What is the latest onset of tuberous sclerosis?

Tuberous sclerosis, the age of onset is unknown, it may occur at any age, tuberous sclerosis complex is a neurological syndrome inherited by all dominant family members, involving other organs of the human body, and is extremely harmful to patients. If you want to minimize the impact, you have to pay attention to the positive**, because the disease itself cannot**.

Tuberous sclerosis complex occurs mostly in childhood, more males than females, and is inherited in an autosomal dominant manner, and sporadic cases are also more common. The typical clinical features of tuberous sclerosis complex are facial hemangioma, seizures and mental retardation, and some patients can also be combined with skeletal lesions and other visceral lesions, such as pulmonary lymphangiomyolipomas. Sirolimus is mainly given for tuberous sclerosis, which can inhibit the excessive activation of the MTOR pathway and control cell proliferation, and has a good effect on subependymal giant cell astrocytoma and pulmonary lymphangiomyolipomas.

Symptoms of tuberous sclerosis** include seizure control, reduction of intracranial pressure, reduction of cerebral edema, etc. ACTH** can be used for infantile spasms, surgery may be considered for obstruction of cerebrospinal fluid circulation**, and cosmetic ** can be used for facial sebaceous adenoma. Patients with tuberous sclerosis complex generally have a good prognosis.

Tuberous sclerosis complex (TSC), also known as Bourneville's disease, is an autosomal dominant neuro** syndrome, and there are also sporadic cases, mostly caused by abnormal organ development of ectodermal tissues, and multiple organs such as brain, **, peripheral nerves, and kidneys can be involved, and the clinical characteristics are facial sebaceous adenoma, seizures and mental decline. The incidence rate is about 1,6000 live babies, with a male-to-female ratio of 2:1.

1] On May 11, 2018, the Notice on the Publication of the First Batch of Rare Disease Catalogues was announced, and tuberous sclerosis complex was among them.

From a medical point of view, tuberous sclerosis is generally benign, but it will not occur unless there are special circumstances. However, it is important to pay attention to regular check-ups to ensure good health.

I don't know this clearly, and I don't know exactly how big it will be at the latest.

I think some of them may not have the disease until they are in their 70s.

Tuberous sclerosis complex is caused by genetic factors. Sebaceous adenoma skates and mental retardation diseases, if given special care. Generally speaking, there is no particular threat to life, but if it is not taken care of by very professional people.

It can be life-threatening in a short period of time. So. It is necessary to suppress it reasonably.

Tuberous sclerosis complex is an autosomal dominant neurological disease that occurs in childhood and causes damage to multiple organs such as the brain, **, peripheral nerves and kidneys. The clinical manifestations are mainly facial sebaceous adenoma, mental retardation and seizures, and the prognosis of the disease is related to the involvement of organs and the degree of lesions

1.Frequent seizures of epilepsy can lead to a variety of complications and can be life-threatening.

2.When the volume or number of cardiac rhabdomyomas are large, it affects the function of the heart, causing arrhythmia, and in severe cases, sudden death.

3.Organ transplantation is required when the lungs or kidneys are severely affected, and can be life-threatening if not done in a timely manner.

4.When the volume of intracranial subependymal nodules is too large, it will cause hydrocephalus, resulting in increased intracranial pressure, and in severe cases, surgery is risky and affects life.

Most patients with tuberous sclerosis complex will not be affected by close monitoring and regular **.

Tuberous sclerosis complex will affect the life expectancy, and positive ** can reach the life span of a normal person, so patients must start regular ** at an early stage, and adhere to the follow-up and regular review of relevant indicators to prevent the occurrence of complications and strive to improve the prognosis.

Tuberous sclerosis complex (TSC), also known as Bourneville disease, is an autosomal dominant neuro** syndrome, and there are also sporadic cases, mostly caused by abnormal organ development of ectodermal tissues, and multiple organs such as brain, **, peripheral nerves, and kidneys can be affected.

A large proportion of people now have tuberous sclerosis, which is a chromosomal dominant neuro** syndrome. When it occurs, it will bring many harms to the body, such as ** damage, mental retardation, and seizures, and in severe cases, it will also affect the function of the kidneys and heart. If you find that you are feeling unwell, you should seek medical attention in time and maintain a good attitude.

Tuberous sclerosis, also known as nodular sclerosis, refers to an autosomal dominant neuro** syndrome, and the main genes that cause the onset of the disease are TSC1 and TSC2, and 80% of patients can find mutation sites in these two gene loci. However, there are also sporadic cases, which are basically caused by abnormal organ development of ectodermal tissues, and what kind of harm will be brought to patients when the disease occurs, the following article will give you a brief introduction to the five harms caused by tuberous sclerosis.

