 30 días (9%), 1 reoperación a los 30 días (1%), 2 TEV a los 90 días (3%). Reingresos a los 30 días: 4 por incapacidad para tolerar la vía oral, 2 obstrucciones de intestino delgado y 1 úlcera anastomótica sintomática. CONCLUSIONES: En nuestros casos, la anticoagulación posoperatoria pudo iniciarse sin aumento del riesgo de sangrado en pacientes con antecedentes de TVE sometidos a cirugía bariátrica.

Incidence of venous thromboembolic disease and risk of bleeding in critically ill patients with hematologic malignancies: A retrospective study.

OBJECTIVE: Our objectives were to describe the use of thromboprophylaxis and the incidence of VTE/bleeding in critically ill patients with hematologic malignancies (HM). DESIGN: Retrospective cohort study (2014-2022). SETTING: Medic-Surgical Intensive Care Unit (ICU) in a tertiary care academic center. PATIENTS: Adult patients admitted to ICU with a concomitant diagnosis of a hematological malignancy. INTERVENTIONS: None. MAIN VARIABLES OF INTEREST: We analyzed demographic data, use of thromboprophylaxis and secondary outcomes that included incidence of VTE (venous thromboembolism), bleeding, mortality, severity scores and organ support. We applied a multivariable logistic regression model to examine the risk of thrombosis in the ICU. RESULTS: We included 862 ICU admissions (813 unique patients). Thromboprophylaxis was given during 65% of admissions (LMWH 14%, UFH 8%, and SCDs 43%); in 21% it was contraindicated due to thrombocytopenia; 14% of cases lacked documentation on prophylaxis. There were 38 unique incident cases of VTE (27 DVT, 11 PE), constituting 4.4% of ICU episodes. Most of VTE cases happened in patients with various degrees of thrombocytopenia. In the multivariable analysis, SOFA score on the first ICU day was independently associated (OR 0.85, 95% CI 0.76-0.96) with the risk of VTE. Bleeding occurred in 7.2% (minor) and 14.4% (major) of episodes; most frequent sites being CNS, abdomen/GI and pulmonary. CONCLUSIONS: In this cohort of critically ill patients with HM, there was considerable variability in the utilization of DVT prophylaxis, with predominant use of SCDs. The incidence of VTE was 4.4% and major bleeding 14%. CLINICAL TRIAL REGISTRATION: NCT05396157. Venous Thromboembolism in Hematologic Malignancy and Hematopoietic Cell Transplant Patients: a Retrospective Study (https://clinicaltrials.gov/).

Thrombotic Event Prevention in Patients Undergoing Posterior Lumbar Arthrodesis: our experiencie. A retrospective case series study. / Prevención de eventos trombóticos en pacientes sometidos a cirugía de artrodesis lumbar por vía posterior: experiencia en nuestro centro. Estudio retrospectivo de casos.

INTRODUCTION: There is no generalized consensus regarding perioperative prophylaxis of venous thromboembolism (VTE), either on using or timing it in patients undergoing spine surgery. VTE is a current concern because, even though being an uncommon event, it can cause serious complications. The aim of the present study is to propose guidelines for the prevention of thrombotic events in posterior spinal surgery, either as deep vein thrombosis or pulmonary thromboembolism. If the number of patients getting prophylaxis drugs is reduced a subsequent reduction of the incidence of epidural hematoma can be expected. MATERIALS AND METHODS: A number of 235 patients who had undergone posterior spinal arthrodesis in the previous five years were studied. Mechanical thromboprophylaxis measures consisting of compression stockings were applied in all of them. Anticoagulant drugs were also applied whenever risk factors for thrombosis were observed. Early weight-bearing was resumed immediately after surgery. Demographic, clinical, and surgical variables were collected, as well as complications appearing during the follow-up period, that was scheduled at one, two, four, six and twelve months after the surgery. Thrombotic events, if present, were diagnosed by clinical and imaging tests such as ultrasound and CT angiography. RESULTS: From the total 235 patients of this series, one hundred and fifty-three cases met the study inclusion criteria. A total of four thrombotic events appeared, one in the form of deep vein thrombosis and other three in the form of pulmonary thromboembolism. These last patients suffering an embolism died because of it. None of the variables studied had statistical significance for the occurrence of a thrombotic event. All four patients who suffered thrombotic events were receiving anticoagulant drugs, in addition to mechanical compression stockings, because of the presence of risk factors for thrombosis. CONCLUSIONS: By applying the fore mentioned protocol, adequate prevention of thromboembolic events was achieved in this study population of patients undergoing posterior spinal surgery.

