Malignancies and Lymphoproliferations in Children With Primary Immune Deficiency-A Single-center Experience.

Primary immune deficiencies (PIDs) are rare genetic disorders characterized by impaired immune function, leading to frequent infections and immune dysregulation. Studies have shown that individuals with PID are at an increased risk of developing malignancies and lymphoproliferative disorders compared with the general population. In this single-center study, we aimed to analyze the occurrence of malignancies and lymphoproliferations in children diagnosed with PID. We retrospectively analyzed the medical records of 550 pediatric patients diagnosed with PIDs at our center. Among them, 17 (3,0%) patients were identified with malignancy and/or benign lymphoproliferation. Eight of the 17 patients (47.0%) had immune dysregulatory diseases, whereas ataxia-telangiectasia was the second most common PID associated with malignancy and/or benign lymphoproliferation (n = 5, 29.4%). Lymphoma was the predominant malignancy (n = 11, 64.7%), and Epstein-Barr virus was identified as the most common viral agent associated with malignancy and/or benign lymphoproliferation in patients with PID (n = 8, 47.0%). Our study highlights the association between PID and malignancies/lymphoproliferations, with immune dysregulation syndromes being the most common subclass associated with malignancies/lymphoproliferations. Early diagnosis, multidisciplinary management, and regular surveillance are crucial in improving patient outcomes and saving lives.

Children with asthma gained more weight during the Coronavirus Disease-2019 pandemic than in previous years: a case-control study.

Objective:The objective of our study to evaluate weight changes and factors influencing weight gain in children with asthma during the coronavirus disease-2019 (COVID-19) pandemic.Methods:The study included 100 children with asthma, aged 5-17, from two regions in Turkey. Face-to-face written questionnaires were used, and siblings closest in age sharing the same household were taken as controls. Only 65 of these children had healthy sibling(s), aged between 2 and 25 years. One hundred children with asthma were compared in terms of weight gain and lifestyle factors before (March 2019 to March 2020) and after the pandemic (March 2020 to March 2021). To determine whether the weight gain differed from the control group, the 65 children with asthma were compared to their siblings.Results:Survey responses indicate that children with asthma performed less physical activity, were exposed to screens more, and slept later during the pandemic period than pre-pandemic (p = 0.003, p < 0.001, and p ≤ 0.001, respectively). Children with asthma and their siblings gained more weight during the pandemic than before the pandemic (both p < 0.001). However, children with asthma gained significantly more weight than their non-asthmatic siblings during the pandemic (p = 0.011). There was no statistical relationship between weight gain and physical activity, screen time, or sleep status.Conclusions:Children with asthma gained more weight during the COVID-19 pandemic than before the pandemic. They also gained more weight than their non-asthmatic siblings during the COVID-19 pandemic.

Current Recommendations on the Management of Pediatric Asthma and Allergic Rhinitis during the COVID-19 Pandemic versus Real-Life Practice.

INTRODUCTION: Several statements and position papers on the management of childhood asthma and allergies during the COVID-19 pandemic have been published of late. The aim of this study was to evaluate the knowledge and awareness of pediatricians and family physicians regarding the management of asthma and allergic rhinitis during the pandemic according to recently published updated guidelines. METHOD: We conducted an online survey among pediatricians and family physicians in Turkey, using a questionnaire designed to evaluate 4 items: (1) the relationship between COVID-19 infection risk and pediatric asthma/allergic rhinitis and medications used in treatment; (2) the follow-up and management of asthma/allergic rhinitis according to published updated recommendations; (3) pediatricians' and family physicians' observations and perceptions of treatment compliance and the attitudes of their pediatric asthma patients; and (4) pediatricians and family physicians' attitudes to using telehealth in the follow-up and management of pediatric asthma patients during the pandemic. RESULTS: A total of 346 participants responded to the survey. The relationship between the risk of COVID-19 and asthma was known by less than 25% of the participants. More than 33% of family physicians and 20% of pediatricians were unaware that asthma medication does not lead to a susceptibility to COVID-19 infection; 55% of family physicians and 48% of pediatricians thought that patients showed better compliance with asthma controller medication; over 33% of pediatricians and approximately 50% of family physicians stated that they could not distinguish between an asthma attack and lung involvement in COVID-19 infection; of the respondents, over 75% stated that they prefer face-to-face visits with patients, even in situations that do not require a physical examination. CONCLUSION: The overall knowledge and awareness of pediatricians and, especially, family physicians regarding the management of pediatric asthma/allergic rhinitis during the pandemic is not at a satisfactory level. There is an urgent need to inform them about updated recommendations appearing in recent guidelines published by allergy organizations.

