A European-Japanese study on peach allergy: IgE to Pru p 7 associates with severity.

BACKGROUND: Pru p 3 and Pru p 7 have been implicated as risk factors for severe peach allergy. This study aimed to establish sensitization patterns to five peach components across Europe and in Japan, to explore their relation to pollen and foods and to predict symptom severity. METHODS: In twelve European (EuroPrevall project) and one Japanese outpatient clinic, a standardized clinical evaluation was conducted in 1231 patients who reported symptoms to peach and/or were sensitized to peach. Specific IgE against Pru p 1, 2, 3, 4 and 7 and against Cup s 7 was measured in 474 of them. Univariable and multivariable Lasso regression was applied to identify combinations of parameters predicting severity. RESULTS: Sensitization to Pru p 3 dominated in Southern Europe but was also quite common in Northern and Central Europe. Sensitization to Pru p 7 was low and variable in the European centers but very dominant in Japan. Severity could be predicted by a model combining age of onset of peach allergy, probable mugwort, Parietaria pollen and latex allergy, and sensitization to Japanese cedar pollen, Pru p 4 and Pru p 7 which resulted in an AUC of 0.73 (95% CI 0.73-0.74). Pru p 3 tended to be a risk factor in South Europe only. CONCLUSIONS: Pru p 7 was confirmed as a significant risk factor for severe peach allergy in Europe and Japan. Combining outcomes from clinical and demographic background with serology resulted in a model that could better predict severity than CRD alone.

Showcasing MESMER-X: Spatially Resolved Emulation of Annual Maximum Temperatures of Earth System Models.

Emulators of Earth System Models (ESMs) are complementary to ESMs by providing climate information at lower computational costs. Thus far, the emulation of spatially resolved climate extremes has only received limited attention, even though extreme events are one of the most impactful aspects of climate change. Here, we propose a method for the emulation of local annual maximum temperatures, with a focus on reproducing essential statistical properties such as correlations in space and time. We test different emulator configurations and find that driving the emulations with global mean surface temperature offers an optimal compromise between model complexity and performance. We show that the emulations can mimic the temporal evolution and spatial patterns of the underlying climate model simulations and are able to reproduce their natural variability. The general design and the good performance for annual maximum temperatures suggest that the proposed methodology can be applied to other climate extremes.

Physical and psychosocial quality of life in children with overweight and obesity from Sri Lanka.

BACKGROUND: While childhood obesity is rising rapidly in South Asia, there is limited research on quality of life (QoL) of children with overweight and obesity from the region. This study assessed physical and psychosocial QoL in Sri Lankan children attending a specialized obesity clinic, from both children's and parents' perspective, and modifiable social factors affecting QoL. METHODS: We performed cross-cultural translation of the Pediatric Quality of Life Inventory (PedsQL™) 4.0 (Child-Self Report and Parent-Proxy forms), and assessed self-reported and parental-perception of physical and psychosocial QoL in 8-12 year-olds with overweight and obesity (n=110), referred for obesity management at a tertiary-care children's hospital in Sri-Lanka. Body mass index (BMI) and pre-selected social factors affecting QoL were also assessed. Data were analyzed by non-parametric tests (Mann-Whitney U test, Wilcoxon test and Spearman correlation). RESULTS: The median physical QoL was lower than psychosocial QoL (78.1vs81.7, p=0.032) and physical QoL was inversely correlated with BMI. Parental-perception of children's physical and psychosocial QoL correlated with child-reported QoL, but was lower. Being bullied (p=0.001) and not getting regular exercise (p=0.031) were associated with lower psychosocial QoL. Both physical and psychosocial QoL were lower in children having difficulties in finding suitable clothes (p< 0.001). CONCLUSIONS: Children with overweight and obesity from Sri Lanka appeared to have greater impairment of physical QoL than psychosocial QoL. Higher BMI, bullying, lack of regular exercise and lack of suitable clothing, negatively affected QoL. Potential strategies to improve QoL include promoting regular exercise, addressing bullying and promoting availability of children's clothes in larger sizes to fit children with overweight and obesity.

Assessment of current status and geospatial analysis of compliance to bacteriological parameters: Tools to ensure access to safe water in Sri Lanka.

