Osteopsathyrosis

OSTEOPSAТИРОЗ, osteopsathyrosis (from Greek osteon--bone and psathyrosis-fragile), Latin fragilitas ossium, bone fragility, a term used to denote such a state of the skeletal system when the bones prove to be weak and easily subject to fractures. Symptomatic and idiopathic O. are distinguished. Symptomatic O. is that bone fragility which is the consequence of various lesions of the skeletal system associated with a loss of bone substance, for example in osteoporosis of various origins, in senile atrophy of the skeleton, in rickets, osteomalacia, fibrous osteitis, in neurogenic osteopathies, in multiple tumors in the bones, etc. Idiopathic O. (idiopathic bone fragility) represents a peculiar disease described for the first time by Lobstein in 1825, expressing itself in increased bone fragility, manifesting itself from early childhood, usually from 1-2 years, for the most insignificant reasons (diapering the child, games, etc.). The disease is more often observed in women: There are indications that it is more widespread in northern countries. Patients suffering from idiopathic O. are usually weak, of delicate build, with a large head and characteristic skull shape, conditioned by a wedge-shaped protrusion of the occiput. From other frequently occurring changes in idiopathic O. one can note weak development of the venous system, increased electrical excitability of the nerves, and premature sexual maturation with hypoplasia of the internal genitalia and with excessive development of secondary sexual characteristics. Intellectually, patients are generally normally developed. In addition to fractures, a tendency to dislocations and sprains of ligaments is also observed. Fractures in idiopathic O. are characterized by slight pain and rapid healing. Improperly united bone fractures often lead to such a sharp deformation of the limbs that the latter sometimes have to be amputated because of this. A feature of the clinical course of the disease is its weakening during puberty, and often its complete cessation after the onset of sexual maturity. Patho-anatomical changes in the skeletal system in idiopathic O. are quite similar to the changes in osteogenesis imperfecta (see), differing from them only by a less pronounced degree. These changes, also connected with reduced activity of the bone-forming elements, are in the main reduced to underdevelopment of the cortical and spongy substance of the bones. The changes in bones in O. give a characteristic picture on X-ray films in the form of a sharp thinning of the cortical layer and rarefaction of the spongy substance of the diaphyses of the bones with a normal appearance of the epiphyses and epiphyseal lines. The etiology of idiopathic O. is still unclear. Some authors consider it to be early osteomalacia or a variety of rickets. The majority of authors, on the basis of patho-anatomical research, consider O. to be identical with osteogenesis imperfecta, considering it only as a weakened and late form of the latter (osteogenesis imperfecta tarda). Therefore, all views on the etiology existing in relation to osteogenesis imperfecta also apply to O. The majority of authors also considers O. as a developmental defect expressing itself in the underdevelopment of all derivatives of the mesenchyme. This is confirmed by the fact that in O. a clearly expressed hereditary familial character of the disease, transmitted through several generations, is often noted. In this connection, sometimes a combination of O. with blue sclera, conditioned by excessive thinness (underdevelopment) of the connective tissue of the sclera, and with progressive deafness, connected with otosclerosis, is noted. The whole triad of signs may not be transmitted by inheritance in full, but sometimes only some of them. The presence of blue sclera can serve as a diagnostic sign for latent forms of the disease, which, in connection with less pronounced patho-anatomical changes, can be clinically characterized only by a slight tendency to fractures or even their complete absence. The prognosis is connected with the onset of the manifestation of the disease: the earlier and the more numerous the fractures appear, the worse the prognosis. For treatment, strontium, arsenic, calcium, phosphorus, fish oil, pituitary preparations, thyroid gland preparations, and milk of castrated goats are applied, but without sufficient effect, since although with the help of some of them it is possible to increase the absorbability of calcium, the disease does not improve. General strengthening treatment is recommended.