Evaluation of a web-based registry of inherited bleeding disorders: a descriptive study of the Brazilian experience with HEMOVIDAweb Coagulopatias.

BACKGROUND: Inherited bleeding disorders (IBD) consist of a group of rare heterogeneous diseases, which require treatment for life. Management of these disorders is complex and costly. Therefore, good quality data of the affected population is crucial to guide policy planning. The aim of this manuscript is to describe the impact of a national, web-based registry - the Hemovidaweb Coagulopatias (HWC) - in the management of the IBD in Brazil. METHODS: The system was developed in PHP 5.0 language and is available on the internet at http://coagulopatiasweb.datasus.gov.br . The system was validated in September 2008 and launched nationally with input from January 1, 2009. HWC collects variables related to socio-demographic, clinical, laboratory and treatment data of patients with IBD. RESULTS: Within 7 years, there was an increment of 90.8% on the diagnosis of IBD altogether, which increased from 11,040 in December 2007 to 21,066 in December 2014. This is now the fourth and third largest world population of patients with haemophilia and von Willebrand's disease (vWD), respectively, according to the most recent (2015) Annual Global Survey of the World Federation of Hemophilia. The data collected provided the basis for planning and implementing home therapy, prophylaxis and immune tolerance induction (ITI), recently initiated in Brazil. CONCLUSION: HWC was an effective tool in the increment of registration of patients with IBD in Brazil. Furthermore, it was essential to support policy planning, monitoring, evaluation and treatment. Future development should focus on surveillance, health outcomes and research. Every country should implement a national registry on IBD.

Hemovida Web Coagulopatias: um relato do seu processo de desenvolvimento e implantação. / Hemovida Web Coagulophaties: a report of its development andimplementation.

As coagulopatias hereditárias são doenças hemorrágicas decorrentes da deficiência de um ou mais fatores de coagulaçãosanguínea. A Coordenação da Política Nacional de Sangue e Hemoderivados, responsável pelo Programa Nacional deCoagulopatias Hereditárias, em parceria com o Departamento de Informática do Sistema Único de Saúde (SUS), desenvolveuo sistema informatizado Hemovida Web Coagulopatias, cujo objetivo é a sistematização do cadastro nacional dospacientes com coagulopatias e outras doenças hemorrágicas hereditárias. Esse cadastro reúne dados sobre o diagnóstico,tratamento e complicações das doenças, bem como o perfil sociodemográfico dos pacientes e os quantitativos de fatoresde coagulação a eles dispensados. Este artigo apresentou um relato sobre o desenvolvimento e implantação desse Sistema,considerando a descrição dos antecedentes, desenvolvimento, processamento dos dados, treinamento de pessoal esituação atual da implantação, além de apontar as potencialidades e perspectivas previstas para o seu aperfeiçoamento. Osistema está disponível na Internet desde janeiro de 2009 e conta com a adesão das Unidades da Federação, em estágiosdiferenciados de utilização. O uso potencial das informações produzidas nesse sistema é de grande necessidade, devido àpossibilidade de se realizar uma gestão eficiente às políticas públicas voltadas a essa população, em função de um gastopúblico de elevado valor na aquisição de hemoderivados.

Inherited coagulopathies are bleeding disorders characterized by the deficiency of one or more coagulation factors. The Coordenaçãoda Política Nacional de Sangue e Hemoderivados, in charge of the National Program of Inherited Coagulopathieshas developed, in conjunction with the Departamento de Informática do Sistema Único de Saúde (SUS), the system named Hemovida Web Coagulopatias, which role is to organize the national registry of patients with inherited coagulopathies and other bleedingdisorders. The registry compiles data on diagnosis, treatment and complications of the disorders, as well as the socioeconomic profile of thepatients and detailed treatment used. This article presented an evaluation of the development and implantation of this system, considering therational for its development, data processing, training of human resources and present situation of implantation. It also discussed its powerand perspectives for improvement. The system is available on the Internet since January 2009 with implantation in all States with differentlevels of utilization. The potential use of the system information is enormous, due to the need of implementing political actions directed to thispopulation of patients, due to a high value of public spending on the purchase of blood products.

Köln's unstable hemoglobin: case report and literature review

Unstable hemoglobins are a group of genetic variants of hemoglobins caused by the mutation of amino acids into alpha and beta globins and, depending on the points and types of mutation, the result can vary from no clinical symptomatology to severe hemolytic anemia. On the present report, we study the case of a female patient who showed a very exuberant hematological picture for the red series, which suggests hemoglobinic changes; this was confirmed following the conduction of the protocol established by Laboratório Médico Santa Luzia for the study of hemoglobinopathies and which was then sent for a reference laboratory: C.D.A. Naoun Laboratórios de Análises Clínicas