Compliance of drinking-water to bacteriological parameters serves as a surrogate measure of the risk of water-borne diseases. Understanding the risk of water-borne diseases could help promote healthy behaviors such as household water treatment and safe water storage practices and advocacy to increase access to centrally-managed piped water. The objective of this research was to assess the current status of compliance and to geospatially analyze the probability of compliance to bacteriological parameters in the Western Province of Sri Lanka. A drinking-water quality survey was conducted among 4508 households representing four water-source types: National Water Supply and Drainage Board (NWSDB), dug wells, Community Water Supply (CWS), and tube wells, and other sources. Besides, a detailed assessment of selected NWSDB and CWS supplies was done. Water samples were tested for the total coliform count, thermotolerant coliform count, and free residual chlorine levels against the Sri Lanka Standards. Indicator kriging was performed using the geospatial analyst tool of ArcGIS version 10.6 for different water source types to interpolate the probability of compliance for both total coliform count and thermotolerant coliform count. The bacteriological compliance decreased from NWSDB to tube wells and other sources to CWS to dug wells. The interpolation maps confirm the relatively higher compliance of NWSDB for bacteriological parameters compared to other sources. Areas with a high probability of compliance for both parameters show considerable overlap with urban areas with a supply of centrally managed water from the NWSDB. It is recommended to expand the coverage of NWSDB water, strengthen the drinking-water quality surveillance system and water safety plans, and promote household water treatment and safe storage practices in the Western Province of Sri Lanka.

Hygiene practices during clinical training: knowledge, attitudes and practice among a cohort of South Asian Medical students.

BACKGROUND: Proper hygienic practices are important in preventing nosocomial infection. This study aimed to determine knowledge, attitudes and practices (KAP) on hand, attire and equipment hygiene during clinical training among medical students at a State Medical Institution in Sri Lanka. METHODS: This cross-sectional study was conducted among 3rd, 4th and final (5th) year medical students of the Faculty of Medicine, University of Colombo, who had undergone at least 6 months of clinical training. KAP on hand hygiene (HH), attire hygiene (AH) and equipment hygiene (EH) were assessed using a pre-tested, self-administered questionnaire with a Likert-type scale. KAP scores were graded as follows: good ≥75; moderate:74.9-50; unsatisfactory:49.9-25; poor:< 25%. KAP based on duration of training and gender were compared using independent samples t-tests. RESULTS: Three hundred thirty-three students participated (mean age 24 ± 1.1 years, male: female = 1: 1.2). Combined KAP scores on hand and attire hygiene were moderate (HH:73%, AH:65%) while equipment hygiene was unsatisfactory (EH:47%). Senior students (5th year) had higher combined KAP and knowledge (K) on hand hygiene (HH KAP 75% vs. 72%, p = 0.01; K:72% vs. 67%, p = 0.001) and equipment hygiene (EH KAP 50% vs. 44%, p = 0.001; K:47% vs. 35%, p = 0.001) compared to junior students (3rd/ 4th years). However, they had lower KAP and P scores on attire hygiene (AH KAP 63% vs. 67%, p = 0.006; P:60% vs. 67%, p = 0.004). Female students had better AH compared to male students (KAP:67% vs. 64% p = 0.01; K 71% vs. 66%, p = 0.048; P:66% vs. 62%, p = 0.05). CONCLUSIONS: Overall, hand hygiene was moderate among medical students and improved with progression of training. Attire hygiene was also graded as moderate but to a lesser extent compared to hand hygiene, lower in males, and declined over time, indicating need for better reinforcement of attire hygiene practices with progression of clinical training. Equipment hygiene was unsatisfactory among most medical students and thus needs to be highlighted as a potential area to be improved during clinical training. This study suggests that knowledge, attitudes and practices on equipment and attire hygiene among medical students was less satisfactory and needs to receive greater emphasis during medical clinical training.

Component-resolved diagnosis and beyond: Multivariable regression models to predict severity of hazelnut allergy.

