Autoantibodies are biomarkers for autoimmune disease diagnosis, monitoring, and prediction. Therefore, this study established the frequency of latent and overt polyautoimmunity in children and adolescents with >6 months of diagnosis of immune thrombocytopenia (ITP). Forty-seven patients with chronic or persistent disease had non–organ-specific and organ-specific autoantibodies assessed. Frequency of latent polyautoimmunity was 36.2%, and, of overt polyautoimmunity, it was 4.3%. Of ITP patients with latent polyautoimmunity, 52.9% were positive for antinuclear antibody (ANA), 47.1% for autoantibodies other than ANA, and 64.7% for multiple autoantibodies. In addition, patients with latent polyautoimmunity and those positive for ANA were significantly older at disease onset. Both ITP patients positive and negative for autoantibodies reported family members with autoimmune diseases. The autoantibodies observed were as follows: ANA, anti-dsDNA, anti-SSA/Ro, IgM aCL, anti-GAD, anti-IA2, anti-IAA, anti-TG, anti-TPO, anti-LKM1, and SMA. Of ITP patients with overt polyautoimmunity, 1 was diagnosed with type 1 diabetes mellitus and the other with thyroiditis. In conclusion, children and adolescents with ITP present high frequency of latent and overt polyautoimmunity even for autoantibodies other than ANA. Therefore, ANA and other non–organ-specific and organ-specific autoantibodies should be considered for assessment during ITP patients’ follow-up.
Background Kidney transplantation is the gold standard treatment for children with end‐stage chronic kidney disease. Graft thrombosis is an important cause of graft failure, with high morbidity, mortality, and impact on quality of life and to the health system. The role of thromboprophylaxis in this setting is still uncertain. We describe the demographic characteristics and thrombotic risk factors in pediatric renal transplant recipients, determining the rate of renal graft thrombosis, and discuss the role of thromboprophylaxis. Methods This retrospective study reviewed 96 pediatric renal transplantations between 2008 and 2017 in a single hospital. Patients were assigned to one of two groups: children who did not receive thromboprophylaxis after transplantation and those who did. We reported their characteristics, comparing the incidence of graft thrombosis and hemorrhagic complications between the groups. Results Forty‐nine patients (51%) received thromboprophylaxis. Thrombosis occurred in 5 patients who did not receive thromboprophylaxis (5.2%) compared with none in the group that did (p = .025). In all patients, renal graft thrombosis resulted in early graft loss. Thirteen patients had hemorrhagic complications. Seven were unrelated to pharmacological thromboprophylaxis (2 major, 1 moderate, and 4 minor bleeding, which either did not receive thromboprophylaxis or had bleeding prior to thromboprophylaxis), while six occurred during heparinization (2 major, 1 moderate, and 3 minor bleeding). There was no significant difference in the rate of hemorrhagic complications between the groups (p = .105). Conclusions The rate of renal graft thrombosis was 5.2%. Thrombosis remains an important cause of early graft loss. Thromboprophylaxis was associated with a reduction in graft thrombosis without increased risk of bleeding.
Essential thrombocythemia is an acquired myeloproliferative disorder characterized by the proliferation of megakaryocytes in bone marrow, leading to a persistent increase in the number of circulating platelets and thus increasing the risk for thrombotic and hemorrhagic events. The disease features leukocytosis, splenomegaly, vascular occlusive events, hemorrhages and vasomotor disorders. The intricate mechanisms underlying the molecular pathogenesis of this disorder are not completely understood and are still a matter of discussion. Essential thrombocythemia is an extremely rare disorder during childhood. We report on a case of essential thrombocythemia in a child and discuss the diagnostic approach and treatment strategy.
Esta dissertação é dedicada: Ao meu amor Marcelo, marido, parceiro de vida, pelo incentivo e por acreditar mais em mim do que eu mesma, por todas as críticas construtivas durante essa jornada: do projeto, passando pelo artigo até a finalização da dissertação. Tenho certeza de que nada disso seria possível sem seu apoio incondicional.Às minhas filhas Laura e Cecília, razões do meu viver, do amor mais genuíno, meu estímulo diário a ser uma pessoa melhor.Muito orgulho da família que formamos. AGRADECIMENTO ESPECIALAo meu orientador Prof. Dr. Jorge David Aivazoglou Carneiro, pela confiança, paciência, disponibilidade e pela oportunidade de aprender hematologia pediátrica com uma pessoa tão dedicada e competente durante todos esses anos. Minha eterna gratidão e admiração. AGRADECIMENTOSAgradeço a Deus pela oportunidade da trajetória seguida até aqui, guiando e protegendo meu caminho. Aos pacientes e seus familiares que fizeram com que a realização desse estudo fosse possível, o motivo de querermos sempre aprender e melhorar. Agradeço aos meus pais, Josefina e João, que me ensinaram a ser uma pessoa correta, que apoiaram minhas decisões e que nunca mediram esforços para me dar uma boa educação. Ao meu irmão Henrique pela amizade. A minha sogra Anita, pelo incentivo e suporte. Vocês são a minha tão necessária rede de apoio. Às queridas amigas, Priscila Grizante-Lopes parceira na pós-graduação e na vida que sempre esteve na torcida e vibrou com cada conquista, e Daniele Martins Celeste, companheira e incentivadora. À toda equipe da Unidade de Hematologia Pediátrica e residentes do ITACI pelo acolhimento e aprendizado nesses anos de convívio. Aos membros da banca examinadora da qualificação, Dra. Vera Hermina Koch, Dr. Durval Damiani e Dr Ioannis Antonopoulos pelas críticas construtivas que tanto contribuíram. À Dra. Andrea Watanabe por disponibilizar meu acesso ao ambulatório de nefrologia pediátrica.Também sou grata à Monica Alves Souza e toda equipe da secretaria da pós-graduação, aos funcionários do SAME do ITACI, Mariza Kazue, Nivaldo e Rosangela, pela paciência, prontidão e pela estimada ajuda na execução e finalização desta dissertação.Esta dissertação está de acordo com as seguintes normas, em vigor no momento desta publicação:Referências: adaptado de International Committee of Medical Journals Editors (Vancouver).
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