Caschin-Baker disease
Introduction
Introduction to Caschin-Beck disease Kaschin-Beckdisease (Kaschin-Beckdisease) is a kind of endemic cartilage and joint deformity disease, which is a deformed osteoarthrosis with cartilage necrosis. Occurs in children and adolescents, mainly invading the bones and joints of children and adolescents, resulting in osteogenesis in the cartilage, shortening of the tubular bone and secondary deformed joint disease, resulting in tubular long bone development disorders, joint thickening, pain, Muscle relaxation, atrophy and movement disorders. The patient was characterized by short stature, short fingers, joint deformity, abnormal gait (typically limp, duck step). The disease is also known as "dwarf disease", "calculation of plate disease", "willow disease" and so on. basic knowledge The proportion of sickness: 0.0001% - 0.0002% Susceptible people: mostly in children and teenagers Mode of infection: non-infectious Complications: dyskinesia
Cause
The cause of Caschin-Beck disease
(1) Causes of the disease
The etiology of Kashin-Beck disease mainly involves the following three types of factors: low selenium in the ecological environment of the ward; organic pollution in drinking water; mycotoxins.
1. The ecological environment of the ward is low. Selenium was originally proposed by the former Soviet Union scholars. The disease was caused by too much or insufficient of one or several elements. In the early days, it was thought that it was related to water, less calcium and more sputum, and more sputum. Later, it was claimed that due to the phosphorus and manganese in the soil and the main and non-staple foods in the ward, the disease was not found from the patient or in the experimental study.
Chinese scientists have found that Kaschin-Beck disease is closely related to environmentally low selenium:
1 The distribution of the diseased area in China is generally consistent with the low-selenium soil zone. The total selenium content of most of the diseased areas is below 0.15mg/kg, and the grain selenium content is less than 0.02mg/kg;
2 The blood, urine and hair selenium content of the ward population is lower than that of the non-patient area, and a series of metabolic changes associated with low selenium can be detected in the patient;
3 When the level of selenium in the hair of the ward is rising, the condition is reduced;
4 selenium supplementation can reduce the new incidence of Kashin-Beck disease and promote the repair of metaphyseal lesions.
But there are also some important facts that do not support low selenium is the cause of this disease:
1 Some areas have low selenium and do not have Kaschin-Beck disease, such as Yulin in Shaanxi, Luonan, and some Keshan disease areas in Yunnan and Yunnan. In some areas, selenium is not very low, but this disease occurs, such as Qingzhou in Shandong. Zuoquan of Shanxi, Huo County, Ankang of Shaanxi, Ban Ma of Qinghai, etc.;
2 after selenium supplementation can not completely control the new development of the disease;
3 cell culture showed that chondrocyte growth has no special need for selenium;
4 Low selenium animal experiments can not cause cartilage necrosis similar to this disease.
At present, more people tend to think that low selenium is only a conditional factor in the onset of this disease.
2. Organic matter pollution in drinking water In many wards in China, folks have long attributed the cause of this disease to poor water quality. Japanese scholar Yan Ze and others studied the relationship between plant organic matter in drinking water and Kashin-Beck disease, and considered it as a ferula in organic matter. Acid or p-hydroxycinnamic acid may be a causative factor.
In our country from 1979 to 1982, the scientific investigation of Kaschin-Beck disease in Yongshou County and other areas, it was found that the total amount of humic acid and hydroxyhumic acid in water was positively correlated with the prevalence of Kashin-Beck disease, and the selenium content was negative. Correlation; the humic acid content in the ward is generally higher than that in the non-disease area. The isolation and identification of the organic matter in the drinking water of the ward shows that there is no significant difference between the humic acid structure and the core part of the non-disease area. Phenolphthalein, thiazolidine-containing compounds containing sulfur and nitrogen appeared more frequently in the drinking water of the ward, and were detected by electron spin resonance (ESR). It was found that there was a significant free radical signal in the drinking water of the ward, and its concentration was significantly high. In the control non-disease area; the concentration of free radicals in drinking water in the ward has a linear correlation with the content of humic acid. Some researchers believe that organic matter pollution in drinking water produces exogenous free radicals (half-free radicals) and increased free radicals. The human body can damage the chondrocytes.
So far, the relationship between organic matter pollution in drinking water and the incidence of Kashin-Beck disease has not been supported by sufficient epidemiological and experimental research data.
