Oxyhemoglobin desaturation in patients with sickle cell disease has been proposed as a possible mechanism in the initiaton of vasco-occlusive pain crises. Nocturnal oxyhemoglobin desaturation (NOD) has been described with a prevalence of up to 40% in children and adolescents with sickle cell disease. The objective of this study was to evaluate the mechanisms of nocturnal oxyhemoglobin desaturation in sickle cell disease and determine the role of obstructive sleep apnea. We performed 16-channel polysomnograms and pulmonary function testing in 20 patients with sickle cell disease (ages 7-21 years) who had documented desaturation on home oximetry studies. The median saturation awake was 94% (quartile range, 88-95). Median saturation during REM sleep was 93.5% (88-95) and during non-REM sleep 93.5% (87.5-95). The median respiratory disturbance index was low (1.35 quartile range, 0.25-2.85). Twelve patients had no obstructive apnea recorded, while 3 patients had a total of 9 or 10 episodes during the entire study. The median snoring time was 5. 65% of total sleep time (quartile range, 1.35-22.65). There was no correlation between number of obstructive apneas and mean sleeping saturation (r = 0.012, p = 0.95). There was no correlation between pulmonary function data and prevalence of NOD. There was a strong, positive correlation between sleeping and awake saturation (r = 0.96, p < 0.001). We conclude that while nocturnal oxyhemoglobin desaturation may be common in children and adolescents with sickle cell disease, upper airway obstruction does not appear to play an important role in its genesis.
"Respiratory complications such as acute chest syndrome (ACS) and pulmonary hypertension (PH) are the most common identifiable causes of premature death in adults with SCD . Many studies of children with SCD showed hypoxia during the day, while sleeping, or after exercising –. The consequences of hypoxia in SCD children are unclear. "
[Show abstract][Hide abstract] ABSTRACT: Hypoxia is a common feature in children with sickle cell disease (SCD) that is inconsistently associated with painful crises and acute chest syndrome. To assess the prevalence and risk factors of hypoxia, we recorded daytime, nocturnal, and postexercise pulse oximetry (SpO2) values in 39 SCD patients with a median age of 10.8 years. Median daytime SpO2 was 97% (range, 89%-100%), and 36% of patients had daytime hypoxia defined as SpO2<96%. Median nocturnal SpO2 was 94.7% (range, 87.7%-99.5%), 50% of patients had nocturnal hypoxia defined as SpO2≤93%, and 11(37%) patients spent more than 10% of their total sleep time with SpO2<90%. Median postexercise SpO2 was 94% (range, 72%-100%) and 44.7% of patients had postexercise hypoxia defined as an SpO2 decrease ≥3% after a 6-minute walk test. Among patients with normal daytime SpO2, 35% had nocturnal and 42% postexercise hypoxia. Compared to 9 patients without daytime, nocturnal, or postexercise hypoxia, 25 patients with hypoxia under at least one of these three conditions had greater anemia severity (P = 0.01), lower HbF levels (P = 0.04), and higher aspartate aminotransferase levels (P = 0.03). Males predominated among patients with postexercise hypoxia (P = 0.004). Hypoxia correlated neither with painful crises nor with acute chest syndrome. Of 32 evaluable patients, 6 (18.8%) had a tricuspid regurgitation velocity ≥2.6 m/s, and this feature was associated with anemia (P = 0.044). Median percentage of the predicted distance covered during a 6-minute walk test was 86% [46-120]; the distance was negatively associated with LDH (P = 0.044) and with a past history of acute chest syndrome (P = 0.009). In conclusion, severe episodes of nocturnal and postexercise hypoxia are common in children with SCD, even those with normal daytime SpO2.
PLoS ONE 05/2014; 9(5):e97462. DOI:10.1371/journal.pone.0097462 · 3.23 Impact Factor
"One mechanism which may account for the presence of executive dysfunction in the absence of obvious neuropathology is low hemoglobin oxygen saturation associated with sleep-disordered breathing (SDB) and the resulting sleep fragmentation caused by increased sleep arousals. SDB is relatively common in children with SCA, with one group reporting 40% affected (Needleman et al., 1999). Sleep disruption has been shown to have a negative impact on attention and executive function in adults (Waters & Bucks, 2011). "
[Show abstract][Hide abstract] ABSTRACT: Previous research has identified cognitive impairment in children with sickle cell anemia (SCA, Hemoglobin SS) compared with controls, partly accounted for by overt neuropathology after clinical stroke, "covert" ("silent") infarction, and severity of anemia. However, cognitive deficits have also been identified in children with SCA with no history of stroke and a normal T2-weighted magnetic resonance imaging (MRI) scan. Our aim was to investigate whether nocturnal hemoglobin oxygen desaturation and sleep fragmentation could be associated with cognitive impairment in children with SCA. We assessed 10 children with SCA (9 with normal MRI) using neuropsychological measures of executive function. Cognitive assessment was immediately followed by overnight polysomnography to record nocturnal hemoglobin oxygen saturation and sleep arousals. Decreases in hemoglobin oxygen saturation and/or increased sleep arousals were associated with reduced performance on cognitive assessment. Nocturnal hemoglobin oxygen desaturation and sleep fragmentation may be a contributing factor to executive dysfunction in SCA.
Journal of the International Neuropsychological Society 11/2011; 18(1):168-73. DOI:10.1017/S1355617711001469 · 2.96 Impact Factor
"Impairment of NO bioavailability over decades leads to chronic vasoconstriction and mild pulmonary hypertension (Reiter et al, 2002; Gladwin et al, 2004; Morris et al, 2005; Rother et al, 2005). Hypoxemia in sickle cell disease may result from anaemia, intrinsic lung disease or upper airway obstruction (Samuels et al, 1992; Rackoff et al, 1993; Needleman et al, 1999). Sickle cell patients with hypoxia have been found to have an increase "
[Show abstract][Hide abstract] ABSTRACT: Elevated pulmonary artery pressures (PAP) occur in approximately 30% of children with sickle cell disease. In adults, pulmonary hypertension is significantly associated with mortality. There are no data on the long term significance in children. Nineteen children with SS/Sbeta(0) thalassaemia had elevated PAP, defined as tricuspid regurgitant jet velocity (TRV) > or =2.5 m/s on screening echocardiograms. They were prospectively followed for 23 months (range 19-31 months). Patients with initial TRV > or = 3 or TRV > or = 2.5 m/s on repeat echocardiogram had cardiopulmonary evaluation and were offered treatment with hydroxyurea. Associated conditions like asthma and obstructive sleep apnea were treated. 18/19 patients had follow-up echocardiograms. These showed normalization of TRV in 8 patients. Risk factors associated with persistent elevation were higher TRV on initial echocardiogram (P = 0.01), lower haemoglobin (P = 0.003) and lower oxygen saturation (P = 0.03). Five patients with persistently elevated PAP were treated with hydroxyurea. Mean right ventricular pressure dropped from 40.16 to 29.26 (P = 0.017) after 3-6 months and to 23.6 mmHg (P = 0.002) after 9-12 months on treatment. In conclusion (i) At borderline elevation of TRV there is intrapatient variability and echocardiograms should be repeated for confirmation. (ii) Elevated PAP are reversible in children with early detection and treatment with hydroxyurea.
British Journal of Haematology 03/2009; 144(5):736-41. DOI:10.1111/j.1365-2141.2008.07501.x · 4.71 Impact Factor
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