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Introduction: Beta-lactam (BL) antibiotics are the most often involved drugs in allergic reactions. Mild cutaneous reactions such as maculopapular exanthema or urticaria are the most common presenting complaints of BL allergy in the pediatric population. However, it can be challenging to distinguish BL-induced allergy from reactions due to infections or other reasons. In this study, we aimed to determine the clinical characteristics and potential risk factors of true BL allergy in children with suspected mild cutaneous reactions to BLs. Methods: We evaluated children who were admitted to our pediatric allergy clinic with suspected BL allergy in between January 2015 and March 2020. Patients with a history suggestive of immediate and non-immediate mild cutaneous reactions were included in the study. The oral challenge test (OCT) with the culprit drug was performed on all patients to confirm the diagnosis. Results: Two hundred fourteen (119 male and 95 female) patients with a median age of 4.9 years were evaluated. BL allergy was confirmed in 10.7% (23) of the patients, according to the OCT results. Most of the proven allergic reactions were of the immediate type (73.9%), and urticaria was the most common presenting complaint (60.8%) in proven BL-allergic patients. The negative predictive value of penicillin-G skin testing was 89.7% for immediate-type penicillin allergy and 93.4% for non-immediate reactions. Also, positive predictive value of penicillin-G skin testing was 50% for immediate and 25% for non-immediate reactions. In the multivariate logistic regression analysis, a history of proven drug allergy (Exp (B): 7.76, 95% CI: 1.88–31.97, p = 0.005) was found to be the risk for BL allergy. Conclusion: This study highlighted that OCTs should be performed to confirm the diagnosis in patients suspected of immediate and non-immediate mild cutaneous reactions to BLs and remove the overestimated “BL allergy” label. In these patients, a history of proven drug allergy might be a risk factor for true BL allergy.

Introduction: Ibuprofen is the most common culprit drug causing nonsteroidal anti-inflammatory drug (NSAID) hypersensitivity in children. We aimed to evaluate the frequency, clinical characteristics, and risk factors of confirmed ibuprofen allergy in children presenting with a history of suspected immediate type ibuprofen-induced hypersensitivity reactions. Methods: We evaluated 50 (35 M, 15 F) children with a median age of 7 years, who were referred to our clinic with suspected immediate ibuprofen hypersensitivity. Patients were subjected to a diagnostic work up including drug provocation tests (DPTs) with the culprit drug. Reactions were classified according to the European Academy of Allergy and Clinical Immunology Task Force recommendations for pediatric patients. Proven ibuprofen allergic patients underwent DPT to find a safe alternative drug. Results: Ibuprofen allergy was confirmed in 34% (n: 17) of children; 9 patients were diagnosed by DPTs and 8 patients diagnosed based on their histories. Angioedema was the most common clinical manifestation (n: 30, 60%). Among patients with proven ibuprofen allergy, 7 of them were classified as cross-intolerant. Cross-intolerance reactions were further classified as NSAID-exacerbated cutaneous disease (n = 1) and NSAID-induced urticaria/angioedema/anaphylaxis (n = 6). As an alternative drug, paracetamol was safely tolerated, whereas 1 patient developed angioedema and urticaria with nimesulide. Older age and male gender were identified as independent risk factors for immediate-type ibuprofen allergy. Conclusion: DPTs should be performed to confirm or exclude ibuprofen allergy in children and to find safe alternative drugs. Male gender and older age are risk factors for ibuprofen allergy. NSAID-induced hypersensitivity reactions in the pediatric population cannot be well defined using the adult classification system.

Asthma is the most common chronic disease of childhood. Management of asthma control mainly depends on compliance to long-term therapy. Art therapy, in which children express their experiences through artistic activities, is one of the psychosocial support treatments in chronic diseases. The aim of the study is to investigate the effects of expressive art therapy on asthma control and quality of life of asthmatic children.A total of 20 children (9F/11M), aged 8 to13 years, had a group art therapy program constructed of 90-minutes sessions per week for 8-weeks. Pulmonary function tests (PFTs) by spirometry, asthma control tests, and asthma quality of life scale for children (PAQLQ) were performed before and after art therapy.Although statistically significant increase in PFTs (FEV1, PEF,p= 0.001) and improvement in the items of quality of life (activity limitation, symptoms, p0.001) were observed in our patients after art therapy, the increase in asthma control was not significant.Expressive art therapy can cause improvement in both pulmonary function tests and quality of life scales in children with asthma. Longer-term art therapies planned by an experienced team may also be beneficial in achieving asthma control.

Background: Several factors that increase the risk of severe food-induced anaphylaxis have been identified. Objective: We aimed to determine the demographic, etiologic, and clinical features of food-induced anaphylaxis in early childhood and also any other factors associated with severe anaphylaxis. Methods: We carried out a medical chart review of anaphylaxis cases from 16 pediatric allergy and immunology centers in Turkey. Results: The data of 227 patients with 266 food-induced anaphylaxis episodes were included in the study. The median (interquartile range) age of the first anaphylaxis episode was 9 months (6‐18 months); 160 of these patients were boys (70.5%). The anaphylaxis episodes were mild in 75 cases (28.2%), moderate in 154 cases (57.9%), and severe in 37 cases (13.9%). The most frequent food allergens involved were cow's milk (47.4%), nuts (16.7%), and hen's egg (15.8%). Epinephrine was administered in only 98 (36.8%) of these anaphylaxis episodes. A logistic regression analysis revealed two statistically significant factors that were independently associated with severe anaphylaxis: the presence of angioedema and hoarseness during the anaphylactic episode. Urticaria was observed less frequently in patients who developed hypotension. In addition, confusion and syncope were associated with 25.9- and 44.6-fold increases, respectively, in the risk of concomitant hypotension. Conclusion: Cow's milk, nuts, and hen's egg caused the majority of mild and moderate-to-severe anaphylaxis episodes. The presence of angioedema and hoarseness in any patient who presents with a history of food-induced anaphylaxis should alert clinicians that the reaction may be severe. In addition, the presence of confusion, syncope, or stridor probably indicates concomitant hypotension.

