Nezelot syndrome
Introduction
Introduction to Nezelot Syndrome Nozelof's syndrome (Nozelof's syndrome), also known as thymic hypoplasia syndrome, was first reported by the French pathologist Nozelof (1964), T-lymphocyte immunodeficiency with thymic hypoplasia, immunoglobulin values can be normal or elevated, Some are accompanied by one or more immunoglobulin selective defects, and even if the immunoglobulin concentration is normal, specific antibody reactions are often involved. basic knowledge The proportion of illness: this disease is rare, the incidence rate is about 0.0002%-0.0004% Susceptible people: no special people. Mode of infection: non-infectious Complications: high immunoglobulin E syndrome
Cause
Causes of Nezelot syndrome
Genetic factors (95%):
This syndrome is an autosomal recessive primary immunodeficiency disease.
Pathogenesis
Pathological manifestations in thymic hypoplasia, lymphoid and spleen-dependent areas lack lymphoid tissue.
Prevention
Nezelot syndrome prevention
Symptomatic treatment supplements with immunoglobulins, transfer factors, thymus transplantation can be temporarily effective, such as bone marrow transplantation may have a lasting effect.
Complication
Nezelot syndrome complications Complications high immunoglobulin E syndrome
Immunoglobulin values may be normal or elevated, and some may be accompanied by one or more immunoglobulin selective defects.
Symptom
Nezelot syndrome symptoms common symptoms repeated infection fever dyspepsia congenital bone dysplasia
Repeated infectious diseases such as pneumonia, candida albicans, viruses, Pseudomonas aeruginosa, Pneumocystis carinii, non-tuberculous mycobacteria infection, oral thrush, fever, indigestion, etc. The child is stunted, and there may be tonsil dysplasia and no abnormal thyroid symptoms.
Examine
Examination of Nezelot syndrome
It can be seen that the lymphocytes are significantly reduced, the total amount of serum IgG, IgA, IgM can be normal, but the individual components can be increased or decreased, and the formation of antibodies is defective, and the skin allergic test for various antigens does not respond, phytohemagglutinin, allogeneic Cellular antigens and the like are not easy to promote lymphocyte transformation in patients. Lymph node biopsy shows lymphoid tissue in the thymus-dependent region, and plasma cells are seen in the bone marrow.
Chest X-rays can show that the thymus becomes smaller or invisible.
Diagnosis
Diagnosis and diagnosis of Nezelot syndrome
According to medical history, clinical manifestations, laboratory tests, chest X-ray examination, can be diagnosed.
Should be differentiated from immunodeficiency diseases.
