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.
RESULTS:Comparative analysis made it possible to identify important advances in the legislation. In 1992, liquid and powdered milk were included in the scope, along with teats and dummies (pacifiers), and also warning phrases in advertising and on product labeling. In 2002, regulations for products were published by the National Agency for Sanitary Surveillance, thereby strengthening supervisory actions and including regulations for baby foods, nutrient formulae for high-risk newborns, and nipple protectors. The phrases used in commercial advertising and on product labeling, including dummies, teats and bottles, became Ministry of Health warnings. The labeling was defined according to product types, on the basis of more restrictive rules. CONCLUSIONS:Significant modifications in the control over the marketing of products aimed at mothers during the lactation period. However, there are still some legislative questions that would make it possible to improve the Brazilian norm, in order to protect breastfeeding. There is also a need for the government to implement systematic monitoring routines to supervise this legislation.
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