The Kinetics of Early T and B Cell Immune Recovery after Bone Marrow Transplantation in RAG-2-Deficient SCID Patients

The kinetics of T and B cell immune recovery after bone marrow transplantation (BMT) is affected by many pre- and post-transplant factors. Because of the profoundly depleted baseline T and B cell immunity in recombination activating gene 2 (RAG-2)-deficient severe combined immunodeficiency (SCID) patients, some of these factors are eliminated, and the immune recovery after BMT can then be clearly assessed. This process was followed in ten SCID patients in parallel to their associated transplant-related complications. Early peripheral presence of T and B cells was observed in 8 and 4 patients, respectively. The latter correlated with pre-transplant conditioning therapy. Cells from these patients carried mainly signal joint DNA episomes, indicative of newly derived B and T cells. They were present before the normalization of the T cell receptor (TCR) and the B cell receptor (BCR) repertoire. Early presentation of the ordered TCR gene rearrangements after BMT occurred simultaneously, but this pattern was heterogeneous over time, suggesting different and individual thymic recovery processes. Our findings early after transplant could suggest the long-term patients' clinical outcome. Early peripheral presence of newly produced B and T lymphocytes from their production and maturation sites after BMT suggests donor stem cell origin rather than peripheral expansion, and is indicative of successful outcome. Peripheral detection of TCR excision circles and kappa-deleting recombination excision circles in RAG-2-deficient SCID post-BMT are early markers of T and B cell reconstitution, and can be used to monitor outcome and tailor specific therapy for patients undergoing BMT

Vampires in the village Žrnovo on the island of Korčula: following an archival document from the 18th century

Središnja tema rada usmjerena je na raščlambu spisa pohranjenog u Državnom arhivu u Mlecima (fond: Capi del Consiglio de’ Dieci: Lettere di Rettori e di altre cariche) koji se odnosi na događaj iz 1748. godine u korčulanskom selu Žrnovo, kada su mještani – vjerujući da su se pojavili vampiri – oskvrnuli nekoliko mjesnih grobova. U radu se podrobno iznose osnovni podaci iz spisa te rečeni događaj analizira u širem društvenom kontekstu i prate se lokalna vjerovanja.The main interest of this essay is the analysis of the document from the State Archive in Venice (file: Capi del Consiglio de’ Dieci: Lettere di Rettori e di altre cariche) which is connected with the episode from 1748 when the inhabitants of the village Žrnove on the island of Korčula in Croatia opened tombs on the local cemetery in the fear of the vampires treating. This essay try to show some social circumstances connected with this event as well as a local vernacular tradition concerning superstitions

Acurácia da avaliação clínica da disfagia orofaríngea na encefalopatia crônica não progressiva

Objetivo: analisar a acurácia da avaliação clínica da disfagia orofaríngea para detectar penetração e aspiração laringotraqueal na encefalopatia crônica não progressiva. Métodos: participaram deste estudo 45 indivíduos com ECNP e disfagia orofaríngea, sendo 28 do sexo masculino e 17 do sexo feminino, faixa etária variando de 3 a 19 anos. A avaliação clínica da deglutição utilizou protocolo específico e a videofluoroscopia de deglutição (VFD) foi utilizada como padrão ouro. Resultados: verificou-se que houve sensibilidade de 80,0% (IC 95%: [82,7;100]), especificidade de 46,67% (IC 95%: [18,1;75,3]), valor preditivo positivo de 77,78% (IC 95%: [62,8;92,8]) e valor preditivo negativo de 77,78% (IC 95%: [45,1;100]). Conclusão: constatou-se que a avaliação fonoaudiológica clínica da disfagia orofaríngea na ECNP apresenta maior sensibilidade que especificidade.Universidade Estadual Paulista ?Júlio de Mesquita Filho?Hospital Estadual Bauru Serviço de DisfagiaUniversidade Federal de Santa CatarinaUNESP Departamento de FonoaudiologiaUniversidade Estadual Paulista ?Júlio de Mesquita Filho?UNESP Departamento de Fonoaudiologi