![]() Sickle cell anemia (SCA, homozygous HbS or HbSS) is the most common and severe manifestation of SCD and double heterozygosis occurs as HbS/β 0-thalassemia (HbSβ 0, severity as HbSS), HbS/β +-thalassemia (HbSβ +), HbSC disease (HbSC), HbSD disease (HbSD) and other less frequent associations. Sickle cell disease (SCD) is a group of hereditary autosomal recessive hemoglobinopathies. The genetic hemoglobin (Hb) mutation caused by the replacement of glutamic acid by valine in position 6 of the beta-globin chain (β6 Glu → Val) produces the variant hemoglobin S (HbS → α2β2 s). After identifying studies that potentially substantiated the recommendation, the level of evidence and degree of recommendation were calculated using the Oxford Classification. The data recovered were critically analyzed using discriminatory instruments (scores) according to the type of evidence. The questions were structured using the Patient/Problem, Intervention, Comparison and Outcome (PICO) system, allowing the generation of evidence search strategies in Medline (via PubMed) and using a manual search. These guidelines result from a systematic evidence-based review centered on the Evidence-Based Medicine movement, in which clinical experience is integrated with the ability to critically analyze and apply scientific information rationally, thereby improving the quality of medical care. Description of the method used to collect evidence This article presents the guidelines on sickle cell disease: secondary stroke prevention in children and adolescents. The attending physician should evaluate all possible medical approaches, considering the patient characteristics and clinical status. The recommendations of this article were elaborated by the Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular (ABHH). The guidelines project is a joint initiative of the Associação Médica Brasileira and the Conselho Federal de Medicina, aiming to bring information in medicine to standardize management and assist in decision-making during treatment. ![]()
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March 2023
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