Incremento del riesgo hemorrágico en pacientes con fibrilación auricular y enfermedad renal crónica en hemodiálisis tratados con anticoagulantes. Serie de cuatro centros / Increased risk of bleeding in patients with atrial fibrillation and chronic kidney disease on hemodialysis treated with anticoagulants. A four center series

Resumen Objetivo: El objetivo de este estudio fue estimar si el uso de anticoagulantes se asociaba con una diferencia en la frecuencia de trombosis de cualquier sitio, hemorragia mayor y mortalidad en adultos con coexistencia de ambas patologías. Método: Se realizó un estudio de cohorte retrospectivo en cuatro centros de alta complejidad. Se incluyeron mayores de 18 años con ERC en hemodiálisis y FA no valvular, con indicación de anticoagulación (CHA2DS2VASc ≥ 2). El desenlace primario fue la ocurrencia de sangrado mayor, evento trombótico (accidente vascular cerebral, infarto agudo al miocardio o enfermedad tromboembólica venosa) o muerte. Se realizó ajuste por variables de confusión por regresión logística. Resultados: De los 158 pacientes incluidos, el 61% (n = 97) recibieron anticoagulante. El desenlace principal se encontró en el 84% de quienes recibieron anticoagulación y en el 70% de quienes no la recibieron (OR: 2.12, IC95%: 0.98-4.57; luego del ajuste OR: 2.13, IC95%: 1.04-4.36). De los desenlaces mayores se presentaron sangrado en el 52% vs. el 34% (OR: 2.03; IC95%: 1.05-3.93), trombosis en el 35% vs. el 34% (OR: 1.03; IC95%: 0.52-2.01) y muerte en el 46% vs. el 41% (OR: 1.25; IC95%: 0.65-2.38). Conclusiones: Los resultados de este estudio sugieren un incremento en el riesgo de sangrado en los pacientes con FA y ERC en hemodiálisis que reciben anticoagulación, sin disminución del riesgo de eventos trombóticos ni de muerte.

Abstract Objective: The aim of this study was to estimate whether the consumption of anticoagulants was associated with a difference in the frequency of thrombosis of any site, major bleeding and mortality, in adults with both diseases. Method: A retrospective cohort study was carried out in four high complexity centers. Patients older than 18 years with CKD on hemodialysis and non-valvular AF, with an indication for anticoagulation (CHA2DS2VASc ≥ 2), were included. The primary outcome was the occurrence of: major bleeding, thrombotic event (cerebrovascular accident, acute myocardial infarction or venous thromboembolic disease) or death. Adjustment for confounding variables was performed using logistic regression. Results: From 158 patients included, 61% (n = 97) received an anticoagulant. The main outcome was found in 84% of those who received anticoagulation and 70% of those who did not (OR: 2.12, 95%CI: 0.98-4.57; after the adjusted analysis OR: 2.13, 95%CI: 1.04-4.36). Separate outcomes were bleeding in 52% vs. 34% (OR: 2.03; 95%CI: 1.05-3.93), thrombosis in 35% vs. 34% (OR: 1.03; 95%CI: 0.52-2-01) and death in 46% vs. 41% (OR: 1.25; 95%CI: 0.65-2.38). Conclusions: The results of this study suggest an increased risk of bleeding in patients with AF and CKD on hemodialysis receiving anticoagulation, without a decrease in the risk of thrombotic events or all-cause mortality.

Primary hyperparathyroidism in pregnancy: a case report highlighting uncommon complication / Hiperparatiroidismo primario en el embarazo: reporte de caso destacando una complicación infrecuente

Abstract Primary hyperparathyroidism (PHPT) is characterized by elevated levels of calcium and parathyroid hormone (PTH). However, the interpretation of diagnostic tests, such as serum calcium and PTH levels, is complex in pregnant women. The aim of this report is to present a case of PHTP in a pregnant adolescent, with a special emphasis on an uncommon complication, as well as diagnostic and treatment strategies. A 17-year-old pregnant female presented with hyper emesis gravidarum and neurological symptoms, leading to the diagnosis of cerebral venous thrombosis. Further investigations revealed hypercalcemia and persistently elevated PTH levels, consistent with PHPT. After local ization studies, the patient underwent an emergency parathyroidectomy with a diagnosis of parathyroid ad enoma. During follow-up, intrauterine growth restric tion and severe preeclampsia developed, necessitating an emergency cesarean section. Both the mother and neonate had favorable outcomes. PHPT is an infrequent condition in the pregnant population, and its diagnosis can be challenging due to the overlap of symptoms with normal physiological changes during pregnancy. The occurrence of uncom mon complications, such as thrombotic phenomena, highlights the need for a comprehensive approach to ensure early detection and management. In most cases, parathyroidectomy is the treatment of choice.

