is the fi rst patient in Australasia to be treated with Leniolisib, a small molecule inhibitor of the p110 δ catalytic subunit of PI3K. Method : Clinical manifestations and blood samples were collected prospectively at multiple time points before and following Leniolisib with a follow up duration of 8 months post-Leniolisib. Multiparameter fl ow cyto-metry was used to track B and T cell phenotypes in response to Leniolisib treatment. Results : Our patient demonstrated signi fi cant clinical improvement with reduced infections, improvement of lung function and control of her lymphoproliferation. Laboratory data supported correction of B cell dysfunction with improvement of her dysregulated B cell subsets and dysgammaglobulinaema, and of T cell activation exhaustion. Conclusion : This case demonstrates the ef fi cacy of precision medicine in controlling the clinical features of APDS1 including its effects on lung function and demonstrates the immune mechanisms that may underly the response to therapy.

nant tick species on the eastern coast of Australia, is responsible for an increasing number of tick anaphylaxis presentations. While it is assumed that they thrive in warm and humid conditions, it is unclear whether environmental conditions increase tick anaphylaxis cases. If tick anaphylaxis presentations correlate with environmental variables, residents of tickendemic regions might be forewarned.

Method: A systematic literature review was conducted in May 2022. Studies were included if they were double-blind placebo-controlled randomised controlled trials (DBPCRCT), included ≥1 commercially available AIT treatment, were >12 weeks duration and reported clinical outcomes. Only studies of the current manufacturer-recommended maintenance dose (MRMD) of the AIT product were included. Narrative synthesis was performed; meta-analysis was not possible.

Introduction: Wiskott-Aldrich Syndrome (WAS) results from mutations in the WAS gene on the short arm of chromosome X, encoding the WAS protein (WASp). WAS is typically described as a triad of microthrombocytopenia, severe recurrent infections and eczema, however not all patients fit this classical phenotype. With greater access to genetic testing, there is increasing evidence for a spectrum of disease from the more classical WAS phenotype to the milder X-linked thrombocytopenias (XLT). Acknowledging the accumulation of clinical features over time, WAS protein expression and genotype can help predict the likely phenotype and thus guide the decision to proceed with haemopoietic stem cell transplant.

Until recently, successful management of atopic dermatitis has proved to be a significant challenge for clinicians, with available treatments often expensive, time consuming and in many cases unable to completely control a patient’s symptoms. The past few years have seen extensive research and innovation in this space, with several novel treatments in clinical trials, and some which have gained TGA or PBS approval for use in the management of severe atopic dermatitis.

Until recently, successful management of atopic dermatitis has proved to be a significant challenge for clinicians, with available treatments often expensive, time consuming and in many cases unable to completely control a patient’s symptoms. The past few years have seen extensive research and innovation in this space, with several novel treatments in clinical trials, and some which have gained TGA or PBS approval for use in the management of severe atopic dermatitis.

Until recently, successful management of atopic dermatitis has proved to be a significant challenge for clinicians, with available treatments often expensive, time consuming and in many cases unable to completely control a patient’s symptoms. The past few years have seen extensive research and innovation in this space, with several novel treatments in clinical trials, and some which have gained TGA or PBS approval for use in the management of severe atopic dermatitis.

Paediatric food allergy practice requires access to an observed food challenge (OFC), the gold standard investigative tool. This process requires supervision by trained health professionals experienced in management of acute allergic reactions, including anaphylaxis. In New Zealand, OFCs are done in both a tertiary care paediatric center (Starship Children’s Hospital – SCH) as well as numerous regional paediatric sites. We present the prospective data obtained from food challenges undertaken in an 18-month period from September 2015 to March 2017. Food challenges were conducted at 6 regional paediatric centers across the North and South Island and at SCH, Auckland. Decision for OFC was made by the treating physician and based on history and testing for food specific IgE (skin prick and/or serum specific IgE). Open label food challenge protocols were standardised to the ASCIA guidelines where possible and published ‘stop’ criteria were employed. Data was prospectively obtained, deidentified, compiled and analysed at the end of the study period. During the 18-month study period 1104 children underwent OFC. Forty three percent of the challenges (473) were conducted at SCH. There was no significant difference between the rates of OFC clinical reaction at SCH (113/473 – 24%) compared to the regional sites, independently or combined, with an average reaction rate of 24% (range 13–31%). Anaphylaxis was seen at all but one site. This occurred in 14% of those that reacted at SCH (16/113) and at an average of 16% (24/154) in regional children (range 0–23%). Paediatricians and experienced allied health professionals are able to successfully and safely undertake OFC for children in regional areas with outcomes that replicate those in a tertiary centre. This reduces travel costs, public expenditure and tertiary waitlists while maintaining optimum allergy care provision.

SummaryBackgroundRetrospective studies of childhood peanut allergy demonstrate serum‐specific IgE (IgE) levels against the peanut allergen Ara h2 may help predict a clinical reaction at food challenge. Fraction of exhaled nitric oxide (FeNO) is a non‐invasive tool correlating to allergic airways inflammation and has been independently associated with increased food‐specific IgE.ObjectiveTo assess the validity of serum‐specific Ara h2 IgE measured prospectively to diagnose peanut allergy and explore the utility of FeNO as a non‐invasive screening tool for childhood food challenge.MethodsWe recruited 53 participants from a cohort of consecutive children scheduled for an open‐labelled peanut food challenge (OFC) by their paediatric allergist. Participants underwent skin prick test (SPT) measurement for sensitization to whole peanut extract, and serum was collected for Ara h2‐specific IgE. FeNO was also measured in all cooperative children before the challenge. OFC and assessment of reaction were undertaken by clinicians blinded to test results.ResultsAra h2‐specific IgE and FeNO each showed improved diagnostic accuracy when compared to SPT. Receiver operator characteristic curve analysis gave an area under the curve (AUC) for Ara h2 sIgE of 0.84 (95% CI, 0.72–0.96). The AUC for FeNO, 0.83 (95% CI, 0.71–0.95), was equivalent to that of Ara h2. Combined AUC for SPT, sIgE to Ara h2 and FeNO was 0.96 (95% CI 0.90–1.00). There was no correlation between FeNO and serum nitrite levels (rs = −0.13, P = 0.6, n = 18).Conclusion and clinical relevanceProspectively measured Ara h2‐specific IgE improves diagnostic accuracy and reduces unsuccessful challenge to peanut. FeNO levels may provide improved diagnostic accuracy in a paediatric population undergoing OFC. The proposed FeNO‐based diagnostic algorithm requires further validation studies.