Vol 22, No 3 (2019)

Hereditary syndromes with signs of premature aging
Golounina O.O., Fadeev V.V., Belaya Z.E.

Aging is a multi-factor biological process that inevitably affects everyone. Degenerative processes, starting at the cellular and molecular levels, gradually influence the change in the functional capabilities of all organs and systems. Progeroid syndromes (from Greek. progērōs – prematurely old), or premature aging syndromes, represent clinically and genetically heterogeneous group of rare hereditary diseases characterized by accelerated aging of the body. Progeria and segmental progeroid syndromes include more than a dozen diseases, but the most clear signs of premature aging are evident in Hutchinson-Guilford Progeria Syndrome and Werner Syndrome. This review summarizes the latest scientific data reflecting the etiology and clinical picture of progeria and segmental progeroid syndromes in humans. Molecular mechanisms of aging are considered, using the example of progeroid syndromes. Modern possibilities and potential ways of influencing the mechanisms of the development of age-related changes are discussed. Further study of genetic causes, as well as the development of treatment for progeria and segmental progeroid syndromes, may be a promising direction for correcting age-related changes and increasing life expectancy.

Osteoporosis and Bone Diseases. 2019;22(3):4-18
Limited joint mobility syndrome as a predictor of the diabetic foot syndrome
Panevin T.S., Alekseeva L.I., Taskina E.A., Kashevarova N.G.

Diabetes mellitus (DM) can lead to the development of late complications. In addition to the traditional late complications, a high prevalence of damage to the musculoskeletal system in diabetes was noted. The most appropriate term that reflects the defeat of the musculoskeletal system in diabetes is «limited joints mobility syndrome» (LJM). Damage to the hands in the presence of open painless stiffness of the joints, fixed flexion contractures, impaired fine motor skills of the hands and grip forces. Subsequently, it became clear that, over time, the restriction of joint mobility develops not only in the small joints of the hands, but also in other large and small joints of the limbs. Traditionally, LJM syndrome pays low attention on the part of practitioners in comparison with other micro- and macrovascular complications of diabetes, even though LJM can significantly impair functional activity, self-care, and impair the quality of life. It is assumed that damage to the periarticular tissues and joints in DM is caused by the accumulation of end glycation products. A decisive place in the diagnosis of LJM is the clinical examination. In the presence of LJM syndrome, the osteoarticular structures of the feet can be affected, timely diagnosis can lead to the development of diabetic foot syndrome.

Osteoporosis and Bone Diseases. 2019;22(3):19-26
A novel calcimimetic evocalcet for the management of secondary hyperparathyroidism with little effect on the gastrointestinal tract and cyp isozymes in vivo and in vitro
Egshatyan L.V.

In the treatment of secondary hyperparathyroidism of end-stage chronic kidney disease, vitamin D receptor activation and allosteric modulators of the calcium-sensing receptor - inhibit glandular hyperplasia; reduce parathyroid hormone levels, impact on bone turnover and mineral density. Cinacalcet, an oral calcimimetic agent has been widely used for the management of secondary hyperparathyroidism in chronic kidney disease. Nevertheless, some patients remain refractory to the treatment, as the dose of cinacalcet cannot be sufficiently increased due to gastrointestinal symptoms and it strong inhibits of cytochrome P450 (CYP) 2D6. In order to resolve this issue, was develop a newly synthesized calcimimetic agent, evocalcet (MT-4580/KHK7580). In a rat model of chronic kidney disease induced by 5/6 nephrectomy, and in multicenter, open-label study phase 3, and in clinical practice oral administration of evocalcet efficiently suppressed the secretion of parathyroid hormone. Evocalcet also demonstrated the less induction of emesis and gastro-intestinal effects, and its pharmacological effects were observed at lower doses because of its higher bioavailability than cinacalcet. In addition, evocalcet showed no substantial direct inhibition of any CYP isozymes in in vitro. These findings suggest that evocalcet can be a better alternative to cinacalcet with a wider safety margin.

Osteoporosis and Bone Diseases. 2019;22(3):27-33
Osteoporosis in the elderly
Dudinskaya E.N., Brailova N.V., Kuznetsova V.A., Tkacheva O.N.

Osteoporosis in the elderly and senile can be compared with the epidemic of the 21st century due to the high prevalence and increased incidence among people who have survived the 50-year threshold, which make up the bulk of patients. Osteoporosis is associated with a significant increase in the risk of falls and fractures, leading to adynamia and an increased risk of death. Despite the insufficient knowledge of the pathogenesis of the disease, the available data have already allowed the development of preventive measures and treatment principles. Currently, there are preventive and therapeutic measures aimed at reducing the risk of falls, fractures and repeated fractures, however, earlier detection of the disease in old age is often difficult due to the characteristic features of geriatric patients. The polymorbidity, unexpressed clinical picture, the development of frailty syndrome, sarcopenia, social and mental maladaptation and an increase in the frequency of depression make the population of elderly and senile people vulnerable to an increased risk of osteoporosis, falls and fractures and associated hospitalizations and mortality. This review highlights the features of pathogenesis, clinical features, principles of treatment and prevention of osteoporosis in the older age group.

Osteoporosis and Bone Diseases. 2019;22(3):34-40

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