© 2022 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.Background: Lipopolysaccharide-responsive beige-like anchor protein (LRBA) deficiency and cytotoxic T-lymphocyte protein-4 (CTLA-4) insufficiency are recently described disorders that present with susceptibility to infections, autoimmunity, and lymphoproliferation. Clinical and immunological comparisons of the diseases with long-term follow-up have not been previously reported. We sought to compare the clinical and laboratory manifestations of both diseases and investigate the role of flow cytometry in predicting the genetic defect in patients with LRBA deficiency and CTLA-4 insufficiency. Methods: Patients were evaluated clinically with laboratory assessments for lymphocyte subsets, T follicular helper cells (TFH), LRBA expression, and expression of CD25, FOXP3, and CTLA4 in regulatory T cells (Tregs) at baseline and 16 h post-stimulation. Results: LRBA-deficient patients (n = 29) showed significantly early age of symptom onset, higher rates of pneumonia, autoimmunity, chronic diarrhea, and failure to thrive compared to CTLA-4 insufficiency (n = 12). In total, 29 patients received abatacept with favorable responses and the overall survival probability was not different between transplanted versus non-transplanted patients in LRBA deficiency. Meanwhile, higher probability of survival was observed in CTLA-4-insufficient patients (p = 0.04). The T-cell subsets showed more deviation to memory cells in CTLA-4-insufficiency, accompanied by low percentages of Treg and dysregulated cTFH cells response in both diseases. Cumulative numbers of autoimmunities positively correlated with cTFH frequencies. Baseline CTLA-4 expression was significantly diminished in LRBA deficiency and CTLA-4 insufficiency, but significant induction in CTLA-4 was observed after short-term T-cell stimulation in LRBA deficiency and controls, while this elevation was less in CTLA-4 insufficiency, allowing to differentiate this disease from LRBA deficiency with high sensitivity (87.5%) and specificity (90%). Conclusion: This cohort provided detailed clinical and laboratory comparisons for LRBA deficiency and CTLA-4 insufficiency. The flow cytometric approach is useful in predicting the defective gene; thus, targeted sequencing can be conducted to provide rapid diagnosis and treatment for these diseases impacting the CTLA-4 pathway.

Antibiotic allergy is a big problem that may affect the treatment and life quality of patients with cystic fibrosis (CF).To evaluate predictive factors for confirmed antibiotic hypersensitivity in children with CF.In this case-controlled study, we examined 15 patients with CF who had been confirmed with antibiotic allergy. Additionally, we included a control group of age- and gender-matched 45 CF patients with no antibiotic allergy. The diagnosis of antibiotic allergy was confirmed in the presence of a compatible history and a positive response in the drug skin test or provocation test. Multiple drug hypersensitivity was classified according to the temporal relationship of antibiotics: (i) distant, (ii) simultaneous, and (iii) sequential. The data were analyzed by conditional logistic regression.β-lactam allergy was confirmed in eight patients (ceftazidime n = 5, piperacillin-tazobactam n = 3) and non-β-lactam allergy was confirmed in two patients (ciprofloxacin n = 1, azithromycin n = 1). Additionally, multiple drug hypersensitivity in five patients (distant n = 4, sequential n = 1), among whom two patients showed hypersensitivity against ceftazidime/piperacillin-tazobactam+ ciprofloxacin/levofloxacin, two patients showed hypersensitivity against ceftazidime+ ciprofloxacin n = 2, and one patient showed hypersensitivity against piperacillin-tazobactam+ amikacin+ trimethoprim-sulfamethoxazole. All patients (n = 13) with confirmed β-lactam allergy were meropenem tolerant. Multivariate analysis indicated that immediate reactions (, p lt; 0.001) and allergic evaluation in the first six months after the reaction (p = 0.036) were significant risk factors for the prediction of antibiotic hypersensitivity.Beta-lactam antibiotic allergy is the most commonly confirmed drug allergy in children with CF. However, unlike normal children, ceftazidime and piperacillin-tazobactam account for the majority.

Atopic dermatitis is a chronic relapsing, inflammatory skin disorder associated with skin barrier dysfunction, the prevalence of which has increased dramatically in developing countries. In this article, we propose a treatment algorithm for patients with mild-to-moderate and severe atopic dermatitis flares in daily clinical practice. An international panel of 15 dermatology and allergy experts from eight countries was formed to develop a practical algorithm for the treatment of patients with atopic dermatitis, with a particular focus on topical therapies. In cases of mild-to-moderate atopic dermatitis involving sensitive skin areas, the topical calcineurin inhibitor pimecrolimus should be applied twice daily at the first signs of atopic dermatitis. For other body locations, patients should apply a topical calcineurin inhibitor, either pimecrolimus or tacrolimus, twice daily at the first signs of atopic dermatitis, such as pruritus, or twice weekly in previously affected skin areas. Emollients should be used regularly. Patients experiencing acute atopic dermatitis flares in sensitive skin areas should apply a topical corticosteroid twice daily or alternate once-daily topical corticosteroid/topical calcineurin inhibitor until symptoms improve. Following improvement, topical corticosteroid therapy should be discontinued and patients switched to a topical calcineurin inhibitor. Maintenance therapy should include the use of pimecrolimus once daily for sensitive areas and tacrolimus for other body locations. This treatment algorithm can help guide clinical decision-making in the treatment of atopic dermatitis.

