Analysis of the costs of the basic care line for sickle cell disease in Brazilian children under 5 years of age

ABSTRACT Objective: To evaluate and estimate the cost of basic care in sickle cell disease (SCD) for patients under five years of age, within the scope of the Unified Health System (SUS) and to discuss the costs related to possible complications of the disease from the literature. Methods: The main management and conduct recommendations in the SCD up to five years of age, with healthy and baseline health status, were extracted from the Basic Guidelines of the Care Line in the SCD of the Ministry of Health. Systematic data regarding costs of medicines were extracted from the Medicine Market Regulation Chamber. The SUS Table of Procedures, Medicines and Orthotics, Prosthetics and Auxiliary Means of Movement Management System was the guide for the values of complementary exams, as well as for medical consultations. The values applied to calculate the vaccination schedule were extracted from the Pan American Health Organization, adopting the perspective of the SUS-paying costs. Results: The total cost obtained for basic care of SCD in children up to five years of age, including the use of antibiotic prophylaxis, immunizations and the performance of transcranial Doppler ultrasound in the prevention and early detection of cerebrovascular accidents was, on average, $1020.96. Conclusion: The cost-effectiveness of prophylaxis in SCD, up to five years of age, exceeds the expenses resulting from hospitalizations due to complications of the disease. The study of expenses associated with SCD could be used to establish public policies, improve prevention strategies and treat the symptoms and complications of the disease.

Liga Acadêmica de Hematologia (HemoLiga): atuação no ensino, pesquisa e extensão / Academic League of Hematology (HemoLiga): performance in Teaching, Research and Extension*

Introdução: As Ligas Acadêmicas (LAs) são organizações universitárias muito presentes nos cursos de medicina no Brasil, são de caráter extracurricular e complementar, fundamentadas no tripé ensino, pesquisa e extensão (EPE). Suas atividades tendem a aprofundar os conhecimentos sobre determinado tema, estimular a criatividade, o autoaprendizado e o espírito crítico dos estudantes a fim de proporcionar uma melhor formação profissional. Objetivo: Relatar o histórico e a atuação da HemoLiga (HL) com foco na produção científica e compará-la com outras LAs de Medicina do Brasil descritas na literatura. Material e Métodos: Leitura e análise das atas de reuniões da HL para a documentação histórica da liga associada a busca dos Currículos Lattes dos atuais e ex-integrantes da HL para levantamento de dados sobre a produção científica da liga. As informações obtidas foram tabeladas em planilhas de acordo com os subtipos de trabalhos. Foram realizados análises e cruzamento de variáveis para confecção de gráficos. Resultados: A HL é uma liga vinculada a três faculdades de medicina e possui 14 anos de existência. Ela atua no ensino através de reuniões e estágios práticos na área de Hematologia. A pesquisa constitui sua principal atuação, integrando, inclusive, linha de pesquisa internacional. Assim, desde a sua fundação, a HL consta de uma lista de 217 trabalhos científicos produzidos, sendo 32 artigos científicos, 180 resumos, três capítulos de livros e dois livros. Na extensão, a liga agrega os projetos Unir para Cuidar, Doador do Futuro, Triagem Neonatal e Amigo de Sangue. Conclusão: A HL por meio do seu escopo de atuação, em consonância com outras LAs, contribui através de seu perfil de iniciação científica na formação acadêmica de seus membros, além de beneficiar a sociedade através de seus projetos de extensão.

