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Objective: Life-threatening anaphylaxis may occur in IgE-mediated food allergy. OralFood Challenge (OFC) is the gold standard in demonstrating tolerance and diagnosingfood allergy; however, these tests may cause anaphylaxis. Predicting the risk ofdeveloping anaphylaxis before performing OFC is valuable information in evaluatingtolerance as in diagnosis. The present study aims to evaluate the effectiveness of the testsused in clinical practice in predicting the risk of anaphylaxis during OFC in IgE-mediatedfood allergy. To our knowledge, this is the first study evaluating skin prick tests in theprediction of anaphylaxis.Material and Methods: In this descriptive cross-sectional study, the history,demographic, clinical and laboratory data of the patients, followed up with the diagnosisof IgE-mediated food allergy, on whom OFC was performed, were evaluatedretrospectively.Results: Of the 254 patients who underwent OFC, 133 were followed up with a diagnosisof IgE-mediated food allergy. The mean age was 21 months (12-120), and anaphylaxisoccurred in nine (6.7%) of them during OFC. According to the frequency, the foodresponsible for IgE-mediated food allergy was determined as milk, egg and egg-milkcombination. Age during the challenge and total IgE levels were higher in the group thatexperienced OFC-related anaphylaxis. The tests that could best determine the risk ofanaphylaxis before the challenge was the skin prick test (SPT) and prick to prick (PTP)test for milk. Milk SPT and PTP test at the time of initial diagnosis and determination ofmilk sIgE and egg white sIgE before challenge were found to predict the risk ofanaphylaxis. The negative predictive value was over 95% in tests that gave significantresults for milk. There was no statistically significant finding associated with otherallergenic foods.Conclusion: In evaluating tolerance development, performing sIgE, SPT and/or PTPtests for milk before OFC is useful in predicting anaphylaxis. Studies with larger numbersof cases are needed to assess the risk of anaphylaxis caused by other foods.

Cystic fibrosis is one of the most common inherited diseases. It presents with recurrent respiratory infections, pancreatic insufficiency, and growth retardation. Neurological involvement becomes more common as life expectancy increases. Chronic hypoxia, impaired glucose intolerance, autoimmune mechanisms, vasculitis changes, and micronutrient deficiencies seem to cause neuropathy in cystic fibrosis. This study was aimed to investigate peripheral neuropathy in pediatric cystic fibrosis patients. Twenty-one cystic fibrosis patients and 19 healthy control subjects between the ages of 7 and 17 years were included. Their nerve conduction study results and laboratory investigations were analyzed. Participants were classified into four groups; 1. Cystic fibrosis with vitamin D deficiency, 2. Cystic fibrosis with normal vitamin D levels, 3. Healthy subjects with vitamin D deficiency, 4. Healthy subjects with normal vitamin D levels. We found statistically significantly lower sensory median nerve sensorial nerve action potential, sensorial sural nerve conduction velocity, and motor peroneal nerve compound motor action potential in cystic fibrosis patients with vitamin D deficiency than in other cases. We also found that the main difference between cystic fibrosis and control groups was especially in patients with low vitamin D levels. Nerve damage starts at an early age, especially in cystic fibrosis patients especially those with vitamin D deficiency. Electrophysiological evaluation to assess neuropathy is important even in asymptomatic patients. Prevention of hypovitaminosis D is important to prevent neuropathy in cystic fibrosis patients.

Objective A misdiagnosed “penicillin allergy” is a common problem in childhood. Recently, skipping skin tests (STs) and performing a direct oral challenge test (OCT) have become an increasingly common approach in children with suspected β-lactam (BL) allergy. In our study, we aimed to evaluate the safety and efficacy of OCT without using ST in children who had a history of hypersensitivity reactions with BL antibiotics. Materials and Methods We retrospectively evaluated direct OCT outcomes in children with both nonimmediate and immediate-type reaction history with BL antibiotics. STs were not performed before the challenge test. The patients were monitored for 4 hours after the challenge and continued using the drug in two divided doses for 3 days at home. Results In this study, 72 patients were included, with median age of 7 years (interquartile range: 4; min: 1 year to max: 16 years), and of these, 56% were male. Forty-five subjects (63%) reported immediate-type adverse reactions. The most common clinical manifestation was urticaria/angioedema (51%, n: 37) and maculopapular exanthema in 46% (n: 33) of patients, respectively. The most commonly suspected drug was 71% amoxicillin-clavulanate. A 3-day OCT without preceding ST was performed in all patients. Only three patients (4.2%) showed a positive response to the oral drug challenge test. None of these reactions observed was more severe than index reactions. Conclusion Performing OCT without STs is a safe and convenient method to exclude BL hypersensitivity in the pediatric age group.

