Pulmonary Hypertension in Children and Adolescents with Sickle Cell Disease.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3787-3787 ◽  
Author(s):  
Aziza Sedrak ◽  
Sreedhar P. Rao ◽  
Scott T. Miller ◽  
Vahid Hekmet ◽  
Madu Rao
Keyword(s):  
Obstructive Sleep Apnea ◽  
Pulmonary Hypertension ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Pulmonary Function ◽  
Sickle Cell ◽  
Pulmonary Function Tests ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Obstructive Sleep

Abstract Pulmonary hypertension occurs in 20–40% of adults with sickle cell disease and has a poor prognosis; age of onset and prevalence in childhood are not well established. In adults, recurrent acute chest syndrome is associated with chronic sickle cell lung disease and perhaps pulmonary hypertension. Hematologically normal children with obstructive sleep apnea are at risk for pulmonary hypertension. We sought to determine the prevalence of pulmonary hypertension in children and adolescents with sickle cell disease, explore potential association with abnormal pulmonary function or sleep apnea, and identify other clinical or laboratory factors associated with this potentially life-threatening complication. Forty-eight patients (age 5–21 years, median 12 years) consented to participate. Thirty-eight (79%) had Hb SS; five each had Hb SC and S-beta thalassemia (three beta plus and two beta zero) (10.4%). Eleven (22.9%) were on chronic transfusion (seven for stroke, three for abnormal TCD velocity screening, and one for recurrent pain crises); nineteen (39.5%) were on hydroxyurea (ten for recurrent acute chest syndrome and nine for recurrent pain). A detailed history and physical examination were done on all enrolled subjects (history was comprehensive, but specifically included cardiopulmonary symptoms; neurological problems; number of pain crises and acute chest syndrome; blood transfusions; use of hydroxyurea; and a screening for obstructive sleep apnea). If apnea history was suggestive, polysomnography was done. In addition, all study subjects had Doppler echocardiography and pulmonary function tests (PFT). Pulmonary hypertension was defined as an age and body mass index-adjusted tricuspid regurgitant jet velocity (TRV) of greater than 2.5 mm/sec. Among the study group, 31 (64.5%) had pulmonary function tests; 17 (54.8%) had restrictive abnormalities on PFT, three (9.6%) had obstructive changes and 11 (35.4%) had normal PFT. Three patients (6.2%) had a history suggestive of apnea, then polysomnography; one was normal and two had OSA. Four of the 48 patients (8.3%) had PHT (TRV values 2.52, 2.55, 2.61, and 2.91). All had Hb SS. Two were age 17 and 18 years and two were 10 and 11. All were asymptomatic. One had restrictive PFT and none had OSA. There was no correlation between the presence of PHT and pulmonary function abnormalities or sleep apnea. Of the various other clinical and laboratory parameters examined, only an elevated serum indirect bilirubin was associated with PHT; there was a trend toward association with elevated reticulocyte count and low fetal Hb levels. In summary, the prevalence of PHT our pediatric sickle cell population is 8.3%. As reported in adults, there may be an association between PHT and more severe hemolysis. We could find no association with abnormal pulmonary function or obstructive sleep apnea. Characteristics of study patients PHT No PHT P Value Fetal Hb(%) (Mean) 3.7 7.9 .154 Age (Mean) 13.5 years 12 years .564 Reticulocytes (%) 13.8 8.25 .087 Indirect bilirubin (mg/dl) (Mean) 5.4 2.2 .027

Adverse Events in Sickle Cell Patients with Nocturnal Hypoxia.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3793-3793
Author(s):  
Andrew D. Campbell ◽  
Megumi Okumura ◽  
Ndidi Unaka ◽  
Sally Hutchinson ◽  
Onyinye Onyekwere
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Case Series ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Obstructive Sleep ◽  
The Relationship

