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Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study.


ABSTRACT: In the US, mortality in sickle cell disease (SCD) increases after age 18-20?years. Biomarkers of mortality risk can identify patients who need intensive follow-up and early or novel interventions. We prospectively enrolled 510 SCD patients aged 3-20?years into an observational study in 2006-2010 and followed 497 patients for a median of 88?months (range 1-105). We hypothesized that elevated pulmonary artery systolic pressure as reflected in tricuspid regurgitation velocity (TRV) would be associated with mortality. Estimated survival to 18?years was 99% and to 25?years, 94%. Causes of death were known in seven of 10 patients: stroke in four (hemorrhagic two, infarctive one, unspecified one), multiorgan failure one, parvovirus B19 infection one, sudden death one. Baseline TRV ?2.7 m/second (>2 SD above the mean in age-matched and gender-matched non-SCD controls) was observed in 20.0% of patients who died vs 4.6% of those who survived (P =?.012 by the log rank test for equality of survival). The baseline variable most strongly associated with an elevated TRV was a high hemolytic rate. Additional biomarkers associated with mortality were ferritin ?2000??g/L (observed in 60% of patients who died vs 7.8% of survivors, P

SUBMITTER: Nouraie M 

PROVIDER: S-EPMC7735042 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature

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Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study.

Nouraie Mehdi M   Darbari Deepika S DS   Rana Sohail S   Minniti Caterina P CP   Castro Oswaldo L OL   Luchtman-Jones Lori L   Sable Craig C   Dham Niti N   Kato Gregory J GJ   Gladwin Mark T MT   Ensing Gregory G   Arteta Manuel M   Campbell Andrew A   Taylor James G JG   Nekhai Sergei S   Gordeuk Victor R VR  

American journal of hematology 20200421 7


In the US, mortality in sickle cell disease (SCD) increases after age 18-20 years. Biomarkers of mortality risk can identify patients who need intensive follow-up and early or novel interventions. We prospectively enrolled 510 SCD patients aged 3-20 years into an observational study in 2006-2010 and followed 497 patients for a median of 88 months (range 1-105). We hypothesized that elevated pulmonary artery systolic pressure as reflected in tricuspid regurgitation velocity (TRV) would be associa  ...[more]

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