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Disease Severity Scoring System (DSSS) of Cappellini et al for Non-Transfusion-Dependent Thalassemia (NTDT): Liver and Gallbladder Status

Description

Cappellini et al for developed a Disease Severity Scoring System (DSSS) for patients with non-transfusion-dependent thalassemia (NTDT). One component deals with liver and gallbladder status.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: non-transfusion-dependent thalassemia (NTDT)

 

Parameters:

(1) serum ALT relative to upper limit of normal (ULN, current or within past 3 months)

(2) liver disease (current or any history)

(3) hepatitis B or C

(4) indirect serum bilirubin relative upper limit of normal (ULN, current or within past 3 months)

(5) gallbladder status

 

Parameter

Finding

Points

serum ALT

<= 3 times ULN

0

 

> 3 times ULN

1

liver disease

absent

0

 

fibrosis

1

 

compensated cirrhosis

2

 

uncompensated cirrhosis

3

 

hepatocellular carcinoma

4

viral hepatitis

negative

0

 

positve ELISA

1

 

significant viral load (RNA positive)

2

indirect bilirubin

<= 3 times ULN

0

 

3.01 to 6 times ULN

1

 

6.01 to 10 times ULN

2

 

> 10 ULN

3

gallbladder

none

0

 

cholecystectomy

1

 

history of cholecystis or cholelithiasis

1

 

active cholecystis  or cholelithiasis

2

 

Where:

• A positive ELISA for hepatitis B should not be due to vaccination.

• The wording of gallbladder disease is ambiguous. It is unclear if cholelithiasis and cholecystitis are graded separately or if both can be active separately.

 

subtotal for liver and gallbladder status =

= SUM(points for all 5 parameters)

 

Interpretation:

• minimum subscore: 0

• maximum subscore: 12

 


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