congenital pangammaglobulinemia
Introduction
Introduction to congenital all-gamma globulin hypoxia Congenital all-gamma globulin hypoplasia is inherited by sexual union, and its defect is that the differentiation of pre-B cells into B cells is impaired. The disease is only found in men, also known as Bruton. After 4 to 6 months of birth, the protective effect of IgG from the mother disappeared, and severe suppurative bacterial infections began to occur repeatedly, especially in the upper respiratory tract and lower respiratory tract. Commonly, there were suppurative bronchitis, pneumonia, otitis media, etc. Meningitis, osteomyelitis and septic arthritis can occur, but there is no special susceptibility to toxins and fungi, lymph node dysplasia in children, small or absent tonsils, although repeated infections, lymph nodes and spleen are not swollen. basic knowledge The proportion of illness: 0.001% Susceptible people: only seen in men Mode of infection: non-infectious Complications: urticaria asthma shock
Cause
Congenital all-gamma globulin hypolipidemia
Congenital all-gamma globulin hypoplasia is inherited by sexual union. The defect is that the differentiation of pre-B cells into B cells is impaired, leading to the formation of antibodies, and the number of antibodies is small or absent.
Prevention
Congenital all-gamma globulin hypoxia prevention
Congenital all-gamma globulin hypotension is a congenital recessive sex chromosome disease. Only boys are sick, consult experts before pregnancy, and purposefully screen offspring sex to avoid male birth, can achieve preventive effects.
Complication
Congenital gamma globulin hypoxia complications Complications urticaria asthma shock
Repeated injection of human serum immunoglobulin for many years can cause scar formation at the local injection site, occasional fever, rash, urticaria,
Symptom
Congenital symptoms of hypogamma globulin hypothyroidism Common symptoms Bacterial infection of tonsils small or lack of meningitis
After 4 to 6 months of birth, the protective effect of IgG from the mother disappeared, and severe suppurative bacterial infections began to occur repeatedly, especially in the upper respiratory tract and lower respiratory tract. Commonly, there were suppurative bronchitis, pneumonia, otitis media, etc. Meningitis, osteomyelitis and septic arthritis can occur, but there is no special susceptibility to toxins and fungi, lymph node dysplasia in children, small or absent tonsils, although repeated infections, lymph nodes and spleen are not swollen.
Examine
Examination of congenital hypogamma globulin hypotension
1. The total amount of serum immunoglobulin is less than 250 mg/dl, IgG is less than 200 mg/dl, and IgA, IgM, IgD and IgE are difficult to detect.
2. B cells with surface Ig or Ia-like antigens in the circulation are significantly reduced, which is different from common variant immunodeficiency diseases.
3. Lack of the same hemagglutinin or low titer.
4. The antibody response to vaccination is extremely low or absent.
5. The number of lymphocytes in the peripheral blood is normal, in which the percentage of T cells rises, while the B cells are rarely (<0.5%) or absent, and the T/B cell ratio increases.
6. The cellular immune function is normal.
Lymph node and tonsil biopsy lacks germinal centers and plasma cells (and normal amounts of pre-B cells in the bone marrow).
Diagnosis
Diagnosis and diagnosis of congenital all-gamma globulin hypotension
Temporary hypogammaglobulinemia in infancy: 1 serum IgG is very low, while IgA and IgM are normal, 2 peripheral blood B cell count is normal, 3 lymph node biopsy lacks mature plasma cells, but plasmacytoid lymphocytes, 4 The ability to synthesize immunoglobulins is generally restored in less than 18 months.
Normal 5-9 months old infants: 1 serum IgG is low, but higher than 350mg / dl, 2 serum can be detected IgM and IgA.
