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Öğe Factors affecting formula compliance of infants with IgE mediated cow's milk protein allergy during the pandemic(Frontiers Media Sa, 2023) Uygun, Dilara Kocacik; Karaatmaca, Betul; Topal, Erdem; Arga, Mustafa; Sancakli, Ozlem; Ozcan, Dilek; Igde, MahirIntroductionCow's milk protein allergy (CMPA) is the most commonly encountered food allergy in the world, usually seen in infants under the age of 2 years. This study aims to determine the factors including COVID-19 affecting formula compliance of CMPA patients.MethodsThis study is a prospective, observational study based on 10 different Paediatric Allergy-Immunology clinics in Turkey. Patients aged between 6 months and 2 years, who were followed up with IgE-mediated CMPA treatment or newly diagnosed and using breast milk and/or formula were included in the study. The sociodemographic characteristics of the patients, their symptoms, the treatments they received, and the effects of the COVID-19 pandemic on adherence to formula were evaluated with a questionnaire administered to the parents.ResultsThe compliance rate for formula-based treatment was 30.8% (IQR: 28.3, SD: 21.86). The number of patients with a single and multiple food allergy was 127 (51.6%) and 71 (28.9%), respectively. Breastfeeding duration, daily amount of prescribed formula and addition of sweetener to the formula were found to reduce compliance (p = 0.010, p = 0.003, and p = 0.004, respectively). However, it was determined that the patient's height, weight, age at diagnosis, and age of formula onset did not have a significant effect on compliance.ConclusionIt was found that the duration of breastfeeding, the increase in the daily amount of formula requirement, and the addition of sweeteners had adverse effects on formula compliance. There was no significant correlation between the formula adherence of CMPA patients and the pandemic.Öğe ILC3 deficiency and generalized ILC abnormalities in DOCK8-deficient patients(Wiley, 2020) Eken, Ahmet; Cansever, Murat; Okuş, Fatma Zehra; Erdem, Şerife; Nain, Ercan; Azizoğlu, Zehra Büşra; Haliloğlu, Yeşim; Karakukcu, Musa; Özcan, Alper; Devecioğlu, Ömer; Aksu, Güzide; Arıkan Ayyıldız, Zeynep; Topal, Erdem; Karakoç Aydıner, Elif; Kıykım, Ayça; Metin, Ayşe; Erol Çipe, Funda; Kaya, Ayşenur; Artaç, Hasibe; Reisli, İsmail; Güner, Şükrü N.; Uygun, Vedat; Tezcan Karasu, Gülsün; Dönmez Altuntaş, Hamiyet; Canatan, Halit; Oukka, Mohamed; Özen, Ahmet; Chatila, Talal A.; Keleş, Sevgi; Barış, Safa; Ünal, Ekrem; Patıroğlu, TürkanBackgroundDedicator of cytokinesis 8 (DOCK8) deficiency is the main cause of the autosomal recessive hyper-IgE syndrome (HIES). We previously reported the selective loss of group 3 innate lymphoid cell (ILC) number and function in a Dock8-deficient mouse model. In this study, we sought to test whether DOCK8 is required for the function and maintenance of ILC subsets in humans. MethodsPeripheral blood ILC1-3 subsets of 16 DOCK8-deficient patients recruited at the pretransplant stage, and seven patients with autosomal dominant (AD) HIES due to STAT3 mutations, were compared with those of healthy controls or post-transplant DOCK8-deficient patients (n = 12) by flow cytometry and real-time qPCR. Sorted total ILCs from DOCK8- or STAT3-mutant patients and healthy controls were assayed for survival, apoptosis, proliferation, and activation by IL-7, IL-23, and IL-12 by cell culture, flow cytometry, and phospho-flow assays. ResultsDOCK8-deficient but not STAT3-mutant patients exhibited a profound depletion of ILC3s, and to a lesser extent ILC2s, in their peripheral blood. DOCK8-deficient ILC1-3 subsets had defective proliferation, expressed lower levels of IL-7R, responded less to IL-7, IL-12, or IL-23 cytokines, and were more prone to apoptosis compared with those of healthy controls. ConclusionDOCK8 regulates human ILC3 expansion and survival, and more globally ILC cytokine signaling and proliferation. DOCK8 deficiency leads to loss of ILC3 from peripheral blood. ILC3 deficiency may contribute to the susceptibility of DOCK8-deficient patients to infections.