Damy et al reported a disease severity score for a patient with hereditary transthyretin amyloidosis (ATTRv). It has several subscores for different organ involvement. The authors are from multiple institutions in Europe.
Patient selection: hereditary transthyretin amyloidosis
Subscore
Maximum
cardiovascular involvement
6.02
cardiac disease
10.44
GI involvement
2.43
urogenital disease
0.87
skin ulceration
0.10
proprioception
0.37
tactile sensation
2.83
motor function
15.86
total score =
= SUM(values of all the subscores)
Interpretation:
• minimum score: 0
• maximum score: 38.92 (39.93 in the paper)
• The higher the score the more severe the amyloidosis.
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Specialty: Hematology Oncology
