flashcards_hereditary_immunodeficiency
What is the first step in managing hereditary immunodeficiency?
Specialist referral.
What antimicrobial prophylaxis is recommended for T-cell and neutrophil defects?
Co-trimoxazole to prevent PCP, itraconazole or fluconazole to prevent other fungal infections.
What antimicrobial prophylaxis is recommended for B-cell defects?
Antibiotic prophylaxis (e.g. azithromycin) to prevent recurrent bacterial infections.
What is the recommended approach for antibiotic treatment in hereditary immunodeficiency?
Prompt treatment of infections, longer courses, low threshold for IV therapy.
What should be screened for in children with antibody deficiency?
End-organ disease, e.g. CT scan to detect bronchiectasis.
What therapy is recommended for children with antibody deficiency?
Immunoglobulin replacement therapy.
What treatment is considered for SCID and chronic granulomatous disease?
Bone marrow transplantation.
What advanced therapy can be considered for hereditary immunodeficiency?
Gene therapy.
When should isolation be considered for patients with hereditary immunodeficiencies?
For severe immunodeficiencies, especially those awaiting haematopoietic stem cell transplant.
What additional support should be provided to caregivers of patients with hereditary immunodeficiencies?
Caregiver counselling and hygiene advice.
What preventive measure should be taken for patients and their close contacts?
Vaccination of patients, family members, and caregivers.
What counselling should be offered to families considering further children?
Genetic counselling.
