Abstract
Patients with primary immunodeficiency diseases (PID) are prone to infectious, and they often determine the course of the disease. Antimicrobial prophylaxis for patients with PID includes co-trimoxazole, amoxicillin and macrolides. Co-trimoxazole is a widely used antimicrobial agent for pneumocystosis prophylaxis in immunocompromised patients, including patients with primary cellular immunodeficiency. Antiinflammatory effect of azithromycin made it a drug of choice in the management of chronic lung diseases in the patients with primary immunodeficiencies, especially in those with antibody deficiencies. Further randomised controlled studies are needed to define the optimal agent for all types of primary immunodeficiencies, its dose, duration of treatment, benefits and safety of long-term use.A recommendation for the prophylactic use of long-term therapy should consider the balance between benefits and risk of adverse events.
 Bangladesh Journal of Medical Science Vol.20(3) 2021 p.530-538
Highlights
Infectious manifestations are one of the main features of primary immunodeficiency disorders (PID).[1]
The French National Reference Center for PIDs recommends co-trimoxazolefor anti-infectious prophylaxis for all patients with chronic granulomatous disease (CGD), at a daily dose of 25 mg/kg/day of sulfamethoxazole and 5 mg/kg/day of trimethoprim; the maximum dose of 800 mg/day of sulfamethoxazole.[3]
In the absence of bronchiectasis co-trimoxazole prophylaxis is recommended for children with common variable immunodeficiency (CVID) and hyper IgM-syndrome.[2, 3]
Summary
Infectious manifestations are one of the main features of primary immunodeficiency disorders (PID).[1] Infections often determine the outcome of PID. Their prevention may resultsin the increased overall survival, improved quality of life, and reduced longterm healthcare costs. Long-term prophylaxis to prevent episodes of certain opportunistic infections, in particular Pneumocystis pneumonia, have become a standard of care in the patients with human immunodeficiency virus infection.[6]. Antimicrobial agents for long-term prophylaxis in patients with PIDs include trimethoprimsulphamethoxazole (TMP-SMX), amoxicillin or macrolides (azithromycin)].1, 2, 8. Prophylactic treatment with these medications has been proposed. Enterobacteriaceae: Escherichia coli, Salmonella spp., Klebsiella spp, Shigella spp, Brucella; Haemophilus influenzae, Haemophilus ducreyi; Burkholderia cepacia, Stenotrophomonas maltophilia
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