Introduction: The Academic Leagues (ALs) are very present organizations in Medicine courses in Brazil. They are extracurricular and complementary activities which are based on tripod education, research, and extension, aiming to promote a more profound knowledge on a given topic. As a result, it stimulates creativity, self-learning, and critical thinking to provide better professional training. Objective: Report the history and performance of HemoLiga (HL), focusing on scientific production and compare it with other Brazilian Medicine ALs presented in the literature. Material and Methods: Reading and analysis of HL meeting minutes for the league's historical documentation associated with the study of the Lattes Curriculum of current and former HL members to collect data on the league's scientific production. The information obtained was tabulated in spreadsheets according to the subtypes of work. Analysis and crossing of variables were performed to produce graphics. Results: HL has been functioning for 14 years and it is linked to three Medicine courses in Juiz de Fora. The aim of education is obtained through meetings and internships in the area of Hematology. Research is its main activity, including an international line of research. Since its foundation, HL has produced a list of 217 scientific works, including 32 scientific articles, 180 abstracts, three book chapters, and two books. Regarding the extension aspect, the league worked on the projects Unir para Cuidar, Doador do Futuro, Triagem Neonatal and Amigo de Sangue. Conclusion: Through its scope of action, as other ALs, HL contributes through its scientific initiation profile to the academic training of its members, in addition to benefiting society through its extension projects.

Factor XIII deficiency, a rare coagulopathy: case reports / Deficiência do fator XIII, uma coagulopatia rara: relato de casos

Introduction: Hereditary Factor XIII (FXIII) deficiency is a rare autosomal recessive hemostatic disorder with an estimated incidence of one case per two million individuals and a higher prevalence in descendants of consanguineous relationships. Possible clinical manifestations include intracranial hemorrhage, umbilical cord bleeding at birth, hematoma, spontaneous abortions, and menometrorrhagia. Objective: To highlight the peculiarities of this hemostatic disorder, as well as the recommended management. Case Report: The authors describe two cases of FXIII deficiency with different hemorrhagic manifestations. Case 1 presented extensive spontaneous hematoma in the right thigh, while Case 2 had umbilical cord bleeding at birth and intracranial hemorrhage, requiring hemotherapy support. Both patients had normal results in screening laboratory tests for coagulation disorders. Coagulation factor serum levels and diagnostic assessments identified mild Factor XIII deficiency in Case 1 and severe deficiency in Case 2. The patient in Case 1 is under regular control and follow-up, while the patient in Case 2 is on a monthly prophylactic regimen with FXIII infusion. Conclusion: The diagnosis of FXIII deficiency in patients with significant bleeding should be considered if screening coagulation tests are normal. The Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis has established an algorithm for laboratory diagnosis and identification of different forms of FXIII deficiency. Quantitative determination of FXIII activity, antigenic assays, and molecular studies are necessary.

Introdução: A deficiência hereditária do Fator XIII (FXIII) é uma rara desordem autossômica recessiva da hemostasia, com uma incidência estimada de um caso a cada dois milhões de pessoas e uma maior prevalência em descendentes de relacionamentos consanguíneos. Possíveis manifestações clínicas incluem: hemorragia intracraniana, sangramento do cordão umbilical no nascimento, hematoma, abortos espontâneos e menometrorragia. Objetivo: Ressaltar as particularidades desse distúrbio hemostático, assim como o manejo preconizado. Relato de caso: Os autores descrevem dois casos de deficiência de FXIII com diferentes manifestações hemorrágicas. O Caso 1 apresentou extenso hematoma espontâneo na coxa direita, enquanto o Caso 2 apresentou sangramento do cordão umbilical ao nascer e hemorragia intracraniana, necessitando de suporte hemoterápico. Ambos os pacientes apresentavam resultados normais nos testes laboratoriais de triagem para distúrbios de coagulação. As dosagens séricas de fatores de coagulação e de diagnóstico identificaram deficiência leve do Fator XIII no Caso 1 e grave no Caso 2. O paciente do Caso 1 está sob controle e acompanhamento regular, enquanto o paciente do Caso 2 está em regime profilático mensal com infusão de FXIII. Conclusão: O diagnóstico de Deficiência de FXIII em pacientes com sangramento importante deve ser considerado se os testes de coagulação de triagem forem normais. O Comitê Científico e de Padronização da Sociedade Internacional de Trombose e Hemostasia estabeleceu um algoritmo para o diagnóstico laboratorial e identificação de diferentes formas de deficiência FXIII. A determinação quantitativa da atividade do FXIII, ensaios antigênicos e estudos moleculares são necessários.