BACKGROUND: Component-resolved diagnosis (CRD) has revealed significant associations between IgE against individual allergens and severity of hazelnut allergy. Less attention has been given to combining them with clinical factors in predicting severity. AIM: To analyze associations between severity and sensitization patterns, patient characteristics and clinical history, and to develop models to improve predictive accuracy. METHODS: Patients reporting hazelnut allergy (n = 423) from 12 European cities were tested for IgE against individual hazelnut allergens. Symptoms (reported and during Double-blind placebo-controlled food challenge [DBPCFC]) were categorized in mild, moderate, and severe. Multiple regression models to predict severity were generated from clinical factors and sensitization patterns (CRD- and extract-based). Odds ratios (ORs) and areas under receiver-operating characteristic (ROC) curves (AUCs) were used to evaluate their predictive value. RESULTS: Cor a 9 and 14 were positively (OR 10.5 and 10.1, respectively), and Cor a 1 negatively (OR 0.14) associated with severe symptoms during DBPCFC, with AUCs of 0.70-073. Combining Cor a 1 and 9 improved this to 0.76. A model using a combination of atopic dermatitis (risk), pollen allergy (protection), IgE against Cor a 14 (risk) and walnut (risk) increased the AUC to 0.91. At 92% sensitivity, the specificity was 76.3%, and the positive and negative predictive values 62.2% and 95.7%, respectively. For reported symptoms, associations and generated models proved to be almost identical but weaker. CONCLUSION: A model combining CRD with clinical background and extract-based serology is superior to CRD alone in assessing the risk of severe reactions to hazelnut, particular in ruling out severe reactions.

Invasive dermatophyte infection with Trichophyton interdigitale is associated with prurigo-induced pseudoperforation and a signal transducer and activator of transcription 3 mutation.

Invasive dermatophyte infection, with extension beyond the dermis, in immunocompetent hosts is exceptionally rare. Dermatophytes are keratinophilic and are usually confined to the stratum corneum, hair and nails. Susceptibility to dermatophyte infections is incompletely understood, but inherited mutations in key signalling pathways of the innate immune system have been identified. We report the first case of an invasive dermatophyte infection associated with abrupt onset of a prurigo-induced pseudoperforation and a loss-of-function mutation in signal transducer and activator of transcription 3 (STAT3).

Corticosteroids in management of anaphylaxis; a systematic review of evidence.

SUMMARY: As anaphylaxis is a medical emergency, there are no randomized controlled clinical trials on its emergency management. Therefore, current guidelines are mostly based on data from observational studies, animal and laboratory studies. Although epinephrine is the mainstay of recommended treatment, corticosteroids are also frequently used. This review evaluates the evidence on the use of corticosteroids in emergency management of anaphylaxis from published human and animal or laboratories studies. Thirty original research papers were found with 22 human studies and eight animal or laboratory studies. The average rate of corticosteroid use in emergency treatment was 67.99% (range 48% to 100%). Corticosteroids appear to reduce the length of hospital stay, but did not reduce revisits to the emergency department. There was no consensus on whether corticosteroids reduce biphasic anaphylactic reactions. None of the human studies had sufficient data to compare the response to treatment in different treatment groups (i.e. corticosteroids, epinephrine, antihistamines). Animal studies demonstrated that corticosteroids act through multiple mechanisms. These modulate gene expression, with effects becoming evident 4 to 24 hours after administration. A much quicker response has been detected within 5 to 30 minutes, through blockade of signal activation of glucocorticoid receptors independent of their genomic effects. Therefore, we conclude that there is no compelling evidence to support or oppose the use of corticosteroid in emergency treatment of anaphylaxis. However, based on the available data, it appears to be beneficial and there was no evidence of adverse outcomes related to the use of corticosteroids in emergency treatment of anaphylaxis.

Effects of antenatal exercise in overweight and obese pregnant women on maternal and perinatal outcomes: a randomised controlled trial.

OBJECTIVE: To assess whether antenatal exercise in overweight/obese women would improve maternal and perinatal outcomes. DESIGN: Two-arm parallel randomised controlled trial. SETTING: Home-based intervention in Auckland, New Zealand. POPULATION AND SAMPLE: Pregnant women with body mass index ≥25 kg/m(2) . METHODS: Participants were randomised to a 16-week moderate-intensity stationary cycling programme from 20 weeks of gestation, or to a control group with no exercise intervention. MAIN OUTCOME MEASURES: Primary outcome was offspring birthweight. Perinatal and maternal outcomes were assessed, with the latter including weight gain, aerobic fitness, quality of life, pregnancy outcomes, and postnatal body composition. Exercise compliance was recorded with heart rate monitors. RESULTS: Seventy-five participants were randomised in the study (intervention 38, control 37). Offspring birthweight (adjusted mean difference 104 g; P = 0.35) and perinatal outcomes were similar between groups. Aerobic fitness improved in the intervention group compared with controls (48.0-second improvement in test time to target heart rate; P = 0.019). There was no difference in weight gain, quality of life, pregnancy outcomes or postnatal maternal body composition between groups. However, compliance with exercise protocol was poor, with an average of 33% of exercise sessions completed. Sensitivity analyses showed that greater compliance was associated with improved fitness (increased test time (P = 0.002), greater VO2 peak (P = 0.015), and lower resting heart rate (P = 0.014)), reduced postnatal adiposity (reduced fat mass (P = 0.007) and body mass index (P = 0.035)) and better physical quality of life (P = 0.034). CONCLUSIONS: Maternal non-weight-bearing moderate-intensity exercise in pregnancy improved fitness but did not affect birthweight or clinical outcomes. TWEETABLE ABSTRACT: Moderate-intensity exercise in overweight/obese pregnant women improved fitness but had no clinical effects.

