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Primary Immunodeficiency

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16 Treatment Table 5. Regimens for Prophylaxis of Bacterial Respiratory Tract Infections Antibiotic Regimen for children Regimen for adults Oral agents a Amoxicillin (consider with clavulanate, if necessary) 10-20 mg/kg daily or twice daily 500-1,000 mg daily or twice daily Trimethoprim (TMP)/ sulfamethoxazole dosing for TMP) 5 mg/kg daily or twice daily 160 mg daily or twice daily Azithromycin 10 mg/kg weekly or 5 mg/kg every other day 500 mg weekly or 250 mg every other day Clarithromycin 7.5 mg/kg daily or twice daily 500 mg daily or twice daily Doxycycline Age >8 y: 25-50 mg daily or twice daily 100 mg daily or twice daily Inhaled agents Gentamicin Age >6 y: 80 mg twice daily, 28 days on, 28 days off, OR 21 days on, 7 days off Tobramycin Age >6 y: 300 mg twice daily, 28 days on, 28 days off a ese are commonly used regimens. If these agents are not effective or are not tolerated, other drugs can be considered, including cefuroxime, cefprozil, cefpodoxime, ciprofloxacin or other quinolone, or others, depending on the individual circumstances of the patient. Autoinflammatory disorders No No No • Cytokine (IL-1, TNF, IL-6) inhibitors: CAPS, DIRA, PAPA, PSMB8, TRAPS • Steroids: Blau syndrome, DITRA, HIDS, TRAPS • Retinoids: DITRA • Colchicine: TRAPS Complement deficiency No No No • Antibiotics: all • Pneumococcal vaccine: C1, C2, C3, C4 • Meningococcal vaccine: C5-C9 • Immunomodulators: C1, C2, C4, factors H and I Cytokine autoantibody- mediated disorders Possible No No • Plasmapheresis • Rituximab • Cytokine supplement a Yes or No indicates whether or not IgG replacement is a component of standard therapy for this disorder. Table 4. Summary of Therapeutic Considerations for Primary Immunodeficiencies and Their Complications (cont'd) Diagnosis IgG a HSCT Gene therapy

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