What does mucopolysaccharidosis bone marrow transplantation mean

summary

Due to congenital defects will lead to some patients with mucopolysaccharide disease, the appearance of this disease will have a great impact on the health of patients, generally doctors will recommend bone marrow transplantation. Now let's talk about the meaning of bone marrow transplantation for mucopolysaccharidosis.

What does mucopolysaccharidosis bone marrow transplantation mean

The first is the disorder of proteoglycan catabolism caused by the congenital defect of proteoglycan degrading enzyme. It is characterized by excessive accumulation and excretion of oligosaccharides. Mucopolysaccharidosis type I (H) is also known as Chengliu disease because it has an ugly face and looks like a monster in the gutter under the eaves of ancient Chinese buildings. The number of male patients was more than that of female patients, most of them were the offspring of close relatives, and most of them had family history. There is no specific treatment, only symptomatic and supportive treatment.

Second: mucopolysaccharide, i.e. amino polysaccharide, is a straight chain polysaccharide, which is composed of hexosamine and uronic acid, and is repeatedly connected to form a long chain. Mucopolysaccharide contains uronic acid and sulfuric acid group, so it is acidic. Hyaluronic acid, chondroitin, dermatan sulfate, heparin and keratin sulfate are common mucopolysaccharides in connective tissue. Under normal conditions, mucopolysaccharide is a non fibrous component of connective tissue, which is firmly bound to protein and widely distributed in cartilage, cornea, vascular wall and subcutaneous tissue.

Third: in vivo, mucopolysaccharide is degraded by a variety of enzymes in lysosomes. Any congenital defect of nucleosidase and phosphatase can affect the decomposition of mucopolysaccharide and make mucopolysaccharide accumulate in lysosomes. After the accumulation of mucopolysaccharide in liver, spleen, cartilage, bone, myocardium and nerve tissue, these organs will have nutritional disorders and functional abnormalities. Due to the different defective enzymes in the process of mucopolysaccharide degradation, the types of mucopolysaccharide deposited in organs and tissues are also different, and the clinical manifestations are also different.

matters needing attention

Before the bone marrow transplantation of mucopolysaccharidosis, it is necessary to find the bone marrow that matches the patient successfully. The general hospital needs more than 100000, and later may also need to carry out regular examination, also need to spend a certain amount of medical expenses. In addition, patients also need to pay attention to diet after surgery.