Cetirizine is a selective H1 histamine receptor antagonist derived from piperazine. Piperazine is a cyclic moiety molecule located in the structure of many drugs, including anxiolytics, antidepressants, and antipsychotics. Drug-induced dystonia is reported mostly due to antipsychotic and antiemetic drugs. It occurs due to the disruption of dopamine and acetylcholine balance in favor of acetylcholine. Although cetirizine is a relatively safe drug, it has rarely been reported to cause a dystonic reaction. To the best of our knowledge, there is no reported case of dystonia due to cetirizine in infancy. Here, we present a 7-month-old patient who developed a dystonic reaction after cetirizine administration., Setirizin, piperazinden türetilen seçici bir H1 histamin reseptör antagonis- tidir. Piperazin, anksiyolitikler, antidepresanlar ve antipsikotikler dahil olmak üzere birçok ilacın yapısında yer alan siklik bir moleküldür. İlaca bağlı distoni, çoğunlukla antipsikotik ve antiemetik ilaçlara bağlı olarak bildirilmektedir. Dopamin ve asetilkolin dengesinin asetilkolin lehine bozulması sonucu oluşur. Setirizin nispeten güvenli bir ilaç olmasına rağ- men, nadiren distonik reaksiyona neden olduğu bildirilmiştir. Şimdiye kadar süt çocukluğu döneminde setirizine bağlı olarak bildirilmiş bir dis- toni vakası yoktur. Burada setirizin uygulaması sonrası distonik reaksiyon gelişen 7 aylık bir hasta sunulmuştur.

Background: The prevalence of allergic disorders is on the rise, affecting about 10% of the population. In this retrospective cohort, we investigated prevalence of allergic disorders, associated risk factors, and the outcome of food allergies. Material and methods: We analyzed data from birth cohorts of two university hospitals' well-child outpatient clinics. Factors related to onset and type of allergic diseases were assessed from demographic, socioeconomic, and clinical data. Results: Analyses were performed on 949 (431F/518M) infants at a mean current age of 28 +/- 6 months. Any allergic disease was established among 177 cases (22%); atopic dermatitis in 123 (12.8%), respiratory allergies in 55 (5.7%), and food allergy in 41 (4.3%). The risk for allergic disorders was found to be significantly increased for male gender (OR: 2.31, 95% CI; 1.54-3.46), and positive parental atopy (OR: 1.94, 95% CI; 1.31-2.86). The risk of food allergies was significantly higher in the male gender (OR: 2.47, 95% CI; 1.21-5.02), who consumed egg-white between 6 and 12 months (OR: 2.34, 95% CI; 1.22-4.48), and who were formula-fed before 6 months (OR: 2.16, 95% CI; 1.14-4.10). We found no significant association between the rate of food allergy outgrowth or food induced-anaphylaxis with regards to the timing of introducing egg-white into the diet. Conclusions: Although the introduction of egg-white into infant diet at 6-12 months of life appeared as an independent risk for any food allergy, none of the patients developed anaphylaxis. Age at symptom onset and outgrowing food allergy were similar compared to those introduced egg-white after 12 months. We recommend promoting exclusive breast-feeding during the first 6 months of life, and avoidance of prolonged restrictive diets for children with food allergy. (C) 2021 Codon Publications. Published by Codon Publications.

The purpose of the study was to determine the effect of two different training styles (with brochure or video) with follow-ups for metered-dose inhaler (MDI) technique on asthma control and quality...

Food allergy is a component of the atopic march and may have effects on asthma. This study aimed to evaluate the risk factors for confirmed immunoglobulin E-mediated food allergies and their impact on the clinical picture in preschool children with asthma.Clinical history and allergic assessment results were obtained from medical records and analyzed retrospectively. Preschool children with asthma were included in the study and the characteristics of food allergy and asthma were evaluated. The patients were grouped as those with food allergy (Group I, n = 60) and those without (Group II, n = 98).In patients with food allergy and asthma, the number of episodes requiring systemic steroids in the last year (p = 0.002), atopic dermatitis (p = 0.001), parental atopic disease (p = 0.009) and aeroallergen sensitivity rates (p0.001) was higher than patients without food allergies. The use of medium or high doses of inhaled steroids to achieve asthma control was more frequent in patients with food allergies (p = 0.014). Parental history of atopic disease [p = 0.007, odds ratio (OR): 3.27, 95% confidence interval (CI) 1.37-7.77)], atopic dermatitis (p = 0.017, OR: 2.80, 95% CI: 1.19-6.57), starting complementary food after 6 months (p = 0.004, OR: 3.9, 95% CI: 1.5-10.0) and having aeroallergen sensitivity (p0.001, OR: 6.01, 95% CI: 2.21-16.29) were identified as significant risk factors for food allergy.Asthmatic preschool children with food allergies are more likely to have a parental atopic disease, atopic dermatitis, aeroallergen sensitivity and starting complementary food after 6 months. These patients experience more asthma attacks and need higher doses of steroids.Food allergy and asthma in preschool children affect each other mutually. Food allergy is a component of the atopic march, a risk factor for the development of asthma, and is also included in the modified asthma predictive index in children with wheezing. Therefore, the manifestation of asthma-related symptoms such as cough and wheezing along with food allergy in this age group may facilitate the decision to start asthma control therapy. On the other hand, having asthma is a risk factor for food-related reactions to be more serious. In the current study, we compared patients with and without food allergies. We determined that other atopic diseases (allergic rhinitis and atopic dermatitis) are more common in children with food allergies, and asthma control can be achieved with higher inhaler steroid doses.

Background Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. Methods In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). Results In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). Conclusions Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations.

