Category: Central DXA: (DXA, TBS)

6 - Measurement of bone microarchitecture by trabecular bone score in patients with Gaucher disease.

Introduction: Gaucher disease is a secondary cause of osteoporosis (OP). The measurement of bone mineral density (BMD) by DXA is the "gold standard" diagnostic study of OP. BMD is one of the major determinants of bone strength and therefore the presence of OP is associated with increased risk to develop fractures. However some pathological fractures occur in presence of osteopenia. The trabecular bone score (TBS) is a bone texture analysis where a score is obtained with the acquisition of lumbar spine DXA. It is also associated with increased risk to develop fractures. The TBS is strongly correlated with trabecular number and connectivity and negatively with the space between the trabeculae.

Objective: To assess TBS in a group of adult GD patients (> 40 years) and relate the results with the diagnosis of OP by DEXA.


Method:
14 GD patients were evaluated with dual X-ray absorptiometry (DXA) and TBS score was performed. The diagnosis of OP was assessed according to the regulations of the International Society Clinical Densitometry. TBS score was used to classify the bone structure: Normal > 1300, slightly degraded 1300-1200 and degraded < 1200. Statistical analysis: Kruskal Wallis test, Sperman’s Coefficient and variable of group.


Results:
28% of patients had a pathological BMD: 21% osteopenia / low bone mass and 7% OP. 100% of these patients had altered TBS. 72% had normal BMD of which 40% had altered the TBS. Of the 14 patients evaluated only 43% (6/14) had normal TBS. We compared the TBS with the semiquantitative bone marrow burden (BMB) scoring system by resonance magnetic and Dussendorf score , and some parameters of therapeutic targets such as hemoglobin, platelets, Splenomegaly, hepatomegaly, DMO and the biomarker of chitotriosidase activity. A significant difference (p = 0.040) was observed in Hemoglobin according to the categories of TBS. A moderate positive correlation was observed between TBS and HB, Spearman's Coefficient = 0.70 (p = 0.017)


Conclusion:
In this preliminary study we can observe that patients with normal BMD have affected bone microarchitecture and are associated with anemia
This is the first report on TBS in EG. This new tool evaluates bone micro architecture can be used to assess these patients

Gabriel Aguilar

PHISYCIAN
NO
BUENOS AIRES, Buenos Aires, Argentina

Guillermo Drelichman

pediatric hematologist
no
BUENOS AIRES, Buenos Aires, Argentina

Nicolas Fernandez Escobar

physician
NO
BUENOS AIRES, Buenos Aires, Argentina

Ignacio Rossi

physician
no
BUENOS AIRES, Buenos Aires, Argentina

Barbara Soberon

physician
NO
BUENOS AIRES, Buenos Aires, Argentina

Maria SILVIA.. Larroude

PHYSICIAN
member of ISCD
Buenos Aires, Buenos Aires, Argentina