Description

Cappellini et al for developed a Disease Severity Scoring System (DSSS) for patients with non-transfusion-dependent thalassemia (NTDT). One component deals with the status of pediatric patients.The authors are from the University of Milan, University College London, American University of Beirut, University of Athens, Mahidol University (Bangkok) and the University of Cagliari.


 

Patient selection: pediatric non-transfusion-dependent thalassemia (NTDT), <= 16 years of age

 

Parameters:

(1) height on standardized states

(2) activity

(3) weight

(4) bone age

(5) school performance

(6) puberty

 

Parameter

Finding

Points

height

normal

0

 

3 to 9.99%

1

 

< 3%

2

activity

normal

0

 

lethargic

1

 

severely affected

2

weight

normal

0

 

3 to 9.99%

1

 

< 3%

2

bone age

normal

0

 

delayed > 2 years

1

school performance

NA

0

 

normal

0

 

suboptimal

1

puberty

NA

0

 

normal

0

 

delayed

2

 

where:

• Delayed puberty: no adrenarche at 14 years for males, no thelarche at 13 years for females

 

subscore for pediatric patients =

= SUM(points for all 6 parameters)

 

Interpretation:

• minimum subscore: 0

• maximum subscore: 10

 


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