The diagnosis and management of patients with idiopathic osteolysis

1. ГБОУ ВПО ВГМУ Минздравсоцразвития Кафедра иностранных языков The diagnosis and management of patients withidiopathic osteolysis Выполнила: студентка 202 гр. П.Ф. Говорун А.С. Научный руководитель: старший преподаватель Огородникова Э.Ю. Владивосток 2012

2. Aimand methods Aim: • to compare the clinical history, phenotypic, and radiographic changes of idiopathic osteolysis syndromes in three unrelated children. • Methods: • detailed phenotypic assessment • radiologic scanning • CT scanning • histological testing • genetic testing

3. classificationofosteolyses by Hardegger hereditary multicentricosteolysis with dominant transmission hereditary multicentric osteolysis with recessive transmission nonhereditary multicentricosteolysis wit h nephropathy OSTEOLYSIS Gorham-Stout syndrome Winchester syndrome

4. Patient I Presenting symptoms: • pain • weakness • aggressive destruction of the hip joints • extensive lytic changes of the spine and the skull base

5. Patient I Figure 1 Anteroposterior pelvis radiograph The fracture was fixed with a long Gamma nail.

6. Patient I Figure 2 Sagittal 3DCT scan of the thoracolumbar radiograph It showed a combination of deformities ranged from severe flattening, fusion, shrinkage and compression fractures.

7. Patient I Figure 3 Axial 3DCT scan of the skull base. S C S- the sphenoid bone C- the occipital part of the clivus (both manifested osteolytic destruction associated with significant irregularities)

8. Patient I Figure 4 Specimen obtained at open biopsy of a lesion in the left proximal femur showed multiple, dilated, vascular spaces replacing normal bone marrow elements.

9. Patient II Presentingsymptoms: • pelvic and tarsal pain • nephropathy • congenital solitary kidney • small hand with contractures

10. Patient II Figure 5 Anteroposterior pelvis radiograph. It showed massive osteolysis crosses the epiphyseal growth centers of the proximal femora bilaterally.

11. Patient II Figure 6 Anteroposterior radiograph of the feet. It showed complete resorption of the tarsal bones and complete ankylosis with severe osteolysis.

12. Patient III Presenting symptoms: • distal arthropathy • generalized osteopenia • right radial head dislocation and metatarsal fractures • 3MCC deficiency

13. Patient III Figure 7 Hands photo showed marked decrease range of motion in wrists and fingers and were mildly puffy. Fifth finger was noted to be curved like C shape.

14. Patient III Figure 8 The extensor tendons in both feet were swollen and her feet showed decreased range of motion in her all toes with eversion of the big toe.

15. Patient III Figure 9 Anteroposterior hand radiograph. Marked widening of the distal portions of the metacarpal metaphyses and diaphyses.

16. Patient III Figure 10 Anteroposterior radiograph of the foot. It showed widespread erosions and shortenings of the big toes.

17. statistics Figure 11 Number of osteolysis patients per 100 000 population in some countries.

18. pathogenetic mechanism • of idiopathic osteolysis ? • Hemangioma • Proteinuria • Peripheral arthropathy • 3-MCC deficiency

19. Thanks For your attention