Original articleImpact of acute chest syndrome on lung function of children with sickle cell disease
Section snippets
Methods
Afro-Caribbean children with SCD and homozygous for sickle hemoglobin were recruited from 2 specialty clinics into a prospective study examining pulmonary abnormalities in children with SCD. The study was approved by the King’s College Hospital Research Ethics Committee, and parents gave informed written consent for their children’s participation. Consecutive children whose parents gave informed written consent were recruited without regard to their asthma status. For the purposes of this
Results
The 20 children in the ACS group had a median age of 11 years (range, 6 to 16 years) (Table I; Figure). Fifteen of the 20 “pairs” were also matched for sex. The children in the ACS group tended to be shorter than the children in the non-ACS group (P = .22), but there were no significant differences in the numbers of males or asthmatics between the 2 groups (Table I). The results of 23 of the children (8 in the ACS group) were reported in a study comparing lung function in children with SCD and
Discussion
We have demonstrated that the lung function results of the children with SCD who had been hospitalized for ACS episodes differed significantly from those of the children who had not experienced an ACS episode. The mean Raw, TLC, and RV were significantly higher in the ACS group than in the non-ACS group. The 2 groups differed (albeit not significantly so) with regard to height; hence we compared the results expressed as the percentages of those predicted for height. The proportions of children
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Cited by (46)
High risk and low prevalence diseases: Acute chest syndrome in sickle cell disease
2022, American Journal of Emergency MedicineCitation Excerpt :In patients who continue to deteriorate despite mechanical ventilation and exchange transfusion, extracorporeal membrane oxygenation (ECMO) may be used but is only reported in case reports and observational studies [103-107]. A variety of complications are associated with ACS, including worsening lung function due to fibrosis (e.g. chronic sickle cell lung disease) and higher risk of recurrent ACS [108-111]. SCD is associated with significant risk of pulmonary hypertension, with a lifetime prevalence of 6–10.5% in those with SCD [112-114].
Hypoxemia in sickle cell disease: Significance and management
2014, Paediatric Respiratory ReviewsCitation Excerpt :Recurrent episodes of ACS can lead to lung parenchymal scarring and sickle cell chronic lung disease, which leads to progressive loss of lung function and/or impaired pulmonary growth (Figure 1). [18] Children with SCD who have recurrent ACS episodes have worse pulmonary lung function compared to aged-matched children with SCD who have not experienced ACS episodes. [19] Further, steady state daytime and/or nocturnal oxyhemoglobin desaturations are risk factors for overt ischemic stroke in children with SCD. [4,20]
Lung function in sickle cell disease
2014, Paediatric Respiratory ReviewsCitation Excerpt :Using a different methodology, in which each patient was classified as “normal”, “obstructive”, or “restrictive” according to pre-specified criteria, Koumbourlis et al. [16] confirmed their findings in infants reporting that over 57% of their patients had normal lung function, 35% had obstructive and only 8% had a restrictive pattern. During the last decade many more studies have been published with information on the lung function of children and adults with SCD [17–34] [Table 1]. While there is now a general recognition that SCD is not associated with a single pattern of lung function and that certain patients may actually have a normal or obstructive pattern, the disease remains mostly associated with a restrictive pattern especially among adult patients.
Asthma and Chronic Sickle Cell Lung Disease: A Dynamic Relationship
2011, Paediatric Respiratory ReviewsCitation Excerpt :Because HbS is insoluble in the deoxygenated state, erythrocytes become rigid and cause painful microvascular occlusion in the capillary beds of many organs including the lungs 1. SCD is the most common inheritable disorder in African-Americans, with an incidence of 1 in 400 births in the United State 38. In parts of sub-Saharan Africa, an estimated 40-60% of the population is heterozygous for SCD 2.
Lung Function Disorders among Sickle Cell Disease Patients in Basrah City, Iraq
2023, Iraqi Journal of Pharmaceutical Sciences
Karl Sylvester and Richard Patey were supported by the Community Fund and the Well Child Trust Medical Research Fund. Karl Sylvester is currently supported by the Amanda Smith Charitable Trust.