Abstract The relationship between hemoglobin dexoygenation and sickling is well known. However, the relationship between hypoxia and severity of disease in sickle cell patients has not been well established. Recently, nocturnal hypoxemia has been associated with higher incidence of CNS events including strokes, and elevated TCDs. We present our case series on 13 patients(12 SS, 1 SC) with sickle cell disease (SCD) who have nocturnal hypoxia. Approximately 75 patients were screened at the University of Michigan Sickle Cell clinic for nocturnal hypoxia either by symptoms of obstructive sleep apnea or by longitudinal baseline clinic 02 saturations (02 Sat <92%). Of the 13 hypoxic pts, median baseline O2 Sat 90%(n=13, mean 90) and the median nocturnal O2sat (Nctnl 02 sat) 84%(n=13, mean 80%) with 10/13 with moderate-severe nocturnal hypoxia (O2sats<85%)based on sleep studies. Multiple adverse events noted in the cohort were pulmonary hypertension (PHTN TRJV>2.5, n=9, median 2.74/mean 2.74,) frequent pain episodes(>3visits to ER or hospitalizations/year, n=7, with 5 pts >5/year ), recurrent acute chest syndrome( ≥ 3 episodes, n= 10), CNS events (n=3 silent infarcts, vascular stenosis), priapism( (n=4, among 6 males ). Also reported were possible causes of the underlying hypoxia including obstructive sleep apnea(OSA)(n=7 of 11 pts), asthma(n= 10 of 13 pts), and chronic lung disease( n=8). In conclusion, the persistence of nocturnal hypoxia in pediatric sickle cell disease could possibly contribute to the development of severe complications of sickle cell disease. Treatment of underlying hypoxia (ie nighttime oxygen, maximize asthma treatment, T&A for OSA)may help prevent complications and lead to the improvement clinical symptoms. Further, chronic nocturnal hypoxia may complicate pulmonary disease and accelerated the development of PHTN. More studies are needed to clarify the mechanism of hypoxia in SCD. Table I. Clinical &Demographic Data of 13 SCD Patients with Nocturnal Hypoxia. Age:(6–22y/o, mean 15) Sex: M=6 F=7 Clinical: Total Mild Mod Sev. Genotype: SS=12, SC=1 Mean Range Nctnl Hypoxia(<%) 13 3 5(<85) 5(<80) Baseline O2 Sat(%) 90 +3.0 86–97 Obstr Sleep Apnea 7 3 3 1 Nctnl 02 sat (%) 80+8.4 59–87 Pulm HTN 9 4 4 1 Total #Apneic Events(11) 65.6+80 6–256 Rest. Lung Ds. 8 2 5 1 Obstr. Apneic Events(7) 27+68.5 0–221 # of Episodes <3 3–4 >4 Hypopneic Events(9) 32.5+38 0–132 ACS 2 5 5 TRJet Velocity 2.74+.42 2–3.5 Severe Pain Crises/yr 1 2 5

scholarly journals Nocturnal Hypoxemia Rather Than Obstructive Sleep Apnea Is Associated With Decreased Red Blood Cell Deformability and Enhanced Hemolysis in Patients With Sickle Cell Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Emeric Stauffer ◽  
Solène Poutrel ◽  
Giovanna Cannas ◽  
Alexandra Gauthier ◽  
Romain Fort ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Pulmonary Hypertension ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Severity ◽  
Cell Disease ◽  
Nocturnal Hypoxemia ◽  
Obstructive Sleep ◽  
Rbc Deformability

Background: Although obstructive sleep apnea (OSA) could act as a modulator of clinical severity in sickle cell disease (SCD), few studies focused on the associations between the two diseases.Research Question: The aims of this study were: (1) to explore the associations between OSA, nocturnal oxyhemoglobin saturation (SpO2) and the history of several acute/chronic complications, (2) to investigate the impact of OSA and nocturnal SpO2 on several biomarkers (hematological, blood rheological, and coagulation) in patients with SCD.Study Design and Methods: Forty-three homozygous SCD patients underwent a complete polysomnography recording followed by blood sampling.Results: The proportion of patients suffering from nocturnal hypoxemia did not differ between those with and those without OSA. No association between OSA and clinical severity was found. Nocturnal hypoxemia was associated with a higher proportion of patients with hemolytic complications (glomerulopathy, leg ulcer, priapism, or pulmonary hypertension). In addition, nocturnal hypoxemia was accompanied by a decrease in RBC deformability, enhanced hemolysis and more severe anemia.Interpretation: Nocturnal hypoxemia in SCD patients could be responsible for changes in RBC deformability resulting in enhanced hemolysis leading to the development of complications such as leg ulcers, priapism, pulmonary hypertension or glomerulopathy.Clinical Trial Registration:www.ClinicalTrials.gov, identifier: NCT03753854.

scholarly journals The burden of obstructive sleep apnea in pediatric sickle cell disease: a Kids’ inpatient database study

SLEEP ◽  
2020 ◽  
Author(s):  
Po-Yang Tsou ◽  
Christopher M Cielo ◽  
Melissa S Xanthopoulos ◽  
Yu-Hsun Wang ◽  
Pei-Lun Kuo ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Obstructive Sleep ◽  
Increased Risk ◽  
Secondary Outcomes ◽  
Inpatient Database

