Osteogenesis Imperfecta Research Paper

Osteogenesis Imperfecta (OI) is a hereditary connective tissue disorder due to a
3 qualitative or quantitative defect of type I collagen. OI is a rare disease, occurring in one
4 case per 15,000 to 20,000 births and affecting one in every 200,000 individuals,
5 however there are no citations in the literature about the predominance of race or gender
6 [1].
7 The main features of OI are ligamentous laxity, osteopenia, short stature, fractures
8 caused by mild trauma, progressive skeletal deformities, and additional clinical
9 manifestations including blue sclera, dentinogenesis imperfecta, and deafness [2,3].
10 Bone fragility in the spine usually leads to serious consequences. The vertebrae can
11 easily break, either in an acute or a chronic …show more content…
15 Our radiographic thoracic kyphosis followed the recommendation of the SRS -Scoliosis
16 Research Society [15,16], who consider normal values for thoracic kyphosis as a range
17 between 20 and 50 degrees, with the curve measured by the method of Cobb modified
18 between the vertebrae T3 to T12. Furthermore, the SRS defines a normal absolute value
19 of 40 degrees in thoracic kyphosis. Even in 37 patients who had thoracic kyphosis
20 within the normal range (between 20 and 50 degrees), the average was 29 degrees
21 below the normal absolute value of 40 degrees. This drew attention to the high number
22 of cases with hypokyphosis, 19, in relation to cases with kyphosis, 6. However, as
23 opposed to the study of Abelin et al [14], a tendency toward decreased thoracic
24 kyphosis was observed in the normal population.
25 Koerber et al [17] introduced a new classification of spinal abnormalities in patients
26 with OI, but used only lateral view X-rays. As in our study, they identified the high
27 incidence of thoracolumbar kyphosis as a risk factor.
28 The presence of thoracolumbar kyphosis is always pathologic [18] (Figure 4). The

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Osteogenesis Imperfecta Research Paper