Zimran et al developed a severity scoring index to evaluate patients with Gaucher's disease. The authors are from Scripps Clinic in La Jolla, California.
(3) history of splenectomy
(5) liver function tests
(6) clinical evidence of liver disease
(7) CNS involvement
(8) other organ involvement
(9) bone objective findings
(10) bone subjective findings
NOTE: This is the modified version from 1992. The previous version of the form published in 1989. Differences include:
(1) removal of age at time of diagnosis (from 0 to 6 points)
(2) addition of CNS disease
(3) distinguishing the number of cytopenias in the splenectomized patient
(4) removal of joint replacement and severe disability from bone assessment
(5) change in liver function tests from degree of abnormality (none, minor, severe)
splenectomized and 1 cytopenia
splenectomized and 2 cytopenias
splenectomized and 3 cytopenias
mild (above level of umbilicus)
moderate (between umbilicus and pelvic rim; does not cross to the right side)
massive (extends below pelvic rim)
moderate (between umbilicus and pelvic rim)
liver function tests
clinical liver disease
other organ involvement
bone, objective findings
no signs or symptoms
imaging studies positive
bone, subjective findings
mild, intermittent pain
chronic pain (not due to fractures)
• Cytopenias include thrombocytopenia, anemia and/or leukopenia.
• Liver function tests: AST, alkaline phosphatase, GGT and LDH.
• Other organ involvements: lungs, kidneys, heart, other
total score =
= SUM(points for all 11 parameters)
• minimum score: 1
• maximum score: 51
• The higher the score the more severe the disease.
Score (1989 Version)
0 to 10
11 to 25
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Purpose: To evaluate a patient with Gaucher's disease using the severity scoring index of Zimran et al.
Objective: severity, prognosis, stage