3. Mycotoxin was as early as 1943 ~ 1945, the former Soviet scholars suggested that the ward grain was contaminated by some Fusarium bacteria and formed a heat-resistant toxic substance. The residents got sick because they ate food containing this poison. After the 1960s, China Scholar Yang Jianbo and others have done a lot of work in this area. In recent years, the following major advances have been made in the T-2 toxin:
1 ELISA method was used to detect the main food flour and T-2 toxin in corn flour, and the content was found to be significantly higher than that of non-disease commercial flour and corn flour; the granular foods such as rice, millet and yellow rice collected from all regions were collected. No or only trace amount of T-2 toxin detected;
2 Inoculate the non-disease corn with Fusarium graminearum to prepare the bacterial grain, and mix the normal feed with 10% proportion. The chicks were fed with the cartilage banded necrosis of the knee joint for 5 weeks. The pure T-2 toxin was applied directly, according to 100g/ Kg body weight was incorporated into the feed to feed the chicks for 5 weeks, and there was a significant degenerative change in the knee cartilage.
Other domestic units have also carried out some work on the relationship between mycotoxins and Kaschin-Beck disease. At present, the dominant fungi in cereals in different wards are different, and there is also a lack of consistent pathogenic fungi and their strains. The detection results of mycotoxins in food are also Inconsistent, in addition, due to technical limitations, T-2 toxins and their metabolites have not yet been detected from patients with Kashin-Beck disease.
4. Experimental animal model research In order to explore the etiology and pathogenesis, many scholars at home and abroad have devoted themselves to the study of experimental animal models of this disease. Chinese researchers generally use cartilage damage as the basic morphological index for determining animal models, but in the past In experiments with rats or dogs, most of the signs seen in the epiphyseal plate and articular cartilage were scattered chondrocyte necrosis, matrix degeneration, and a small cell-free area with minor changes in characteristics, compared with the control group. Lack of qualitative differences, it is difficult to judge its value. In recent years, it has been more successful in the experiment of food and water in the gorge of the rhesus monkey. After feeding the water or grain in the ward for 6 months or 18 months, it is large. In some monkeys, the articular cartilage and the epiphyseal cartilage in the deep layer showed focal, banded necrosis and a series of secondary changes after necrosis (Figures 1-4).
It basically reproduces the pathological development process and main pathological features of Kaschin-Beck disease. The experimental results suggest that there are pathogenic factors in the water and food in the ward; the pathogenic effect of such pathogenic factors on experimental animals does not follow the disease. The condition of the district is reduced and weakened.
So far, it has not been proven that there is a naturally occurring Kaschin-Beck disease in the animal kingdom. In the past, the joints of livestock or dogs in the ward have been swollen and cautious, which is far from the Kaschin-Beck disease of humans. It is mentioned in foreign countries that it can be compared with this disease. : 1 Osteochondrosis of livestock: This disease is similar to Kaschin-Beck disease in the development of articular cartilage and epiphyseal cartilage necrosis and can develop secondary osteoarthrosis, but the most typical change is cartilage. Cell differentiation disorder and local hypertrophic chondrocyte accumulation. Although the thickness of the epiphyseal plate is uneven, the existing materials have not proved that before the cartilage necrosis, the accumulation of hypertrophic chondrocytes is the basis, and the development of the tibial cartilage of 2 birds. Poor (tibial dyschondroplasia): its basic lesion is that the epiphyseal cartilage matrix can not be calcified, cartilage internalization bone stops, although sometimes considered to be equivalent to osteochondrosis of livestock, but because there is no cartilage necrosis, and the articular cartilage is not tired, so and Human Kaschin-Beck disease has nothing in common.
(two) pathogenesis
1. A large number of T-2 toxins (Fusarium oxysporum toxin) were detected directly from the ward, the patient and the locally marketed cereals (corn and wheat), and the pure T-2 toxin was used at 100 g/d. The dose is mixed with normal feed to feed the chicks, the cartilage necrosis of the chicks, and the cartilage joint disease occurs. The lesion characteristics are similar to those of the mammalian cartilage joint disease. The disease mainly occurs in the young animals, and the joint cartilage and the epiphyseal cartilage appear selectively. Necrosis, separation of osteochondritis, and subsequent cartilage joint deformation, it has been clarified that the mechanism of action of T-2 toxin on various tissues and organs of the body is to inhibit the synthesis of protein and DNA, leading to osteonecrosis of the bone. Experimental pathological studies prove that T-2 The toughness of articular cartilage caused by toxins is in the transition zone of rapid growth of cell growth and hypertrophy. The deep changes of the epiphyseal cartilage seen in human large bone lesions are essentially the same, the chondrocyte damage caused by T-2 toxin, Mainly damage of the cell membrane system, mitochondrial swelling, endoplasmic reticulum expansion, which is due to membrane permeability change, intracellular ion and water balance disorder, cell edema, mitochondria Injury will inevitably affect its energy supply function, thus causing cell metabolism disorders. Chondrocyte membrane structure damage is the main feature of early ultrastructural changes before chondrocyte necrosis. Further damage will lead to chondrocyte necrosis. The causative agent of the disease is the T-2 toxin produced by Fusarium contaminated by food in the ward. The carrier is wheat (flour) and corn in the ward, not rice. The related factors are the unique natural, social and ward. Living environment, etc.