Ayse Suleyman, Zeynep Tamay, Nermin Guler Istanbul University, Istanbul Faculty of Medicine, Department of Pediatrics, Division of Pediatric Allergy and Immunology, Istanbul, TurkeyCorrespondence: Ayse Suleyman, Istanbul University, Istanbul Faculty of Medicine, Department of Pediatrics, Division of Pediatric Allergy and Immunology, Capa, Istanbul, Turkey, Tel +90 535 4160702, Email draysesuleyman@yahoo.comBackground: Local anesthetics (LA) are relatively safe and rarely cause immediate hypersensitivity reactions. The data on immediate LA hypersensitivity and its risk factors in children are limited.Aim: To evaluate risk factors of immediate LA hypersensitivity.Methods: The retrospective case-controlled study included 17 patients with confirmed immediate LA hypersensitivity. For each patient, three age- and gender-matched control subjects were included in the study. LA hypersensitivity was excluded by skin tests and subcutaneous drug provocation tests in all control subjects.Results: The most common allergic assessment in the patient/control group was for lidocaine (n=5; 29.4%, vs n=23; 45.1%). Suspected LA hypersensitivity reactions were found to be associated with cutaneous manifestations in 14 (82.4%) patients and in 7 (13.7%) of the controls. A history of exposure to local anesthetics twice or more was present in 11 (64.7%) patients vs 6 (11.8%) controls. In conditional regression analysis, repeated LA exposure (≥ 2) and cutaneous findings were determined as significant risk factors (Odds Ratio [OR]:5.7; 95% Confidence Interval [CI]:1.2– 27.1; P=0.029 and (OR:17.3; 95% CI:3.6– 82.5; P< 0.001, respectively).Conclusion: Cutaneous manifestations and a history of LA exposure twice or more were predictive factors for LA allergy confirmed by skin test in children.Keywords: local anesthetic, immediate reactions, hypersensitivity, predictive factors, skin test, subcutaneous provocation test, children

Amaç: Bu çalışmada amacımız okul öncesi astımlı çocuklarda yatmadan önceki son beslenme saati, uykuda beslenmeleri, gece yatma saati, sabah kalkma saati ve sosyal çevresi ile ilgili faktörlerin üst solunum yolu semptomları ve astım kontrolü üzerine etkilerini araştırmaktadır. Gereç ve Yöntem: Çalışma, okul öncesi astımlı çocuklar ile benzer cinsiyet, yaş dağılımına sahip sağlıklı çocuklarda yapıldı. Bulgular: Çalışma gurubuna 103’ü (%47,5) astım, 114’ü (%52,5) sağlıklı olmak üzere 217 çocuk alındı. Gece yatmadan önce son 2 saat içinde beslenme sıklığı astımlı çocuklarda %47,6 iken sağlıklı çocuklarda %82,5 idi (p, Objective: In this study, our aim was to investigate the effects of feeding habits such as the last feed before bedtime, feeding during sleep, bedtime, waking time and factors associated with the social environment on upper airway tract symptoms and asthma control. Material and Method: The study was conducted on children with preschool asthma and their age and sex matched healthy children. Results: The study group included 217 children, 103 of whom have asthma and 114 were (52.5%) healthy. The frequency of feeding in the last 2 hours before going to bed was 47.6% in children with asthma and 82.5% in healthy children (p

Background Clarithromycin hypersensitivity is reported as the most common cause of non-β-lactam antibiotic allergy in children. Clarithromycin is frequently prescribed in cases of suspected β-lactam hypersensitivity. Oral provocation tests stand as the gold standard to confirm drug hypersensitivity as diagnostic value of skin tests is variable. We analyzed the frequency of true clarithromycin hypersensitivity ratio and its relationship with β-lactam allergy among children with suspected clarithromycin hypersensitivity and evaluated the diagnostic value of skin tests. Methods The study included 160 children referred with suspected clarithromycin hypersensitivity. Clinical history and allergy workups including skin tests or/and oral provocation tests were retrieved from medical records. Results Oral provocation test confirmed clarithromycin hypersensitivity rate was 5.6% (n = 9/160). Skin tests with clarithromycin showed positivity in 32.6% (n = 29/89) of the tested patients. The sensitivity of clarithromycin skin tests was negligible, and specificity was 73.9% (95% confidence interval [CI], 64.7-81.8). Eighty-eight of the patients (55%) reported that they had previously tolerated a β-lactam antibiotic. β-lactam hypersensitivity was suspected in 40% (n = 64/160) of the patients (simultaneous [n = 10], sequential [n = 19], distant form [n = 35]) in relation with clarithromycin usage. β-lactam hypersensitivity (95% CI, 2.1-70.6, p = .005) and sequential usage of clarithromycin after the development of a rash with amoxicillin-clavulanic acid (95% CI, 2.0-96.4, p = .007) were found as risk factors for confirmed clarithromycin hypersensitivity. Conclusion The frequency of confirmed clarithromycin hypersensitivity was found low among suspected patients. Oral provocation test is crucial for definite diagnosis. Confirmed β-lactam allergy may be attributed as a risk factor for clarithromycin hypersensitivity, particularly clarithromycin treatment after a developing rash with amoxicillin-clavulanic acid in sequential usage.

Background: Cow's milk is one of the most common of the foods that cause food allergies in children. Here, we present a 10-month-old male who was diagnosed with having an allergy to cow's milk and who developed an anaphylactic reaction after being recently vaccinated with a measles vaccine. Case: The patient had been diagnosed with atopic dermatitis and cow's milk allergy at 40 days old after a rash appeared on his face and arms while exclusively breastfeeding. At 9 months, on his routine welfare outpatient appointment, he developed a facial rash and swelling, wheezing, difficulty breathing, and cyanosis within 10 min of having his first measles vaccination (M-VAC(®); Serum Institute of India, Hadapsar, Pune, India). After an allergy evaluation and a physical examination that showed that he was otherwise healthy, he was diagnosed with an allergy to cow's milk, which was then eliminated from his diet. Laboratory evaluations were as follows: serum immunoglobulin E (IgE) to cow's milk: 36.2 kU/L, α-lactalbumin: 9.39 kU/L, β-lactoglobulin: 8.74 kU/L, casein: 34.2 kU/L, latex-specific (sp)IgE: 0.10 kU/L, gelatin spIgE

Clarithromycin and azithromycin are the most frequently prescribed oral macrolide antibiotics for children. Macrolides are relatively safe antibiotics due to their low allergenicity, but are nevertheless known to cause hypersensitivity reactions. Azithromycin appears to be responsible for severe reactions more often than clarithromycin. The most common reactions are maculopapular exanthems, and macrolide-induced anaphylaxis is exceptionally rare. Skin tests are not enough for the diagnosis of macrolide allergies, and oral provocation tests are necessary for a definite diagnosis. Here, we present a case of anaphylaxis during oral provocation with azithromycin, in which intolerance to amoxicillin-clavulanic acid and clarithromycin have been verified by provocation testing previously.

