Punita Ponda

X-linked agammaglobulinemia presenting as polymicrobial pneumonia, including Pneumocystis jirovecii

Pneumocystis jirovecii pneumonia is an opportunistic pulmonary infection usually associated with T-cell defects. Clinical and murine studies demonstrate that B-cells and Pneumocystis-specific antibodies also contribute to effective immune responses.1 Patients with primary humoral immunodeficiencies, including X-linked agammaglobulinemia (XLA), may develop P . jirovecii pneumonia despite normal T-cell number and function.2,3 XLA arises from Bruton tyrosine kinase (BTK) gene mutations, leading to pre-B-cell differentiation arrest, absence of immunoglobulin expression, and increased susceptibility to bacterial/enteroviral infections.4 Although more than 600 BTK mutations have been described, new variations/mutations continue to be identified. We describe an infant with a novel BTK kinase domain variation who presented with polymicrobial pneumonia, including P. jirovecii.

Comparison of Pediatric and Adult Systemic Reactions to Subcutaneous Immunotherapy

BACKGROUND Subcutaneous immunotherapy (SCIT) has been used to treat allergic rhinitis for over a century, and current regimens have wide variability with an array of practice styles and dosing strategies. Although there are some statements about contraindications and cautions, no specific formal age- or weight-based dosing guidelines are utilized when administering SCIT. OBJECTIVE The primary objective of this study was to estimate the overall incidence rate of any reaction to SCIT and to consider the severity of the reaction by grade in children and adults. METHODS A retrospective chart review was conducted to document the number and severity of episodes of systemic reactions (SRs) in pediatric and adult subjects. Crude incidence rates were estimated as the number of SRs relative to the total injections administered. Adjusted incidence rate ratios were generated using a generalized estimating equation approach, which accounted for multiple visits within subjects. RESULTS The incidence rate for any SR was 0.2%. The unadjusted incidence rate of any SR was significantly higher in children compared with adults (P < .001), although not significant when adjusted for asthma, gender, and phase of SCIT (P < .054). However, the incidence rate for grade 1 and 2 SRs in children was 1.89 times the incidence rate for adults, adjusting for these variables (P < .015). CONCLUSIONS These results suggest that current SCIT practices are associated with a higher rate of SRs, specifically of grade 1 and 2 SRs, in children than adults. Further studies are necessary to evaluate if changes in dosing strategies for children, such as a lower starting dose, a decrease in target maintenance dose, or a slower build-up phase, are warranted.

198 AAAAI Survey on Immunotherapy Practice Patterns Concerning Dosing, Dose-Adjustment after Missed Doses and Duration of Immunotherapy.

Background Several practical issues dealing with the exact application of allergen immunotherapy (AIT) among European and US allergists are not well known. Guidelines on AIT give recommendations and suggestions for only some of them. We present this unique survey with worldwide response. Methods The AAAAI immunotherapy committee conducted a web-based practice patterns survey (program: Survey Monkey) among all members in&outside US on dosing, dose-adjustment after missed doses and duration of AIT. Results 1201 Returned questionnaires (almost 25% response rate). 21% were non-US-Canada members. Maintenance doses in USCan are (mean/median): Dermatophagoides farinae (Df) combined with Dermatophagoides pteronyssinus (Dpt): 2155/1000AU; Df solo 2484/1000AU. Dpt when combined with Df 1937/1000AU; Dpt solo: 2183/1000AU.Cat 3224/2000BAU. Grass 11,410/4000BAU. 57-65% of the dosing falls within the recommended Practice Parameters recommended ranges. Non-USCan allergists expressed maintenance doses in many different units making analysis impossible. Dose-adjustment after missed doses is based on ‘time elapsed since the last applied dose’ by 77% of USCan and 58% of non-USCan allergists and on ‘time since missed scheduled dose’ by the rest. Doses are adjusted when a patient comes in more than 14 d/5 wk after the last administration at build-up/maintenance by both USCan and non-USCan colleagues. The mostly followed dose-adjustment schedules after 1, 2, 3 missed doses are: Build-up: repeat last dose, reduce by one dose, reduce by 2 doses; maintenance: reduce by one dose, reduce by 2 doses, reduce by 3 doses. 26% uses a different approach reducing doses by a certain percentage or volume. AIT is restarted after a gap in build-up of >30 days and of >12 weeks during maintenance in both groups (median). Outside USCan AIT is prescribed for 3 years (Median). However, 75% of USCan allergists prescribes AIT for 5 years. Main reasons why to continue AIT beyond 5 years: ‘symptoms came back after stopping’ or “patient afraid to relapse.” Conclusions These results show regional differences on some points (especially AIT duration) and they suggest in which direction to plan further research in 2 areas to establish universal dose-adjustment plans for missed applications and define the usefulness (or lack of) of long-term AIT. Moreover, there is still room for improvement in the way AIT is dosed.