Whether it is from macroscopic or microscopic chemical environment, the population of Kaschin-Beck disease is in a low-selenium ecological environment, that is, the selenium content of ecological substances in the ward, from water, soil, grain to human hair, blood content is significantly lower than In non-disease areas, supplementation with selenium can effectively correct the selenium nutrition level of children in the ward and promote the repair of dry sputum. Selenium and its compounds are an antioxidant. Selenium-containing glutathione peroxidase can catalyze lipids. Reductive decomposition of hydroperoxide and hydrogen peroxide to prevent damage to the cell membrane, maintain cell membrane integrity and stability, tissue selenium deficiency, reduced antioxidant capacity, in addition to causing oxidative damage of the cell membrane system, can also lead to protein , free radical damage of nucleic acids, etc., resulting in decreased tissue enzyme activity, collagen denaturation and decreased DNA. Children's plasma sulfhydryl (ie, total plasma sulfhydryl, non-protein sulfhydryl and protein-bound sulfhydryl groups) is associated with a lack of selenium, suggesting that children The antioxidant defense system in the body is damaged. It is believed that selenium has an antagonistic effect on T-2 toxin (including other Fusarium toxins), which can reduce lipid peroxidation damage. The composition of glycopeptide peroxidase.
It can participate in many biological processes to protect the body from a series of foreign compounds. The antioxidant effect of selenium is mainly reflected in the inhibition of peroxidation, the decomposition of peroxides to eliminate harmful free radicals, the repair of cell membrane molecules, etc. Because T-2 toxin mainly affects the ATP content of hepatocytes, selenium has a protective effect on the reduction of ATP in the liver caused by T-2 toxin, which can protect liver cell mitochondrial membrane, microsomal membrane and lysosomal membrane from damage. Further research is needed to confirm that the relationship between low selenium and Kashin-Beck disease is still controversial. It has been found that there are non-disease points in the low selenium area, and there are disease points in the high selenium area. On the one hand, the degree of early lesions of the disease is found. There is a significant dose-effect relationship between selenium levels. On the other hand, selenium is administered for 1 to 3 years in 3 different types (ie, moderately active, mildly active, and relatively stationary), with less than 1% per year. New cases of metaphyseal disease and new cases of osteophytes less than 4%, and found that the content of selenium is not directly related to X-ray metaphyseal lesions. From the effect of selenium prevention and treatment for many years, the X-ray film is used to improve the rate of dryness. measure Compared with the control group, the selenium group has a net improvement rate of 10% to 30%, so it cannot be said that the disease is caused by selenium deficiency alone. This disease is also related to the lack of various elements such as manganese, phosphorus and zinc. Research confirmed.
Switching to deep water wells, drinking boiled water, the incidence of Kashin-Beck disease is significantly reduced, water can prevent the occurrence of Kaschin-Beck disease, so that the patient's condition is reduced or relatively stable, alanine aminotransferase in children after the change of water The activity of lactate dehydrogenase and alkaline phosphatase decreased, which was significant compared with that before water change. Therefore, it is considered that Kaschin-Beck disease is related to organic poisoning in water. It is also considered that the occurrence of Kashin-Beck disease is not related to drinking water. Further research is still needed.
2. Mechanism of cartilage damage Chinese researchers mainly have the following three kinds of insights:
One opinion is that the biochemical basis of cartilage damage in this disease is a disorder of sulfur metabolism. Chondroitin sulfate (Chs) is an important component of cartilage matrix. Researchers with this knowledge found that the excretion of Chs in the urine of patients with this disease increased. The degree of sulfation is reduced, the molecular weight is reduced, and the proportion of various aminopolysaccharides in the urine is dysregulated. They believe that these changes suggest a sulphur utilization disorder, and the sulfation of Chs in the body is affected by the sulfation factor (SF) produced by organs such as the liver and kidney. Regulated, they found that the serum SF activity of children with this disease was significantly lower than that of local healthy control children, who were lower than non-disease control children. They believed that sulfur metabolism disorder was the result of decreased SF activity, and the pathogenic factor of this disease was A series of cartilage damage is caused by interfering with the biological function of SF.
Another opinion is that the membrane defect state of cells is the biochemical basis for the pathogenesis of this disease. They found that the phospholipids of the erythrocyte membrane lipid composition decreased, the molecular ratio of cholesterol/phospholipid increased, and phospholipids in phospholipids. The acetylcholine (PC) is mainly decreased, the sphingomyelin (SM) changes are small, and the molecular ratio of SM/PC is increased. These changes mean the aging of the biofilm. The above situation is also seen in the cartilage of the autopsy material of the patient. In the analysis, they believe that the combination of low temperature, low selenium and monotonous food (insufficient intake of phospholipids) leads to weak membrane system and reduced antioxidant capacity.
