Clinical and care profiles of children and adolescents with Sickle Cell Disease in the Brazilian Northeast region

• Main Author: Marques,Taciana
• Publication Date: 2019
• Other Authors: Vidal,Suely Arruda, Braz,Adriana Farrant, Teixeira,Maria de Lourdes Holanda
• Format: Article
• Language: eng
• Source: Revista Brasileira de Saúde Materno Infantil (Online)
• Download full: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1519-38292019000400881
• topic: Sickle cell disease; Complications; Epidemiology; Child health; Adolescent health; Neonatal screening

Abstract Objectives: to describe the clinical epidemiological and care profiles of pediatric patients with sickle cell disease. Methods: a descriptive study of all (48) children and adolescents with sickle cell disease, assisted at a public referral hospital in Paraíba State. The information were obtained from the patients’ medical records and interviews were analyzed by using the Epi-Info program 7.2 version, frequency tables were built for the categorical variables and the central measurements and dispersion tendencies were calculated for the variables related to age and hospitalizations. Results: the patients’ age ranged from 15 months to 19 years old (median 8.6 years old); 91.7% considered their skin color mixed/black; 81.3% belong to D and E social class; 48% of the responsible guardians reported to have less than nine years of schooling; 70.9% lived in other cities; 93.8% received late diagnosis and 87.5% had irregular outpatient follow-up, 62,5% had an incomplete or outdated vaccination record. There were 226 hospitalizations; painful crises were the most common causes (55.7%). Each patient was hospitalized, about 5.2 times in the period; the median of total days for being hospitalized was 28. There were no deaths. Cardiac (56.2%) and hepatobiliary (54.3%) were the most common chronic compli-cations. Conclusions: sickle cell disease is a neglected clinical condition in the Brazilian Northeast region, where the appropriate political support for the patients is not fulfilled.