Hyper-igm syndrome

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Hyper-IgM syndrome is characterized by normal or high levels of immunoglobulin M (IgM) and decreased levels or absence of other immunoglobulins. As a result, people are more susceptible to bacterial infections.

Children with hyper-IgM syndrome have frequent sinus and lung infections.
Doctors diagnose the disorder by measuring levels of immunoglobulins in the blood and by doing genetic tests.
Treatment involves immune globulin , antibiotics to prevent infections, and, when possible, stem cell transplantation.

(See also Overview of Immunodeficiency Disorders.)
Hyper-IgM syndrome is a primary immunodeficiency disorder. It may be inherited in one of the following ways:

As an X-linked disorder: That is, it is due to a mutation in a gene on the X (sex) chromosome. X-linked disorders usually affect only boys.
As an autosomal recessive disorder: That is, two genes for the disorder, one from each parent, are required.

Most cases of hyper-IgM syndrome are X-linked.
How the disorder affects people varies depending on which gene is affected.
Levels of IgM may be high or normal. Other immunoglobulins may be absent, or levels may be low. The lack of other immunoglobulins makes people with hyper-IgM syndrome less able to fight off infections.