There is also a view that exogenous free radicals can cause both chondrocyte necrosis and abnormal metabolism of chondrocytes. The latter will synthesize and secrete abnormal matrix rich in type I collagen, which occurs rapidly, with small particle size and crystallinity. Low abnormal mineralization, which led to the pathological chemical process of the disease, the mice were fed with ward and water, and the type I collagen in the cartilage matrix was increased, and the ratio of type I/II was increased.
The above aminopolysaccharide, collagen and cell membrane system changes provide useful clues for exploring the mechanism of cartilage damage, but there is still a large distance to explain how the disease selectively acts on specific parts of cartilage and initiates a series of characteristic changes. .
3. Pathology The disease is mainly invading the bone and joint system. Other tissues and systems, such as muscle tissue, endocrine gland, digestive system, and circulatory system can also be involved. The nature of the lesion is generally changed to malnourished degeneration, mainly invading cartilage. The hyaline cartilage part of the bone type (such as the limb bone) is internalized, the lesion is symmetrical, the joint is thickened, and the short limb deformity is usually negative. The joints with more activity are the most affected. The pathological changes of the cartilage tissue are mainly the following two. Kind:
(1) Basic pathological changes of cartilage: firstly invade the epiphyseal cartilage plate, secondly involving the articular cartilage, osteophyte curvature, uneven thickness, uneven arrangement of chondrocytes, ossification disorder and growth delay, pause, etc., capillary of bone marrow Invading the deep layer of the cartilage plate, often part of the cartilage band is cut off or divided into cartilage islands. This is a characteristic of this disease. Sometimes it can be calcified in the matrix of part of the cartilage band to form a transverse bone beam. These phenomena can cause bone growth to pause or Delayed, the cartilage matrix of the epiphyseal cartilage plate is also softened, where the chondrocytes can completely disappear and the nearby chondrocytes aggregate.
The disease mainly involves the bones of the cartilage osteogenesis, especially the limb bones, which are characterized by degeneration and necrosis of the hyaline cartilage and accompanying absorption, repair changes, common coagulative necrosis of chondrocytes, nucleus pyknosis, fragmentation, dissolution disappearance, Residual red-stained cell shadow (Fig. 5), and the residual image disappears, the matrix is red-stained, becomes a foci-like, band-like acellular zone, and the necrotic area can be further disintegrated, liquefied, and the chondrocytes viable around the necrotic foci often have Reactive hyperplasia, the formation of cartilage cell clusters of different sizes (Fig. 6), pathological calcification can occur in the necrotic site near the bone tissue; the blood vessels and connective tissue of the primary bone marrow invade the necrotic foci, appearing ossification, ossification, Finally, the cartilage necrosis of the bone tissue is mainly involved in the mature chondrocytes (hypertrophic chondrocytes), showing a near-bone distribution. When the necrosis is enlarged, it will also affect other levels of chondrocytes. The necrotic foci are often multiple and vary in size. , in the form of dots, flakes or strips.
(2) tarsal cartilage lesions:
The necrosis of the epiphyseal cartilage mainly occurs in the mast cell layer. The severe one can penetrate the whole layer of the tarsal plate. After the necrosis occurs in the deep layer of the tarsal plate, the blood vessels from the metaphysis can not be invaded, and the normal cartilage osteogenesis stops. The proliferating layer of chondrocytes above the necrotic foci can continue to proliferate and differentiate, resulting in this local thickening of the epiphyseal plate. Degenerative calcification often occurs at the proximal edge of the necrotic foci, and bone can be deposited along the metaphysis of the necrotic foci. Quality, the formation of irregular bone or transverse beam, indicating that the normal ossification process pauses, while other parts of the tarsal osteogenesis continue, resulting in uneven thickness of the tarsal plate and uneven ossification lines.
When the necrotic foci runs through the entire tarsal plate, the absorption of necrotic material in both directions of the nucleus and the metaphysis is mechanized and ossified, which finally leads to the early closure of the tarsal plate. The longitudinal growth of the tubular bone stops early, resulting in Short finger (toe) or short limb deformity.
See the corresponding relationship between the pathological changes of the epiphyseal cartilage and the X-ray changes.
Due to the rich blood vessels in the metaphysis, the absorption, mechanization and ossification of the epiphyseal cartilage after necrosis are more rapid, so the X-ray image can be significantly aggravated or improved in a short period of time (months to 1 year).
