うちの子よく熱を出すんです: 乳児一過性低ガンマグロブリン血症の話



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乳児一過性低ガンマグロブリン血症と比較検討して 除外すべき主な疾患は、IgGサブクラス欠損症、X連鎖性無ガンマグロブリン血症、常染色体劣性無ガンマグロブリン血症、複合免疫不全症、分類不能型免疫不全症(CVID)などがあります。それぞれ早期診断と専門的な加療を必要とする疾患です。

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The following is an English translation.

“My child has frequent fevers. He is often treated with antibiotics. Isn’t there something wrong with his immune system?
I often receive this kind of consultation at outpatient clinics.
Normally, when babies are in their mother’s body, they receive antibodies from their mother through the placenta (called “maternal antibody”). Antibodies are proteins produced by B cells (or plasma cells), and theit main function is to attack viruses and bacteria.

In the first several months after birth, the body is protected from many infections by the maternal antibodies, and then the antibodies gradually disappear, but the baby continues to grow and develop its own antibodies. With the help of various vaccines, babies by themselves can acquire antibodies against highly pathogenic viruses and bacteria more efficiently.

If a child is born with an abnormality in a gene that is important for immunity, this process of antibody acquisition may not function properly.

In this article, I would like to talk about “transient hypogammaglobulinemia of infancy (THI)” a condition in which the production of antibodies is transiently delayed only during infancy, despite the absence of such specific abnormalities.

Transient hypogammaglobulinemia of infancy is a condition characterized by low levels of antibodies that can only be diagnosed in retrospect after the laboratory findings and symptoms have disappeared. In other words, as a rule, it is not possible to confirm the diagnosis in a child with low antibody levels in front of us. However, a physician specializing in this field can make an educated guess based on the combination of examination findings and medical history.

In transient hypogammaglobulinemia of infancy, persistent low level of IgG antibodies beyond the age of six months is observed. It is also important to note that IgG level rises within normal range by the age of four. Since the normal levels of antibodies in children are lower than in adults, it is necessary to evaluate the amount of antibodies in a way that is appropriate for each age group.

The first infection that tends to occur frequently is acute upper respiratory infection, or what is commonly referred to as “the flu”. On the other hand, urinary tract infections (infections of the pee path), gastrointestinal infections, and severe infections such as meningitis and bacteremia are rare. About one in three children with infantile transient hypogammaglobulinemia have allergic diseases such as food allergy, bronchial asthma, and allergic rhinitis. Bronchitis and otitis media may also occur frequently.

As for treatment, there is no essential treatment for transient hypogammaglobulinemia of infancy. In cases of repeated bacterial infections such as otitis media, prophylactic antibiotics may be administered. The first is a small dose of a drug called Vacta. If this does not stop the repeated infections, immunoglobulin may be given as a supplement, but this should be done with caution because it may delay the production of antibodies by the body.

The main diseases that should be excluded in comparison with infantile transient hypogammaglobulinemia are IgG subclass deficiency, X-linked agammaglobulinemia, autosomal recessive agammaglobulinemia, combined immunodeficiency, and unclassifiable immunodeficiency (CVID). Each of these diseases requires early diagnosis and specific treatment.


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