The Challenges in the Follow-Up and Treatment of Brazilian Children with Hereditary Angioedema.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
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Araújo-Simões Joanna, Boanova Aline Gisele Pena, Constantino-Silva Rosemeire Navickas, Fragnan Nyla Thyara Melo Lobão, Pinto Jorge Andrade, Minafra Fernanda G, Gonçalves Rozana Fátima, Valle Solange Rodrigues do, Alonso Maria Luiza Oliva, Dortas Sergio Duarte, Goudouris Ekaterine Simões, Rêgo-Silva Almerinda Maria, Marques Mayara Madruga, Serpa Faradiba S, Chong-Neto Herberto Jose, Nelson Rosario Filho, Mansour Eli, Moreira Iramirton Figuerêdo, Moreno Adriana S, Arruda Luisa Karla, Roxo Junior Pérsio, Ferriani Mariana Paes Leme, Silva Jane, Ferreira Janaira Fernandes Severo, Giavina-Bianchi Pedro, Takejima Priscila M, Ensina Luis Felipe, Campos Regis de Albuquerque, Toledo Eliana, Pesquero João Bosco, Palma Sandra Mitie Ueda, Veronez Camila Lopes, Grumach Anete Sevciovic
Аннотация
METHODS: Medical records from 18 reference centers of HAE patients under 18 years of age were evaluated after confirmed diagnosis was performed by quantitative and/or functional C1-INH.
RESULTS: A total of 95 participants (51 M:44 F; mean age: 7 years old) out of 17 centers were included; 15 asymptomatic cases were identified through family history and genetic screening. Angioedema attacks affected the extremities (73.5%), gastrointestinal tract (57%), face (50%), lips (42.5%), eyelids (23.7%), genitals (23.7%), upper airways (10%), and tongue (6.3%). Family history was present in 84% of patients, and the mean delay in the diagnosis was 3.9 years. Long-term prophylaxis (51/80) was performed with tranexamic acid (39/80) and androgens (13/80); and short-term prophylaxis (9/80) was performed with tranexamic acid (6/80) and danazol (3/80). On-demand therapy (35/80) was prescribed: icatibant in 7/35, fresh frozen plasma in 16/35, C1-INH plasma-derived in 11/35, and tranexamic acid in 12/35 patients.
CONCLUSIONS: This is the first study on HAE pediatric patients in Latin America. Clinical manifestations were similar to adults. Drugs such as androgens and tranexamic acid were indicated off-label, probably due to restricted access to specific drugs. Educational programs should address pediatricians to reduce late diagnosis and tailored child therapy.