Pulmonary function abnormalities in children with sickle cell disease

K P Sylvester, R A Patey, P Milligan, M Dick, G F Rafferty, D Rees, S L Thein, A Greenough
Thorax 2004, 59 (1): 67-70

BACKGROUND: Adults with sickle cell disease (SCD) have restrictive lung function abnormalities which are thought to result from repeated lung damage caused by episodes of pulmonary vaso-occlusion; such episodes start in childhood. A study was therefore undertaken to determine whether children with SCD have restrictive lung function abnormalities and whether the severity of such abnormalities increases with age.

METHODS: Sixty four children with SCD aged 5-16 years and 64 ethnic matched controls were recruited. Weight and sitting and standing height were measured, and lung function was assessed by measurement of lung volumes and forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and peak expiratory flow (PEF) before and after bronchodilator.

RESULTS: Compared with the control subjects, the children with SCD had lower mean (SD) sitting height (69 (6.3) cm v 73 (7.7) cm; p=0.004), sitting:standing height ratio (0.50 (0.02) v 0.51 (0.01); p<0.0001), weight (33 (10.9) kg v 41 (14.9) kg; p=0.001), functional residual capacity measured by a helium gas dilution technique (1.2 (0.3) l v 1.3 (0.4) l; p=0.04), FEV1 (1.5 (0.5) l v 1.9 (0.7) l; p=0.0008), FVC (1.7 (0.6) l v 2.1 (0.8) l; p=0.001), and PEF (3.9 (1.3) l/s v 4.8 (1.5) l/s; p=0.0004). The effect of age on lung function differed significantly between the children with SCD and the controls for total lung capacity and vital capacity measured by plethysmography and functional residual capacity measured by helium gas dilution.

CONCLUSION: Lung function differs significantly in children with SCD compared with ethnic matched controls of a similar age. Our results suggest that restrictive abnormalities may become more prominent with increasing age.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"