When does progressive muscular dystrophy usually occur?

It is recommended to do ** physiotherapy with oral Chinese medicine conditioning for a period of time.

Duchenne muscular dystrophy, also known as pseudohypertrophic muscular dystrophy and Duchenne muscular dystrophy, is an X-linked recessive inherited disease. The annual incidence rate is about 1 in every 3500 live births. The disease-causing gene DMD is located at XP21, which occurs in males and carries the disease-causing gene in females.

Children with DMD generally develop muscle weakness at the age of 3 to 5 years, and the condition progressively worsens, losing the ability to walk independently at about 12 years of age, and dying of muscle weakness and respiratory failure at about 20 years of age. Becker's muscular dystrophy is also caused by a defect in the DMD gene, and the clinical symptoms appear later than Duchenne muscular dystrophy, and the progression is relatively slow, and after the age of 18, it can still walk independently, and can survive to adulthood 40 to 50 years old or even longer.

At present, there is no specific drug for completely progressive muscular dystrophy in the world, but relevant studies have shown that such diseases can be improved and restored muscle function through the use of some drugs. Because different patients with progressive muscular dystrophy have different genes and different symptoms, if the child can be detected in the early stage of the disease, or can control the disease through drugs, it will not pose too much threat to life in a short period of time, and parents do not need to worry too much.

Progressive muscular dystrophy** myth:

1. Malnutrition = undernutrition.

Many people think that malnutrition is insufficient nutrition, often after the child is diagnosed with progressive muscular dystrophy, will give the child myopia a lot of food, encourage the child to eat more, in fact, the child suffering from this disease itself is very little exercise, if you eat a lot of food, a large amount of fat will accumulate on the body, resulting in obesity, which will further reduce the patient's mobility and affect the effect of recovery.

2. Rush to the doctor when you are sick.

Many parents find out that their children are sick and anxious, so they will seek medical treatment in a hurry, and some people even believe in rumors that such a disease can be **, spend a lot of money, and the child's disease ** is also delayed, I hope everyone has to face the reality, understand the current level of medical care, and think about this kind of disease, it will take time.

3. The amount of exercise.

The main manifestation of children with progressive muscular dystrophy in the early stage is difficulty in exercising, and some parents will strengthen their children's exercise, if the amount of exercise is excessive, it is easy to cause fatigue, which is likely to aggravate the condition.

What is the cause of muscular dystrophy, the occurrence of muscular dystrophy is mainly related to genetic factors, when the patient's body has a large number of deletions and duplications of genes will lead to the occurrence of this disease, once the disease is serious, it will lead to paralysis of the patient, so the patient must pay attention to this disease.

Muscular dystrophy is a disease that generally occurs in childhood, and then as the patient gets older, the severity of the disease will become more and more serious, and the patient's condition is likely to lead to paralysis after reaching a certain level, so it can be seen that the disease is extremely harmful to the patient's body, so we must pay attention to this disease. So what causes muscular dystrophy?

In fact, the occurrence of muscular dystrophy is mainly caused by genetic factors, and the way of inheritance is diverse, although there are many genes and have been located and cloned, and some gene products have also been clarified, but there are also some pathogenic genes so far are also a lot of very clear, but according to the research found that the occurrence of this disease may be caused by the mutation of the gene locus, caused by the defects and abnormalities of the expression product muscle membrane structural protein. Once the disease occurs, it will directly affect the patient's physical health.

In addition, there are some patients who have mutations due to the deletion of large fragments of genes or repeated mutations, which can easily lead to dysfunction or structural dysfunction, which will also cause muscular dystrophy. The BMD gene and DMD are in the same area, and DYS is located in the inner layer of the muscle fiber membrane, which can play a role in stabilizing the muscle fiber membrane, when the DYS is abnormal, it will naturally lead to the occurrence of muscular dystrophy, so the occurrence of muscular dystrophy is directly related to heredity, so we should do a good job in prenatal diagnosis at ordinary times, so as to play a preventive role in this disease.

The initial symptoms of progressive muscular dystrophy are muscle atrophy, weakness, etc., which can be used under the guidance of a doctor.

It is a large group of inherited diseases caused by defects in related genes.

Different types of progressive muscular dystrophy have different genes that cause mutations.

What are the causes of progressive muscular dystrophy?

All types of progressive muscular dystrophy are caused by defects in the associated genes, resulting in changes in protein structure, affecting protein function and ultimately causing progressive destruction of muscles.

Taking the most common Duchenne Becker muscular dystrophy as an example, the cause of the disease is a defect in the DMD gene located on the X chromosome, which makes the expression product dystrophin (dystrophin) loss or expression disorder, resulting in impaired stability of the muscle cell membrane, necrosis and loss of function of muscle cells.

