Correlates and associations of pulse pressure with kidney function and hemolysis.

<p><b>A</b>, Pulse pressure has a significant positive correlation with the hemolytic component in HbSS patients, but not in HbSC patients. <b>B</b>, Pulse pressure has a significant positive correlation with serum creatinine in both HbSS and HbSC patients. <b>C</b>, Elevated pulse pressure is significantly associated with presence of proteinuria in HbSS patients, while the association is not significant in HbSC patients.</p

Independent predictors of pulse pressure.

<p>*transformed using the natural log function.</p><p>Independent predictors of pulse pressure.</p

Correlations of pulse pressure with clinical and laboratory characteristics by hemoglobin genotype.

<p>*p values <0.002 remained significant after Bonferroni's adjustment for multiple comparisons.</p>†<p>N-terminal prohormone of brain natriuretic peptide.</p><p>Correlations of pulse pressure with clinical and laboratory characteristics by hemoglobin genotype.</p

Severe Painful Vaso-Occlusive Crises and Mortality in a Contemporary Adult Sickle Cell Anemia Cohort Study

<div><p>Background</p><p>Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort.</p> <p>Methods</p><p>Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation.</p> <p>Results</p><p>Higher baseline hematocrit (p = 0.0008), ferritin (p = 0.005), and HDL cholesterol (p = 0.01) were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03). Higher tricuspid regurgitatant jet velocity (TRV) (RR 2.41, 95% CI 1.5-3.9, p < 0.0001), elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001) and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001) were also independent risk factors for mortality. </p> <p>Conclusions</p><p>Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies.</p> <p>Trial Registration</p><p>ClinicalTrials.gov NCT00011648 <a href="http://clinicaltrials.gov/" target="_blank"><u>http://clinicaltrials.gov/</u></a></p> </div

Kaplan Meier (KM) curve showing survival in sickle cell anemia by severe pain crises requiring an ED visit/ hospitalization in past year.

<p>Kaplan Meier (KM) curve showing survival in sickle cell anemia by severe pain crises requiring an ED visit/ hospitalization in past year.</p

Kaplan-Meier Analysis of Survival Time by TRV and BNP.

<p>Longer survival times were observed for a) subjects with TRV less than 3.0 m/sec (p<0.0001) and b) for subjects with BNP levels less than 160 pg/mL (p = 0.0003).</p

Study Flowchart.

<p>Study Flowchart.</p

Characteristics of Patients in Screening Cohort.

1<p>Unless otherwise indicated.</p

Cox Proportional Hazards Regression Analysis of Mortality for Demographic, Clinical, and Laboratory Characteristics.

1<p>Hazard ratios presented for 75^th relative to the 25^th percentile, unless otherwise indicated. All results are unadjusted.</p>2<p>Transformed using the log or square root function.</p>3<p>Adjusted for site-specific differences in normal ranges.</p

Cox Proportional Hazards Regression Analysis of Mortality for Composite of TRV and BNP.

1<p>HR is given for the combined influence of TRV and BNP on mortality as defined by TRV levels ≥3.0 or BNP levels ≥160, or both, relative to TRV<3.0 and BNP<160.</p>2<p>Adjusted for ferritin.</p>3<p>HR presented for 75^th relative to the 25^th percentile, calculated as e^{coefficient×(75th percentile–25th percentile)}, and adjusted for TRV/BNP composite.</p