Resumen El hiperparatiroidismo primario (HPTP) se caracteriza por niveles elevados de calcio y hormona paratiroidea (PTH). Sin embargo, la interpretación de pruebas diag nósticas, como los niveles de calcio sérico y PTH, es compleja en mujeres embarazadas. El objetivo de este re porte es presentar un caso de HPTP en una adolescente embarazada, con especial hincapié en una complicación infrecuente, así como en las estrategias diagnósticas y de tratamiento. Una mujer embarazada de 17 años presentó hiperé mesis gravídica y síntomas neurológicos, lo que llevó al diagnóstico de trombosis venosa cerebral. Posterio res investigaciones revelaron hipercalcemia y niveles persistentemente elevados de PTH, consistentes con HPTP. Tras la realización de estudios de localización, la paciente fue sometida a una paratiroidectomía de emergencia con diagnóstico de adenoma de paratiroi des. Durante el seguimiento, se desarrolló restricción del crecimiento intrauterino y preeclampsia grave, lo que resultó en la necesidad de realizar una cesárea de emergencia. Tanto la madre como el neonato evolucio naron favorablemente. El HPTP es una condición infrecuente en la población embarazada y su diagnóstico puede ser desafiante por la superposición de síntomas con los cambios fisiológicos normales del embarazo. La aparición de complicaciones infrecuentes, como fenómenos trombóticos, resalta la necesidad de un abordaje integral para garantizar la detección y el manejo temprano. En la mayoría de los casos, la paratiroidectomía es el tratamiento de elección.

Trombosis espontánea de plexo pampiniforme: Reporte de un caso y revisión de literatura

Introducción: La trombosis de plexo pampiniforme es una entidad poco conocida, solo existen 31 casos reportados a la fecha. Objetivo: Presentación de un caso de una patología poco frecuente, descripción de las publicaciones anteriores y revisión de la literatura. Reporte de caso: Paciente de 20 años, sin antecedentes, con trombosis de plexo pampiniforme derecho. Sospecha inicial de hernia inguinal derecha complicada, el diagnóstico se realizó con ecografía Doppler y recibió tratamiento con anticoagulantes. Se complementó con tomografía y estudio de trombofilias. Discusión: El estudio y manejo ha sido variable, antes prevalecía la estrategia quirúrgica. Actualmente, algunos autores recomiendan el manejo conservador y otros proponen diferenciar el tratamiento según segmento anatómico comprometido. Conclusión: Al ser una entidad poco frecuente, el tratamiento es una decisión que debe ser tomada según cada caso, ya que no existen pautas de tratamiento, pero la tendencia es cada vez mayor hacia el tratamiento conservador. El rol de la anticoagulación está por definir. Es importante realizar un adecuado diagnóstico diferencial y búsqueda de causas subyacentes.

Introduction: Pampiniform plexus thrombosis is a rare entity, with only 31 cases reported to date. Aim: Presentation of a case of a rare disease, description of previous publications and review of the literature. Case report: 20-year-old patient, with no history, with right pampiniform plexus thrombosis. Initial suspicion was complicated right inguinal hernia, diagnosis was made with Doppler ultrasound and treatment with anticoagulation. It was complemented with tomography and study of thrombophilias. Discussion: The study and management has been variable, where the surgical strategy prevailed. Currently some authors recommend conservative management and others differentiate the treatment according to the compromised anatomical segment. Conclusion: Being a rare entity, conservative treatment or surgery continues to be a decision that must be made according to each case since there are no treatment guidelines, but the trend is increasing towards conservative treatment. The role of anticoagulation remains to be defined. It is important to carry out an adequate differential diagnosis and search for underlying causes.

