Description

Nouraie et al reported biomarkers associated with death in young patients with sickle cell disease. These can help to identify a patient who may benefit from more aggressive management. The authors are from multiple institutions in the United States.


Patient: pediatric patient or young adult with sickle cell disease (SS, SC, etc)

 

The most common cause of death was stroke.

 

Parameters:

(1) tricuspid regurgitation velocity (TRV) in m/s

(2) serum ferritin concentration

(3) FEV1/FVC ratio

(4) neutrophil count

 

Parameter

Finding

Points

TRV

< 2.7 m/s

0

 

>= 2.7 m/s

1

serum ferritin

< 2,000 µg/L

0

 

>= 2,000 µg/L

1

FEV1 to FVC ratio

< 0.8

1

 

>= 0.8

0

neutrophil count

< 10,000 per µL

0

 

>= 10,000 per µL

1

 

number of risk factors for mortality =

= SUM(points for all of the parameters)

 

Interpretation:

• minimum number of risk factors: 0

• maximum number of risk factors: 4

• The risk of death increases with the number of risk factors present.


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