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LUNG FUNCTION IN CHILDREN AND ADOLESCENTS WITH SICKLE CELL ANEMIA: A COMPARISON BETWEEN UK AND ITALY
Author(s):
Carlo De Pieri
,
Carlo De Pieri
Affiliations:
Department of Clinical and Experimental Medical Sciences,University Hospital of Udine,Udine,Italy
Michele Arigliani
,
Michele Arigliani
Affiliations:
Department of Clinical and Experimental Medical Sciences,University Hospital of Udine,Udine,Italy
Raffaella Colombatti
,
Raffaella Colombatti
Affiliations:
Clinic of Pediatric Hematology-Oncology, Department of Child and Maternal Health,Azienda Ospedaliera-Università di Padova,Padova,Italy
Laura Sainati
,
Laura Sainati
Affiliations:
Clinic of Pediatric Hematology-Oncology, Department of Child and Maternal Health,Azienda Ospedaliera-Università di Padova,Padova,Italy
Katy Vecchiato
,
Katy Vecchiato
Affiliations:
University of Trieste,Trieste,Italy
Sharon Ndoro
,
Sharon Ndoro
Affiliations:
Department of Pediatrics,Evelina Children's Hospital,London,United Kingdom
Paola Cogo
,
Paola Cogo
Affiliations:
Department of Clinical and Experimental Medical Sciences,University Hospital of Udine,Udine,Italy
Baba Inusa
Baba Inusa
Affiliations:
Department of Pediatrics,Evelina Children's Hospital,London,United Kingdom
(Abstract release date: 05/18/17) EHA Library. De Pieri C. 05/18/17; 182855; PB2142
Carlo De Pieri
Carlo De Pieri
Contributions
PDF Abstract
Abstract

Abstract: PB2142

Type: Publication Only

Background
Acute and chronic respiratory complications are common in sickle cell anemia (SCA). Subjects with SCA often have a progressive decline of lung function with age that could be influenced by the quality of healthcare and by environmental factors, such as the level of exposure to air pollution.

Aims

To compare lung function, evaluated cross-sectionally through spirometry, in children and adolescents attending sickle cell centers in UK and Italy.

Methods

Anthropometry and spirometry were recorded in patients with SCA (SS,Sb0) aged 6-17 years of African ancestry followed at the Evelina Children’s Hospital, London, UK, and at the University Hospitals of Padova and Udine, northeast of Italy. Subjects from the British cohort lived in an urban area while those from Italy came from urban and non-urban areas. Exclusion criteria were the presence of SCA-related morbidity within the last two weeks and the inability to perform a spirometry meeting the European Respiratory Society acceptability and repeatability criteria (Miller, Eur Respir J 2005;26:319–338), modified for children (Kirkby, Pediatr Pulmonol 2008;43:1233–1241). Portable spirometers (Pony FX, Cosmed-IT, Easy-on PC, NDD-CH) were used. Z-scores of anthropometric and spirometric data were derived, respectively, from CDC2000 and from the Global Lung Initiative 2012 predictive equations for African Americans (Quanjer, Eur Respir J 2012; 40:1324–1343). Spirometry patterns were classified as normal, obstructive (zFEV1/FVC<-1.64) or restrictive (zFVC<-1.64 + zFEV1/FVC ≥ -1.64). Differences between groups were assessed by t-tests and considered statistically significant for p values <0.05.

Results
A total of 101 children and adolescents were included (n. 62 in UK; n. 39 in Italy; 42% girls; age-range: 6.2-17.9 years). We didn’t find significant differences in mean spirometry indices between the SCA cohort from London and northeast Italy (Table). Nevertheless while an obstructive spirometry pattern was more common in the British cohort compared to the Italian one (respectively 22.5% vs 7.7%), the picture was the opposite for the restrictive pattern (respectively 11.2% and 20.5%) (Table). In the whole sample age was negatively correlated with both zFEV1 (Spearman’s rho -0.20) and zFVC (Spearman’s rho -0.24).

