Olupot-Olupot et al reported a simple score to screen a child being admitted to a hospital in Africa for sickle cell disease. The authors are from multiple institutions in Uganda, Kenya, and England.
Patient selection: child being admitted to hospital with severe anemia, not previously diagnosed with sickle cell disease.
Prevalence of sickle cell disease in sub-Saharan Africa: 1%
Parameters:
(1) age in months
(2) sibling with sickle cell disease
(3) malaria status
(4) WBC count in 10^9/L
(5) MCV in femtoliters
(6) platelet count in 10^9/L
Parameter
Finding
Points
age
< 12 months
1
>= 12 months
0
sibling with sickle cell
no
0
yes
1
malaria
negative
2
positive
0
WBC count
< 10 * 10^9/L
0
10 to 19 * 10^9/L
1
20 to 29 * 10^9/L
2
>= 30 * 10^9/L
3
MCV
< 90 fL
0
>= 90 fL
1
platelet count
< 50 * 10^9/L
0
50 to 250 * 10^9/L
2
> 250 * 10^9/L
1
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 10
• A score >= 5 was 73% sensitive and 85% specific.
Other risk factors for sickle cell disease:
(1) gross splenomegaly
(2) history of previous blood transfusion
Performance:
• The area under the ROC curve is 0.86.
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