Destructive processes in the bones of the extremities (Fig. 3) may be the cause of their pathological fractures. Changes in the lungs are characterized more often by bilateral small-focal shadows, and sometimes by hyperplasia of the intrathoracic lymph nodes.
The volume of surgical intervention depends on the localization of the focus, its size, age and general condition of the patient. The operation consists in curettage or resection of the affected area of the bone. As a bone-plastic material for defect replacement, auto-bone or allo-bone in the form of shavings, gravel, cortical plates is used (see Bone grafting). With small marginal defects, plastic is not produced. Large defects (in the long bones, supraacetabular region of the pelvis) are filled with cortical allografts. In tubular and flat bones, defects not exceeding 2-4 cm in diameter are carefully plugged with autoshavings. When the spine is affected, reclination is indicated (see Kyphosis) and fixation with metal staples. In the bones of the cranial vault, plastic surgery is carried out with plates of allograft or perforated plastic.
In the postoperative period (see) the immobilization by a plaster bandage before reorganization or a bone reparation is shown. It was noted that surgical treatment of one of the foci often leads to spontaneous closure of several foci in other bones. There are usually no recurrences after surgery.
With generalized lesions of the skeletal system, antitumor agents are prescribed - vincristine, vinblastine, cyclophosphamide (cyclophosphamide), chlorbutine (leukeran), prednisone, mercaptopurine, methotrexate; in some cases, their combinations. The prognosis is favorable in most cases. It worsens with damage to the lungs and the addition of intercurrent diseases.
Prevention has not been developed. Diagnosis: molecular cytogenetic method, hormonal studies (decrease in the level of estrogens, progesterone, FSH, LH, growth hormone, TSH; positive gonadotropin test). As treatment, a diet with lipid restriction, hormone replacement therapy is prescribed.
Hereditary diencephalic-reticular degeneration, caused by multiple gene defects, is often familial. Frequency 1: 60,000. The nuclei of the hypothalamus undergo degenerative changes, the number of ganglion cells decreases, and glia grows in their place.
Treatment: diet with restriction of fats and carbohydrates, exercise therapy, hormone therapy: thyroidin 0.05-0.2 g per day for 5 days with breaks of 2-3 days, gonadotropic hormones, cyclic hormone therapy with sex steroids from 12-13 years.
Diagnosis: molecular cytogenetic method, hormonal studies (decrease in the level of viagra, progesterone, FSH, LH, TSH; positive gonadotropin test). Defeat of an adenohypophysis in the childhood. The disease is characterized bythe current production of all tropic hormones of the pituitary gland with a predominant deficiency of growth hormone. There is a delay in growth and sexual development. The growth of an adult woman does not exceed 120 cm, the proportions of the body are preserved, mental development is not disturbed, the genitals are sharply underdeveloped.
Among the most common signs of the disease it is worth noting: diabetes insipidus, urination insipidus, changes in the structure of the skull and the skeletal system as a whole, forward displacement of the eyeballs. exophthalmos In cases of exacerbation - modifications of all bones, various skin modifications. Vomiting, decreased appetite, fatigue In children, inhibition is observed, both in physical and mental development.
The disease is analyzed as a chronic form of reticulohistiocytosis with a secondary violation of cholesterol metabolism, while some leading experts believe that the disease progresses as a result of changes of a neoplastic nature occurring in the system of mononuclear phagocytes, followed by a violation of cholesterol metabolism.