(3) Articular cartilage lesions: Articular cartilage begins with the same dystrophic lesions, cartilage destruction gradually occurs, the articular surface is rough and uneven, cartilage ulcer often occurs, affecting joint activity, inducing pain, and some cartilage can fall off to form "articular rat "In severe cases, the edge of the articular cartilage proliferates and has a discoidal bulge. After that, there is often a bone, so the joint becomes thicker, the synovial membrane also has a villous proliferation, the villi detachment can become a free body, and the synovium around the cartilage is also Usually hypertrophy, can constitute cartilage and tibia, and the bone end is more enlarged, the edge is irregular, in addition, the bone is often generally loose, trabecular bone destruction, absorption, can form vacuoles and cyst-like structures, and can be accompanied Proliferative changes, bones are significantly thinner, can be loose and spongy, bone marrow cavity dilatation, necrosis and vacuoles often appear in the marrow, muscles, organs and endocrine glands are accompanied by malnutrition degeneration The lesion.
Like the necrotic foci of the epiphyseal cartilage, the lesions of the articular cartilage are also near-bone, that is, the cartilage cells in the deep maturation are first involved, often forming a typical banded necrosis, due to the slow absorption of necrotic material, necrosis. It has a long time, so the proliferative chondrocyte mass in the peripheral part of the necrotic foci is often more noticeable. In the larger necrotic foci, when the necrotic material disintegrates and liquefies, it forms a fissure or cyst (Fig. 8), in gravity and friction. Under the action of mechanical action, the surface cartilage tissue is easy to peel off (separate osteochondritis), forming a joint free body (articular rat), while the local articular surface leaves ulcers of different sizes, and the severe articular cartilage can be The destruction of the whole layer disappears, causing a large piece of bone to be exposed. At the edge of the articular surface, there is often a cartilage hyperplasia reaction with cartilage necrosis, which leads to thickening of the edge of the joint and can be ossified to form a bone edge growth organism. It causes the patient's bone end to enlarge, joint deformation and activity limitation, late joint synovial connective tissue hyperplasia, calcification and ossification, and more serious joints, due to the degeneration of articular cartilage The process of death, disintegration and repair of hyperplasia was repeated, so that the advanced cases showed changes in osteoarthritis, but bone ankylosis was never seen. Monoclonal immunohistochemistry showed that type II of articular cartilage Collagen expression is reduced, type I collagen is increased; proliferating chondrocyte mass is expressed by type I, II, III, and VI collagen.
See the corresponding relationship between the pathological changes of articular cartilage and X-ray changes in this disease. The absorption mechanism of articular cartilage necrosis can only start from the normal gap of the bone plate shell, the repair reaction is relatively weak, and the lesion development is slow. Therefore, the lesion of the articular surface (bone end) under the X-ray is often better than the lesion of the metaphysis. The development was late, the repair process developed slowly, and it changed little over a long period of time.
Prevention
Caschin-Beck disease prevention
Based on the current understanding of the etiology of this disease, comprehensive preventive measures are still advocated, including:
1. Improving water and grain quality In view of research, water in the ward and food are related to the disease, especially the serious pollution of Fusarium in food, changing water, changing grain or improving the quality of grain, is the main measure to prevent this disease. The 10-year prevention and treatment experience of relevant areas in Heilongjiang Province shows that this Law is effective.
(1) Water improvement: The improvement of water quality can be started from two aspects:
1 Adding medicine to water, such as adding halogen base, sodium sulfate, sulfur, gypsum or grass ash extract;
2 Purify water quality, such as deep wells, spring water, drinking filtered water or boiling water, etc., in view of the low salinity of drinking water in the ward, and the heavy natural pollution, efforts should be made to improve water quality. The conditions can be based on local hydrogeology. Conditions to deep wells, or to introduce good quality spring water into the village, should strengthen the protection of drinking water sources, prevent pollution, poor water quality, high organic content can be built according to local conditions, water filtration facilities, centralized filtration, unified water supply.
(2) Changing the grain: changing the rations of the residents in the ward from the grain produced by the ward to the treasury grain or changing the dry land to the paddy field, and changing the staple corn to rice, the disease can be basically controlled. (Corn, wheat) Fusarium is seriously polluted, but the shell of rice makes the rice isolated from pollution and does not cause disease. However, large-scale control uses rice for food or change of staple food for rice. It is often restricted by objective conditions, and some children's canteens are established. To make children susceptible to children, children eat non-disease food or staple rice.
(3) Food protection: improve the harvesting conditions, improve the quality of cereals, reduce the entry of pathogenic substances into the human body, and reduce the incidence rate. Specific measures include improving agricultural operations, increasing mechanization levels, and reducing the pollution of grain contact with soil during grain harvesting. Opportunity, advocate cutting, transport, hit, cool four fast, strictly control the moisture of the grain into the warehouse below 15%, improve storage conditions.