El papel del estetrol en la anticoncepción: una revisión integral

Esta revisión narrativa explora el papel potencial del estetrol (E4), un esteroide estrogénico natural, en la anticoncepción, analizando sus propiedades farmacológicas, su efectividad y su seguridad. Se revisaron estudios preclínicos, ensayos clínicos y evaluaciones de seguridad del E4 como anticonceptivo oral combinado (AOC). Se investigó el impacto en parámetros endocrinos, metabólicos y hemostáticos, así como su tolerabilidad. En los resultados, el E4 tiene menor afinidad por el receptor de estrógeno-α de membrana, pero mantiene la actividad agonista en los receptores nucleares. E4/DRSP (drospirenona) demostró ser un AOC eficaz, con ciclos de sangrado regulares y predecibles en la mayoría de las mujeres. La tolerabilidad fue favorable, con eventos adversos leves o moderados y bajas tasas de interrupción. El sangrado fue el evento adverso más común, y se reportaron casos raros de migrañas con aura, trombosis venosa profunda, hiperpotasemia y depresión. E4/DRSP tuvo mínimo impacto en los parámetros lipídicos, hepáticos, de globulina fijadora de hormonas sexuales y de metabolismo de hidratos de carbono, y efecto neutral o mínimo en los parámetros hemostáticos. Se concluye que E4/DRSP parece ser una opción anticonceptiva eficaz y segura, con reducido riesgo trombótico y mínimo impacto en los parámetros endocrinos y metabólicos. Se requiere más investigación para confirmar su seguridad y eficacia a largo plazo.

This narrative review explores the potential role of estetrol (E4), a natural estrogenic steroid, in contraception, analyzing its pharmacological properties, effectiveness, and safety. Preclinical studies, clinical trials, and safety assessments of E4/DRSP (drospirenone) as a combined oral contraceptive (COC) were reviewed. The impact on endocrine, metabolic, and hemostatic parameters, as well as tolerability, was investigated. In results, E4 exhibits lower affinity for estrogen transmembrane receptor-α but maintains agonistic activity on nuclear receptors. E4/DRSP proved to be an effective COC with regular and predictable bleeding cycles in most women. Tolerability was favorable with mild or moderate adverse events and low discontinuation rates. Bleeding was the most common adverse event, and rare cases of aura migraines, deep vein thrombosis, hyperkalemia, and depression were reported. E4/DRSP had minimal impact on lipid, hepatic, sex hormone-binding globulin, and carbohydrate metabolism parameters, with a neutral or minimal effect on hemostatic parameters. The conclusion is that E4/DRSP seems to be an effective and safe contraceptive option, with reduced thrombotic risk and minimal impact on endocrine and metabolic parameters. Further research is needed to confirm long-term safety and efficacy.

Utilización de escalas no invasivas en la detección de varices esofágicas en pacientes con trombosis venosa portal / Use of non-invasive scales for detecting esophageal varices in paediatric patients with portal vein thrombosis

Introducción La trombosis portal (TVP) es la causa más frecuente de hipertensión portal en población pediátrica. El Consenso de Baveno VI considera la ligadura endoscópica de varices como segunda opción terapéutica tras el meso-Rex-bypass (shunt quirúrgico). Objetivo Analizar la rentabilidad diagnóstica de escalas no invasivas para predecir el riesgo de varices esofágicas en niños con TVP. Material y métodos Estudio descriptivo retrospectivo donde se incluyeron endoscopias digestivas altas (EDA) en pacientes<15 años con TVP no cirróticos. Se dividieron según la presencia de varices esofágicas y se estudiaron sexo, etiología, edad, hemorragia digestiva o tratamientos previos, resultados de EDA y las escalas (Regla Predicción Clínica-CPR, Regla Predicción Varices-VPR, King's Variceal Prediction Score-K-VaPS y ratio plaquetas/bazo-RPB). Las variables cualitativas se expresaron mediante frecuencia absoluta y porcentaje, y las cuantitativas mediante mediana y rango intercuartílico. Para las comparaciones se emplearon los test U de Mann-Whitney y Hanley-McNeil. Resultados Se realizaron 45 EDA. Un 80%(n=36) presentaron varices esofágicas: mediana de 3(2 – 3) y un 33,3%(n=12) precisó ligadura endoscópica de varices. Se demostraron diferencias estadísticamente significativas entre ambos grupos: CPR (142,39 [132,22 - 166,53] vs. 122,75 [115,24 – 133,15] p=0,003), VPR (9,91 [9,36 – 11,75] vs. 5,6 [3,34 – 8,39] p=0,001), K-VaPS (117,86 [99,66 - 126,58] vs. 99,64 [94,88 - 110,18] p=0,019), RPB (2384,62 [1902,22 - 3201,63] vs. 1252,5 [579,6 - 2144,42] p=0,05), con un área bajo la curva>75%, sin demostrarse diferencias entre escalas. Conclusiones En pacientes pediátricos con TVP no cirróticos se pueden emplear escalas no invasivas como herramienta para predecir la presencia de VE y plantear con ello la indicación de EDA. (AU)