1. There is ** damage, and 90% of the symptoms of this kind generally occur before the age of four. Mainly on the upper part of the limbs of the hands and feet on the trunk of the body, the diseased area will be rough, hard, thick, and higher than the normal **, gray-brown, slightly brown patches will appear. There are also a small number of triangular areas of the mouth and nose, ** the surface appears light red or reddish-brown, and there are pimples the size of a needle tip or broad bean, and it is relatively hard, and the color will become lighter when pressed by hand.

2. It can cause seizures, and it has been found that it can start from infantile spasms at the earliest. Symptoms are localized at first, but as the disease worsens, it progresses to a generalized grand mal seizure. If seizures are frequent or persistent, they may trigger epileptic personality disorder or have some effect on personality.

3. Mental retardation, this symptom will mainly include emotional instability, childish behavior, and also very impulsive, or chaotic thinking. People with this type of mental retardation generally have some epilepsy symptoms.

4. It will affect the function of the kidneys, which will have a certain relationship with the number or size of nodules.

5. It will affect the function of the heart, and also include the tuberous sclerosis of the lungs and bones. Nodules in different parts of the body can cause different injuries to the body.

The above five are the hazards caused by tuberous sclerosis, and when the symptoms are detected in the early stage, it is necessary to go to the hospital for examination in time to confirm the diagnosis and correct the symptoms, so as not to delay the best time for the disease. In the process of recovery, it is also crucial to adjust the mental state, do not produce excessive fear or psychological burden, relax your mentality, and actively deal with the disease.

There are two drugs used, one is rapamycin and the second is everolimus. These two drugs are a large class of drugs, and everolimus is a derivative of rapamycin, and the new generation of derivatives may be a little safer.

The two drugs are almost as effective, and there are some indications that can be used. Now, after some observations and studies at home and abroad, it has been proved that for example, this class of drugs, their indications, including renal AML (angiomyolipoma) can cause end-stage renal damage, and such a lesion as kidney dialysis or kidney transplantation is referred to as AML.

Like LAM (lymphatic leiomyomatosis) in the lungs, which is a lesion that can cause end-stage lung transplantation, these fatal TSC (tuberous sclerosis) lesions, after using these drugs, the MTOR pathway inhibitors can stop its progression.

After long-term medication, it will be able to reverse and reduce, which means that it can improve his kidney function and improve his lung function. Therefore, this should be fought for as early as possible, and if it is discovered, it should be analyzed whether the patient has damage to these important organs.

Tuberous sclerosis is not usually an advanced stage of cancer, but it can develop, so it is important to take measures to prevent the disease by having regular check-ups once it is detected.

At present, tuberous sclerosis is more common, is a disease that can not be ignored, easy to induce obvious sebaceous adenoma, and will bring inconvenience and distress to patients, affecting the normal life of patients, every friend should pay attention to the disease, in-depth understanding of the symptoms of the disease, the disease of the patient's ** damage is more obvious, most patients have a sebaceous adenoma in the oral and nasal triangle, and will show a symmetrical butterfly-shaped distribution, showing light red or reddish-brown, slightly faded when pressed, Most patients develop before the age of four, worsen with age, and generally do not affect the upper lip.

Some children will produce more than three long leaf-shaped, ovoid loss spots after birth, and if observed under the ultraviolet lamp, it is particularly obvious, mostly occurring in the patient's limbs and trunk, and after growing to the age of ten, shark skin spots will occur in the lumbosacral area, slightly higher than the patient's normal **, local ** thickening is obvious, and will show gray-brown or slightly brown plaques, bringing more trouble and inconvenience to the patient. After the onset of the disease, it is necessary to cooperate with the relevant nursing work as soon as possible**.

Tuberous sclerosis is complicated, long, and tricky, and there is no ideal way of Western medicine at present. In some patients with tuberous sclerosis, many of them have epilepsy since infancy, and 1 3 of them will have infantile spasms, which seriously affects the intelligence of the child. As people get older, people after the age of 40 are more likely to have the disease.

For people with tuberous sclerosis, some complications are inevitable, such as depigmentation in the normal population, but as high as 97% in the sclerosis population, and epilepsy in 80-95%. Studies have found that 80-100% of patients may develop **damage, such as sebaceous adenoma. Tuberous sclerosis complex is a disease that can develop in infancy, but in addition to this, it can also develop in adults.

This disease will induce lesions in the internal organs, which will bring very serious dangers to the patient, so it is necessary to pay attention to it, and when the disease is found to be abnormal, it is best to ** in time.

Tuberous sclerosis complex is an immunosuppressive disease of the nervous system that can occur at any age, and if it runs in families, the age of onset will be relatively earlier.