Breast cancer inequities between Maori and non-Maori women in Aotearoa/New Zealand.

Maori women have one of the highest incidences of breast cancer in the world. This high incidence is generally unexplained although higher rates of obesity and alcohol intake are modifiable risk factors that may be important. Maori women are less likely to attend mammographic breast screening and are likely to be diagnosed with more advanced disease. This is one of the reasons for the excess mortality. Another factor is differences in the treatment pathway. Maori women are more likely to experience delay in receiving treatment, are less likely to receive radiotherapy, are more likely to be treated with a mastectomy and are less likely to adhere to long-term adjuvant endocrine therapy. However, genetic factors in Maori women do not seem to impact significantly on mortality. This review looks at the inequity between Maori and non-Maori women and addresses the causes. It proposes ways of reducing inequity through primary prevention, increased participation in breast screening and greater standardisation of the treatment pathway for women newly diagnosed with breast cancer. We believe that health system improvements will decrease barriers to health care participation for Maori women and suggest that further research into identifying and modifying obstacles within health systems is required.

Spectrum and management of complement immunodeficiencies (excluding hereditary angioedema) across Europe.

INTRODUCTION: Complement immunodeficiencies (excluding hereditary angioedema and mannose binding lectin deficiency) are rare. Published literature consists largely of case reports and small series. We collated data from 18 cities across Europe to provide an overview of primarily homozygous, rather than partial genotypes and their impact and management. METHODS: Patients were recruited through the ESID registry. Clinical and laboratory information was collected onto standardized forms and analyzed using SPSS software. RESULTS: Seventy-seven patients aged 1 to 68 years were identified. 44 % presented in their first decade of life. 29 % had C2 deficiency, defects in 11 other complement factors were found. 50 (65 %) had serious invasive infections. 61 % of Neisseria meningitidis infections occurred in patients with terminal pathway defects, while 74 % of Streptococcus pneumoniae infections occurred in patients with classical pathway defects (p < 0.001). Physicians in the UK were more likely to prescribe antibiotic prophylaxis than colleagues on the Continent for patients with classical pathway defects. After diagnosis, 16 % of patients suffered serious bacterial infections. Age of the patient and use of prophylactic antibiotics were not associated with subsequent infection risk. Inflammatory/autoimmune diseases were not seen in patients with terminal pathway, but in one third of patients classical and alternative pathway defects. CONCLUSION: The clinical phenotypes of specific complement immunodeficiencies vary considerably both in terms of the predominant bacterial pathogen, and the risk and type of auto-inflammatory disease. Appreciation of these phenotypic differences should help both immunologists and other specialists in their diagnosis and management of these rare and complex patients.

C1 inhibitor deficiency: 2014 United Kingdom consensus document.

C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient patients, representing a joint venture between the United Kingdom Primary Immunodeficiency Network and Hereditary Angioedema UK. To develop the consensus, we assembled a multi-disciplinary steering group of clinicians, nurses and a patient representative. This steering group first met in 2012, developing a total of 48 recommendations across 11 themes. The statements were distributed to relevant clinicians and a representative group of patients to be scored for agreement on a Likert scale. All 48 statements achieved a high degree of consensus, indicating strong alignment of opinion. The recommendations have evolved significantly since the 2005 document, with particularly notable developments including an improved evidence base to guide dosing and indications for acute treatment, greater emphasis on home therapy for acute attacks and a strong focus on service organization.

IgE recognition patterns in peanut allergy are age dependent: perspectives of the EuroPrevall study.

