Avaliação da função pulmonar em lactentes com doença falciforme

• Ano de defesa: 2017
• Autor(a) principal: Ivankovich, Danilo Turcato [UNIFESP]
• Tipo de documento: Dissertação
• Tipo de acesso: Acesso aberto
• Idioma: por
• Instituição de defesa: Universidade Federal de São Paulo (UNIFESP)
• Palavras-chave em Português: Sickle cell anemia; Hemoglobin SC disease; Pulmonary function tests; Pulmonary volumes; Infant; Anemia falciforme; Doença da hemoglobina SC; Testes de função respiratória; Medidas de volume pulmonar; Lactente
• Link de acesso: https://sucupira.capes.gov.br/sucupira/public/consultas/coleta/trabalhoConclusao/viewTrabalhoConclusao.jsf?popup=true&id_trabalho=5199532; http://repositorio.unifesp.br/handle/11600/50381

Objective: To assess lung function in infants with sickle cell disease and to compare with a group of infants who did not have sickle cell or lung diseases. Methods: A cross-sectional study was carried out with patients between 6 and 18 months of age that, after being submitted to a clinical evaluation and sedation with chloral hydrate, underwent the pulmonary function testing through lung volume analysis by whole-body plethysmography, and forced expiratory flows using rapid thoracoabdominal compression with raised lung volume. The parameters were recorded in absolute value, z score and percentage of predicted. Statistical analysis tests were considered significant with p <0.05. Results: 33 patients with sickle cell disease were evaluated, with a mean age of 12.6 months (± 3.3), 67% were males, and compared to 37 controls, with a mean age of 10.3 months (± 1.2), 41% male. The groups differed in asthma family history and gestational smoking, these being more prevalent in the control group. The adjusted analysis of the forced expiratory flows showed significantly lower values in the sickle cell group compared with the controls, this occurred in different parameters, such CVF (-0,39 ± 0,8 vs 0,58 ± 0,5), VEF0,5 (-0,43 ± 0,8 vs 0,82 ± 0,5), FEF25-75 (0,03 ± 0,9 vs 1,03 ± 0,7), FEF50 (-0,04 ± 0,8 vs 0,99 ± 0,7), all with p<0,001. No differences were observed in lung volumes and pulmonary capacities. Serum levels of lactic dehydrogenase were correlated only with CRF (r = -0.32 p = 0.04), hemoglobin levels and reticulocytes did not correlate with pulmonary function values. In the comparison between sickle cell genotypes (HbSS, n = 22 or HbSC, n = 11), FEF50 was significantly lower in patients with SS genotype (p = 0.046). Both genotypes presented significant differences in relation to the control group, being these differences greater in the SS genotype. The pulmonary function testing was altered in 27% of the patients, 78% of them with obstructive pattern. Conclusions: Pulmonary function may be reduced in the first years of life in sickle cell disease, with great variability in flow values and predominance of obstructive alterations. Although we observed most significant differences in patients with HbSS, patients with HbSC also might have altered lung function.