Does Drug Allergy Workup in Children Satisfy Their Parents?

INTRODUCTION: Although drug allergy workup for pediatric patients (skin and drug provocation tests [DPT]) is performed, the procedures are troublesome, painful, and time-consuming. The aim of this study was to assess parents' perception of and satisfaction with their child's drug allergy workup. Further, to evaluate parents' tendency to use the tested drug following a negative DPT and the consequences of re-exposure. METHODS: Parents of children that underwent drug allergy workup from January 2017 to August 2020 in the Pediatric Allergy Department of Trakya University Medical Faculty were included. Satisfaction levels were assessed via a telephone questionnaire (3 open-ended, 7 multiple-choice questions). Satisfaction was scored on a Likert scale (1: very unsatisfied and 5: very satisfied). RESULTS: A total of 102 parents participated the study. Fifty-two (51%) of patients were male, and median age was 6.2 years and a median time interval between admission and test appointment of 1 month; 16.7% had a positive drug allergy workup, with 88.3% reporting being satisfied (56.9%) or very satisfied (31.4%) with the workup. Satisfaction levels were inversely related to the time between admission and test appointment (rho: -0.254, p = 0.01), with 8.2% reporting they did not use the tested drug when necessary despite a negative result. The tested drug was used by 35 individuals (41.1%), 33 (94.2%) tolerating without reaction. CONCLUSION: Most parents were satisfied with the workup, but levels were inversely related to the admission and procedure time interval. Drug allergy workup should be performed as soon as possible to relieve parents' anxiety and achieve more patient satisfaction.

Think Twice before Interpreting the Skin Prick Test as Age, Body Mass Index, and Atopy Affect Reaction Time and Size.

INTRODUCTION: The skin prick test (SPT) is a reliable method to confirm sensitization in IgE-mediated allergic diseases; however, it has been reported to be affected by several personal and environmental factors. Our objective was to determine the factors affecting the skin reactivity to histamine and allergens and investigate whether it differs according to age in terms of reading time. METHODS: A total of 500 patients, aged 4 months-18 years, were enrolled in the study. Wheal and flare reaction sizes were documented as the mean of the longest and the midpoint perpendicular diameter in the 5th, 10th, 15th, and 20th min. Skin reactivity was compared between children >24 and ≤24 months of age. RESULTS: We found larger histamine and allergen wheal sizes in children >24 months than the ones ≤24 months of age (p < 0.001 and p = 0.007, respectively). The duration of maximum histamine reactivity was 15 min for children >24 months whereas 10 min for children ≤24 months of age. The number of children losing their histamine reactivity after 15 and 20 min was significantly higher in the smaller age-group. Multiple regression analysis revealed a larger histamine reactivity in children >24 months of age, having obesity, and having allergen sensitization (p = 0.002, p = 0.003, and p = 0.018, respectively). CONCLUSION: It seems more accurate to evaluate SPT after 10 min in children ≤24 months of age. Cutoff values and ideal measurement time according to individual factors such as age, body mass index, or atopy are needed.

Relationship between arginase genes polymorphisms and preschool wheezing phenotypes.

BACKGROUND: The association between arginase I (ARG1) and arginase II (ARG2) genes and asthma has been reported in previous studies, but associations between polymorphisms in ARG genes and preschool wheezing (PSW) phenotypes are still unknown. OBJECTIVE: To examine the association between genetic variation in ARG1 and ARG2 genes and PSW phenotypes. METHODS: We enrolled 83 patients and 86 healthy controls. The patient group included two subgroups: episodic wheezing (EW) (n = 42, median age 41 months) and multiple-trigger wheezing (MW) (n = 41, median age 39 months). We genotyped six single nucleotide polymorphisms (SNPs) in ARG1 and six SNPs in ARG2. Eighteen haplotypes for ARG1 and 31 haplotypes for ARG2 were constituted, and the distributions of SNPs and haplotypes in patients and controls were analyzed. RESULTS: The frequency of the homozygote cytosine-cytosine (CC) genotype of ARG1 rs2781667T>C SNP and the ARG1 haplotype 4 in the MW group was significantly higher than the EW group (p = .002; odd ratios [OR]: 5.25; confidence interval [CI]: 1.9-14.51 and p < .001; OR: 7.77; CI: 2.54-23.74, respectively). The frequency of the ARG1 haplotype 5 was significantly higher but the frequency of ARG1 haplotype 9 was significantly lower in the all patients than in the healty controls (p = .019; OR: 10.34; CI: 1.28-83.53 and p = .015; OR: 0.093; CI:0.01-0.74, respectively). The frequency of the ARG1 haplotype 2 was significantly higher in the EW group than in the MW group (p = .014; OR: 5.68; CI: 1.48-21.8). CONCLUSION: Variations in ARG1 may potentially be related to phenotypes and risk of PSW.

