Kashin-Bek’s disease

Kashin-Bek disease (Level disease)
Kashin-Beck’s disease is an endemic disease accompanied by multiple degenerative changes of the joints and spine. It is distributed mainly in Eastern Siberia. It is provoked by a violation of the balance of trace elements due to the peculiarities of local water and food. It is manifested by pains, which are subsequently joined by deformations, crunching, restriction of movements. At an early start, low growth and a change in body proportions are revealed. The diagnosis is made according to anamnesis, physical examination, radiography. Treatment – physiotherapy, drug therapy, removal of articular bodies, elimination of contractures. In severe cases, endoprosthesis is indicated.

General information
Kashin-Bek’s disease (level disease) is a pathology that develops due to a violation of the balance of mineral substances entering the human body with water and food. The first mention belongs to the Russian doctor N.I. Kashin (1861), a detailed description was made by domestic specialists, the Beck spouses in 1899-1902. The disease is common in Transbaikalia, Buryatia, and some other regions. It is found in Korea, China. Usually manifests in childhood, less often begins in adults. After 5 or more years after moving to an endemic area, it can be detected in an erased form in visitors. Men and women suffer with the same frequency.

Reasons
The etiology is not precisely established. Most researchers believe that the main cause of the disease is an imbalance of trace elements in the local water and soil. According to the Soviet doctor A.P. Vinogradov, pathology is potentiated by a lack of calcium, an excess of silver, iron, manganese, zinc. In support of his theory, he cites the results of studies of the bone tissue of patients in which such a violation of the ratio of mineral substances is observed.

Some modern experts point to a lack of selenium with an excess of manganese and phosphorus. the authors believe that hyperphosphatemia provokes a violation of hormonal regulation of osteogenesis. Chinese scientists name the lack of selenium as the most significant factor. Hypovitaminosis plays a certain role, especially vitamin D deficiency. There are studies indicating the possible significance of hereditary predisposition, which is realized in the presence of unfavorable endemic conditions.

Pathogenesis
The main morphological manifestation of Kashin-Beck’s disease is the degeneration of the bones of the skeleton with a predominant lesion of the epiphyseal and metaphyseal zones. Due to damage to the growth zones, bone growth in children’s patients slows down, stunting is formed. Articular surfaces are deformed, which explains the early development of arthrosis.

Violation of the congruence of the joints and the shape of the articular surfaces causes subluxations, external disfigurement, restriction of movements, the appearance of contractures. With necrosis of areas of cartilage tissue, separation of cartilage sections and their migration into the articular cavity, reactive synovitis develops. Vertebrae and intervertebral discs are also deformed, osteochondrosis, arthrosis of intervertebral joints, intervertebral hernias occur. Sometimes there is ossification of the anterior longitudinal ligament.

Classification
Kashin-Bek’s disease is traditionally classified according to the severity of its manifestations. Taking into account the severity of functional disorders, the level of disability, there are three degrees of pathology:

1 degree. The ability to work is preserved, there is a soft crunch, joint pain or increased fatigue during physical exertion. External signs are limited to thickening of the joints of the fingers of the hand.
2nd degree. The ability to work is limited, short-fingered, reduced growth, impaired flexion of the fingers of the hand, pathological changes in the ankle and knee joints, muscle atrophy are detected. Shoulder and hip joints are unchanged, pain is possible.
3rd degree. The ability to work is significantly limited or lost. Short-fingered, stunted, gross limitations of finger movements, contractures of the wrist, elbow, hip joints, pathological lordosis are determined.
At a late onset, Kashin-Beck’s disease of the 2nd and 3rd degrees can manifest itself mainly by neuropisceral disorders without forming a typical picture described in the classification.

Symptoms
1 degree
The disease usually begins at the age of 6 to 10 years, develops gradually. The first sign of pathology is common aching pains in the joints, vertebral column. The pain syndrome is unstable, expressed slightly or moderately, accompanied by a feeling of stiffness, a soft crunch during movements. Possible cramps and paresthesia in the fingers, muscles of the lower leg.

At first, the interphalangeal joints are affected, especially the proximal joints of the II-IV fingers. They thicken, deform. With a favorable course, the disease is limited to the listed manifestations or their combination with a slight restriction of movements in the elbow and wrist joints, there is no progression of symptoms.

2nd degree
More often, the disease passes into the second stage, in which most joints are involved in the process to one degree or another. The joints of the lower extremities – knee and ankle – mainly suffer. Thickening and external deformation are combined with limited mobility. The axes of the lower extremities are curved. Moderate muscular atrophy is detected.

The remaining joints are affected symmetrically, but there are no external manifestations, the symptoms are limited to pain and crunch. The pain is intermittent, non-intense, intensifying in the afternoon, at night. Patients lag behind in growth, the average indicators for men are 160 cm, for women – 146 cm. Violation of the normal growth of the fingers of the hand causes the formation of short fingers. The nail phalanges do not reach the palm when bending. Rapid extension is difficult.

3rd degree
At the third stage, widespread symmetrical deformities of all joints are revealed against the background of significant restriction of movements. Average growth rates remain the same. Changes in the appearance of patients are aggravated by a short neck, increased lordosis of the lumbar region, significant valgus or varus curvature of the lower extremities.

Due to the lag in growth and simultaneous expansion of the foot, they take the form of “bear paws”, flat feet are formed. When walking, patients waddle from side to side (duck gait). Many women have a narrow pelvis.

