Pulmonary function studies in sickle cell disease.

Femi-Pearse, D; Gazioglu, Kuddusi; Yu, Paul N.

doi:10.1152/jappl.1970.28.5.574

Cited by 44 publications

(39 citation statements)

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“…Earlier studies [4,5,10,17,18] found a restrictive ventilatory defect in patients with SCLD. It has been suggested that endothelial injury induced by sickled red cells may initiate a chain of reactions leading to pulmonary oedema, which may in turn give rise to the fibrotic process [1,2].…”

Section: Discussionmentioning

confidence: 91%

“…An obstructive pattern has also been reported [4,6] in a few patients with SCLD. Alterations in diffusion capacity (transfer factor of the lung for carbon monoxide (TL,CO)) were found in most studies [4,5].…”

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confidence: 99%

“…Other factors, including infections and infarctions, are superimposed on this background [3], further worsening the lung damage and leading to pulmonary hypertension [1,2]. Most studies of lung function in SCLD have demonstrated a restrictive defect [1,4,5], and a reduction in total lung capacity (TLC) of ð50% has been reported in advanced forms [1]. An obstructive pattern has also been reported [4,6] in a few patients with SCLD.…”

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confidence: 99%

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Pulmonary function in sickle cell disease with or without acute chest syndrome

Santoli

Zérah²,

Vasile³

et al. 1998

Eur Respir J

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Recurrent acute chest syndrome (ACS) has been suggested as a risk factor for chronic lung dysfunction in sickle cell disease. To investigate this hypothesis, lung function tests were performed in 49 sickle cell disease outpatients whose condition was stable, including 23 patients with a history of two to four episodes of ACS (ACS+) and 26 with no history of ACS (ACS-). The two groups were comparable regarding the sex ratio, body mass index, smoking history, physical characteristics, clinical history and usual lung function tests. Respiratory resistance (Rrs), measured using the forced oscillation technique, increased with the number of ACS episodes (r=0.55, p<0.0001) and a significant relationship was observed between Rrs as an independent variable and the expiratory flow rates at 25, 50 and 25-75% of the forced vital capacity as explanatory variables (r= 0.36, p<0.02; r=0.35, p<0.02; and r=0.4, p<0.006, respectively), with higher Rrs being associated with lower expiratory flow rates. The transfer factor (TL,CO) and transfer coefficient (KCO) for CO were significantly higher in the ACS+ group than in the ACS-group (TL,CO=84+/-4 versus 71+/-3%, p<0.004 and KCO=102+/-5 versus 90+/-3%, p<0.05, respectively). The data demonstrate that obstructive lung dysfunction is fairly common in sickle cell disease and suggest that recurrent acute chest syndrome may contribute specific obstructive defects. The increase in respiratory resistance associated with acute chest syndrome was accompanied by an increase in diffusion capacity, suggesting that it may have been related to an increase in lung blood volume.

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Section: Discussionmentioning

confidence: 91%

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confidence: 99%

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confidence: 99%

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Pulmonary function in sickle cell disease with or without acute chest syndrome

Santoli

Zérah²,

Vasile³

et al. 1998

Eur Respir J

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“…Several studies characterize SCD lung manifestations [5,6,[8][9][10][11]. The presence of a restrictive lung pattern have been reported in adults [2,6,[12][13][14][15][16]]. An obstructive pattern has been reported in pediatric studies, with increased prevalence over time approximating a 3 % loss in forced expiratory volume in one second (FEV1)% of predicted/ year [7].…”

Section: Introductionmentioning

confidence: 99%

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

Williams

Nussbaum

Yoonessi

et al. 2014

Lung

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“…Studies of lung function to date in this population have been of modest size, often involving fewer than 50 patients (4,(6)(7)(8)(9)(10)(11) and largely inconclusive. Their results have yielded a spectrum of abnormalities, including restrictive lung disease, abnormal diffusion capacity for carbon monoxide (Dl CO ), obstructive disease, and hypoxemia (4,6,7,9,12).…”

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Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

Klings

Wyszynski

Nolan

et al. 2006

Am J Respir Crit Care Med

180

166

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Rationale: Pulmonary complications of sickle cell anemia (Hb-SS) commonly cause morbidity, yet few large studies of pulmonary function tests (PFTs) in this population have been reported. Objectives: PFTs (spirometry, lung volumes, and diffusion capacity for carbon monoxide [DL CO ]) from 310 adults with Hb-SS were analyzed to determine the pattern of pulmonary dysfunction and their association with other systemic complications of sickle cell disease. Methods: Raw PFT data were compared with predicted values. Each subject was subclassified into one of five groups: obstructive physiology, restrictive physiology, mixed obstructive/restrictive physiology, isolated low DL CO , or normal. The association between laboratory data of patients with decreased DL CO Sickle cell anemia (Hb-SS) results from homozygosity for a point mutation in the ␤-globin gene (HBB; Glu6Val) causing the resultant sickle hemoglobin (Hb S) to be less soluble when deoxygenated than normal hemoglobin (1). Even with improved treatment, including the early use of prophylactic antibiotic regimens, judicious transfusions, and the administration of hydroxyurea in selected patients, mortality remains high for this population. The median age at death is 42 yr for males and 48 yr for females with Hb-SS. Pulmonary complications, including acute chest syndrome (ACS), pulmonary hypertension (PH), and pulmonary fibrosis, account for 20-30% of deaths in the Hb-SS population and are often underrecognized by the health care community (2, 3).Dyspnea is a frequent complaint amongst patients with sickle cell disease, the etiology of which is unclear and likely multifacto- 5). Studies of lung function to date in this population have been of modest size, often involving fewer than 50 patients (4, 6-11) and largely inconclusive. Their results have yielded a spectrum of abnormalities, including restrictive lung disease, abnormal diffusion capacity for carbon monoxide (Dl CO ), obstructive disease, and hypoxemia (4,6,7,9,12). No definitive profile for pulmonary function in sickle cell disease has emerged. As a result, clinicians find pulmonary function tests (PFTs) difficult to interpret in this population and their clinical utility for directing further investigation and therapy has not been well evaluated.Of growing concern is the link between obstructive lung disease and ACS, particularly in children (7,13,14). The few published studies suggest that obstructive lung disease, in some cases, plays a role in the pathogenesis of ACS (7,13,15). Moreover, obstructive lung disease could be a long-term sequela of recurrent episodes of ACS (6, 9). Larger scale studies are necessary to elucidate more clearly the interaction between lung function and ACS. In addition, certain findings on PFTs might be a marker of other complications of sickle vasculopathy. For example, isolated decreased Dl CO is a well-established finding associated with PH (16, 17). However, its role as a marker or predictor of PH in the Hb-SS population is unknown. The purpose of this study is to evalua...

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Pulmonary function studies in sickle cell disease.

Cited by 44 publications

References 0 publications

Pulmonary function in sickle cell disease with or without acute chest syndrome

Pulmonary function in sickle cell disease with or without acute chest syndrome

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

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