Serum levels of S-100 protein are directly proportional to the size, number, thickness and degree of cellularity of congenital melanocytic nevi

To the Editor: Some patients with congenital melanocytic nevi (CMN) present progressive growth and thickening, extracutaneous involvement (neurocutaneous melanocytosis, NCM) or neoplastic transformation (melanoma); and others remain stable or even regress. There are no markers to assess progression or follow-up. Recently, we found S-100, a protein which acts on cell differentiation and proliferation, elevated in CMN.1 S-100 is a ligand of the RAGE pathway (related to the MAPK-pathway), and low serum levels of soluble-RAGE were related to poor survival in melanoma.2 Also SOX10, expressed in melanocytes with high specificity, is useful in detection, prognosis and treatment assessment of melanoma.3 We explored if S-100, RAGE and SOX10 serum levels vary in children’s CMN and assessed clinical or pathological correlations

Sirolimus Early Treatment in Vascular Anomalies Leads to a Better Response

Objectives:. Sirolimus has become a relevant drug in the treatment of vascular anomalies, initially relegated to rescue therapy for refractory lesions but more recently has become first line. Our objective is to determine when it is best to start sirolimus treatment. Methods:. A retrospective review of patients with vascular anomalies treated with sirolimus during a 9-year period was performed. The variables analyzed included subtype of vascular anomaly, age at the start of treatment, sirolimus dosage and levels, response, and duration of treatment among others. Results:. One hundred twenty-two patients were included and classified according to ISSVA in: tumor (7), lymphatic malformation (60), venous malformation (16), combined malformations (18), associated overgrowth (8), and others (15). Overall response was positive in 90.8%. Median duration of treatment was 32 months (0–116). Response was not influenced by dosage or levels, neither by the subtype of vascular anomaly, location, extension, symptoms, genetic testing nor previous treatments. However, the lower the age of starting sirolimus, the better the response, mainly under 5 years of age (P = 0.004). At 6 months, 67% of patients had responded while at 12 months >84% did. By age, patients 5 months from older patients. Time until a positive response was not influenced by subtype or severity. Conclusion:. Overall response to sirolimus was good and most patients responded irrespective of their severity in location, extension or symptoms. Patients <5 years old respond better and faster making our goal to attempt for an early treatment

Exhibition Catalog - UCM Tree Ecosystems. Painting as a learning service for tree ecosystems

Responsable del proyecto: Paloma Peláez Bravo Coordinadores de la exposición: Paloma Peláez Bravo María Dolores Fernández Martínez Soraya Triana Hernández Joaquín Millán Rodríguez Laura Ríos Villar Juan Antonio Tinte Moreno María Lourdes Castro Cerón. Diseño de Catálogo: Soraya Triana Hernández Fotografías: Sandra Malvar Tortajada; Lidia Fernández Martínez Diseño de Cartelería: Roberto Herrero García; Gabriel Jímenez Sánchez-TiradoEl catálogo de la exposición Aedes arboribus ecosystem-UCM.La pintura como aprendizaje servicio de los sistemas arbóreos", tiene como objetivo mostrar una pequeña selección de los estudios realizados por los alumnos del grado en Bellas Artes que han estado vinculados desde sus asignaturas correspondientes al proyecto Innova 137: “La pintura como instrumento aprendizaje-servicio de los ecosistemas arbóreos en la Avd. Complutense”. Así mismo recoge la reflexión teórica de los docentes e investigadores participantes. Responsable del proyecto y edición del vídeo Paloma Peláez Bravo.The catalog of the exhibition "Aedes arboribus ecosystem-UCM. Painting as a learning service for tree ecosystems", aims to show a small selection of the studies carried out by the students of the Fine Arts degree that have been linked from their subjects corresponding to the Innova 137 project: “Painting as a learning instrument service of the arboreal ecosystems in Avd. Complutense ”. It also collects the theoretical reflection of the participating teachers and researchers. Responsible for the project and editing of the video Paloma Peláez Bravo.Depto. de Pintura y Conservación-RestauraciónFac. de Bellas ArtesFALSEpu

Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan-1 study

The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D-dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation. Methods: With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan-1 was determined by ELISA. In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan-1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium. For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.Sin financiación3.2 Q2 JCR 20220.992 Q1 SJR 2023No data IDR 2022UE

Successful Sirolimus Treatment for Recurrent Pericardial Effusion in a Large Cervico-Mediastinal Provisionally Unclassified Vascular Anomaly: Case Report

Provisionally unclassified vascular anomalies (PUVA) are a group of diseases with unique characteristics that make them unclassifiable within vascular tumors or malformations. We describe a PUVA as the cause of recurrent pericardial effusion and its response to sirolimus. A 6-year-old girl was referred with a cervico-thoracic vascular anomaly, a violaceous and irregular lesion in the neck and upper chest, diagnosed as "hemangioma". She had pericardial effusion at the neonatal age that required pericardiocentesis, propranolol and corticosteroids. She remained stable for 5 years, when she presented with a severe pericardial effusion. A magnetic resonance visualized a diffuse vascular image in the cervical and thoracic region with mediastinal extension. The pathological study showed a vascular proliferation in the dermis and hypodermis with positive staining for WT1 and negative for Glut-1. Genetic testing found a variant in GNA14, for which the diagnosis of PUVA was established. When a pericardial drain was placed without response, treatment with sirolimus was started with resolution of the effusion. Sixteen months later, the malformation is stable and there has been no recurrence of pericardial effusion. In a significant group of patients, definitive diagnosis is not possible despite pathological and genetic analysis. mTOR inhibitors may become a therapeutic option if symptoms are severe enough, with a low rate of reported side effects. New Insights and the Importance for the Pediatric Surgeon In patients with vascular anomalies in whom it is not possible to reach a diagnosis despite multiple tests, a safe and effective option is to try mTOR inhibitors if symptoms are severe enough, considering the low rate of reported side effects with their use

Serum levels of S-100 protein are directly proportional to the size, number, thickness and degree of cellularity of congenital melanocytic nevi

To the Editor: Some patients with congenital melanocytic nevi (CMN) present progressive growth and thickening, extracutaneous involvement (neurocutaneous melanocytosis, NCM) or neoplastic transformation (melanoma); and others remain stable or even regress. There are no markers to assess progression or follow-up. Recently, we found S-100, a protein which acts on cell differentiation and proliferation, elevated in CMN.1 S-100 is a ligand of the RAGE pathway (related to the MAPK-pathway), and low serum levels of soluble-RAGE were related to poor survival in melanoma.2 Also SOX10, expressed in melanocytes with high specificity, is useful in detection, prognosis and treatment assessment of melanoma.3 We explored if S-100, RAGE and SOX10 serum levels vary in children’s CMN and assessed clinical or pathological correlations