Objective: β-lactam antibiotic allergy is the most common drug allergy in children. Most of the patients with suspected reactions to β-lactam antibiotics can actually tolerate these drugs. The aim of this study is to evaluate clinical and laboratory characteristics of children with β-lactam allergy and to determine cross-reactivity between penicillin and cephalosporins. Methods: The diagnosis of β-lactam allergy was made based on the results of skin tests and/or drug provocation tests (DPT). Penicillin allergy skin tests were performed with DAP penicillin® (Diater laboratories, Madrid, Spain), penicillin G, and ampicillin/amoxicillin preparations. Skin and provocation tests were performed with the culprit cephalosporin in addition to the penicillin skin and/or provocation tests to evaluate cephalosporin allergy. Results: We found that 87.7% (71/81) of patients with β-lactam allergy were able to tolerate the culprit drug. Among ten patients with confirmed diagnosis, two had cross-reactivity (penicillin and cephalosporin) and 8 had a various β-lactam (aminopenicillin n=6, ceftriaxone n=2) allergies. We identified older age and early-type clinical reactions as risk factors for a confirmed β-lactam allergy. Conclusion: Skin tests and DPT appear to be useful procedures in the diagnosis, and determination of an alternative safe antibiotic in patients with β-lactam allergy. Most of the patients tolerated the drugs. A minority of the patients with confirmed allergy should avoid all β-lactam antibiotics due to the probability of cross-reactivity.

Giris: Kronik spontan urtiker (KSU) tanisi, esas olarak detayli alinmis bir oyku ve fizik muayene ile konulur. Tani icin sinirli sayida laboratuvar testlerinin yapilmasi onerilir. Ancak altta yatan hastaliklari dislamak ve/veya neden olan faktorleri belirlemek amaci ile uzmanlar tarafindan siklikla laboratuvar testleri onerilenden daha fazla istenmektedir. Amac: Calismamizda KSU tanili hastalarda laboratuvar bulgularinin hastaligin tanisi ve yonetimi uzerine olan etkisini degerlendirmeyi amacladik. Gerec ve Yontem: Kronik spontan urtiker tanisi konulan hastalarin demografik ve klinik bulgulari, laboratuvar test sonuclari ve tedavileri degerlendirildi. Bulgular: Kronik spontan urtikerli toplam 113 hasta (61 E, 52 K) degerlendirildi. Medyan yas 7 yil, (ceyrekler arasi acikligi; IQR 7-10) idi. Toplam 911 testten sadece 156’si (%17,1) anormal idi. En sik anormallik tam kan sayiminda saptandi. Hastalarin 83’u (%73,4) birinci basamak tedavi almakta idi. Laboratuvar test sonuclari hicbir hastanin tedavisini etkilemedi ve tedavi protokollerinde bir degisiklik yapilmasina neden olmadi. Sonuc: Detayli olarak yapilan laboratuvar testleri KSU’li hastalarda urtikerin seyrini degistirmese de laboratuvar tetkikleri her hastanin kisisel ozellikleri goz onune alinarak planlanabilir.

This review by pediatric gastroenterology and allergy-immunology experts aimed to address the biological roles of human milk oligosaccharides (HMOs) and the potential utility of HMOs in prevention of allergy with particular emphasis on cow's milk protein allergy (CMPA). The participating experts consider HMOs amongst the most critical bioactive components of human milk, which act as antimicrobials and antivirals by preventing pathogen adhesion to epithelial cells, as intestinal epithelial cell modulators by enhancing maturation of intestinal mucosa and intestinal epithelial barrier function, as prebiotics by promoting healthy microbiota composition and as immunomodulators by modulating immune cells indirectly and directly. Accordingly, the participating experts consider the proposed link between HMOs and prevention of allergy to be primarily based on the impact of HMO on gut microbiota, intestinal mucosal barrier, immunomodulation and immune maturation. Along with the lower risk of respiratory and gastrointestinal infections, HMO supplemented formulas seem to be promising alternatives in the management of CMPA. Nonetheless, the effects of individual as well as complex mixtures of HMO in terms of clear clinical and immunological effects and tolerance development need to be further explored to fully realize the immunomodulatory mechanisms and the potential for HMOs in prevention of allergic diseases and CMPA. Abbott Nutrition Turkey The authors would like to thank Cagla Ayhan, MD and Prof. Sule Oktay, MD, PhD from KAPPA Consultancy Training Research Ltd, Istanbul who provided editorial support funded by Abbott Nutrition Turkey.