Introduction Portal vein thrombosis (PVT) is the most frequent cause of portal hypertension in paediatric population. Baveno VI Consensus considers endoscopic variceal ligation as the second therapeutic option after meso-Rex bypass (surgical shunt). Aim Analyse the diagnostic profitability of non-invasive scales in order to predict the risk of oesophageal varices (OV) in children with PVT. Material and methods Descriptive retrospective study where every upper gastrointestinal endoscopy (UGE) carried on patients <15 years old with non-cirrhotic PVT were included. There were divided according to the presence of OV and sex, cause, age, previous gastrointestinal bleeding or treatments, results of UGE and scales (Clinical Prediction Rule – CPR), Varices Prediction Rule – VPR), King's Variceal Prediction Score – K-VaPS) and Platelet count/Spleen diameter Ratio – PSR). Qualitative variables were expressed as absolute frequency and percentage, and quantitative variables as median and interquartile range. U Mann–Whitney and Hanley–McNeil tests were used for comparisons. Results Forty-five UGE were analysed. 80% (n=36) presented OV: median of 3 (2–3) and 33.3% (n=12) required endoscopic variceal ligation. Statistical differences were demonstrated between both groups: CPR (142.39 [132.22-166.53] vs. 122.75 [115.24-133.15]; p=0.003), VPR (9.91 [9.36-11.75] vs. 5.6 [3.34-8.39]; p=0.001), K-VaPS (117.86 [99.66-126.58] vs. 99.64 [94.88-10.18]; p=0.019), PSR (2384.62 [1902.22-3201.63] vs. 1252.5 [579.6-2144.42]; p=0.05), with and area under the curve AUROC>75%, without statistical differences between scales. ConclusionsIn paediatric patients with non-cirrotic PVT non-invasive scales can be used as a tool to predict the presence of OV and raise the indication of UGE. (AU)

Disasters that develop in the liver due to hydatid cyst: a case report.

Hilar cavernous transformation is the formation of venous structures rich in collateral around the portal vein. Portal vein thrombosis is a rare entity. Although there are many reasons for its etiology, few cases have been reported secondary to hydatid cysts in the liver. Here, we present a 24-year-old patient with complaints of abdominal pain and swelling. Her CT and MRI scans show cholelithiasis with portal vein thrombosis and hilar cavernous transformation due to giant hydatid cyst compression in the lateral liver sector.

La transformación cavernosa hiliar es la formación de estructuras venosas ricas en colaterales alrededor de la vena porta. La trombosis de la vena porta es una afección poco frecuente. Aunque existen muchas razones en su etiología, se han descrito pocos casos secundarios a quiste hidatídico en el hígado. Aquí se presenta el caso de una paciente de 24 años con quejas de dolor abdominal e hinchazón. La tomografía computarizada y la resonancia magnética mostraron colelitiasis con trombosis de la vena porta y transformación cavernosa hiliar por compresión del quiste hidatídico gigante en el sector lateral del hígado.

Proteomic biomarkers for noninvasive left atrial appendage thrombus prediction in patients with atrial fibrillation.

INTRODUCTION AND OBJECTIVES: The CHA2DS2-VASc score, used to assess the risk of left atrial appendage thrombus (LAAT) formation in patients with atrial fibrillation (AF), has limited predictive value. Moreover, transesophageal echocardiography imaging, the gold standard diagnostic method to identify thrombi, is semi-invasive. Consequently, there is a need for alternative and noninvasive diagnostic methods for LAAT risk assessment. METHODS: Deep proteomic analysis was conducted in plasma samples from 8 patients with nonvalvular AF, divided into thrombus and control groups (4 patients in each group) based on the presence or absence of LAAT. Biomarkers associated with LAAT were validated using an enzyme-linked immunosorbent assay in a cohort of 179 patients with available clinical, transthoracic, and transesophageal echocardiography data. Predictive models were developed to assess the improvement in LAAT identification. RESULTS: The LAAT group had higher CHA2DS2-VASc scores, larger LA diameter, and lower LAA flow velocities. Deep proteomic analysis identified 30 differentially expressed proteins, including myosin light chain 4, prenylcysteine oxidase 1 (PCYOX1), and decorin as potential diagnostic biomarkers of LAAT. The model showed that PCYOX1 and decorin provided an area under the curve (AUC) of 0.970 for LAAT prediction compared with 0.672 in a model including the CHA2DS2-VASc score and LAA cauliflower morphology. The incremental value of proteomic biomarkers for LAAT in patients with nonvalvular AF was further confirmed with the net reclassification improvement and integrated discrimination improvement indices. CONCLUSIONS: Protein levels of PCYOX1 and decorin improve the predictive performance for LAAT in patients with nonvalvular AF.