2. Selenium supplementation In view of epidemiological investigation and experimental pathological research, the occurrence and development of Kashin-Beck disease is related to insufficient intake of selenium. Selenium supplementation has become the main measure for mass prevention of Kaschin-Beck disease. Experience in many areas shows that Selenium supplementation can not only accelerate the repair of metaphyseal lesions, but also prevent the increase of lesions, and it is effective for preventing new bone disease. The specific selenium supplement methods are as follows:
(1) Selenium tablets: Each tablet contains 1mg of sodium selenite, generally 0.5mg for 5 years old, 1mg for 5~10 years old, 2mg for 11 years old and above, and once a week, this method is simple, but the drug price is more expensive. Not suitable for large-scale prevention and control.
(2) Aqueous solution of selenium: use 0.1% concentration, 0.5ml for 5 years old, 1ml for 6~10 years old, 2ml for 11 years old and above, once a week, the aqueous solution of selenium is better absorbed in the body than the tablet, and the price is cheap. However, large-scale control applications are inconvenient.
(3) Selenium salt: Add 16mg of sodium selenite tablets to 1kg of salt and mix well, close to 1/60,000, ie 16.67ppm; some use sodium selenite powder 0.5kg plus original salt 45kg, stir well and then process according to proportion 1/60,000 selenium salt or selenium iodide salt; or 15ppm selenium aqueous solution sprayed with salt to eat, such selenium salt has the advantages of selenium living, low price, no side effects, etc., easy to carry out large-area prevention and treatment.
(4) Selenium grain: sprayed with sodium selenite solution in the flowering stage of crops, using sodium selenite 0.5g to 1.0g per acre, this method can increase the selenium content in grain by 4 to 10 times, and can make inorganic Selenium is converted to organic selenium, which is beneficial to the body's absorption. However, this method is more labor-intensive, and there is still no effective management method. It is inconvenient to use in arid areas.
(5) Other high-selenium foods: such as selenium-enriched yeast, high-selenium eggs, selenium-enriched foods and other selenium-enriched foods (fresh garlic, fresh mushrooms, fish, shrimp and pigs, beef kidney, etc.), all according to local conditions Adapt to local conditions and supplement them appropriately.
In short, because the incidence of Kashin-Beck disease is subject to a variety of factors, effective prevention requires comprehensive measures from multiple links, such as Yongshou County, which is famous for this disease, as an example, by taking selenium, changing water, managing food, and eating miscellaneous. "The disease of Kashin-Beck disease has dropped by about 60% from 1980 to 1986, and there are no new cases in the next 3 years."
Complication
Caschin-Beck disease complications Complications
The joints of the extremities are thickened, deformed, dyskinesia, bone destruction and hyperplasia.
Symptom
Kaschin-Beck disease symptoms Common symptoms Joint pain refers to (toe) and calf... Limb weakness bow finger
It can occur in all seasons, but it has the most incidence in spring, and the symptoms are heavier. In autumn and winter, the incidence is not related to gender. It occurs mostly in children and adolescents whose bones are not closed, but adults can also develop the disease. It is 3 to 8 months, and the most is 6 to 18 years old. The onset and development of this disease are relatively slow, only about 3% of patients with acute or subacute procedures. Light patients or early lesions often have no obvious symptoms, sometimes feel tired and inflexible limbs, especially in the morning, often need activities Something can be exercised as usual, and there are sputum and pain in the fingers (toes) and calves. This is a prodromal symptom of the disease. During the examination, the fingers, wrists, elbows, knees, ankle joints have tenderness and irregular hairshots. Friction sound, the tip of the finger is slightly curved to the palm side, especially in the index finger. Symptoms and signs: The disease often starts unconsciously. The patient may be consciously fatigued at first, with limb weakness, abnormal skin (such as ants walking, numbness, etc.), muscle numbness, pain, etc. These symptoms are often not constant. Not obvious, its main, typical clinical manifestations are closely related to osteochondral damage and joint function.
1. Early manifestations in the joints become significantly larger, short-term (toe) deformity, early symptoms, physical signs and more lack of characteristics, according to a large number of investigations and follow-up observations, the following performance is worthy of attention.
(1) joint pain: often multiple, symmetrical, often appear in the large amount of active knuckles and negative weight of the knee, ankle joint, the patient feels pain, soreness or "stomach pain."
(2) refers to the bending of the distal section: that is, the distal knuckles of the 2nd, 3rd, and 4th fingers are bent toward the palm of the hand, often greater than 15°, which is the earliest sign of the disease, and has certain significance in the early diagnosis of the diseased area, but not A small number of children in the ward may also have a lighter degree (less than 15 °) bending of the fingertips; adolescents with no bending in the ward may also have this disease, and the bending of the fingertips often coexists with the fingers, and the skew is more common with the index finger. Followed by the middle finger, ring finger.
(3) Arched fingers: the fingers are bowed to the volar side.
(4) Suspected knuckles thickening: generally occurs in the middle section.