Purpose: Recently, a new form of congenital neutropenia that is caused by germline biallelic loss-of-function mutations in the SMARCD2 gene was described in four patients. Given the rarity of the condition, the clinical spectrum of the disease has remained elusive. We here report a new patient with a novel frameshift mutation and compare our patient with the previously reported SMARCD2-mutant patients, aiming to provide a more comprehensive understanding of the natural course of the disease. Methods: Clinical and laboratory findings of all reported patients were reviewed. Next-generation sequencing was performed to identify the causative genetic defect. Data on the hematopoietic stem cell transplantation including stem cell sources, conditioning regimen, engraftment, graft-versus-host disease, and infections were also collected. Results: An 11-year-old female patient had a variety of infections including sepsis, deep tissue abscesses, otitis, pneumonia, gingivitis, and diarrhea since infancy. A novel homozygous mutation in SMARCD2 (c.93delG, p.Ala32Argfs*80) was detected. Bone marrow examination showed hypocellularity and decreased neutrophils with diminished granules and myeloid dysplasia, but no blast excess as in previously reported patients. The neutropenia was non-responsive even to higher doses of granulocyte colony-stimulating factor (G-CSF); therefore, the patient was transplanted at 10 years of age from a HLA-A allele–mismatched unrelated donor using a reduced toxicity conditioning regimen and recovered successfully. Compared with the previous four cases, our patient showed longer survival before transplantation without blastic transformation. Conclusion: Distinctive myeloid features and long-term follow-up including therapy options are presented for the newly described case of SMARCD2 deficiency. This disorder is apparent at infancy and requires early transplantation due to the unrelenting disease course despite conventional therapy. WOS:000575728800002 33025377 Q1

Background: Urticaria can be the only sign of a food allergy or can be seen together with other signs and symptoms of a food allergy. Objective: To determine the demographic, etiologic, and clinical features of food-induced acute urticaria in childhood. Methods: Patients suspected of food-induced acute urticaria were included in this prospective cross-sectional multicenter study. Results: Two hundred twenty-nine urticaria cases were included in this study. Seventeen patients who did not meet the inclusion criteria of the study were excluded. Of the 212 included cases, 179 (84.4%) were diagnosed with definitive food-induced acute urticaria. The most common foods causing acute urticaria were cow’s milk, hen’s eggs, and nuts in 56.4, 35.2, and 19% of cases, respectively. The positive predictive value of a history of milk-induced acute urticaria together with a milk-specific IgE >5 kU/L for cow’s milk-induced acute urticaria was 92% (95% CI: 81–96%). A history of cow’s milk-induced and/or hen’s egg-induced acute urticaria was consistent with a definitive diagnosis of food-induced urticaria (Chen’s kappa: 0.664 and 0.627 for milk and eggs, respectively). Urticaria activity scores were higher in patients with food-induced acute urticaria (p = 0.002). Conclusion: Cow’s milk, hen’s eggs, and nuts were the most common allergens in the etiology of childhood food-induced acute urticaria. Although the urticaria activity score provides guidance for diagnosis, an oral food challenge is often essential for the definitive diagnosis of a patient with a history of food-induced acute urticaria.

Background: Cystic Fibrosis Registry of Turkey shows various CFTR mutations due to the geographical location and historical background of our country, and also high prevalence of consanguineous marriages. Method:All mutations detected in the Cystic Fibrosis Registry of Turkey 2017 (CFRT2017) data were screened in CFTR1 and CFTR2 databases. Mutations which were not found in both were identified and characteristics of these patients were compared with F508del homozygous patients. Results: Among 1170 registered patients, 978 were genotyped and 200 different mutations were shown in 1270 alleles.29 mutations were not reported in both databases; 58 mutations have been reported in CFTR1 but not in CFTR2. Demographic and phenotypic characteristics of the 112 patients with 87 different alleles those were not previously reported in the CFTR2 database (nonCFTR2 group) were compared with F508del homozygous 103 patients in CFRT2017. In the nonCFTR2 group, mean age was younger (5.81 vs 7.69; p:0.015), mean age at diagnosis was older (1.88 vs 1.08; p:0.041), sweat test was lower (75.12 vs 98.28; p Conclusion: We suggest that patients in the nonCFTR2 group have a mild clinical course, but in some patients, further investigations and functional studies are required for the exact diagnosis.

Pandemic of COVID-19 which has recently influenced our country and the global world, has been an important public health problem. It is possible that patients with asthma may also be affected in this outbreak. At this stage, the correct approach of physicians to patients with asthma can be achieved by revealing the relationship between asthma and COVID-19. Up to now, it has been reported that COVID-19 controlled asthma does not pose an additional risk for controlled asthma COVID-19. However, it is still important to ensure that patients with asthma follow social isolation and personal hygiene rules. Treating asthma according to current guidelines is an appropriate approach, until new information is available., Son günlerde ülkemiz ve tüm dünyayı etkisi altına alan COVID-19 pandemisi önemli bir halk sağlığı sorunu olmuştur. Salgında astımlı hastaların da etkilenmesi mümkündür. Bu aşamada hekimlerin astımlı hastalara doğru yaklaşımı astım COVID-19 arasındaki ilişkinin ortaya konması ile sağlanabilir. Şu ana kadar kontol altındaki astımlılar için COVID-19’un için ek risk getirmediği oluşturmadığı bildirilmişti. Ancak yine de astım hastalarının sosyal izolasyon ve kişisel hijyen kurallarına uymalarının sağlanması önemlidir. Yeni bilgiler elde edilinceye kadar astımın mevcut rehberlere göre tedavi edilmesi uygun bir yaklaşımdır.

Objective: Many perinatal risk factors such as mother’s age and premature birth have been identified for childhood asthma and wheezy child. Some observational studies have shown a relationship between childhood asthma and hyperbilirubinemia and/or phototherapy. We aimed to investigate the relationship between neonatal jaundice and the incidence of wheezing and other respiratory tract diseases in the first year. Material and Method: Term newborns (n=254) who were admitted to the primary NICU between January 2017 and December 2018 due to the indirect hyperbilirubinemia also who received phototherapy, and healthy newborns (n=254) who were without anomalies and hyperbilirubinemia as control group were included in the study. Infants in both groups were evaluated for 12 months in terms of respiratory diseases and wheezing attacks. The clinical and demographic data obtained from patient records and the type/frequency of respiratory diseases were confirmed by calling the families. Results: Of the patient’s group, 142 (55.9%) were male, the mean birth week was 38.07±1.57 and 158 (62.2%) were delivered by cesarean section. The total bilirubin level was 15.26±2.66 mg/dl at hospitalization and 8.20±2.51 mg/dL at discharge. The mean phototherapy time was 2.91±1.83 days. In the one-year follow-up, of the patients, 94 (37%) had wheezing, 54 (21.2%) had only upper respiratory tract infections, and 6 (2.3%) had other lower respiratory tract problems. Infants who were diagnosed as wheezy child had significantly higher phototherapy time than infants without wheezing (p

Sismanlar Eyuboglu, Tugba/0000-0001-7284-4999; Dogru, Deniz/0000-0001-9931-9473; Aslan, Ayse Tana/0000-0002-5360-8517; COBANOGLU, NAZAN/0000-0002-3686-2927; ORHAN, FAZIL/0000-0002-4850-932X WOS: 000530442700001 PubMed: 32364312 Background Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. Methods The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. Results Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. Conclusion PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.