BACKGROUND: We tested the hypothesis that specific molecular sensitization patterns correlate with the clinical data/manifestation in a European peanut-allergic population characterized under a common protocol. METHODS: Sixty-eight peanut-allergic subjects and 82 tolerant controls from 11 European countries were included. Allergy to peanut and lowest symptom-eliciting dose was established by double-blind placebo-controlled food challenge in all but anaphylactic subjects. Information of early or late (before or after 14 years of age) onset of peanut allergy was obtained from standardized questionnaires. IgE to peanut allergens rAra h 1-3, 6, 8-9, profilin and CCD was determined using ImmunoCAP. RESULTS: Seventy-eight percent of peanut allergics were sensitized to peanut extract and 90% to at least one peanut component. rAra h 2 was the sole major allergen for the peanut-allergic population. Geographical differences were observed for rAra h 8 and rAra h 9, which were major allergens for central/western and southern Europeans, respectively. Sensitization to rAra h 1 and 2 was exclusively observed in early-onset peanut allergy. Peanut-tolerant subjects were frequently sensitized to rAra h 8 or 9 but not to storage proteins. Sensitization to Ara h 2 ≥ 1.0 kUA /l conferred a 97% probability for a systemic reaction (P = 0.0002). Logistic regression revealed a significant influence of peanut extract sensitization and region on the occurrence of systemic reactions (P = 0.0185 and P = 0.0436, respectively). CONCLUSION: Sensitization to Ara h 1, 2 and 3 is usually acquired in childhood. IgE to Ara h 2 ≥ 1.0 kUA /l is significantly associated with the development of systemic reactions to peanut.

The EuroPrevall outpatient clinic study on food allergy: background and methodology.

BACKGROUND: The EuroPrevall project aimed to develop effective management strategies in food allergy through a suite of interconnected studies and a multidisciplinary integrated approach. To address some of the gaps in food allergy diagnosis, allergen risk management and socio-economic impact and to complement the EuroPrevall population-based surveys, a cross-sectional study in 12 outpatient clinics across Europe was conducted. We describe the study protocol. METHODS: Patients referred for immediate food adverse reactions underwent a consistent and standardized allergy work-up that comprised collection of medical history; assessment of sensitization to 24 foods, 14 inhalant allergens and 55 allergenic molecules; and confirmation of clinical reactivity and food thresholds by standardized double-blind placebo-controlled food challenges (DBPCFCs) to milk, egg, fish, shrimp, peanut, hazelnut, celeriac, apple and peach. RESULTS: A standardized methodology for a comprehensive evaluation of food allergy was developed and implemented in 12 outpatient clinics across Europe. A total of 2121 patients (22.6% <14 years) reporting 8257 reactions to foods were studied, and 516 DBPCFCs were performed. CONCLUSIONS: This is the largest multicentre European case series in food allergy, in which subjects underwent a comprehensive, uniform and standardized evaluation including DBPCFC, by a methodology which is made available for further studies in food allergy. The analysis of this population will provide information on the different phenotypes of food allergy across Europe, will allow to validate novel in vitro diagnostic tests, to establish threshold values for major allergenic foods and to analyse the socio-economic impact of food allergy.

Assessment of future changes in water availability and aridity.

Substantial changes in the hydrological cycle are projected for the 21st century, but these projections are subject to major uncertainties. In this context, the "dry gets drier, wet gets wetter" (DDWW) paradigm is often used as a simplifying summary. However, recent studies cast doubt on the validity of the paradigm and also on applying the widely used P - E (precipitation - evapotranspiration) metric over global land surfaces. Here we show in a comprehensive CMIP5-based assessment that projected changes in mean annual P - E are generally not significant, except for high-latitude regions showing wetting conditions until the end of the 21st century. Significant increases in aridity do occur in many subtropical and also adjacent humid regions. However, combining both metrics still shows that approximately 70% of all land area will not experience significant changes. Based on these findings, we conclude that the DDWW paradigm is generally not confirmed for projected changes in most land areas.

Risk factors associated with mortality from breast cancer in Waikato, New Zealand: a case-control study.

