Publication
Sickle cell disease in children: chronic complications and search of predictive factors for adverse outcomes
| dc.contributor.author | Silva, IV | |
| dc.contributor.author | Reis, AF | |
| dc.contributor.author | Palaré, MJ | |
| dc.contributor.author | Ferrão, A | |
| dc.contributor.author | Rodrigues, T | |
| dc.contributor.author | Morais, A | |
| dc.date.accessioned | 2014-09-10T22:19:23Z | |
| dc.date.available | 2014-09-10T22:19:23Z | |
| dc.date.issued | 2014 | |
| dc.description.abstract | BACKGROUND: Sickle cell disease (SCD) has extremely variable phenotypes, and several factors have been associated with the severity of the disease. OBJECTIVES: To analyze the chronic complications of SCD and look for predictive risk factors for increased severity and number of complications. METHODS: Retrospective study including all children followed for SCD in the Paediatric Haematology Unit of a tertiary hospital in Portugal, who completed 17 yr old between the years 2004 and 2013. RESULTS: We identified 44 patients, 55% female and 98% black. Chronic complications occurred in 80% of cases. Slight dilatation of the left ventricle was the most frequent complication (47.7%), followed by respiratory function disturbs (43.2%), microlithiasis or cholelithiasis (40.9%), increased flow velocity of cerebral arteries (31.8%), enuresis, delayed puberty and bone abnormalities (6.8% each), sickle cell retinopathy and leg ulcer (4.6% each) and recurrent priapism (2.3%). We identified a statistically significant association between leukocytes >15 000/μL and a higher number of hospitalizations (P < 0.001) and chronic complications of the disease (P = 0.035). The occurrence of dactylitis in first year of life was also significantly associated with a higher number of hospitalizations (P = 0.004) and chronic complications (P = 0.018). The presence of α-thalassemia was associated with a lower number of chronic complications (P = 0.036). CONCLUSIONS: Leucocytosis and dactylitis in the first year of life can be predictors of SCD severity, while the presence of α-thalassemia can be protective. The determination of early predictors of chronic complications of SCD may improve the comprehensive care of these patients. | por |
| dc.identifier.citation | Eur J Haematol. 2015 Feb;94(2):157-61. | por |
| dc.identifier.uri | http://hdl.handle.net/10400.26/6705 | |
| dc.language.iso | eng | por |
| dc.peerreviewed | yes | por |
| dc.publisher | Wiley | por |
| dc.subject | Criança | por |
| dc.subject | Anemia de Células Falciformes | por |
| dc.title | Sickle cell disease in children: chronic complications and search of predictive factors for adverse outcomes | por |
| dc.type | journal article | |
| dspace.entity.type | Publication | |
| rcaap.rights | openAccess | por |
| rcaap.type | article | por |