Manejo de la trombosis venosa cerebral en España: estudio descriptivo MOTIVATE / Management of cerebral venous thrombosis in Spain: MOTIVATE descriptive study

Introducción: La trombosis venosa cerebral (TVC) es una causa poco común de ictus que afecta principalmente a adultos jóvenes. Un diagnóstico precoz y preciso puede reducir la tasa y gravedad de las complicaciones. Objetivo: Analizar las características clínicas, manejo y tratamiento de la TVC en diferentes centros de nuestro país. Métodos: Estudio descriptivo retrospectivo multicéntrico de pacientes hospitalizados por TVC entre 2008 y 2017 en 11 centros sanitarios en nuestro país. Resultados: Se incluyeron 256 pacientes, edad media 49,8 ± 18,7 años y el 51% fueron mujeres. Los síntomas más frecuentes fueron: cefalea (73%), déficits focales (50%), crisis epilépticas (33%) y encefalopatía (21%). Las localizaciones más frecuentes fueron: seno longitudinal superior (12,5%), transverso (10,9%) y afectación de dos o más senos o venas (66,4%). La etiología conocida más frecuente fue la trombofilia (24%), siendo la mutación de la protrombina G20210A la más común (19%). El 46% fue tratado con antitrombóticos durante 3-6 meses, el 21% durante un año y un 22,6% de los pacientes requirieron anticoagulación indefinida. En un 5% de los sujetos fue preciso terapia endovascular y un 33% requirió neurocirugía. En relación al pronóstico, el 75% fueron independientes a los 3 meses con una puntuación en la escala de Rankin modificada (mRS) ≤ 2 y la presencia de papiledema (p = 0,03), déficit focal (p = 0,001) y encefalopatía (p < 0,001) se relacionaron significativamente con mal pronóstico (mRS > 3). La tasa de mortalidad intrahospitalaria fue del 4,3% y el 6,3% de los pacientes fallecieron a los 3 meses. Conclusión:La diversidad de factores de riesgo y la presentación variable suponen un desafío en el diagnóstico y tratamiento de la TVC. Para mejorar el pronóstico y reducir la mortalidad es fundamental la instauración de protocolos en el manejo de esta patología.(AU)

Introduction: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults. Early, accurate diagnosis can reduce the rate and severity of complications. Objective: The aim of this study was to analyse the clinical characteristics, management, and treatment of CVT in different centres in Spain. Methods: We conducted a multicentre, retrospective, descriptive study of patients hospitalised due to CVT between 2008 and 2017 at 11 Spanish centres. Results: We included 256 patients, with a mean age (SD) of 49.8 (18.7) years; 51% of patients were women. The most frequent symptoms were headache (73%), focal deficits (50%), epileptic seizures (33%), and encephalopathy (21%). The most frequent localisations were the superior sagittal sinus (12.5%), the transverse sinus (10.9%), and 2 or more sinuses or veins (66.4%). Thrombophilia was the most frequent known aetiology (24%), and was most commonly associated with the prothrombin G20210A mutation (19%). Forty-six percent of patients were treated with antithrombotics for 3-6 months, 21% for one year, and 22.6% required indefinite anticoagulation. Endovascular therapy was performed in 5% of cases, and 33% required neurosurgery. Regarding outcomes, 75% of patients were independent at 3 months (modified Rankin Scale [mRS] score ≤ 2), with papilloedema (P = .03), focal deficits (P = .001), and encephalopathy (P < .001) showing a statistically significant association with poor prognosis (mRS > 3). The in-hospital mortality rate was 4.3%, with a 3-month mortality rate of 6.3%. Conclusion: The diverse risk factors and variable presentation of CVT represent a challenge in the diagnosis and treatment of this condition. To improve prognosis and reduce mortality, it is essential to establish management protocols for this entity.(AU)

Trombosis venosa cerebral / Cerebral venous thrombosis

La trombosis venosa cerebral forma parte de las llamadas trombosis en sitios inusuales. Se define como una oclusión en el territorio venoso cerebral. Su incidencia se encuentra en aumento progresivo, especialmente en países en vías de desarrollo. Se observa con más frecuencia en mujeres jóvenes, siendo los factores hormonales, como el embarazo o la anticoncepción hormonal, factores de riesgo principales en el desarrollo de esta afección. La clínica va a depender fundamentalmente de la topografía de la trombosis, con un diagnóstico de confirmación basado principalmente en las pruebas de imagen. El tratamiento consiste generalmente en la anticoagulación, pudiendo plantearse otras opciones según la gravedad del cuadro. En general, el pronóstico es mejor que el de otros trastornos vasculares intracraneales. En esta revisión se describe la evidencia actual disponible acerca de la trombosis venosa cerebral.(AU)

