الفهرس 
?-ThalassemiaOnly 14 pages are availabe for public view
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Abstract
Sleep-disordered breathing is described in children with SCD and nocturnal desaturation is related to pain episodes, as well as extramedullary hematopoiesis causes sleep apnea syndrome in thalassemic patients.
Sleep-disordered breathing is associated with LV hypertrophy, endothelial dysfunction, diastolic dysfunction and a pro inflammatory state leading to increased cardiovascular risk.
In the present study we aimed at studying the frequency of sleep apnea in chronic hemolytic anemia patients and its effect on left ventricular diastolic functions and dimensions in comparison to normal children.
One-hundred children with chronic hemolytic anemia (β-thalassemia major, sickle cell disease patients and thalassemia intermedia) were eligible for the study who were following in Ain shams university hematology and oncology clinic and 100 age and sex matched healthy subjects were all subjected to PSQI.
26 patients (26%) habitually snored as proved by PSQI score (15 β- thalassemia major (42.3%) and 11 Sickle cell disease patients (57.6) had score ≥5). There were 15(60%) males and 10(40%) females their mean age was 14.6± 2.8 (range 8-18).
Twenty five age and sex matched healthy individuals served as a control group. They were 17(68%) males and 8(32%) females with mean age 13.58±2.7 years (range 7-18).
from the course of this study we found the following:
The average awake SpO2 was 93.15±2.48% (range 89-97%) and the average sleep SpO2was 91.5%±3.44% (range 85-96%) in thalassemia major patients.
While in sickle cell disease patients the average awake SpO2 was 93.18 %±3.74 % (range 87-98%) and the average sleep SpO2 was 92.8% ±3.54% (range 87-97%).
73.3% of thalassemia major patient had sleep O2 saturation <95%, 45.5% of sickle cell disease patients have sleep O2 saturation <95%.
AHI was >1 in 40% and 27.3% of thalassemia and sickle cell disease patients respectively,We found that 13.3% of thalassemic patients and 9% of sickle patients have OAHI ≥1 and they were of the mild type.
The average sleep efficiency was 91.1±3.73% (range 70-98%) and 89.8%±4.76 (range 66-95%) with sleep efficiency <90% in 33.3% and 45.5% in thalassemia and sickle patients respectively.
The median arousal index was 12.9/hour and 7.5/hour with arousal index >5 in 33.3% and 9% in thalassemia major and sickle cell disease patients respectively, with significant difference between two groups (p =0.043)
As regards ECHO findings
In thalassemia major patients
• All have increased myocardial performance indices as assessed by TDI.
• All have increased LVMI.
• All have normal LV systolic functions as assessed by FS% and EF% but decreased systolic functions assessed by TDI (Sm).
• 72.7% have LV diastolic dysfunction and increased estimated LV filling pressure (E/Em).
• 80%have Em/Am ratio<1.
• All have increased pulmonary pressure (RVESP>30mmHg). Mild (38.46%), moderate (57.6%) and sever (3.8%).
• 53.3% have TRjet >2.5m/sec.
In sickle cell disease patients:
• All have increased myocardial performance indices as assessed by TDI.
• All have increased LVMI.
• All have normal LV systolic functions as assessed by FS% and EF% but decreased systolic functions assessed by TDI (Sm) in (45.4%).
• 76% have LV diastolic dysfunction and increased estimated LV filling pressure (E/Em).
• 54.5%have Em/Am ratio<1.
• All have increased pulmonary pressure (RVESP>30mmHg). Mild (18%), moderate (82%) and sever (0%).
• 81.8% have TRjet >2.5m/sec.
Systolic functions assessed by TDI was worse in thalassemia major patients than sickle patients as measured by Sm (p= 0.005).
Statistical significant increase in LVMI in thalassemic patients compared to sickle patients (p=0.027).
LV diastolic functions as assessed by E/Em ratio was increased in sickle patients (76%) than in thalassemic one (72.7%) with (p=0.033).
LVMPI was statistical significantly higher in sickle patients than thalassemia major patients (p<0.001).
In thalassemia major patients we found:
• Statistically significant positive correlation was found between RVESP, TR jet and systolic and diastolic blood pressure (p= 0.026, <0.001).
• Statistically significant negative correlation was found between RVESP, TRjet and awake and sleep O2 saturation (P = 0.001 and 0.008 respectively).
• Statistically significant positive correlation was found between LVMPI and blood pressure (p = 0.021, 0.011).
• Statistically significant negative correlation was found between LVMPI and awake O2 saturation, sleep maintenance efficiency (P = 0.015 and 0.050 respectively).
• Statistically significant positive correlation was found between LVMI and diastolic blood pressure (p = 0.004).
• Statistically significant negative correlation was found between LVMI and awake O2 saturation, sleep maintenance efficiency (P = 0.006, 0.044 respectively).
• Statistically significant positive correlation was found between AHI and RVESP and LVMI (p = 0.049 and 0.023 respectively).
• RVESP and E/Em were statistically significant higher in thalassemia major patients with O2 saturation <95% when compared to those with O2 saturation ≥95% (p=0.016 and 0.011).
• RVESP and E/Em were statistically significant higher in thalassemia major patients with sleep O2 saturation <95% when compared to those with sleep O2 saturation ≥95% (p=0.016 and 0.011).
In multivariable analysis we found:
• A significant positive correlation was found between LV mass index and BP (p = 0.041).
• A significant negative correlation was found between sleep O2 saturation and LVEDD (p = 0.021).
• A significant positive correlation between LV mass index and BP (p=0.041).
In sickle cell disease patients we found:
A statistically significant negative correlation was found between RVESP, TR jet and E/Em and awake O2 saturation (p = 0.031, 0.026 and 0.025 respectively).
A statistically significant negative correlation was found between RVESP and E/EmO2 and saturation sleep (p = 0.024 and 0.041).
A statistical significant negative correlation was found between both LVEDD and E/Em and sleep O2 saturation (p<0.001, 0.041 respectively).
LVMPI was statistically significant higher in sickle patients with sleep O2 saturation <95% when compared to those with sleep O2 saturation ≥95%. (p = 0.036).
LVMPI was statistically significant higher in sickle patients with O2 saturation <95% when compared to those with O2 saturation ≥95%. (p = 0.036).
In SCD patients there is statistically significant positive correlation between mean serum ferritin and AHI, and statistically significant negative correlation when compared to awake O2 saturation (p=0.039, 0.013 respectively).
In multi variable analysis we found:
A statistically significant negative correlation was found between LVEDD and sleep maintenance efficiency (p<0.001).
A statistically significant positive correlation was found between LVEDD and blood pressure, heart rate and BMI (P= 0.003, 0.001 and 0.006 respectively).
A statistical significant negative correlation was found between both LVEDD and E/Em and sleepO2 saturation (p<0.001, 0.041 respectively).
A statistically significant negative correlation was found between LVEDD and sleep maintenance efficiency (p<0.001).
A statistical significant negative correlation was found in sickle patients between both LVESD and E/Em and awakeO2 saturation (p =0.007 and 0.045 respectively).
In conclusion this study has showed that:
1- (72.7%) ofthalassemia major patients and (76%) of SCD patients have left ventricular diastolic dysfunction and all patients have left ventricular hypertrophy.
2-There is Correlation between pulmonary hypertension, increased myocardial performance index assessed by TDI and low sleep O2 saturation in thalassemia major patients and correlation between increased left ventricular dimensions, diastolic dysfunctions and low sleep O2 saturation in sickle cell disease patients.