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Chest. 2005 Nov;128(5):3336-44.

Factors associated with dyspnea in adult patients with sickle cell disease.

Author information

1
Service de Physiologie, Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris, Créteil, France. christophe.delclaux@egp.aphp.fr

Abstract

OBJECTIVE:

The aim of this prospective study was to determine the cardiorespiratory factors associated with dyspnea in patients with sickle cell SS-hemoglobin disease, with a specific interest in lung vascular involvement.

MEASUREMENTS:

Forty-nine patients (29 women and 20 men; mean [+/- SD] age: women, 29 +/- 6 years; men, 31 +/- 11 years) underwent direct evaluations (Borg scale evaluation during a 6-min walk test) and indirect evaluations (modified Medical Research Council [MRC]score) of their dyspnea, pulmonary function tests (PFTs) [spirometry, volumes, diffusing capacity of the lung for carbon monoxide (Dlco), diffusing capacity of the alveolar-capillary membrane, and pulmonary capillary blood volume measurements], echocardiography, and biological evaluation.

RESULTS:

Thirty-four patients complained of significant breathlessness (MRC score, > 1). Indirect and direct evaluations of dyspnea were correlated. PFT results depicted a very mild restrictive pattern (mean total pulmonary capacity, 86 +/- 11% predicted) and an impairment of Dlco (mean Dlco corrected for the degree of anemia, 69 +/- 13% predicted). The statistical analysis demonstrated that dyspnea and exercise performance were closely linked to indexes of Dlco but not with any echocardiographic or biological measure including anemia. Nevertheless, only approximately 25% of the variability was explained by these associations. Despite having a similar history of vasoocclusive crisis events, women had more severe anemia, dyspnea, decreases in Dlco (corrected for the degree of anemia), and a higher capillary blood volume (corrected for alveolar volume) than men.

CONCLUSION:

Lung vascular disease contributes to dyspnea and the exercise limitation of patients with sickle cell disease. A sequential assessment of Dlco would therefore constitute one of the objective functional end points for follow-up studies of these patients.

PMID:
16304281
DOI:
10.1378/chest.128.5.3336
[Indexed for MEDLINE]

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