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L M Nieto-Benito, Beatriz Berenguer-Fröhner, Verónica Parra-Blanco, Minia Campos-Domínguez. Rev Chil Pediatr 2020 Feb;91(1):99-104. Epub 2019 Dec 3. DOI: 10.32641/rchped.v91i1.1303.  Abstract Introduction: Bednar tumor is a rare low-grade sarcoma considered the pigmented variant of dermatofibrosarco ma protuberans (DFSP). Objective: To describe the clinical and histopathological characteristics, treatment and evolution of this rare neoplasm. Clinical case: A 9-year old female presented with a 2-year history of an indurated, asymptomatic papule on the back of her fourth left toe. The incisio nal biopsy was compatible with pigmented DFSP. The immunohistochemical study showed intense positivity for CD34 throughout the lesion, with negative factor XIIIa. We complemented the study with molecular… Leer más »Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case.

Angela Hernández-Martín, Rebeca Kennedy-Batalla, Elvira Cañedo, Esther Bernaldo-de-Quirós, Begoña Carazo-Gallego, Angel Vera, Antonio Torrelo, Lucero Noguera-Morel, Rogelio González-Sarmiento, Marieke Bolling, Marta Martínez-Bonet, Marjorie Pion, Rafael Correa-Rocha. N Engl J Med  2019 Nov 28;381(22):2176-2178. DOI: 10.1056/NEJMc1908531 The SAM syndrome, which is characterized by severe dermatitis, multiple allergies, and metabolic wasting (Online Mendelian Inheritance in Man number, 615508), is a congenital skin disease caused by mutations in the genes encoding either desmoglein-1 (DSG1) or desmoplakin (DSP). These mutations result in reduced epidermal integrity, which may be lethal in children because of recurrent sepsis and extreme undernourishment. The immunopathogenic mechanisms remain uncertain, and no treatments have been available. A 9-month-old girl was referred to our practice… Leer más »Imbalance in T-Helper 17 Cells and Targeted Therapy in an Infant with SAM-like Syndrome.

Carolina Gutiérrez, Jacobo Lopez-Abente, Verónica Pérez-Fernández, Adrián Prieto-Sánchez, Rafael Correa-Rocha, Santiago Moreno-Guillen, María-Ángeles Muñoz-Fernández, Marjorie Pion. PLoS One 2019 Mar 27;14(3):e0213744. eCollection 2019. DOI: 10.1371/journal.pone.0213744 Abstract This study examines the relationship between regulatory B (Breg) and T (Treg) compartments, which play crucial roles in the maintenance of immune homeostasis in the context of HIV. Using flow cytometry, the phenotypes of different Breg and Treg subsets from HIV-infected and healthy individuals were analyzed, along with the suppressive capacity of Breg. Peripheral blood samples of thirteen HIV+ treatment-naïve individuals, fourteen treated-HIV+ individuals with undetectable viral load and twelve healthy individuals were analyzed. The absolute counts of Breg and Treg subsets… Leer más »Analysis of the dysregulation between regulatory B and T cells (Breg and Treg) in human immunodeficiency virus (HIV)-infected patients.

Jacobo López-Abente, Esther Bernaldo-de-Quirós, Manuela Camino, Nuria Gil, Esther Panadero, Minia Campos-Domínguez, Elena Seoane-Reula, Juan M Gil-Jaurena, Marjorie Pion, Rafael Correa-Rocha. Am J Transplant 2019 May;19(5):1536-1544. Epub 2019 Jan 25. DOI: 10.1111/ajt.15245 Abstract Atopic dermatitis (AD) has a high incidence in heart-transplant children, and the reason why there is more AD after transplantation is still unknown. We conducted a cross-sectional study comparing 11 AD and 11 non-AD age-matched heart-transplant children, to assess which immune alterations are related to AD in these patients. AD patients had been transplanted at a younger age compared to non-AD, indicating that age at transplant may be determinant in the onset of AD. The earlier thymectomy… Leer más »Immune dysregulation and Th2 polarization are associated with atopic dermatitis in heart-transplant children: A delicate balance between risk of rejection or atopic symptoms.

Jacobo Lopez-Abente, Adrián Prieto-Sanchez, Maria-Ángeles Muñoz-Fernandez, Rafael Correa-Rocha, Marjorie Pion. Cell Mol Immunol. 2018 Oct;15(10):917-933. Epub 2017 Jul 17.  DOI: 10.1038/cmi.2017.48.  Abstract Individuals infected with human immunodeficiency virus type-1 (HIV-1) usually show a general dysregulation and hyper-activation of the immune system. A direct influence of HIV-1 particles on B-cell phenotypes and functions has been previously described. However, the consequences of B-cell dysregulation are still poorly understood. We evaluated the phenotypic changes in primary B cells after direct contact with HIV-1 particles in comparison with different types of stimuli. The functionality of treated B cells was challenged in co-culture experiments with autologous CD4+ and CD8+ T… Leer más »Human immunodeficiency virus type-1 induces a regulatory B cell-like phenotype in vitro.