Among them, the function of the DMD gene in Becker-type muscular dystrophy is not completely lost, so the age of onset is later and the disease is significantly less severe than that of Duchenne muscular dystrophy.

Progressive muscular dystrophy (pseudohypertrophy) is a genetic disorder controlled by a recessive pathogenic gene located on the X chromosome, characterized by progressive atrophy of skeletal muscle, gradual loss of muscle strength, and finally complete loss of exercise capacity. Occurs mainly in boys; Females are carriers of the gene and have a significant family history of the disease.

If you talk about the causes of progressive muscular dystrophy, then you must say that there are many aspects, after all, you can't find the cause, like this pathological cause, try not to talk about it, it may be related to your usual habits, and that kind of long-term genetic factors are related.

Progressive muscular dystrophy is a group of inherited disorders that can be sporadic in the form of genetic mutations in which different proteins do not play exactly the same role in the structure of muscle cells, resulting in different types of muscular dystrophy.

Progressive muscular dystrophy is a group of inherited muscle degenerative diseases. The clinical features are slowly progressive, symmetrical muscle weakness and atrophy, and elevated serum CK, which is hereditary. Electromyography showed that the myogenic damage was present, and the pathology showed extensive myofiber atrophy in a small circle, accompanied by myofiber degeneration, necrosis and regeneration, and in severe cases, it was accompanied by a large amount of fat and connective tissue hyperplasia.

There is currently no effective method. According to the genetic mode, age of onset, distribution of atrophic muscles, the rate of progression and prognosis, progressive muscular dystrophy can be divided into the following types: pseudohypertrophic muscular dystrophy, facimoscapulohumeral muscular dystrophy, limb-girdle muscular dystrophy, congenital muscular dystrophy, etc.

The study of the pathogenesis of this disease has attracted worldwide attention. Over the past decades, there have been theories such as vascular, neurotic, muscle fiber regeneration disorder and cell membrane defects, but a large number of research evidence shows that cell membrane defects play an important role in the occurrence of this disease. One-third of newborn boys are caused by genetic mutations.

With the in-depth development of molecular biology research, the pathogenesis and pathogenesis of this disease have been further elucidated. It has been clarified that this disease is a type of monogenic inherited disease, and the inheritance mode is diverse, many pathogenic genes have been located and cloned, some gene products have been elucidated, and some pathogenic genes are still unknown. Mutations in related gene loci can cause defects and abnormalities in the structural proteins of the muscle membrane that are the products of their expression.

There is also a new understanding of the molecular mechanisms of different types and subtypes. Among them, Duchenne and Becker muscular dystrophy (DMD, BMD) are the most deeply studied. DMD is an X-linked recessive genetic disease, and the pathogenic gene has been mapped in the 1 band 2 3 sub-zone of the short arm of the X chromosome, and the cDNA of the gene has been cloned, with a total length of 14kb, 60 65 exons, and the gene expression product is dystrophin (dystrophin, dys).

When there is a deletion, duplication, or other form of mutation such as point mutation in a gene, the absence of dys or structural dysfunction is the root cause of DMD. The BMD gene is in the same region as DMD and is an allele of each other. DYS is located in the inner layer of the myofibrous membrane and is a cytoskeletal protein that stabilizes the myofibrous membrane.

In DMD patients, due to the lack of dys in muscle fibers, the integrity of the muscle membrane structure is destroyed, and a large number of extracellular components rich in calcium ions flow into the muscle cells, which eventually leads to myofiber degeneration, necrosis and morbidity. The causative gene of Emery-Dreifuss muscular dystrophy is mapped to XQ28, and its encoding protein is Emerin. In recent years, it has been found that the occurrence of limb-girdle muscular dystrophy (LGMD) is related to genetic defects in the dystrophin-glycoprotein complex (DGC) attached to the muscle fiber membrane.

DGC plays an important role in maintaining the stability of myofibrous membranes and preventing membrane damage and necrosis. Facicoscapulohumeral muscular dystrophy (FSHD) is the most common autosomal dominant myopathy in adults. The gene localization is 4q35, the gene has not been cloned, and the encoded protein has not been isolated, but FSHD has been shown to be related to the deletion of copy number of tandem repeats at the end of the long arm of chromosome 4.

Molecular mechanisms of different subtypes of other subtypes, such as distal muscular dystrophy, are also being recognized.

Progressive muscular dystrophy is a primary skeletal muscle disorder caused by genetic factors. Slow-moving muscle atrophy, muscle weakness, and varying degrees of dyskinesia.