Hutchinson-Guilford syndrome: features of the course, treatment and causes of the onset of the disease
Hutchinson-Guilford syndrome is a genetica disease that is characterized by premature and very rapid aging, which begins with the birth of the child. It should be noted that there are very few such patients in the world. Pathology has another name - progeria.
Symptomatology of the disease
Hutchinson-Guilford syndrome has obvious signs:
- small growth;
- The skull has abnormally enlarged dimensions;
- lack of hair, eyebrows and eyelashes;
- "bird's face";
- bone and other body systems are deformed;
- absence of a subcutaneous fat layer;
- a strong lag in physical development, while thinking and psyche are normal.
It should be said that people have a certainaverage life expectancy: only 14 years, although a unique case is known when a person with such a diagnosis lived up to 45 years. Death most often occurs due to heart failure, as well as excessive fat deposition in the brain tissues.
The causes of the appearance of the disease and the peculiarities of treatment
Hutchinson-Guilford syndrome is a pathology,provoked by violations in the human chromosomes, that is, she can not cure the complete cure at the moment. The meaning of the defect lies in the fact that the number of cell divisions significantly decreases, in comparison with the norm.
It should be noted that the syndromeHutchinson-Guilford (children's form) can be affected by any child (regardless of the age and lifestyle of parents). He becomes noticeable at the age of two. There is also Werner's disease, which affects already adult people. The growth of the organism stops at the age of three.
There is no special diagnosis of this pathology, soas it is not thoroughly studied. In addition, it is clearly visible on the listed symptoms. As for the treatment of the disease, there are no effective drugs that could stop it and return a person to a normal life. However, children who have Hutchinson-Guildford syndrome should be registered with a doctor and be constantly inspected. Certain procedures can help slow the progression of pathology.
The treatment plan for the syndrome is compiled separately forof each patient. It includes an increase in the motor activity of the child, physiotherapy procedures. Due to the use of small doses of aspirin in a baby, the risk of a stroke is reduced. Sometimes the patient undergoes surgical interventions that are aimed at shunting the vessels, removing the milk teeth.
Features of development of children suffering from the presented disease
If a child can not grow physically, his thinking and psyche do not suffer. A kid can learn to read, can learn. Naturally, the learning process will take place at home.
As for the subsequent forecast, heIt is disappointing. Such children do not live long. The fact is that they spend 6-8 years in one year. Naturally, scientists are working to uncover the mechanism of the appearance of the disease and ways to stop it. However, the current level of development of medicine can only slightly improve the quality of life of the patient, as well as slow the aging process.
