Airway hyperreactivity in children with sickle cell disease

Leong, Mila A.; Dampier, Carlton; Varlotta, Laurie; Allen, Julian L.

doi:10.1016/s0022-3476(97)70166-5

Cited by 132 publications

(115 citation statements)

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“…It is possible that our definitions of asthma and allergy, based on parent report of symptoms, were not reliable enough to pick up this association. A parent report of doctordiagnosed asthma has been associated with an increased rate of pain and acute chest syndrome, [42][43][44][45][46] so this definition has clinical relevance in this SCA sample. By using more quantitative measures of allergy and respiratory disease, we found that atopy was not associated with OSAS, but reduced FEV 1 and FEV 1 :FVC were, suggesting an association of lower airway disease with OSAS.…”

Section: Discussionmentioning

confidence: 91%

Obstructive Sleep Apnea and Sickle Cell Anemia

et al. 2014

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WHAT'S KNOWN ON THIS SUBJECT: Obstructive sleep apnea syndrome (OSAS) prevalence in children with sickle cell anemia is not well described. Although these children often experience nocturnal oxygen desaturation, it is unclear whether they are more likely to have OSAS.WHAT THIS STUDY ADDS: Children with sickle cell anemia have a high prevalence of OSAS with typical symptoms, beyond just nocturnal oxyhemoglobin desaturation. This study supports the need for increased efforts to screen for, diagnose, and treat OSAS in this vulnerable population. abstract OBJECTIVE: To ascertain the prevalence of and risk factors for obstructive sleep apnea syndrome (OSAS) in children with sickle cell anemia (SCA). METHODS:Cross-sectional baseline data were analyzed from the Sleep and Asthma Cohort Study, a multicenter prospective study designed to evaluate the contribution of sleep and breathing abnormalities to SCArelated morbidity in children ages 4 to 18 years, unselected for OSAS symptoms or asthma. Multivariable logistic regression assessed the relationships between OSAS status on the basis of overnight inlaboratory polysomnography and putative risk factors obtained from questionnaires and direct measurements. RESULTS:Participants included 243 children with a median age of 10 years; 50% were boys, 99% were of African heritage, and 95% were homozygous for b S hemoglobin. OSAS, defined by obstructive apnea hypopnea indices, was present in 100 (41%) or 25 (10%) children at cutpoints of $1 or $5, respectively. In univariate analyses, OSAS was associated with higher levels of habitual snoring, lower waking pulse oxygen saturation (SpO 2 ), reduced lung function, less caretaker education, and non-preterm birth. Lower sleep-related SpO 2 metrics were also associated with higher obstructive apnea hypopnea indices. In multivariable analyses, habitual snoring and lower waking SpO 2 remained risk factors for OSAS in children with SCA. CONCLUSIONS:The prevalence of OSAS in children with SCA is higher than in the general pediatric population. Habitual snoring and lower waking SpO 2 values, data easily obtained in routine care, were the strongest OSAS risk factors. Because OSAS is a treatable condition with adverse health outcomes, greater efforts are needed to screen, diagnose, and treat OSAS in this high-risk, vulnerable population.

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Section: Discussionmentioning

confidence: 91%

Obstructive Sleep Apnea and Sickle Cell Anemia

et al. 2014

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“…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).…”

mentioning

confidence: 99%

“…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).…”

mentioning

confidence: 99%

“…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).…”

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confidence: 99%

“…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). No definitive profile for pulmonary function in sickle cell disease has emerged.…”

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confidence: 99%

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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

187

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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Dysregulated Arginine Metabolism, Hemolysis-Associated Pulmonary Hypertension, and Mortality in Sickle Cell Disease

Morris¹

2005

JAMA

657

682

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L-ARGININE, THE SUBSTRATE FOR nitric oxide (NO) synthesis, is deficient in sickle cell disease (SCD). [1][2][3][4] Increased NO consumption by cell free plasma hemoglobin 5 and reactive oxygen species 6,7 leads to decreased NO bioavailability 8,9 that is exacerbated by decreased availability of the NO synthase substrate L-arginine. This state of resistance to NO is accompanied by a compensatory up-regulation of NO synthase and non-NO-dependent vasodilators. [10][11][12][13] Under conditions of low arginine concentration, NO synthase is uncoupled, producing reactive oxygen species in lieu of NO, 14,15 potentially further reducing NO bioavailability in SCD and enhancing oxidative stress. Recent reports of elevated arginase activity in SCD [16][17][18] offer another avenue for decreasedargininebioavailability.Arginase, an enzyme that converts L-arginine to ornithine and urea, can limit NO bioavailability through increased consumption of the substrate for NO synthase. [19][20][21] Arginase, which is found predominantly

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Airway hyperreactivity in children with sickle cell disease

Cited by 132 publications

References 24 publications

Obstructive Sleep Apnea and Sickle Cell Anemia

Obstructive Sleep Apnea and Sickle Cell Anemia

Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

Dysregulated Arginine Metabolism, Hemolysis-Associated Pulmonary Hypertension, and Mortality in Sickle Cell Disease

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