How to prevent Gitlin syndrome?

summary

The gene of Guillain's syndrome is located on the X chromosome, which is a sex linked recessive severe combined immunodeficiency disease. The pathogenesis is not clear. Most of the patients were male, and were prone to upper respiratory tract infection, otitis media, pyoderma, mucocutaneous candidiasis and virus infection. The diagnosis depends on genetic investigation and X chromosome examination. See severe combined immunodeficiency disease for treatment. Most of the children died in early childhood, but the survival time was longer than that of severe combined immunodeficiency disease. So how to prevent Gitlin syndrome?

How to prevent Gitlin syndrome?

Prevention of infection should pay attention to isolation, try to reduce contact with pathogens. For severe combined immunodeficiency disease, children must be placed in sterile warehouse for a long time until the immune function is reconstructed. Vaccination should be avoided. Live vaccines such as vaccinia and BCG should be prohibited for newborns with suspected immune deficiency, so as to avoid systemic vaccinia caused by vaccinia and death caused by systemic dissemination caused by BCG. Measles and polio vaccines should also be avoided.

The gene of Guillain's syndrome is located on the X chromosome, which is a sex linked recessive severe combined immunodeficiency disease. The cause of the disease is not clear. Due to the short discovery time, few cases, unclear etiology and epidemiology, various theories have not been generally recognized, and are still under exploration and observation.

The clinical manifestations of the disease are different, and the mild patients have no clinical symptoms. Some patients have similar clinical manifestations of AIDS related syndrome (ARC). The typical manifestations of idiopathic CD4 + T lymphocytopenia are similar to those of AIDS, but only 40% of patients have typical aids like symptoms. The common manifestations of the disease are emaciation, fatigue, chronic diarrhea and unexplained abdominal lesions. About 50% of the patients have skin symptoms, most of which are symptoms of various infections, such as herpes zoster, herpes simplex, wart, infectious soft wart, condyloma acuminatum, oral candidiasis, tinea cruris, skin cryptococcosis, skin abscess, folliculitis, etc. There are also skin basal cell tumor, squamous cell carcinoma, Kaposi sarcoma, non Hodgkin's lymphoma, psoriasis, chronic prurigo, eczema, contact dermatitis, contact urticaria, etc. In addition, idiopathic thrombocytopenic purpura may occur. Patients can have a variety of conditional infections, such as extrapulmonary cryptococcosis, Pneumocystis carinii, atypical mycobacillosis, central nervous toxoplasmosis, cytomegalovirus enteritis, cytomegalovirus retinitis, histoplasmosis and so on. Severe cases of this disease can cause death, but it is generally stable and can survive for a long time.

matters needing attention

The disease gene of Gitlin syndrome is located on the X chromosome, which is a sex linked recessive severe combined immunodeficiency disease. The pathogenesis is not clear. It is an infectious disease caused by human immunodeficiency virus invading into the lymphatic system of children. Its pathogenesis is the selective depletion of CD4 T lymphocytes, leading to the deficiency of immune function. After about 5 years of incubation period, most of the intrauterine HIV infected infants were born without clinical symptoms and had normal physical examination. 15% ～ 25% of perinatal HIV infected infants get sick in a few months after birth, and then increase by about 10% every year. The patients died within 1-5 years after onset, and a few of them survived for 9 years or longer.