Objective Interferon gamma receptor 1 (IFNGR1) deficiency is a primary immunodeficiency that causes systemic infections with weakly virulent nontuberculous mycobacteria, which are very difficult to control with combined antimycobacterial agents. Methods Disseminated mycobacterial infection that could not be controlled with combined antimycobacterial treatments in a 13-year-old patient with a novel mutation in the IFNGR1 gene. Despite receiving intravenous antibiotic therapy, fever continued, bone involvement gradually increased, and proteinuria and microscopic hematuria occurred. Peg-IFNα-2a was administered as an adjuvant therapy that resulted in good clinical response with no side effects. Fever improved and no new bone lesions occurred with edema of existing lesions regressing after 2 weeks of peg-IFNα-2a therapy. Conclusion Aggressive treatment with antimycobacterial antibiotics and hematopoietic stem cell transplantation remains the single accepted method for the management of patients with complete IFNGR1 deficiency. Peg-IFNα-2a treatment may be an adjunct treatment in this disorder.

Background: Cystic fibrosis (CF) is an inherited autosomal recessive disorder that causes chronic airway disease. In addition to genetic factors, environmental factors may affect the clinical phenotype of CF. In this study, the presence of aeroallergen sensitivity in our patients with CF and its effects on clinical findings are evaluated. Methods: In this study, patients included were diagnosed with CF and followed in the Pediatric Respiratory and Allergy Clinic of the Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey. Demographic characteristics, clinical and laboratory findings, skin prick test (SPT) results, and modified Shwachman-Kulczycki (MSK) scores of the patients were evaluated. Results: We evaluated 51 patients with CF with a median age of 10 (6-18) years. The mean MSK score of the patients was 72.54 +/- 11.50, and the mean predictive value of forced expira-tory volume (FEV1) in the initial (1st) second was 80.43 +/- 19.50. According to SPT, aeroallergen sensitivity was detected in 17 (33.3%) patients. The prevalence of bacterial colonization and bronchiectasis was higher, and MSK scores were lower in Aspergillus fumigatus (AF)-sensitive patients (P

Background: Atopic dermatitis (AD) is a chronic, relapsing, inflammatory skin disease. In this study, the relationship between the severity of AD and the quality of life (QoL) of patients and their families were evaluated; also, the factors that predict the severity of AD and the QoL index were determined. Methods: Infants with AD were enrolled in the study. Pruritus, sleep disturbance, and dermatitis severity scores were obtained. The QoL of infants was assessed using the Infants’ Dermatitis Quality of Life Index (IDQOL), while the Family Dermatology Life Quality Index (FDLQI) was used to assess the impact of disease on the QoL of parents. Results: 122 patients diagnosed with AD were enrolled in the study. The correlation analysis of score showed a positive correlation between IDQOL and FDLQI (r = 0.444, p < 0.0001). Positive correlations between the pruritus, sleep disturbance, Severity Scoring of Atopic Dermatitis (SCORAD), Eczema Area and Severity Index (EASI), and dermatitis severity scores and the QoL indexes were found. While pruritus, sleep disturbance scores, and EASI were the most significant parameters for predicting a severe SCORAD score, IDOQL, FDLQI, and SCORAD were the most significant parameters for predicting severe EASI. In the analysis of QoL indexes, sleep disturbance and FDLQI were the most significant parameters for predicting severe IDQOL index, while IDQOL was the most significant parameter for predicting severe FDLQI scores. Conclusions: AD has a negative effect on the QoL of infants and their parents. Pruritus and sleep disturbance should be evaluated during clinical practice due to their strong relationship with disease severity and QoL index.