LOW BONE MINERAL
DENSITY IN ADOLESCENTS AND YOUNG PATIENTS WITH JOINT HYPERMOBILITY
SYNDROME: PRELIMINARY CLINICAL STUDY OF 68 PATIENTS
AUTHORS: Bravo J, Wolff C and Rojas P
Introduction. The Joint Hypermobility Syndrome
(JHS) or Ehlers-Danlos (EDS) type III, is a forme fruste of the Hereditary
Diseases of the Connective Tissues (HDCT). Osteoporosis is frequent in HDCT
(EDS, Marfan Syndrome and Osteogenesis Imperfecta, reaching 100% in the latter
and in EDS type VI).
Objective. To describe the frequent alteration of bone mineral density (BMD) in
adolescents and young adults with JHS.
Patients and Methods. In 600 patients with HDCT (Brighton Criteria) younger than 50 years old,
studied during the last 4 years, we found 68 with low BMD. Secondary
osteoporosis was ruled out with the clinical picture and laboratory tests.
Results. Mean age 33 years old (13-49). Males 32%. Marfanoids 32%. Laboratory tests were normal, except for mild
elevation of PTH in 16 of 29 patients (55%). Significantly no fractures were
seen.
|
BMD |
Lumbar spine % |
|
Hip % |
||||
|
Femoral neck |
|
Total |
|||||
|
Normal |
( > - 1,00 ) |
13,24 |
|
19,12 |
|
15,87 |
|
|
Osteopenia: |
|
|
|
|
|
|
|
|
Mild (-
1,00 a - 1,49 ) |
10,29 |
|
19,12 |
|
23,81 |
||
|
|
|
|
|
|
|
|
|
|
Moderate (- 1,50 a - 1,99 ) |
32,36 |
|
20,59 |
|
23,81 |
||
|
|
|
|
|
|
|
|
|
|
Severe (-
2,00 a - 2,49 ) |
26,47 |
|
20,59 |
|
19,05 |
||
|
|
|
|
|
|
|
|
|
|
Osteoporosis (
= < - 2,5 ) |
17,65 |
|
20,59 |
|
17,46 |
||
Conclusion. Patients with JHS, especially Marfanoids presented
a significant decrease in BMD. This was noted with equal frequency in
trabecular as well as cortical bone, and affected 32% of males. The bone
alteration presented itself from early age promoting the need to further study
the problem, in this frequent and usually undiagnosed condition.