Chuang Gung Medical Foundation, Division of Pediatric Allergy, Asthma and Rheumatology - UNREGISTERED VERSION

Management principle:
(By functional defects)

B-cell defects:
1. If patients with low IgG or IgG2 deficiency have recurrent sinopulmonary infections receive regular IVIG infusion (0.5g/kg/dose per month).
2. If patients with bronchiectasis may take prophylactic antibiotics (Amoxil, Augmentin, Cefuroxine or Aziothromycin) and elevate IVIG dose to 0.8g /kg/dose to prevent exacerbation of bronchiectasis.

T-cell defects (usually combine B-cell defects):
1. Prophylactics for opportunity infections such as pneumocystic jiroveci (carinii) infection (bactar)
2. If patients with hypogammaglobulinemia or recurrent sinopulmonary infections should receive treatment as those patients with B-cell defects

PMN defects
For example, prophylactics of CGD patients include catalase-positive Staphy. aureus skin infections (suggestively bactar) and pulmonary or deep aspergillous infections (suggestively itraconazole)。IFN-γ is suggestive for severe infections, especially aspergillous infection. -

Based on individual immune cell defects, patients with severe profound T and PMN defects should consider “curable” treatment by hematopoietic stem cell transplantation in addition to prophylactics for opportunity virus, fungus and bacterial infections. Alive vaccination including BCG, polio or others vaccine should be prohibited. Special immunologist will first evaluate their immunity to design proper vaccination schedule if necessary.