2. After the development of the disease, after the disease progresses, in addition to the early manifestations of joint pain and other factors continue to increase, the main symptoms and signs appear:
(1) joint thickening: the most common is multiple, symmetrical interphalangeal joint thickening, often first appeared in the first interphalangeal joint of the 2nd, 3rd, and 4th fingers, generally the right finger joint thickening is more obvious than the left hand. The knuckles of mechanically damaged joints or women wearing thimbles are thicker and heavier.
(2) joint movement disorder: the performance is that the morning fist feels stiff, the fist is not tight, the fingertip can not touch the palm horizontal stripes, the fist can not be stretched quickly, the elbow joint flexion and extension is limited, the flexion contracture, the shoulder joint involvement patient The hand can not touch the opposite side of the ear from the back of the head, and even wash the face can not wash the forehead, knee inversion or eversion, O-leg or X-leg, due to knee, hip flexion deformation, patient kneeling difficulty, lumbar spine Compensatory lordosis, hip kyphosis, small stride when walking, sway or abduction, "duck gait", ankle joint flexion and extensional obstacles, patient's pain and movement disorder often appear as after rest Or the morning is aggravated, and the symptoms of the activity are alleviated. After many patients get up in the morning, they need to step on the bed and then walk.
(3) Joint friction sound: from the small sputum pronunciation to the rough friction sound, due to the joint surface is not smooth, the joint capsule synovial villi hyperplasia, shedding and other factors.
(4) Free joints of the joints: they can be derived from exfoliated articular cartilage fragments, or from the proliferating synovial villi. The latter are mostly small rice granules, and the free body may be stuck in the joint cavity. The joint lock is formed to cause severe pain; the loose body is relieved as the joint activity loosens.
(5) Skeletal muscle atrophy: The limb muscles of the disease, especially the flexor muscles of the calf and forearm, are often atrophied, sometimes even before the joint changes significantly. The disease is restricted due to pain and joint activity, and is more misused. Sexual factors are involved, so that the contraction is more serious.
(6) Short-finger (toe) malformation: the development of knuckles is shorter than that of ordinary people, the hand is small, or the degree of developmental disorders of each finger (toe) is different, and its length is lost to normal proportional relationship.
(7) short limb deformity, short stature: the degree of developmental instability of each tubular bone is often uneven, and some patients have early growth of the tibia, the ulna is relatively long, the ulnar styloid is displaced to the lower back, and the hand is tilted to the temporal side, resulting in Madelung's deformity, the age of onset is small and the lesions can form a Kaschin-Becky dwarf. The limbs of the patient are not proportional to the head and trunk. Generally, the upper arm is significantly shorter than the forearm, the lower leg is obviously shorter than the thigh, and the trunk is close to normal.
3. The stage of the disease is classified according to the severity of the disease. The disease can be divided into early stage, I degree, II degree, and III degree. The main clinical manifestations are shown in Table 3. According to the retrospective survey of patients with Kashin-Beck disease for more than 15 years, It is found that some patients may become normal in the early stage, and some may evolve into I degree, II degree or even III degree, but none of the cases after the age of 7 become III degree. The early clinical signs are reversible, and patients with I degree or higher can It remains the same or continues to increase, so it is extremely important for the treatment of early patients. It can be seen from Table 3 that the main demarcation point between the early stage and the working degree is whether there are multiple knuckles thickening; the main dividing point of I degree and II degree is whether there is short finger deformity; the main dividing point of II degree and III degree lies in Whether there are short limb deformities and short stature.
The disease progresses further, the patient's fatigue is aggravated, the joints of the extremities are painful, the calves and forearms are aggravated after work or walking, and the joint movements of the extremities are more inflexible. The finger joints or ankle joints are slightly thickened, which is common in hands 2, 3, 4 The first interphalangeal joint, finger, wrist, elbow, knee, ankle joint has mild stretching and flexion difficulty, the elbow joint can not be fully extended, mostly 170°, and there is a clear and constant burst-like rubbing sound in the joints of the limbs. The hand, forearm and calf muscles are slightly atrophied. At this time, the height of the body is as normal as normal, and the general physical labor can be afforded. The patient can have a flat foot, which is the first degree of the disease. The disease progresses to the second degree, the patient's mental and physical strength is worse, the walking is inconvenient, especially when going downhill, it is very difficult, and can only bear light labor, refers to the wrist, elbow, knee, ankle joint activity is limited, accompanied by severe pain. In the knee, the ankle pain is more common. In this period, the patient may have a certain degree of short-finger deformity. The finger flexion is difficult. When the fist is fisted, the finger can't touch the palm surface. The elbow joint is flexed flexibly, and the angle can be formed. The forearm pre-rotation After significant obstacles, the muscles of the limbs are obviously atrophied, often causing severe joint pain due to the formation of joint small bodies in the joints, and the flat feet are heavier.
