Sickle cell disease retinopathy: characterization among pediatric and teenage patients from northeastern Brazil

Objective: The aim of the present study was to characterize sickle cell disease retinopathy in children and teenagers from Bahia, the state in northeastern Brazil with the highest incidence and prevalence of sickle cell disease. Methods: A group of 51 sickle cell disease patients (36 hemoglobin SS and 15 hemoglobin SC) with ages ranging from 4 to 18 years was studied. Ophthalmological examinations were performed in all patients. Moreover, a fluorescein angiography was also performed in over 10-year-old patients. Results: The most common ocular lesions were vascular tortuosity, which was found in nine (25%) hemoglobin SS patients, and black sunburst, in three (20%) hemoglobin SC patients. Peripheral arterial closure was observed in five (13.9%) hemoglobin SS patients and in three (13.3%) hemoglobin SC patients. Arteriovenous anastomoses were present in six (16.5%) hemoglobin SS patients and six (37.5%) hemoglobin SC patients. Neovascularization was not identified in any of the patients. Conclusions: This study supports the use of early ophthalmological examinations in young sickle cell disease patients to prevent the progression of retinopathy to severe disease and further blindness...

Associação entre hipertrofia adenotonsilar, tonsilites e crises álgicas na anemia falciforme / Association between adenotonsillar hypertrophy, tonsillitis and painful crises in sickle cell disease

OBJETIVOS: Determinar a prevalência da hipertrofia adenotonsilar obstrutiva em crianças e adolescentes portadores de anemia falciforme; investigar possível associação entre presença de mais de cinco episódios de tonsilite nos últimos 12 meses e episódios de crise álgica no mesmo período; e comparar a hemoglobina anual média entre os que apresentam e os que não apresentam hipertrofia adenotonsilar obstrutiva. MÉTODOS: Trata-se de estudo prospectivo, observacional do tipo corte transversal, com 85 crianças e adolescentes com anemia falciforme. Todos responderam questionário e avaliação otorrinolaringológica, incluindo endoscopia nasossinusal. Para o diagnóstico da hipertrofia adenotonsilar obstrutiva foram adotados os critérios de Brodsky. RESULTADOS: A prevalência da hipertrofia adenotonsilar obstrutiva foi de 55,3 por cento. A hipertrofia adenotonsilar obstrutiva associou-se à história de dificuldade para alimentar-se (76,7 versus 23,5 por cento; p = 0,003), presença de mais de cinco episódios de tonsilites nos últimos 12 meses (70,6 versus 29,4 por cento; p = 0,021), roncar alto (73,0 versus 27,0 por cento; p = 0,004) e apneia do sono assistida (71,8 versus 28,2 por cento; p = 0,005). Portadores de hipertrofia adenotonsilar obstrutiva apresentaram maior número de infecções das vias aéreas superiores (62,5 versus 37,5; p = 0,010). Também foi observada associação entre presença de mais de cinco episódios de tonsilite nos últimos 12 meses e episódios de crise álgica no mesmo período (mediana = 12 versus 2; p = 0,017). Não houve diferença significante da hemoglobina anual média entre portadores de hipertrofia adenotonsilar obstrutiva versus hipertrofia adenotonsilar não-obstrutiva (7,6 versus 8,2 g/dL; p = 0,199). CONCLUSÃO: A prevalência da hipertrofia adenotonsilar obstrutiva foi de 55,3 por cento em crianças e adolescentes com anemia falciforme. A presença de mais de cinco episódios de tonsilite nos últimos 12 meses associaram-se com...

OBJECTIVES: To determine the prevalence of obstructive adenotonsillar hypertrophy in children and adolescents with sickle cell anemia; to investigate possible association between the presence of more than five episodes of tonsillitis in the last 12 months and episodes of painful crises in the same period; and to compare the mean annual hemoglobin level in children and adolescents with and without obstructive adenotonsillar hypertrophy. METHODS: Prospective, observational, cross-sectional study involving 85 children and adolescents with sickle cell anemia. All patients answered a questionnaire and underwent a standard otolaryngology examination, including endoscopic endonasal approach. The diagnosis of obstructive adenotonsillar hypertrophy was made according to the Brodsky scale. RESULTS: The prevalence of obstructive adenotonsillar hypertrophy was 55.3 percent. Obstructive adenotonsillar hypertrophy was associated with history of difficulty in eating (76.7 vs. 23.5 percent, p = 0.003), presence of more than five episodes of tonsillitis in the last 12 months (70.6 vs. 29.4 percent, p = 0.021), loud snoring (73.0 vs. 27.0 percent, p = 0.004), and sleep apnea (71.8 vs. 28.2 percent, p = 0.005). Patients with obstructive adenotonsillar hypertrophy had more episodes of recurrent upper airway tract infection (62.5 vs. 37.5; p = 0.010). The presence of more than five episodes of tonsillitis in the last 12 months was associated with episodes of painful crises (median = 12 vs. 2, p = 0.017). There was no significant difference between mean annual hemoglobin levels of patients with obstructive adenotonsilar hypertrophy vs. nonobstructive adenotonsillar hypertrophy: 7.6 vs. 8.2 g/dL, p = 0.199. CONCLUSION: The prevalence of obstructive adenotonsillar hypertrophy was 55.3 percent in children and adolescents with sickle cell anemia; the presence of more than five episodes of tonsillitis in the last 12 months was associated with episodes of painful...