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What is the prevalence of Agammaglobulinemia?

How many people does Agammaglobulinemia affect? Does it have the same prevalence in men and women? And in the different countries?

Prevalence of Agammaglobulinemia

Agammaglobulinemia is a rare genetic disorder characterized by the absence or extremely low levels of immunoglobulins, leading to a compromised immune system. It primarily affects males, with an estimated prevalence of 1 in 190,000 to 1 in 380,000 individuals. The condition is typically diagnosed in early childhood, presenting with recurrent bacterial infections, particularly in the respiratory tract. Agammaglobulinemia can significantly impact the quality of life and requires lifelong management through regular intravenous immunoglobulin replacement therapy. Early diagnosis and appropriate medical intervention are crucial for individuals with this condition to prevent severe infections and associated complications.



Agammaglobulinemia is a rare genetic disorder characterized by the absence or extremely low levels of immunoglobulins, which are essential antibodies for fighting infections. This condition primarily affects the immune system, leaving individuals highly susceptible to recurrent bacterial infections.



The prevalence of Agammaglobulinemia is estimated to be around 1 in 100,000 to 1 in 250,000 individuals worldwide. It is more commonly observed in males than females, with a male-to-female ratio of approximately 3:1. Agammaglobulinemia can manifest in early childhood, typically becoming evident within the first year of life.



Although Agammaglobulinemia is considered a rare disorder, it is important to note that there are different types and subtypes of the condition, each with varying prevalence rates. For instance, X-linked Agammaglobulinemia (XLA), the most common form, accounts for approximately 85% of all cases. Other forms, such as autosomal recessive Agammaglobulinemia, are less prevalent.



Early diagnosis and appropriate management, such as regular administration of immunoglobulin replacement therapy and prophylactic antibiotics, can significantly improve the quality of life for individuals with Agammaglobulinemia.


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