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Inborn Errors of Immunity

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8 Diagnosis Table 2B. Recommendations for the Care of Infants With SCID Diagnosis a Recommendation Frequency Comment Test cytomegalovirus (CMV) polymerase chain reaction (PCR) in urine and in blood Baseline and every 3 to 4 mo If present, treat CMV infection (valganciclovir, 16 mg/kg/dose, orally twice a day or ganciclovir, 6 mg/ kg/dose, intravenously twice a day). Alternatives are cidofovir and foscarnet. Test serum anti-CMV IgG in patient's mother Baseline If suspected or confirmed maternal CMV infection, avoid breastfeeding. Test EBV PCR in blood Baseline and every 3 to 4 mo If present, treat EBV infection (valganciclovir or ganciclovir, dose similar to CMV). Test HIV PCR or antigen tests in blood Baseline Treat HIV infection Test PCR-based respiratory viral pathogen panel (e.g., influenza A and B, parainfluenza 1, 2, and 3, adenovirus, rhinovirus, human metapneumovirus). When there is an increase of nasal secretions or respiratory symptoms such as cough or shortness of breath Treat respiratory viral infection: oxygen support, beta-agonists, antiviral drugs (e.g., oseltamivir) if indicated Environment with reduced exposure to infections At all times Inpatient (with reverse isolation) or outpatient (close distance to a medical center) Prophylaxis for Pneumocystis jirovecii pneumonia (PJP) Starting at 1 mo of age Trimethoprim/sulfamethoxazole (TMP/SMX) 5 mg (of trimethoprim component)/ kg/d oral 2 or 3 times a week Prophylaxis for candidiasis Daily Fluconazole 3 mg/kg/d oral Prophylaxis for herpes infection Daily Acyclovir 12.5 mg/kg/d oral Prophylaxis for respiratory syncytial virus (RSV) infection During RSV season Palivizumab or nirsevimab-alip Immunoglobulin replacement therapy Monthly or weekly Intravenous or subcutaneous routes (400 mg/kg/mo) Avoid live viral vaccines Includes: rotavirus, measles, mumps, rubella (MMR), bacillus Calmette- Guerin (BCG), oral polio. Household members are encouraged to receive all scheduled vaccines. a From Dorsey et al. Not an exhaustive list, alternative recommendations exist.

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