Tesch et al reported the Immune Deficiency and Dysregulation Activity (IDDA) score to evaluate patients with LRBA deficiency. The authors are from multiple institutions from around the world, European Society for Blood and Marrow Transplantation and European Society for Immunodeficiencies.
Parameters:
(1) Karnofsky Performance Scale or Play Performance Scale of Lansky et al (for pediatric patients), from 0 to 100
(2) hospitalization days in percent (days per 100 days), including day clinic, excluding ICU
(3) days receiving intensive care in percent (days per 100 days)
(4) immunoglobulin substitution therapy, intravenous or subcutaneous
(5) any chronic or recurrent infestation or infection
(6) any other organ dysfunctions (cardiomyopathy, etc)
(7) nutritional status and habits
(8) malignancy including lymphoma (yes or no)
|
Parameter
|
Finding
|
Points
|
|
hospitalization days
|
< 40
|
0.1 * (percent)
|
|
|
>= 40
|
4
|
|
ICU days
|
< 10
|
0.8 * (percent)
|
|
|
>= 10
|
8
|
|
immunoglobulin
|
no
|
0
|
|
|
sporadic
|
2
|
|
|
regular
|
3
|
|
infestation/infection
|
no
|
0
|
|
|
asymptomatic
|
1
|
|
|
oligosymptomatic
|
2
|
|
|
recurring, requires intermittent therapy
|
3
|
|
|
requires continuous therapy
|
4
|
|
|
refractory to therapy
|
4
|
|
organ dysfunction
|
no
|
0
|
|
|
mild, transient
|
1
|
|
|
mild, chronic
|
2
|
|
|
moderate to severe
|
3
|
|
|
clinically compromising
|
4
|
|
nutrition
|
normal
|
0
|
|
|
modified, disease-adjusted
|
1
|
|
|
part formula, medically advised
|
2
|
|
|
tube feeding (full formula), partial parenteral
|
3
|
|
|
total parenteral
|
4
|
subscore for items 2 through 7 =
= SUM(points for parameters)
Interpretation:
• minimum subscore: 0
• maximum subscore: 25
• Karnofsky or Lansky score recorded separately.
• Malignancy recorded but not scored.
• These parameters are combined with the graded manifestations for the final IDDA.