Abstract Study Objectives Obstructive sleep apnea (OSA) is associated with cardiovascular and cerebrovascular morbidity. Patients with sickle cell disease (SCD) are at increased risk for both neurologic complications (NC) and OSA. However, the relationship between OSA and SCD complications is unclear. We hypothesized that there would be an association between OSA diagnosis and SCD complications. Methods Hospital discharge records of patients with SCD aged < 19 years were obtained for the years 1997, 2000, 2003, 2006, 2009, and 2012 from the Kid’s Inpatient Database. The primary outcome, NC, a composite of stroke, transient ischemic attack, and seizures. Secondary outcomes included acute chest syndrome (ACS), vaso-occlusive crisis, length of hospital stay, and inflation-adjusted cost of hospitalization. Multivariable regression was conducted to ascertain the association of OSA with primary and secondary outcomes. Analyses were adjusted for the use of noninvasive mechanical ventilation (NIMV) to determine its role as NC risk modifier. Results There were 203,705 SCD discharges included in the analysis, of which 2,820 (1.4%) and 4,447 (2.2%) also included OSA and NC diagnoses. Multivariable logistic regression indicated that OSA was associated with NC (adjusted odds ratio [OR], 1.50 [95% CI 1.02–2.21], p = 0.039) and ACS (OR, 1.34 [95% CI 1.08–1.67], p = 0.009) in children with SCD. In the multivariable analysis adjusted for NIMV, the significant association between OSA and NC was no longer observed (OR, 1.39 [95% CI 0.94–2.05], p = 0.100). Conclusions OSA is associated with a 50% increase of odds of NC in children with SCD in this nationwide dataset. The use of NIMV to treat OSA may modify the risk of OSA-associated NC.

Screening for Obstructive Sleep Apnea in Children With Sickle Cell Disease: A Pilot Study

2020 ◽  
Author(s):  
Andrew J. Maroda ◽  
Matthew N. Spence ◽  
Stephen R. Larson ◽  
Jeremie H. Estepp ◽  
M. Boyd Gillespie ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Pilot Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Obstructive Sleep

Sickle Cell Disease Is a Risk Factor for Obstructive Sleep Apnea

CHEST Journal ◽  
2010 ◽  
Vol 138 (4) ◽  
pp. 322A
Author(s):  
JoAnn Eng ◽  
George A. Apergis ◽  
Samir Fahmy
Keyword(s):  
Obstructive Sleep Apnea ◽  
Risk Factor ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Obstructive Sleep

Abnormal Pulmonary Function in Adults with Sickle Cell Disease: Association of Decreased DLCO with Systemic Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 316-316 ◽  
Author(s):  
Elizabeth S. Klings ◽  
Diego F. Wyszynski ◽  
Vikki G. Nolan ◽  
Martin H. Steinberg
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Function ◽  
Sickle Cell ◽  
Pulmonary Function Tests ◽  
Systemic Disease ◽  
The United States ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Restrictive Physiology ◽  
Abnormal Pulmonary Function

Abstract Pulmonary complications of sickle cell disease (SCD), including acute chest syndrome, pulmonary hypertension (PH) and pulmonary fibrosis, are common. Dyspnea and hypoxemia are equally common in this population. It is likely that pulmonary function tests (PFT) are abnormal in the SCD population, however, no extensive study has been reported to date. Moreover, the relationship between abnormal pulmonary function and other manifestations of SCD, such as PH, is unclear. We hypothesized that abnormalities of pulmonary function, particularly a low diffusion capacity for carbon monoxide (DLCO), may be associated with other complications of SCD. The Cooperative Study of Sickle Cell Disease (CSSCD) enrolled and followed more than 4,000 SCD patients who had visited one of 23 participating clinical centers across the United States between 1978 and 1998. Data were collected on many complications of the disease, and standardized collection of PFTs were part of the protocol. From the more than 1300 CSSCD patients who had the results of PFTs recorded, 310 adults (age≥ 20 years of age) homozygous for the Hb S gene without coincident α thalassemia and with sufficient data were identified. Predicted values for FEV1, FVC, FEV1/FVC, TLC, RV and DLCO were calculated using algorithms that accounted for gender, age, and height in the African American population (using STATA, version 9); data are presented as percent predicted. Based on criteria established by the American Thoracic Society, subjects were sub-classified into 7 groups: obstructive physiology; restrictive physiology; mixed obstructive/restrictive disease; low lung volumes with normal spirometry (LLV); LLV with a low DLCO, isolated low DLCO, or normal. The association of blood counts and serum chemistries between patients with low DLCO compared with those with a normal DLCO was assessed by multivariate linear regression (using SAS software version 8.2). Normal PFTs were present in only 31 of 310 (10 %) SCD patients. Overall, the adult SCD population was characterized by decreased total lung capacities (70.2 + 14.7% predicted) and DLCO (64.5 + 19.9 % predicted adjusted for hemoglobin concentration). The most common PFT patterns observed were restrictive physiology (35.8%), LLV with normal spirometry (34.2% of patients), and an isolated low DLCO (12.9%). The presence of a low DLCO was associated with an elevated platelet count (p=0.05), hepatic dysfunction [elevated ALT (p=0.07) with elevated AST (p=0.01)] and renal dysfunction [elevated BUN and creatinine (p=0.05, 0.07)]. Restrictive disease is marginally associated with a decrease in hematocrit (p=0.07) and Hb F levels (p=0.07). Pulmonary function is abnormal in 90% of adult SCD patients. Common abnormalities include restrictive physiology, LLV with normal spirometry and a decreased DLCO. The presence of a decreased DLCO may be a marker of more severe systemic disease that includes impaired renal and hepatic function and possibly complications of hemolytic anemia such as PH.

Sign in / Sign up

Export Citation Format

Share Document