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Lung function in infants with sickle cell disease
Author(s) -
Koumbourlis Anastassios C.,
HurletJensen Anne,
Bye Michael R.
Publication year - 1997
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/(sici)1099-0496(199710)24:4<277::aid-ppul6>3.0.co;2-h
Subject(s) - medicine , functional residual capacity , acute chest syndrome , nitrogen washout , bronchospasm , pulmonary function testing , lung volumes , respiratory disease , airway obstruction , airway resistance , cardiology , gastroenterology , lung , anesthesia , asthma , sickle cell anemia , airway , disease
We performed pulmonary function testing in 20 infants (11 male and 9 female; ages 3–30 months) with sickle cell disease to assess whether abnormal lung function develops early in life. Respiratory system compliance ( C rs ) and resistance ( R rs ) were measured by the passive occlusion technique, functional residual capacity (FRC) was measured by the nitrogen washout technique, and tidal flow‐volume loops and partial expiratory flow‐volume curves were obtained by the thoracoabdominal compression technique to detect airway obstruction. Patients with Hb SS (Group I, n = 12) had significantly lower hemoglobin levels and a higher (but not significant) incidence of acute chest syndrome (ACS), vasoocclusive crisis (VOC), splenic sequestration, transfusions, and history of intermittent bronchospasm compared to with patients with hemoglobinopathies Hb SC, Hb Sbt and Hb SF (Group II; n = 8). Both groups had elevated FRC, decreased maximum expiratory flows at FRC ( V ′ max,FRC ), and decreased time needed to reach peak expiratory flow ( t me / t E ), suggesting lower airway obstruction (LAO) and hyperinflation. Restrictive disease was found in only three patients of Group I. Our findings suggest that in sickle cell disease (especially among patients with Hb SS), abnormal lung function (predominantly LAO) may be present in early infancy. Airway reactivity may play a role in the pathogenesis, but the relation to VOC or ACS remains unclear. Pediatr. Pulmonol. 1997;24:277–281. © 1997 Wiley‐Liss, Inc.