The disease progresses to the third degree, the patient's activity is difficult, typical duck steps appear when walking, there is a very obvious short-finger deformity, short stature, can become a dwarf with a height of only about 1 meter, hands can not make a fist, elbow flexion is very obvious, Can not stretch to 150 °, limb muscles are extremely atrophy, there is obvious lumbar spine compensatory foreturn, labor ability is extremely reduced, and even lose labor, but mental development is normal. If the patient is from a prevalent area of the disease, it is obviously chronic, the symmetry joint is coarse and deformed, and the body is short and the diagnosis is not difficult. In the epidemic area, if the child has joint pain, activity is not working, or there is friction sound, it should first Think of this disease, can further X-ray examination of bones and joints, especially X-ray examination of the fingers, in order to determine the diagnosis early, laboratory examination has only auxiliary diagnostic effect on the disease. Based on years of research and practical experience, China developed the diagnostic criteria for this disease in 1995.
Examine
Kaschin-Beck disease check
1. General routine examination of blood is normal, there may be biochemical metabolic abnormalities:
(1) The patient's blood, urine, selenium in the hair, vitamin E and glutathione peroxidase activity are low.
(2) The activity of lactate dehydrogenase, alanine aminotransferase, creatine kinase, -hydroxybutyrate dehydrogenase and alkaline phosphatase in blood is increased.
(3) The total amount of erythrocyte membrane phospholipids decreased, lecithin decreased significantly, neurophospholipid increased, the ratio of cholesterol/phospholipid to neurophospholipid/lecithin increased, erythrocyte fragility increased, fluidity decreased, anti-oxidation and anti-hemolytic Reduced ability, increased red blood cell lipid peroxidation products, etc.
(4) urinary creatine, urinary hydroxyproline, urinary mucopolysaccharide and urinary total sulphur increased, creatinine decreased, these biochemical metabolic changes can occur before X-ray changes, epidemiological observation confirmed, population monitoring in epidemic areas The above indicators can be used for early diagnosis and can be used as a reference to reflect the degree of activity.
Liver and kidney function are mostly in the normal range, and immune function is generally low, such as E-rosette formation rate, T lymphocyte transformation rate and C3 are low, and adrenal cortical stress reserve function also declines.
2. Examination of bone and cartilage metabolism In addition to osteochondral damage and skeletal muscle atrophy, other organ organs have not been proven to have any regular changes, and there is still no reliable for cartilage and secondary bone changes. In addition, the simple and easy laboratory testing methods, in addition, the bone disease that has formed in this disease can last for life, some laboratory tests done by the researchers in the early years, which are inherent in the disease, Which are the results of secondary reactions or combined with other diseases, and are not easy to distinguish. In recent years, some tests have been carried out for the study of pathogenesis, and have significance for the observation group. The application to individuals is of little value. Here are some of the main Research result.
(1) The activity of plasma alkaline phosphatase (ALP) is increased, especially in children with Kashin-Beck disease with typical changes of X-ray. Compared with healthy controls in non-disease areas and healthy controls in non-disease areas, there is no significant liver, kidney, etc. In the case of organ damage, ALP is mainly derived from bone, reflecting the active function of osteoblasts.
(2) Hydroxylysine in urine increased significantly, and increased with the severity of the disease reflected by X-ray, but the hydroxyproline change of collagen degradation products was not regular, and some reported urinary hydroxyproline. There is an increasing trend in active critically ill areas, and some reports are the opposite.
(3) The excretion of chondroitin sulfate (Chs) in urine increased, reflecting the decomposition of cartilage matrix and the degree of sulfation of Chs. The electrophoretic mobility of Chs in urine was significantly changed by cellulose acetate membrane electrophoresis. Large, indicating that the molecular weight of Chs becomes smaller.
(4) The blood sulfation factor is low in activity (see above).
3. Examination related to muscle metabolism Because the skeletal muscle atrophy occurs earlier in this disease, some people have measured some components that reflect muscle metabolism. The results of early and recent years are similar. The basic changes are blood creatine and creatinine. Reduced, urinary creatine content increased significantly, urine creatinine was low or the change was not significant.
4. Red blood cell morphology and function changes Under the light microscope, the frequency of target red blood cells in the blood of children with this disease increases, and the deformed red blood cells (thorn cells and oral cells) increase under scanning electron microscopy, which all suggest the disease. The erythrocyte membrane has structural and functional abnormalities. We have already discussed the reduction of the total phospholipids in the erythrocyte membrane of the patient, and the proportion of various components in the phospholipids is abnormal, indicating that the lipid bilayer structure of the erythrocyte membrane has a certain change. In terms of function, it was found that the activity of Na, K-ATPase on the erythrocyte membrane decreased, and the actin of one of the erythrocyte skeleton proteins increased. The fluorescence polarization technique showed that the fluidity of the erythrocyte membrane was slightly decreased.
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