Neuro-visceral disorders
Asthenoneurotic syndrome is detected in patients with grade 1 of the disease. Then encephalopathy develops, there is a gradually progressive decrease in intelligence. In the atypical course of Kashin-Beck’s disease, typical asthenoneurotic and encephalopathic manifestations are determined against the background of unchanged or slightly altered joints. Similar cases can be found both among local residents and natives of other regions who have been living in this area for a long time.

Headaches, dizziness, decreased appetite are possible. Due to neurotrophic disorders, the condition of the skin appendages is disturbed – early wrinkles appear, hair and nails become dull, brittle. Possible visceral manifestations include myocardiodystrophy, gastritis, enterocolitis, bronchitis, diseases of the ENT organs (pharyngitis, rhinitis, otitis media). Anemia is detected in a number of patients.

Complications
After the development of pronounced arthrotic changes and the appearance of free articular bodies due to the separation of pieces of altered cartilage, recurrent effusions and joint blockages manifest. Over time, contractures form that limit the ability to work and self-service opportunities. Secondary arthritis is possible. When the spine is affected, radiculitis occurs.

Diagnostics
The diagnosis is made by orthopedic doctors. In the process of diagnosis, anamnestic data, the results of external examination and additional studies are used. The survey plan includes:

Survey. Characteristic signs of the disease are living in a certain area, early onset, slow progression, a certain sequence of lesions of various parts of the skeleton, the absence of episodes of fever.
Physical examination. In favor of Kashin-Beck’s disease, there is evidence of a decrease in growth, short-fingered feet, typical deformities of the feet, hyperlordosis, symmetry of joint damage.
Radiography of the hands. Brushes are involved first of all, so the technique is informative even at the initial stages of the disease. The expansion of the epiphyses in the form of flat bowls and goblet-shaped deformation of the metaphyses are revealed. The heterogeneity of the bone substance in the metaphysical zone, subchondral sclerosis is determined.
Radiography of large joints. When examining adolescent patients, premature closure of germinal zones is detected. Other features are similar to changes in the hands. Osteophytes, narrowing of the articular gap are viewed. Joint lesions are symmetrical.
Radiography of the spine. Vertebrae suffer less often than other parts of the skeleton. Uneven height of intervertebral discs with a predominant tendency to decrease may be detected. The closure plates of the vertebrae are deformed, but retain continuity. Sometimes osteophytes, areas of calcification in the ligament area are visualized.
Differential diagnosis
Differential diagnosis is performed with osteochondropathy, rheumatoid arthritis, chondrodystrophy and epiphyseal dysplasia. Sometimes Kashin-Beck’s disease must be distinguished from hypothyroidism and rickets. In first-time adults and elderly patients with 1-2 degrees of the disease, it may be necessary to exclude paraneoplastic syndrome.

Treatment of Kashin-Bek’s disease
Pathology treatment can be conservative and operative. Conservative therapy is long-term, usually carried out on an outpatient basis. Surgical interventions, as a rule, are performed in the final stages of the disease, with the development of irreversible changes that grossly violate the functions of the joints.

Conservative therapy
The goal of conservative measures in the early stages is to stop the pathological process and complete recovery. Subsequent treatment is aimed at reducing the severity of symptoms, increasing the ability to work and self-care, slowing the progression of the disease. Apply:

Drug therapy. Patients are prescribed phosphorus and calcium preparations, vitamin complexes and means to stimulate regeneration (ATP, vitreous, etc.).
Non-drug methods. Radon baths provide a good effect. Among other physiotherapy techniques, UHF, ultrasound and diathermic currents are used. Massage and regular physical therapy sessions are shown.
Since Kashin-Beck’s disease is rarely accompanied by inflammation and intense pain, medications from the NSAID group are not used. Spa treatment in specialized sanatoriums is recommended.

Surgical treatment
Operations are performed at stages II and III of the disease. The purpose of the interventions is to eliminate contractures and other obstacles to movement. Depending on the nature and severity of pathological changes are performed:

Removal of intra-articular bodies. It is shown in the presence of articular mice that are pinched between the surfaces of the joint, causing blockages. It is usually performed using arthroscopic equipment.
Redressation. Forcible rupture of the scar tissue of the capsule to increase the volume of movements. It is carried out under general anesthesia. It can be performed in addition to the removal of intra-articular bodies or independently. In the latter case, no incisions are required.
Arthroplasty. It is recommended for pronounced changes in articular surfaces that create obstacles to movement. It includes the removal of altered tissues, modeling of the articular ends of bones and placing between them a pad of soft tissues of the patient that prevents fusion.
Endoprosthetics. It is the most effective method for severe violations of joint function. Allows you to restore the volume of movements and ability to work. Most often, knee and hip joint prostheses are installed, less often – joints of the upper extremities.
In the postoperative period, therapeutic gymnastics is prescribed to prevent contractures and muscular atrophy. Massage, physiotherapy procedures, multivitamins, mineral preparations are used.

Forecast
The prognosis is determined by the degree of Kashin-Bek’s disease. At the initial stage, complete recovery can be achieved in 30% of patients, in other cases, stabilization of the process is observed. In the subsequent stages, recovery is impossible due to the presence of widespread organic changes, but treatment allows you to prevent the progression of the disease, maintain working capacity or achieve a significant improvement in functionality.

Prevention
Preventive measures are comprehensive, including soil treatment, fertilization of farm animals with mineralized additives, delivery of imported products and water with a balanced mineral content to endemic regions. Regular examinations of children are of great importance for the timely detection and treatment of the disease. Children, young people and pregnant women are prescribed preventive courses of vitamins and minerals.

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