Lung Function in Sickle Cell Disease

Koumbourlis, Anastassios C.

doi:10.1016/j.prrv.2013.10.002

Cited by 21 publications

(26 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Although airway hyper-reactivity and asthma contribute to the decline in respiratory function, in SCD [23,24,25], other factors include ACS and pulmonary hypertension [5,6,9]. However, quite often, no predisposing factors can be identified other than the chronic vasculopathy that characterizes the disease [26]. Pulmonary function abnormalities develop early in the disease and while the respiratory pattern may be normal or obstructive, it is mostly associated with a restrictive pattern in adults [26].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Correlates of Pulmonary Function in Children with Sickle Cell Disease and Elevated Fetal Hemoglobin

Adekile¹,

Azab²,

Owayed³

et al. 2017

Med Princ Pract

View full text Add to dashboard Cite

Objective: The current study was carried out to compare pulmonary function tests (PFTs) in pediatric Kuwaiti sickle cell disease (SCD) patients to age-matched normal controls and to investigate the association of PFTs with selected clinical and laboratory parameters. Subjects andMethods: There were 38 patients with SCD and 36 controls in the study. The patients were recruited from the Pediatric Hematology Clinics of Mubarak Al-Kabeer and Al-Amiri Hospitals, Kuwait, and were studied in steady state. The controls were healthy, non-sickle cell siblings of the patients. Forced expiratory volume in 1 s (FEV₁), forced vital capacity (FVC), total lung capacity, and other PFT parameters were obtained using a constant-volume, variable-pressure, body plethysmograph. Hemoglobin, fetal hemoglobin, serum bilirubin, and lactate dehydrogenase were determined using standard methods. Results: The mean ages of the patients and controls were 10.5 ± 3.2 and 10.5 ± 3.5 years, respectively. The FEV₁% predicted of 84.1 ± 15.4% among the patients was significantly lower than the 92.1 ± 11.8% in the controls (p = 0.003). The FVC% predicted was also significantly lower (p = 0.022) in the patients than in the controls, although the values were generally within the normal range. There was no association of FEV₁ with pain phenotype, acute chest syndrome (ACS), or blood transfusions. Also, there was no significant correlation with reticulocytes, bilirubin, or lactate dehydrogenase. Conclusions: In this study, changes in PFT, especially FEV₁, developed early in the SCD patients. There was no demonstrable association with frequent vaso-occlusive crisis, ACS, and other variables. Hence, there is a need for follow-up studies with serial PFTs to identify vulnerable patients, who might need intervention to prevent early mortality.

show abstract

Section: Discussionmentioning

confidence: 99%

“…However, quite often, no predisposing factors can be identified other than the chronic vasculopathy that characterizes the disease [26]. Pulmonary function abnormalities develop early in the disease and while the respiratory pattern may be normal or obstructive, it is mostly associated with a restrictive pattern in adults [26]. …”

Section: Discussionmentioning

confidence: 99%

Correlates of Pulmonary Function in Children with Sickle Cell Disease and Elevated Fetal Hemoglobin

Adekile¹,

Azab²,

Owayed³

et al. 2017

Med Princ Pract

View full text Add to dashboard Cite

show abstract

“…Equally important, in a prospective cohort design, having an abnormal lung function pattern (obstructive, restrictive, non-specific) was not associated with future pain or ACS events Prior studies have examined the prevalence and progression of lung function abnormalities in children (14). The most common abnormal pattern was obstruction (found in 16%), with fewer patients having restriction (7%).…”

Section: Discussionmentioning

confidence: 99%

Pattern of Lung Function Is Not Associated with Prior or Future Morbidity in Children with Sickle Cell Anemia

Cohen

Strunk

Rodeghier³

et al. 2016

Annals ATS

View full text Add to dashboard Cite

show abstract

“…The current consensus in the pediatric literature is that SCD does affect the lung function, that its effects may start early in life (even during infancy), and that changes do occur as the patients grow older. However, there is still no consensus on whether SCD is causing a specific lung function abnormality . Several mechanisms have been implicated in the pathogenesis of the pulmonary complications including airway reactivity and asthma but the exact timing and mechanism(s) involved remain unclear .…”

Section: Comparison Of Longitudinal Changes In Lung Function Indices mentioning

confidence: 99%