Rafael Correa-Rocha, Jacobo Lopez-Abente, Carolina Gutierrez, Verónica Astrid Pérez-Fernández, Adrián Prieto-Sánchez, Santiago Moreno-Guillen, María-Ángeles Muñoz-Fernández, Marjorie Pion. PLoS One 2018 Aug 30;13(8):e0203419. eCollection 2018. DOI: 10.1371/journal.pone.0203419.  Abstract In our work, we analyzed the role of the CD100/CD72 and PD-1/PD-L1 axes in immune response dysfunction in human immunodeficiency virus (HIV)-1 infection in which high expressions of PD-1 and PD-L1 were associated with an immunosuppressive state via limitation of the HIV-1-specific T-cell responses. CD100 was demonstrated to play a relevant role in immune responses in various pathological processes and may be responsible for immune dysregulation during HIV-1 infection. We investigated the function of CD72/CD100, and PD-1/PDL-1 axes on T… Leer más »CD72/CD100 and PD-1/PD-L1 markers are increased on T and B cells in HIV-1+ viremic individuals, and CD72/CD100 axis is correlated with T-cell exhaustion.

de Quiros Plaza, Esther Bernaldo; López-Abente, Jacobo; Camino, Manuela; Gil, Nuria; Panadero, Esther; Campos, Minia; Seoane, Elena; Pion, Marjorie; Correa-Rocha, Rafael. Transplantation: July 2018 – Volume 102 – Issue – p S143 DOI: 10.1097/01.tp.0000542766.40479.8a Abstract Introduction  Heart transplantation is a successful strategy for the treatment of end-stage pediatric heart diseases. In recent years, improved therapies and clinical practice have led to increased graft survival, and consequently, chronic complications secondary to organ transplantation due in part to long-term immunosuppression have also emerged. Allergies and atopic diseases, such as atopic dermatitis (AD) are among these chronic complications. Despite that AD constitutes a frequent… Leer más »Th1/Th2 Imbalance and Early Age to Transplantation is Associated to Atopic Dermatitis in Heart-Transplanted Pediatric Patients.

Esther Bernaldo de Quirós, Manuela Camino, Nuria Gil, Esther Panadero, Constancio Medrano, Juan Miguel Gil-Jaurena, Marjorie Pion, Giovanna Lombardi, Megan K Levings, Lori J West, Rafael Correa-Rocha. Transplantation: July 2018 – Volume 102 – Issue – p S205 DOI: 10.1097/01.tp.0000542859.38902.af Abstract Immune allograft rejection remains the main obstacle to reach successful transplants. Transfer of regulatory T cells (Treg) has acquired growing interest in attempts to prevent rejection. However, the limited number and the differentiated phenotype of Tregs isolated from blood constitute important drawbacks for the effectiveness of this strategy. In collaboration with several teams, we have explored the use of the… Leer más »“First-In-Human” Clinical Trial Employing Adoptive Transfer of Autologous Thymus-Derived Treg Cells (thyTreg) to Prevent Graft Rejection in Heart-Transplanted Children.

Jorge Gallego-Valle, Verónica Astrid Pérez-Fernández, Rafael Correa-Rocha, Marjorie Pion. Int J Mol Sci 2018 Jun 12;19(6):1737. DOI: 10.3390/ijms19061737 Abstract Regulatory B cells (Bregs) participate in auto-tolerance maintenance and immune homeostasis. Despite their impact on many diseases and due to the difficulty to define them, knowledge about their origin and their physiological inducers is still unclear. The incomplete understanding about the generation of Bregs and their limited numbers in periphery make it difficult to develop Breg-based therapy. Therefore, identifying factors that promote their development would allow their ex-vivo production in order to create new immunotherapy. This project aims to test the capacity of several cytokines… Leer más »Generation of Human Breg-Like Phenotype with Regulatory Function In Vitro with Bacteria-Derived Oligodeoxynucleotides.

E Bernaldo de Quiros, E Seoane-Reula, E Alonso-Lebrero, M Pion, R Correa-Rocha. Allergol Immunopathol (Madr)Nov-Dec 2018;46(6):612-618. Epub 2018 May 5. DOI: 10.1016/j.aller.2018.02.002 Abstract Food allergy is a pathological immune reaction that identifies certain harmless food proteins, usually tolerated by the majority of the people, as a threat. The prevalence of these food allergies is increasing worldwide and currently affects 8% of children. Exacerbated reactions to milk, egg and peanut are the most frequent in the pediatric population. It is well known that allergic diseases are a type 2 T-helper (Th2) immune response, characterized by the elevated production of IgE antibodies. However, little is known about… Leer más »The role of regulatory T cells in the acquisition of tolerance to food allergens in children.