Recently, a new form of congenital neutropenia that is caused by germline biallelic loss-of-function mutations in the SMARCD2 gene was described in four patients. Given the rarity of the condition, the clinical spectrum of the disease has remained elusive. We here report a new patient with a novel frameshift mutation and compare our patient with the previously reported SMARCD2-mutant patients, aiming to provide a more comprehensive understanding of the natural course of the disease.Clinical and laboratory findings of all reported patients were reviewed. Next-generation sequencing was performed to identify the causative genetic defect. Data on the hematopoietic stem cell transplantation including stem cell sources, conditioning regimen, engraftment, graft-versus-host disease, and infections were also collected.An 11-year-old female patient had a variety of infections including sepsis, deep tissue abscesses, otitis, pneumonia, gingivitis, and diarrhea since infancy. A novel homozygous mutation in SMARCD2 (c.93delG, p.Ala32Argfs*80) was detected. Bone marrow examination showed hypocellularity and decreased neutrophils with diminished granules and myeloid dysplasia, but no blast excess as in previously reported patients. The neutropenia was non-responsive even to higher doses of granulocyte colony-stimulating factor (G-CSF); therefore, the patient was transplanted at 10 years of age from a HLA-A allele-mismatched unrelated donor using a reduced toxicity conditioning regimen and recovered successfully. Compared with the previous four cases, our patient showed longer survival before transplantation without blastic transformation.Distinctive myeloid features and long-term follow-up including therapy options are presented for the newly described case of SMARCD2 deficiency. This disorder is apparent at infancy and requires early transplantation due to the unrelenting disease course despite conventional therapy.

Harmanci, Koray/0000-0002-8494-648X; Sismanlar Eyuboglu, Tugba/0000-0001-7284-4999; Aslan, Ayse Tana/0000-0002-5360-8517 WOS: 000535323700001 PubMed: 32453906 Background A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. Methods Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. Results Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). Conclusions The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.

Background Paracetamol, a non-steroidal anti-inflammatory drug, is commonly being used for fever and pain relief worldwide. The aim of this study was to evaluate children with a suspected history of paracetamol hypersensitivity. Methods Sixty patients who were referred to our clinic in between January 2015 and December 2018 with a suspected history of paracetamol hypersensitivity were included. Reactions were classified according to the European Network for Drug Allergy (ENDA)/Global Allergy and Asthma European Network classification and European Academy of Allergy and Clinical Immunology (EAACI)/ENDA Position Paper. Diagnoses were confirmed by skin tests and oral challenge tests (OCTs). In those with verified paracetamol hypersensitivity, an OCT with a strong COX-1 inhibitor was performed to classify the type of the reaction to refer as either selective or cross-intolerance hypersensitivity. A subsequent OCT with a selective COX-2 inhibitor was performed in those cross-intolerant patients to find out a safe alternative drug. Results Sixty OCTs with paracetamol were performed to patients with a median age of 8.5 years, and hypersensitivity to paracetamol was verified in 8 patients. Four children were classified as selective responders, and 3 were classified as cross-intolerant after OCT with a COX-1 inhibitor. Overall, skin test positivity for paracetamol was detected in only one patient, in whom OCT with paracetamol was negative. In all 3 cross-intolerant patients, a safe alternative non-steroidal anti-inflammatory drug was identified after an OCT with a selective COX-2 inhibitor. Conclusion OCT stands as the gold-standard procedure in verifying the diagnosis of patients with paracetamol-induced drug hypersensitivity, as well as, in defining the type of reactions and finding out safe alternative drugs.

Introduction Chronic spontaneous urticaria (CSU) is characterized by the onset of symptoms which are not induced by specific triggers, but are rather spontaneous. A considerable number of patients report that foods or food additives might be responsible for their chronic urticaria. Aim To determine the prevalence of sensitization to food additives in children with CSU using atopy patch tests (ATP). Material and methods Atopy patch tests for 23 different food additives were applied to 120 children with CSU and 61 healthy controls. Results Seventeen (14.1%) children with CSU were sensitized with food additives. None of the control group had positive APT. Azorubine and Cochineal red were the food additives detected with the highest sensitization rates (5.8% (n = 7) and 6.7% (n = 8), respectively). Conclusions There can be an association between food additives and CSU. APT tests may be a helpful tool in the assessment and management of CSU so that easier to follow diets and effective treatments can be offered to families.

Background Beekeepers and their families are at an increased risk of life-threatening anaphylaxis due to recurrent bee-sting exposures. Objective The aim of this study is to evaluate the demographic features, previous history of anaphylaxis among beekeepers and their family members, and their knowledge about the symptoms and management of anaphylaxis. Methods A standardized questionnaire was administered to beekeepers during the 6th International Beekeeping and Pine Honey Congress held in 2018, in Mugla, Turkey. Additionally, food-service staff from restaurants were surveyed as an occupational control group about their knowledge about anaphylaxis. Results Sixty-nine beekeepers (82.6% male, mean age 48.4 ± 12.0 years) and 52 restaurant staff (46.2% male, mean age 40.5 ± 10.0 years) completed the questionnaire. Awareness of the terms ‘anaphylaxis’ and ‘epinephrine auto-injector’ among the beekeepers were 55.1% and 30.4% and among the restaurant staff were 23.1% and 3.8%, respectively. Of the beekeepers, 74% were able to identify the potential symptoms of anaphylaxis among the given choices; 2.9% and 5.8% reported anaphylaxis related to bee-stings in themselves and in their family members, respectively. None of the restaurant staff had experienced or encountered anaphylaxis before but 3.8% of their family members had anaphylaxis and those reactions were induced by drugs. Conclusion It is essential that implementation of focused training programs about anaphylaxis symptoms and signs as well as practical instructions of when and how to use an epinephrine auto-injector will decrease preventable morbidities and mortalities due to bee-stings in this selected high-risk population of beekeepers and their family members, as well as other fieldworkers under risk.

