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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Table 5.

X-Linked Agammaglobulinemia: Treatment of Manifestations

Manifestation/ConcernTreatmentConsiderations/Other
Acute infections Antibiotic treatment that is at least twice as long as that used in otherwise healthy personsGenerous use of antibiotics is recommended & treatment should be given w/o unnecessary delay.
Risk of bacterial infections Prophylactic antibiotics are used in some centers for prevention of bacterial infections.Amoxicillin combined w/clavulanic acid (an inhibitor of beta-lactamase enzyme) can be used, or alternatively sulfamethoxazole & trimethoprim. Ciprofloxacin may be used as a third option.
Vaccines, apart from live vaccines, are recommended. 1Protective antibodies will not be generated but cellular immune responses likely will.
Risk assoc w/live vaccines Children w/XLA should only be given inactivated polio vaccine (IPV).The sibs of children w/XLA should also be given IPV rather than oral polio vaccine (OPV) to avoid infecting their affected sib w/live polio virus.
1.

For example, an individual w/XLA contracted tick-borne encephalitis and developed severe disease, which might have been avoided by previous vaccination [Hedin et al 2024].

From: X-Linked Agammaglobulinemia

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