Inherited coagulopathies are bleeding disorders, which require treatment for life. Keeping an updated registry on these diseases is crucial for planning care, documenting prevalence of diseases and evaluating effectiveness of resources. We have analysed data from 26 treatment centres on coagulopathies in Brazil. Information included socio-demographic data, diagnosis of coagulopathies, severity of haemophilias A and B, presence and quantification of inhibitors in haemophilia, type of von Willebrand disease (VWD) and infection status for viral diseases. On 1 July 2007, there were 10 982 patients with inherited coagulopathies in Brazil, of which 6881 (62.7%) corresponded to haemophilia A, 1291 (11.7%) to haemophilia B, 2333 (21.2%) to VWD, 258 (2.4%) to other coagulopathies and 219 (2.0%) to undiagnosed bleeding disorders. Haemophilia A and B inhibitors were present in 9.9% and 1.9% of the patients, respectively. Human immunodeficiency virus infection was present is 6.5%, 4.8% and 1% of patients with haemophilia A, B and VWD, respectively. Hepatitis C virus infection was present in 34.9%, 29.7% and 12% of patients with haemophilia A, B and VWD, respectively. Infection by hepatitis B and human T-cell leukemia-lymphoma virus was also reported. This is the first report on the registry of patients with inherited coagulopathies in Brazil, supposed to be the third largest population of patients with haemophilia.

This article describes a serological survey for rickettsiosis in the county of NovoCruzeiro, Minas Gerais State, Brazil, in 1998, testing

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The development of alloantibodies that inhibit or neutralise the function of factor VIII is considered the most serious complication of the treatment of congenital haemophilia A. In order to describe their course without immune tolerance induction (ITI), we documented data on all performed inhibitor tests with dates as well as on clotting factor infusions of all consecutive patients who were treated in our centre between 1993 and 2006. Patients were tested every 7.1 months (95% confidence interval [CI], 6.6-7.8). A 'sustained negative inhibitor status' was defined as consistent non-positive inhibitor measurements for two years or longer. A total of 60/486 (12%) patients tested had a positive inhibitor titre in two or more occasions. Most of the patients (56%) with a maximum inhibitor titre of < 5 Bethesda unit (BU)/ml (named "low titre inhibitor") developed a sustained negative inhibitor status. Among patients with high (5-9.9 BU/ml) and very high (≥ 10 BU/ml) inhibitor titres, the proportions were 50% and 3%, respectively. Our findings suggest that ITI might not be needed for all patients with non-transient inhibitors, especially when their maximum inhibitor titre is below 10 BU/ml. Further studies in countries where ITI is not available are needed to examine predictors of the natural sustained negative inhibitor status.

Pulmonary embolism is a serious and potentially fatal disorder. Pulmonary embolism risk stratification may allow early hospital discharge and outpatient treatment for low-risk patients. Also, it may prevent death by early medical intervention in high-risk groups. We evaluated objectively confirmed pulmonary embolism in 126 patients by multidetector computed tomographic pulmonary angiography at a single center from January 2008 to January 2010. The Pulmonary Severity Embolism Index (PESI), the right ventricle (RV) to left ventricle (LV) diameter (RV/LV) ratio and the vascular obstruction index (VOI) were derived from data extracted from electronic hospital records and image database. A total of six out of 96 patients (6.3%) died during follow-up. There was an association between PESI and mortality (P-value < 0.001 χ² test). PESI class I-II had a 100% negative predictive value for death in 90 days. No association was found between the RV/LV ratio, the VOI and mortality (P-value > 0.05 χ² test). Also, no association was found between the RV/LV ratio and the VOI and PESI (P-value > 0.05 χ² test). PESI is an accurate tool for pulmonary embolism prognostic stratification. It safely discriminates low-risk from high-risk patients regarding death outcome. We were unable to demonstrate an association between image scores and mortality.