Radiologic Findings of Korean Gaucher Disease.
10.3348/jkrs.1999.40.4.759
- Author:
Jae Hyun CHO
1
;
Byoung Suck KIM
;
Moon Kyun KIM
;
Yoon Sok CHUNG
;
Doo Hoe HA
;
Jung Ho SUH
;
Hyon J KIM
Author Information
1. Department of Radiology, Ajou University, Suwon, Korea.
- Publication Type:Original Article
- Keywords:
Metabolism;
Gaucher disease;
Bone marrow MR
- MeSH:
Alanine Transaminase;
Biopsy;
Bone Diseases, Metabolic;
Bone Marrow;
Bone Regeneration;
Femur;
Follow-Up Studies;
Gaucher Disease*;
Humans;
Infarction;
Liver;
Magnetic Resonance Imaging;
Metabolism;
Radiography;
Spine;
Spleen
- From:Journal of the Korean Radiological Society
1999;40(4):759-767
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: To document the radiologic characteristics of Korean Gaucher disease. MATERIALS AND METHODS: Fifteen bone marrow biopsy and laboratory data confirmed Gaucher disease patients (age 1 -21, mean 10.9 yr) wereundertaken plain X ray and MRI. Number of type I were 10, type II, 2, type III, 3. Seven were splenectomized oninitial evaluation or during follow up. Five enzyme treated patient were undertaken follow-up MR examinationduring 6 -40 month with 6 month interval. Conventional T1 and T2WI of spine and femur was performed and FMPSPGR inand out of phase image was also done. Volume of liver and spleen were measured, and bone marrow infiltration andpresence of infarction were scored according to 6 scale scoring system. Clinical data were also reviewed andcorrelated with the MR findings. RESULTS: Marrow infiltration was noted in 71.4% of all patients in MRI, while itwas in 45.7% with plain radiography. Type I group showed marrow infiltration in all but one cases, which wasparallel with ages, SGPT, and presence of osteopenia, reversely correlated with spleen size. Severe bonecomplications (infarction or fracture) were noted in 7 of 10 type I group, and 6 patients showed severe growthretardation (below 3rd percentile). Follow up MR examination of 5 patient showed decrease in liver and spleen sizefirst without bone change until 6 months. There showed bone regeneration in 2 patient 1 year after, and increasedfat signal in one patient 3.5 years after. In and out of phase images couldn't help in quantifying fatcomposition in bone marrow. CONCLUSION: Korean Gaucher patients revealed as more severe skeletal complicationsthan others reported from Western groups. MR examination is a effective modality to evaluate and monitor ofGaucher patients.