Cerebral venous thrombosis is part of the so-called thrombosis in unusual sites. It is defined as an occlusion in the cerebral venous territory. Its incidence is progressively increasing, especially in developing countries. It is more frequently observed in young women, with hormonal factors such as pregnancy or hormonal contraception being significant risk factors in the development of this condition. The clinical presentation will depend fundamentally on the topography of the thrombosis, with a confirmatory diagnosis based mainly on imaging tests. The treatment generally consists of anticoagulation, and other options may be considered depending on the severity of the case. Overall, the prognosis is better than that of other intracranial vascular disorders. This review describes the current evidence available regarding cerebral venous thrombosis.(AU)

Recomendaciones SER sobre el tratamiento del síndrome de Behçet refractario / SER recommendations on treatment of refractory Behçet's syndrome

Objetivo: Elaborar recomendaciones multidisciplinares, basadas en la evidencia disponible y el consenso de expertos, para el manejo terapéutico de los pacientes con síndrome de Behçet refractario (difícil de tratar, resistente grave, recidivante grave) al tratamiento convencional. Métodos: Un panel de expertos identificó preguntas clínicas de investigación relevantes para el objetivo del documento. Estas preguntas fueron reformuladas en formato PICO –paciente, intervención, comparación, outcome o desenlace–. A continuación, se realizaron revisiones sistemáticas; la evaluación de la calidad de la evidencia se realizó siguiendo la metodología del grupo internacional de trabajo Grading of Recommendations, Assessment, Development, and Evaluation. Tras esto, el panel multidisciplinar formuló las recomendaciones. Resultados: Se seleccionaron 4 preguntas PICO relativas a la eficacia y seguridad de los tratamientos farmacológicos sistémicos en los pacientes con síndrome de Behçet con manifestaciones clínicas refractarias a terapia convencional, relacionadas con los fenotipos mucocutáneo y/o articular, vascular, neurológico-parenquimatoso y gastrointestinal. Se formularon un total de 7 recomendaciones estructuradas por pregunta, con base en la evidencia encontrada y el consenso de expertos. Conclusiones: El tratamiento de las manifestaciones clínicas más graves del síndrome de Behçet carece de evidencia científica sólida y no existen documentos de recomendaciones específicas para los pacientes con enfermedad refractaria a la terapia convencional. Con el fin de aportar una respuesta a esta necesidad, se presenta el primer documento de recomendaciones de la Sociedad Española de Reumatología específicas para el abordaje terapéutico de estos pacientes, que servirá de ayuda en la toma de decisiones clínica y la reducción de la variabilidad en la atención.(AU)

Objective: To develop multidisciplinary recommendations based on available evidence and expert consensus for the therapeutic management of patients with refractory Behçet's syndrome (difficult to treat, severe resistant, severe relapse) to conventional treatment. Methods: A group of experts identified clinical research questions relevant to the objective of the document. These questions were reformulated in PICO format –patient, intervention, comparison and outcome–. Systematic reviews of the evidence were conducted; the quality of the evidence was evaluated following the methodology of the international working group Grading of Recommendations, Assessment, Development, and Evaluation. After that, the multidisciplinary panel formulated the specific recommendations. Results: Four PICO questions were selected regarding the efficacy and safety of systemic pharmacological treatments in patients with Behçet's syndrome with clinical manifestations refractory to conventional therapy related to mucocutaneous and/or articular, vascular, neurological parenchymal and gastrointestinal phenotypes. A total of 7 recommendations were made, structured by question, based on the identified evidence and expert consensus. Conclusions: The treatment of most severe clinical manifestations of Behçet's syndrome lacks solid scientific evidence and, besides, there are no specific recommendation documents for patients with refractory disease. With the aim of providing a response to this need, here we present the first official recommendations of the Spanish Society of Rheumatology for the management of these patients. They are devised as a tool for assistance in clinical decision making, therapeutic homogenisation and to reduce variability in the care of these patients.(AU)

Diagnosis and treatment of deep vein thrombosis of the lower and upper limbs. 2024 recommendations of the venous thromboembolism group of the Spanish Society of Internal Medicine.

Deep vein thrombosis (DVT) of the limbs is a common disease and causes significant morbidity and mortality. It is frequently the prelude to pulmonary embolism (PE), it can recur in 30% of patients and in 25-40% of cases they can develop post-thrombotic syndrome (PTS), with a significant impact in functional status and quality of life. This document contains the recommendations on the diagnosis and treatment of acute DVT from the Thromboembolic Disease group of the Spanish Society of Internal Medicine (SEMI). PE and thrombosis of unusual venous territories (cerebral, renal, mesenteric, superficial, etc.) are outside its scope, as well as thrombosis associated with catheter and thrombosis associated with cancer, which due to their peculiarities will be the subject of other positioning documents of the Thromboembolic Disease group of the Spanish Society of Internal Medicine (SEMI).

