What Is the Treatment for IGG Deficiency?

Antibody-mediated immunodeficiency disease is the most common primary immunodeficiency disease, accounting for more than 50%. The types are as follows: 1. Total gamma globulin hypoemia, which refers to a primary antibody deficiency disease whose serum gamma globulin is greater than 3-4 g / L.

Antibody deficiency

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Antibody-mediated immunodeficiency disease is the most common primary immunodeficiency disease, accounting for more than 50%. The types are as follows: 1. Total gamma globulin hypoemia, which refers to a primary antibody deficiency disease whose serum gamma globulin is greater than 3-4 g / L.

Chinese name

Antibody deficiency

Nature

Primary immunodeficiency disease

Definition

Hypoglycemia

First case time

1952

X-linked agammaglobulinemia

It is the earliest known immunodeficiency disease. The first case was called Bruton's disease in 1952. Onset after more than 4 to 8 months of age, manifested as repeated infection of extracellular bacteria such as Streptococcus pneumoniae, hemolytic streptococcus and Haemophilus. The most common are sinusitis, pneumonia, otitis media, rickets, meningitis, and sepsis. Without neutropenia, chronic mold infections rarely occur. Pneumocystis carinii pneumonia is rare. The infections caused by hepatitis virus and enterovirus are more serious and the course of the disease is prolonged; while the responses to other virus infections are normal. About one-third of patients develop juvenile idiopathic arthritis.

Immunodeficiency disease (CVID)

Patients' family members have abnormal serum Ig concentrations and are prone to autoantibodies and autoimmune diseases or tumors, suggesting that the etiology is related to heredity. The clinical manifestations and susceptible bacterial species of this disease are similar to X-linked agammaglobulinemia, except that the age of onset of CVID is uncertain, more common in young adults, both men and women, and the susceptibility to infection is lower. There may be recurrent purulent respiratory infections. Some patients may have unusual enterovirus infections, chronic meningoencephalitis, and dermatomyositis-like syndromes. CVID patients are susceptible to gastrointestinal disorders and often secondary to Giardia infection; and

Infant transient hypogammaglobulinemia

It is a self-limiting disease that can occur in both men and women. Occasionally family history, the baby's own immune globulin production function is often delayed until 9-18 months after birth, and its content is only 2-4 years old. Reached normal levels. Patients are susceptible to bacterial skin diseases, infections of the lungs, meninges, and upper respiratory tract. The total amount of serum IgG, IgA, IgM is usually less than 4g / L, IgG is less than 2.5g / L, but can still synthesize low levels of blood group antibodies (anti-A, anti-B) and anti-toxin of diphtheria or tetanus; inherent in the rectal mucosa Layer biopsy can find plasma cells. These are the points of identification with Bruton's disease. 2. Selective Ig deficiency.

Selective IgA deficiency: Only lacks IgA (<0.05g / L) and secreted IgA, which are common primary immunodeficiency diseases. It can occur in both men and women, and multiple people in the family can develop it. Patients with this disease can be asymptomatic or have recurrent respiratory infections in infancy and childhood, and gastrointestinal and urinary tract infections can also occur, the cause of which is the same as other antibody deficiency diseases; often accompanied by autoimmune diseases such as systemic Lupus erythematosus, rheumatoid arthritis) and allergic diseases (such as asthma, allergic rhinitis). Except for severe infections, most of them can survive to middle age or old age, and individual cases of IgA content can spontaneously return to normal. In recent years, selective IgA deficiency with repeated infections has often been combined with IgG2 and IgG4 defects. Patients' serum IgG and IgM levels are normal or elevated, and local IgM is often compensatory.

Selective IgM deficiency: The serum IgM concentration is less than 100 mg / L. Patients often have severe bacterial infections, especially septicemia and meningitis caused by Gram-negative bacteria, which cause the patient to die in childhood.

Selective IgG subclass defects: The total serum IgG is generally normal (or nearly normal) and the level of one or more IgG subclasses is lower than normal. It does not produce antibodies to certain antigens. Easily infected. It is difficult to distinguish the physiological IgG subclass before the age of 2 years, so it is not appropriate to diagnose the disease prematurely.