Marie-Bamberger syndrome
Introduction
Introduction to Marie-Bamberger Syndrome Marie-Bamberger syndrome (Marie-Bambergesyndrome) is also known as pulmonary hypertrophic osteoarthrosis, chronic pulmonary osteoarthrosis, systemic hypertrophic osteoarthrosis, secondary pulmonary osteoarthrosis, the syndrome first by Bamberger (1889) reported that in the following year, Marie has already considered patients with acromegaly, with changes in bone deformation combined with chronic lung disease, and another named pulmonary proliferative osteoarthrosis, hence the name of the syndrome. . Berman's definition of this syndrome in 1963 supplemented three essential signs: clubbing; periosteal proliferation of long tubular bone; swelling, pain and tenderness of joints. The clubbing accounts for 18% to 33% of chronic lung disease, and up to 60% of primary lung cancer. basic knowledge The proportion of sickness: 0.002%-0.003% Susceptible people: no specific population Mode of infection: non-infectious Complications: joint pain
Cause
Causes of Marie-Bamberger syndrome
Causes:
The cause is not clear.
The pathogenesis of Marie-Bamberger syndrome is not well understood, but hypertrophic osteoarthrosis is recognized as a special response to certain disease states. There are several hypotheses:
1. Body fluid theory: Under normal circumstances, the lungs can remove or inactivate certain factors from the patient's organs or tissues, but in the case of lung disease, the lungs cannot clear or inactivate this factor, causing it to enter the circulation. It causes characteristic bone and soft tissue hyperplasia, but the existence of this factor has not been confirmed so far. A number of recently discovered tumor-derived growth-promoting polypeptide factors have provided support for the development of this theory.
2. Neurology: It is believed that the diseased organ transmits an impulse through the vagus nerve, and the blood vessels of the fingertips are dilated and deformed by the reflex mechanism. When the vagus nerve is cut, the pain and signs can be alleviated, and the blood flow of the affected part is also reduced.
3. Receptor theory: In recent years, it has been found that glucocorticoid receptors and epidermal growth factor receptors in patients with hypertrophic osteoarthrosis increase the level of urinary epidermal growth factor. It was also found that changes in glucocorticoid receptor and epidermal growth factor receptor are associated with characteristic skin changes in this disease, and increased levels of urinary epidermal growth factor may be associated with systemic changes such as subperiosteal new bone formation. It has also been found that increased blood flow in the lesions of secondary hypertrophic osteoarthrosis is thought to be due to increased blood supply and increased concentration of deoxygenated hemoglobin, leading to periosteal hyperplasia and ossification of hypertrophic osteoarthrosis caused by tissue hypoxia. The blood flow of the primary hypertrophic osteoarthrosis lesions was slow and local hypoxia, which was significantly different from the change of secondary hypertrophic osteoarthrosis, but the lesions were the same. The mechanism is still unclear. Some people think that the two should belong to different diseases.
Prevention
Marie-Bamberger Syndrome Prevention
Active treatment of the primary disease.
Avoid cold and cold diet. To quit smoking and stop drinking. Chinese medicine believes that moderate drinking, phlegm and cold, blood circulation, and phlegm fitness, and modern medical research proves that drinking white wine, beer is harmful to the human body and no benefit, only drinking a small amount of wine has a softening effect Patients with osteoarthrosis will feel relieved after drinking alcohol. This is because drinking alcohol inhibits the function of the central nervous system, and the symptoms are aggravated after alcoholism. Long-term dependence on alcohol and pain is more harmful to the human body, especially for patients with femoral necrosis. Stop drinking. Smoking is harmful to health, everyone knows, but smoking is harmful to bone disease. Everyone should also know that nicotine in the smoke dissolves in the blood, which can poison vascular endothelial cells, leading to abnormal blood vessel contraction and thrombosis. Osteoporosis, bone hyperplasia, intervertebral disc degeneration.
Complication
Marie-Bamberger syndrome complications Complications, joint pain
Can be complicated by joint effusion.
When the joint produces lesions or some systemic diseases occur, the joint fluid increases to form joint effusion, causing joint pain and discomfort. There is a small amount of joint fluid in the knee joint to nourish the articular cartilage, lubricate the joints, and reduce the friction during joint movement. The joint fluid is secreted by the synovial membrane, and the joint fluid continuously circulates and renews during joint movement. When the joint fluid exceeds 10 ml, the floating raft test is positive.
Symptom
Symptoms of Marie-Bamberger syndrome Common symptoms Joint swelling, shortness of breath, joint pain, clubbing (toe), subcutaneous nodules
1. Clubbing: The syndrome has almost all clubbing (toe), the degree of severity is different, the lighter only sees the nail slightly lightly curved, and the severe one can be a typical drumstick.
2. Joint symptoms: joint pain and swelling of the extremities, the most common for the forearm and lower limbs, the pain is mostly persistent, joint tenderness, but rare redness and subcutaneous nodules.
3. Pulmonary chronic disease manifestations: such as chronic bronchitis, obstructive emphysema, lung cancer, etc., the occurrence of bone disease often precedes lung diseases, such as lung cancer.
Examine
Examination of Marie-Bamberger syndrome
Laboratory examination of a small number of patients with obvious joint symptoms, may have elevated body temperature, increased ESR.
X-ray examination of lesions is most common in the palm, sputum, ulnar, sputum, sputum, sputum and other bones, the lesion is bilateral symmetry of periosteal hyperplasia, generally from the distal end of the backbone to the proximal end of the spread, gradually weakened, severe cases can affect the whole body The diaphysis, even the end of the bone, the periosteum of the hyperplasia is lace-like or scallion-like, with a thickness of about 1 to 10 mm. In addition, the X-ray can show osteoporosis of the long tubular bone, thinning of the cortex, and end finger (toe). The bones are enlarged.
Diagnosis
Diagnosis and diagnosis of Marie-Bamberger syndrome
It is distinguished from Keshan disease.
Keshan disease: It is an unidentified disease characterized by cardiomyopathy, also known as endemic cardiomyopathy. First discovered in Keshan County, Heilongjiang Province in 1935, it was named after Keshan disease. In the past, the mortality rate of this disease was high. After the founding of New China, the disease was actively prevented, and the incidence and mortality of the disease were greatly reduced. Some important advances have been made in prevention, treatment and etiology research. At present, the annual incidence rate has dropped below 0.07/100,000, and the incidence type has changed from emergency type to sub-acute type to potential type and slow type. Mainly. A large number of epidemiology, pathological anatomy, clinical prevention and laboratory research results show that the disease is an independent endocardial disease.
