Kashin-Beck's syndrome

Description:

Kashin's disease - Beck - a local degenerative disease of the musculoskeletal device which cornerstone primary disturbance of enchondral growth of tubular bones and processes of ossification is. Developing then deformations of joints with an osteophytosis and without signs of an inflammation give the grounds to consider Kashin's disease - Beck as the local deforming osteoarthrosis.

The first mentioning of this disease belongs to M. A. Dokhturov (1839), but the detailed description it was made by N. I. Kashin (1859) and E. V. Beck (1906). The disease is most widespread in Transbaikalia, near the river Urov, from here and its second name - an urovsky disease. Later it was found out that the disease meets also in other areas of Eastern Siberia in the form of the separate local centers, and also in China, Korea. Separate cases of a disease are noted in Primorsky Krai, Kyrgyzstan and the European part of Russia. In the endemia centers, according to E. V. Beck, the disease is stated at 32% of the population, and in certain settlements - at 46,5%. Now in connection with development of methods of its treatment and prevention spread of a disease decreased. So, according to L. F. Kravchenko, she comes to light at 95,7 on 1000 inhabitants of the local center.

Children and young men both men's, and a female get sick. The disease develops only in a growth period, most often at the age of 6-14 years when the largest growth of a skeleton is observed. The disease never comes to light at children more young than 4 years, and after 25 summer age develops extremely seldom.

The chronic progressing current with multiple symmetric damages of joints, with formation of the expressed deformations is characteristic of a disease. The most characteristic symptoms - the low-tallness and a korotkopalost caused by a growth disorder of bones at children and teenagers.

Symptoms of the Syndrome of Kashin-Beck:

The disease develops slowly and imperceptibly at children and teenagers. The first displays of a disease - the non-constant, aching joint pains, in muscles, in a backbone, constraint and a crunch in sustvo, paresthesia and spasms in fingers and in gastrocnemius muscles. First of all the II-IV interphalangeal joints of hands are surprised, and in certain cases on it process also stops. However progressing of a disease with damage of radiocarpal, elbow and all other joints is more often observed. Damage of joints multiple and symmetric with gradual development in them of thickenings, deformations, restrictions of mobility and a small muscular atrophy. The curvature of axes of an extremity and fingers is often observed. The joint pains aching, non-constant, are expressed very moderately, is more often in the evenings and at night, they can appear much later deformation development. At infringement of "a joint mouse" there is a syndrome of "blockade of a joint" - sharp pain, impossibility of the movement and quickly taking place swelling, most often in a knee joint.

Distinguish three stepenityazhesta of a disease.

I. A moderate thickening of proximal interphalangeal joints II, III, IV fingers of brushes, pains at loading, small restriction of movements in radiocarpal, elbow and ankle joints.
II.  Multiple damage of joints with their thickening, deformation, a crunch and restriction of movements. Atrophy of muscles and muscular contractures. Low growth (at men 160,2 ± 0,6, at women 146 ± 0,5). Korotkopalost.
III. All joints are deformed and thickened. The movements in them are sharply limited. Low growth (at the man 160,2 ± 0,6, at women 144 ± 0,5), a short neck, a bear paw, genu valgum and genu varum, flat-footedness, a hyperlordosis of lumbar department, duck gait. Women have often narrow basin.
Except these changes, at Kashin-Beck's disease II and III severity nervnodistrofichesky disturbances can be observed: headaches, heartaches, small appetite, wrinkled skin, dim nails and hair. Chronic atrophic rhinitis, pharyngitis, otitis, bronchitis, emphysema of lungs, gastritis, a coloenteritis, vegetovascular dystonia, a myocardial dystrophy, deformations of teeth and jaws, neurologic disturbances (an asthenoneurotic syndrome, encephalopathy) are often observed. Intellectual spoyosobnost in process of progressing of a disease decrease. It should be noted that if Kashin-Beck's disease develops at adults, her clinical picture can be erased. Body temperature at patients usually normal. In blood tests and urine is not found pathological deviations. Sometimes note hypochromia anemia, a small lymphocytosis, a neutropenia.

The research of mineral composition of blood shows that the content of calcium is increased, and phosphorus is lowered. In urine opposite changes are noted.