Index
Sickle cell UK
Sickle cell ITA
Diff between means (95% CI)
n (%male)
62 (54%)
39 (61%)
Age (years)
11.9 (2.7)
11.3 (3.5)
0.6 (-0.6 to 1.9)
Height z-score
-0.11 (1.23)
-0.08 (1.09)
-0.03 (-0.53 to 0.47)
BMI z-score
-0.11 (1.71)
-0.55 (1.17)
0.44 (-0.03 to 0.91)
FEV1 z-score
-1.10 (1.04)
-0.80 (0.97)
-0.30 (-0.72 to 0.10)
FVC z-score
-0.71 (1.03)
-0.71 (0.83)
0.00(-0.43 to 0.39)
FEV1/FVC z-score
-0.43 (1.09)
-0.27(0.93)
-0.16 (-0.58 to 0.25)
Spirometry pattern
Obstructive (% of total)
14 (22.5%)
3 (7.7%)
Restrictive (% of total)
7 (11.2%)
8 (20.5%)

Conclusion

Lung function of pediatric subjects with SCA living in London and in the northeast of Italy is overall comparable. Obstructive lung disease is more common among subjects with SCA living in London than in urban and non-urban areas in Italy. Differences in the level of exposure to ambient air pollution and in the prevalence of allergies between the rural and urban environment might have contributed to this finding and need to be further investigated.

Session topic: 25. Sickle cell disease

Keyword(s): Lung, Children

Abstract: PB2142

Type: Publication Only

Background
Acute and chronic respiratory complications are common in sickle cell anemia (SCA). Subjects with SCA often have a progressive decline of lung function with age that could be influenced by the quality of healthcare and by environmental factors, such as the level of exposure to air pollution.

Aims

To compare lung function, evaluated cross-sectionally through spirometry, in children and adolescents attending sickle cell centers in UK and Italy.

Methods

Anthropometry and spirometry were recorded in patients with SCA (SS,Sb0) aged 6-17 years of African ancestry followed at the Evelina Children’s Hospital, London, UK, and at the University Hospitals of Padova and Udine, northeast of Italy. Subjects from the British cohort lived in an urban area while those from Italy came from urban and non-urban areas. Exclusion criteria were the presence of SCA-related morbidity within the last two weeks and the inability to perform a spirometry meeting the European Respiratory Society acceptability and repeatability criteria (Miller, Eur Respir J 2005;26:319–338), modified for children (Kirkby, Pediatr Pulmonol 2008;43:1233–1241). Portable spirometers (Pony FX, Cosmed-IT, Easy-on PC, NDD-CH) were used. Z-scores of anthropometric and spirometric data were derived, respectively, from CDC2000 and from the Global Lung Initiative 2012 predictive equations for African Americans (Quanjer, Eur Respir J 2012; 40:1324–1343). Spirometry patterns were classified as normal, obstructive (zFEV1/FVC<-1.64) or restrictive (zFVC<-1.64 + zFEV1/FVC ≥ -1.64). Differences between groups were assessed by t-tests and considered statistically significant for p values <0.05.

Results
A total of 101 children and adolescents were included (n. 62 in UK; n. 39 in Italy; 42% girls; age-range: 6.2-17.9 years). We didn’t find significant differences in mean spirometry indices between the SCA cohort from London and northeast Italy (Table). Nevertheless while an obstructive spirometry pattern was more common in the British cohort compared to the Italian one (respectively 22.5% vs 7.7%), the picture was the opposite for the restrictive pattern (respectively 11.2% and 20.5%) (Table). In the whole sample age was negatively correlated with both zFEV1 (Spearman’s rho -0.20) and zFVC (Spearman’s rho -0.24).

Index
Sickle cell UK
Sickle cell ITA
Diff between means (95% CI)
n (%male)
62 (54%)
39 (61%)
Age (years)
11.9 (2.7)
11.3 (3.5)
0.6 (-0.6 to 1.9)
Height z-score
-0.11 (1.23)
-0.08 (1.09)
-0.03 (-0.53 to 0.47)
BMI z-score
-0.11 (1.71)
-0.55 (1.17)
0.44 (-0.03 to 0.91)
FEV1 z-score
-1.10 (1.04)
-0.80 (0.97)
-0.30 (-0.72 to 0.10)
FVC z-score
-0.71 (1.03)
-0.71 (0.83)
0.00(-0.43 to 0.39)
FEV1/FVC z-score
-0.43 (1.09)
-0.27(0.93)
-0.16 (-0.58 to 0.25)
Spirometry pattern
Obstructive (% of total)
14 (22.5%)
3 (7.7%)
Restrictive (% of total)
7 (11.2%)
8 (20.5%)

Conclusion

Lung function of pediatric subjects with SCA living in London and in the northeast of Italy is overall comparable. Obstructive lung disease is more common among subjects with SCA living in London than in urban and non-urban areas in Italy. Differences in the level of exposure to ambient air pollution and in the prevalence of allergies between the rural and urban environment might have contributed to this finding and need to be further investigated.

Session topic: 25. Sickle cell disease

Keyword(s): Lung, Children

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