OBJECTIVES: The aim of this study is to identify key characteristics associated with mortality from breast cancer among women with newly diagnosed breast cancer in New Zealand (NZ). STUDY DESIGN: Case-control study. METHODS: All primary breast cancers diagnosed between 01/01/2002 and 31/12/2010 in Waikato, NZ, were identified from the Waikato Breast Cancer Register. A total of 258 breast cancer deaths were identified from 1767 invasive cancers diagnosed over this period. RESULTS: Breast cancer deaths (n = 246) were compared with an age and year of diagnosis matched control group (n = 652) who were alive at the time of the death of the corresponding case and subsequently did not die from breast cancer. Diagnosis through symptomatic presentation, advanced stage, higher grade, absent hormone receptors (i.e. oestrogen and progesterone) and HER-2 amplification were associated with significantly higher risks of breast cancer mortality in bivariate analysis. Tumour stage, grade and hormone receptor status remained significant in the multivariable model, while mode of detection and HER-2 status were non-significant. In the bivariate analysis, Maori women had a higher risk of breast cancer mortality compared to NZ European women (OR 1.34) which was statistically non-significant. However in the adjusted model, risk of mortality was lower for Maori compared to NZ European women, although this was not significant statistically (OR 0.85). CONCLUSIONS: Mortality pattern from breast cancer in this study were associated with established risk factors. Ethnic inequity in breast cancer mortality in NZ appears to be largely attributable to delay in diagnosis and tumour related factors. Further research in a larger cohort is needed to identify the full impact of these factors on ethnic inequity in breast cancer mortality.

Systematic land climate and evapotranspiration biases in CMIP5 simulations.

[1] Land climate is important for human population since it affects inhabited areas. Here we evaluate the realism of simulated evapotranspiration (ET), precipitation, and temperature in the CMIP5 multimodel ensemble on continental areas. For ET, a newly compiled synthesis data set prepared within the Global Energy and Water Cycle Experiment-sponsored LandFlux-EVAL project is used. The results reveal systematic ET biases in the Coupled Model Intercomparison Project Phase 5 (CMIP5) simulations, with an overestimation in most regions, especially in Europe, Africa, China, Australia, Western North America, and part of the Amazon region. The global average overestimation amounts to 0.17 mm/d. This bias is more pronounced than in the previous CMIP3 ensemble (overestimation of 0.09 mm/d). Consistent with the ET overestimation, precipitation is also overestimated relative to existing reference data sets. We suggest that the identified biases in ET can explain respective systematic biases in temperature in many of the considered regions. The biases additionally display a seasonal dependence and are generally of opposite sign (ET underestimation and temperature overestimation) in boreal summer (June-August).

Cardiomyopathy and cardiac computed tomography: what the radiologist needs to know.

Coronary computed tomographic angiography (CCTA) is an established tool for the investigation of shortness of breath and chest pain. Although CCTA is performed to assess vessels that could well be diseased, one must review the study for evidence of cardiomyopathy, which can provoke similar symptoms. Cardiomyopathies can coexist with various causes of chest pain including obstructive coronary artery disease and may, therefore, be identifiable at CCTA. Furthermore, symptoms such as shortness of breath and chest pain that the clinician may suspect are secondary to coronary disease leading to investigation with CCTA, may be secondary to cardiomyopathy. We review several important causes of cardiomyopathy that may be detected by CCTA, which are important for radiologists to identify given the implications for further management and prognosis.

Autosomal recessive chronic granulomatous disease presenting with cutaneous dermatoses and ocular infection.

Dermatoses such as eczematous dermatitis and cutaneous infection are recognized presentations of primary immunodeficiency (PID). However, atopic dermatitis affects approximately 10% of infants, and cutaneous infections are not uncommon in children, therefore the challenge for the dermatologist is to distinguish the few patients that have PID from the many that do not. We report on a 6-year-old girl who was ultimately diagnosed with autosomal recessive chronic granulomatous disease (AR-CGD) after presenting to various hospitals with dermatitis, scalp plaques recalcitrant to treatment, and recurrent infections over a 3-year period, and describe some aspects of her diagnosis and management. This report highlights the importance of considering rare disorders such as AR-CGD in the differential diagnosis of recurrent or recalcitrant dermatological infections in children.

Penile granulomatosis presenting as pseudoangioedema.

Many conditions may present as angioedema. We report a case of a 46 year-old man presenting with intermittent episodes of penile swelling. Following a series of investigations, he was diagnosed with genital granulomatosis. Ano-genital granulomatosis is a rare chronic inflammatory condition and that can present as diffuse penile, scrotal, vulvar or ano-perineal swelling with non-caseating non-necrotising granulomas on histology.