Reasons of the Syndrome of Kashin-Beck:

The etiology of a disease of Kashin - Beck is found insufficiently out. The geobiochemical (mineral) theory offered by A. B. Vinogradov according to which Kashin's disease - Bekya - result of unbalanced contents in an organism of calcium and microelements owing to a lack of calcium and the increased content of iron, strontium, manganese of the soil, water and food stuffs of those areas where the disease is widespread is submitted to the most reasonable. In a bone tissue of patients deficit of calcium and high content of iron, manganese, zinc and silver are found.

A. B. Vinogradov, V. V. Kowalewski and V. G. Hobaryev, studying soils in Urovsky district, found that where there are fields of limestone (in water a lot of calcium), among the population few patients with an urovsky disease come to light. Value of a giposelenizm - a lack of water and the soil of cynthion is discussed also (especially Chinese authors).

Staff of Problem nauchnoissledovatelsky laboratory on studying of an urovsky disease in Chita established that the imbalance macro and microelements in local areas is caused mainly by the increased content of phosphates in the soil, water and local food stuffs. At inspection of the population of these areas increase in level of inorganic phosphorus in blood serum and in urine of patients with an urovsky disease is revealed. Chronic phosphoric intoxication caused in experimental animals the fosfatemiya, delay of growth of bones and change of joints similar to those which are observed at patients with an urovsky disease. Authors of a "phosphatic" hypothesis believe that as a result of excess receipt in an organism of phosphates hormonal regulation of fosfatnokaltsiyevy exchange is broken and there is "failure" of hormonal bone formation.

All these observations confirm a paramount role of microelements in disturbance of processes of bone formation at an urovsky disease. To Povidimy, surplus or a lack of certain mineral salts results in deficit of calcium in a bone tissue of an epiphysis and to a course of a disease. According to L. F. Kravchenko's data, at the same time exchange of collagen is broken and there occurs braking of a fibrinolysis that promotes disturbance of microcirculation in metaepiphyseal departments of an epiphysis, to braking of processes of regeneration of connective tissue elements, disturbance of processes of bone formation and an early sinostozirovaniye with the subsequent development of degenerative changes of joint fabrics and internals.

The role of heredity is not proved, however there are data indicating value of this factor. So, F. P. Sergiyevsky found that at patients with an urovsky disease of parents twice more often sick children, than at healthy faces are born. Yu. A. Domayev, having investigated 461 families where there were patients with an urovsky disease, established higher percent of incidence among blood relatives, than among distant. The author believes that it demonstrates hereditary genesis of an urovsky disease with incomplete manifestation of a genotype in which implementation the big role belongs to the environment of the local area.

The contributing factor, according to many authors, is rickets as hypoavitaminosis of D worsens digestion of calcic salts and, thus, promotes earlier and more expressed development of an urovsky disease. Also physical overwork and overcooling can be the factors favoring to a course of a disease.

Treatment of the Syndrome of Kashin-Beck:

Treatment of a disease of Kashin - Beck is directed to improvement of function of joints, increase in working capacity, reduction of pains and muscular contractures. At timely and persistent treatment in an early stage of a disease at 30% of patients, according to L.F.Kravchenko and соавт., it is possible to achieve full reversibility of process, and in late - reduction of pains, improvement of mobility of joints and delay of progressing of process. It is reached by use of drugs of calcium and phosphorus (phosphene, a calcium gluconate, lactic calcium - on 2-3 tablets a day, etc.), vitamins C, B1, Bia, and also means applied at arthrosis - biological stimulators (an aloe, a vitreous, ATP, FIBS) in combination with fiziobalneoterapiya methods, remedial gymnastics and massage. In view of lack of the expressed pain syndrome, the rare and quickly passing inflammatory phenomena in purpose of anesthetics and anti-inflammatory means these patients usually do not need joints. Action of radonic bathtubs (3-6 courses) in combination with paraffin and mud applications on joints is very effective. Also brine bathtubs, a diathermy, ultrasound and UVCh on joints are applied. Treatment by radonic bathtubs in the resort of Yamkun located in the center of one of local areas of Eastern Siberia allows to achieve improvement, according to F. P. Sergiyevsky, from 80% of patients.

After 2-3 courses of radonic bathtubs (18-30 procedures with the water temperature of 36-40 °C) L. F. Kravchenko observed not only reduction, but disappearance of "niches" on the roentgenogram.

In a late stage of a disease at the expressed irreversible changes of joints recommend ortopedokhirurgichesky treatment for elimination of contractures and recovery the movement, and at repeated blockade of a joint - removal joint "niche".