Better understanding of CFTR biology has led to the development of modulator drugs. Lumacaftor/Ivacaftor (L/T) is approved for people ages ≥2 who have two copies of the F508del mutation. Tezacaftor/Ivacaftor (T/I) is approved for people ages ≥12 who have two copies of the F508del mutation and also approved for people who have a single copy of one of 26 specific mutations. Ivacaftor (I) is approved for people ages ≥1 who have a single copy of one of the mutations approved for T/I and additionally for 12 specific mutations. We aimed to find out the number of patients recorded in Cystic Fibrosis Registry of Turkey (CFRT) in 2018 who are eligible for modulator therapy. Of 1170 patients, 128 are homozygote F508del (22 mths-36 yrs), and 123 (10.51%) are aged ≥2 yrs and eligible for L/T. Among 128 patients with homozygote F508del, 48 are aged ≥12 yrs and among 42 patients (1-31 yrs) who have one or two copies of 3849+10kbC→T, A455E, D110H, D579G, F1052V, R74W, R347H, S945L, 13 are aged ≥12 yrs, and totally 61 (5.21%) patients are eligible for T/I. Finally, total 68 (5.81%) patients (1-31 yrs) have one or two copies of D110H, G178R, G1069R, G1349D, R117H and S549R mutations in addition to the mutations stated in T/I group and all of them are older than one year old and eligible for I therapy. According to the data, approximately one fifth of CF patients are eligible for modulator drug therapy. Compared to North European and North American CF patients, less Turkish patients are eligible for modulator therapies, so new treatment modalities are necessary for them.

WOS: 000486613600003, This review addresses the current strategies of inducing tolerance development in infant and childhood cow's milk protein allergy (CMPA). The change in prevention strategies for CMPA has been emphasized based on the lack of evidence to support the efficacy of food allergen avoidance in infancy and the concept of the dual-allergen-exposure hypothesis, which suggests that allergen exposure through the skin leads to sensitization, whereas early oral consumption of allergenic food protein induces oral tolerance. The new approach is based on the likelihood of early introduction of allergenic foods to the infant's diet to reduce the development of food allergies through oral tolerance induction. The latest treatment guidelines recommend the continuation of breast feeding and the elimination of cow's milk and products from the maternal diet in exclusively breast-fed infants with CMPA, the use of an extensively hydrolyzed infant formula (eHF) with proven efficacy in CMPA as the first elimination diet in formula-fed infants with CMPA and the use of amino acid-based formula (AAF) in severe cases, such as anaphylaxis, enteropathy, eosinophilic esophagitis, and food protein-induced enterocolitis syndrome (FPIES), as well as cases of multiple system involvement, multiple food allergies, and intolerance to extensively hydrolyzed formula (eHF). In conclusion, this paper presents the current knowledge on tolerance development in infants and children with CMPA to increase the awareness of the clinicians concerning the new approaches in CMPA treatment Tolerance development is considered a relatively new concept in CMPA, inducing a shift in interventions in CMPA from a passive (avoidance of responsible allergen) toward a proactive (tolerance induction) strategy., Abbot Nutrition Turkey, We thank Cagla Ayhan, MD and Prof. Sule Oktay, MD, PhD. from the KAPPA Consultancy Training Research Ltd., Istanbul, who provided editorial support funded by Abbot Nutrition Turkey. We also thank Saglik Bahcesi Design & Communication-Learning & Development-Digital Solutions, Istanbul, Turkey, for providing scientific background support and moderation for the panel meeting.

Dilek F, Ozceker D, Guler EM, Ozkaya E, Yazici M, Tamay Z, Kocyigit A, Guler N. Plasma lipoxin A4 levels in childhood chronic spontaneous urticaria. Turk J Pediatr 2018; 60: 527-534. Chronic spontaneous urticaria (CSU) is an idiopathic inflammatory disorder. Despite great research progress, the pathogenesis of the disease is still not fully understood. Lipoxins (LXs) are autacoid lipid metabolites that are the first discovered members of a new genus named called `specialized proresolving mediators`. In this study, we aimed to investigate the possible role of LXA4 in the pathogenesis of CSU. Forty-two children with CSU and 25 healthy children were enrolled in the study. The demographic and clinical features of patients were evaluated, autologous serum skin tests (ASSTs), and routine laboratory assessments were performed. Disease activity was determined using the urticaria activity score. An enzyme-linked immunosorbent assay was used to evaluate LXA4 plasma levels. The median value of plasma LXA4 was found to be 60.8 ng/ml (interquartile range, 48.1-71.8) in CSU patients and 137.4 ng/ml (121.4-150.8) in the control group. The difference between the groups was statistically significant (p < 0.001). Additionally, the median plasma LXA4 levels in the ASST-positive patients were significantly reduced compared to the ASST-negative ones (45.8 [36.7-67.6] versus 63.8 [58.3-78.9] ng/ml, respectively, p < 0.05). Our results showed that diminished LXA4 biosynthesis may be a critical part of CSU pathogenesis in children, especially in patients with an autoimmune component.

Can, Demet (Balikesir Author), Background Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017. Methods The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively. Results There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients. Conclusions Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future.