SER recommendations on treatment of refractory Behçet's syndrome.

OBJECTIVE: To develop multidisciplinary recommendations based on available evidence and expert consensus for the therapeutic management of patients with refractory Behçet's syndrome (BS) (difficult to treat, severe resistant, severe relapse) to conventional treatment. METHODS: A group of experts identified clinical research questions relevant to the objective of the document. These questions were reformulated in PICO format (patient, intervention, comparison and outcome). Systematic reviews of the evidence were conducted, the quality of the evidence was evaluated following the methodology of the international working group Grading of Recommendations Assessment, Development, and Evaluation (GRADE). After that, the multidisciplinary panel formulated the specific recommendations. RESULTS: 4 PICO questions were selected regarding the efficacy and safety of systemic pharmacological treatments in patients with BS with clinical manifestations refractory to conventional therapy related to mucocutaneous and/or articular, vascular, neurological parenchymal and gastrointestinal phenotypes. A total of 7 recommendations were made, structured by question, based on the identified evidence and expert consensus. CONCLUSIONS: The treatment of most severe clinical manifestations of BS lacks solid scientific evidence and, besides, there are no specific recommendation documents for patients with refractory disease. With the aim of providing a response to this need, here we present the first official Recommendations of the Spanish Society of Rheumatology for the management of these patients. They are devised as a tool for assistance in clinical decision making, therapeutic homogenisation and to reduce variability in the care of these patients.

Guidelines for the management of coagulation disorders in patients with cirrhosis.

Coagulation management in the patient with cirrhosis has undergone a significant transformation since the beginning of this century, with the concept of a rebalancing between procoagulant and anticoagulant factors. The paradigm that patients with cirrhosis have a greater bleeding tendency has changed, as a result of this rebalancing. In addition, it has brought to light the presence of complications related to thrombotic events in this group of patients. These guidelines detail aspects related to pathophysiologic mechanisms that intervene in the maintenance of hemostasis in the patient with cirrhosis, the relevance of portal hypertension, mechanical factors for the development of bleeding, modifications in the hepatic synthesis of coagulation factors, and the changes in the reticuloendothelial system in acute hepatic decompensation and acute-on-chronic liver failure. They address new aspects related to the hemorrhagic complications in patients with cirrhosis, considering the risk for bleeding during diagnostic or therapeutic procedures, as well as the usefulness of different tools for diagnosing coagulation and recommendations on the pharmacologic treatment and blood-product transfusion in the context of hemorrhage. These guidelines also update the knowledge regarding hypercoagulability in the patient with cirrhosis, as well as the efficacy and safety of treatment with the different anticoagulation regimens. Lastly, they provide recommendations on coagulation management in the context of acute-on-chronic liver failure, acute liver decompensation, and specific aspects related to the patient undergoing liver transplantation.

Target sign in COVID-19, radiological interpretation and diagnostic contribution of digital thoracic tomosynthesis.

INTRODUCTION: Our objectives are: To describe the radiological semiology, clinical-analytical features and prognosis related to the target sign (TS) in COVID-19. To determine whether digital thoracic tomosynthesis (DTT) improves the diagnostic ability of radiography. MATERIAL AND METHODS: Retrospective, descriptive, single-centre, case series study, accepted by our ethical committee. Radiological, clinical, analytical and follow-up characteristics of patients with COVID-19 and TS on radiography and DTT between November 2020 and January 2021 were analysed. RESULTS: Eleven TS were collected in 7 patients, median age 35 years, 57% male. All TS presented with a central nodule and a peripheral ring, and in at least 82%, the lung in between was of normal density. All TS were located in peripheral, basal regions and 91% in posterior regions. TS were multiple in 43%. Contiguous TS shared the peripheral ring. Other findings related to pneumonia were associated in 86% of patients. DTT detected 82% more TS than radiography. Only one patient underwent a CT angiography of the pulmonary arteries, positive for acute pulmonary thromboembolism. Seventy-one per cent presented with pleuritic pain. No distinctive laboratory findings or prognostic worsening were detected. CONCLUSIONS: TS in COVID-19 predominates in peripheral and declining regions and can be multiple. Pulmonary thromboembolism was detected in one case. It occurs in young people, frequently with pleuritic pain and does not worsen the prognosis. DTT detects more than 80 % of TS than radiography.