Residents and interns in the 3 university hospitals: their knowledge of and attitudes to drug allergy.

BACKGROUND: Previous studies revealed there are many gaps in the awareness and knowledge regarding the diagnosis and management of drug allergy (DA) among various health-care professionals. OBJECTIVE: To assess the knowledge, attitudes, and practices towards DA among residents and interns of 3 university hospitals in the region of Trakya (Thrace), Turkey. METHODS: A cross-sectional survey was conducted at university hospitals of 3 cities in the Trakya region: 1. Trakya University Medical Faculty, Edirne (n = 405), 2. Namik Kemal University Medical Faculty, Tekirdag (n = 114), and 3. Canakkale 18 Mart University Medical Faculty, Canakkale (n = 111). A Turkish questionnaire was developed based on previous published studies. RESULTS: The majority of participants (67%) had not received education on DA as a separate subject during their medical education. Less than a third (28.3%) of all respondents were satisfied with their knowledge concerning patients with clinical signs of DA. Of the 6 knowledge questions on DA, the average score for the study was 3.51 out of 6 (58.5%). Residents had a higher knowledge score (3.93 ± 1.1) compared with interns (3.05 ± 1.2) (p < 0.001). The lowest percentage of correct answers were in response to questions on laboratory confirmation of drug-related anaphylaxis (14.5%) and the possibility of penicillin toleration in patients with a history of penicillin allergy (11.8%). The majority of participants (92%) believe that medical doctors should be educated in DA. There were no significant correlations between taking DA histories and the frequency during daily practice of encountering patients. CONCLUSION: Our study revealed that DA knowledge and attitudes are not at satisfactory level among respondents, and we concluded the importance and necessity of reinforcement of DA education in pre- and postgraduate education of medical doctors.

Assessment of regular drug use and inhaler technique skills in asthmatic children.

OBJECTIVE: This study aimed to assess the regular use of long-term asthma-control medication and to determine inhaler techniques in asthmatic children. METHODS: The study was conducted on asthmatic children aged 6-18 years. Information on rescue and controller medications was given and the proper inhalation technique was demonstrated. One month later, patients and parents were asked to answer a questionnaire on drug use and to demonstrate their inhaler techniques. RESULTS: One hundred children and/or their parents were interviewed for the study. All of the patients identified long-term asthma-control medications while quick-relief asthma medications were identified by 93% of the patients. Of the patients, 34% described the dose of their quick-relief medication correctly. All steps in the inhalation technique were correctly carried out by 60.6% of patients using a metered-dose inhaler (MDI), 80% of patients using a Turbuhaler, and 58% of patients using a capsule-based dry-powder inhaler (DPI). Of the participants, 73% reported regular use of long-term asthma-control medications. While the mean age of the patients regularly using long-term asthma medications was 9.05±2.5 years, that of patients not compliant with the regular treatment was 10.29±3.26 years (p=0.04). The most common reason for irregular drug use was forgetting to take the drug. CONCLUSION: Adherence to long-term asthma-control medications tends to be better in younger patients. Since the most common cause of irregular drug use is forgetting to take the drug, repeated training is necessary to ensure asthma control and the successful treatment of asthmatic children.

Successful Desensitization of a Patient with Possible IgE-Mediated Anaphylactic Reaction to FVIII/VWF Concentrate.

Background: Type 3 von Willebrand disease (VWD) is a severe bleeding disorder with a prevalence of 1:1 million live births. There are several von Willebrand factor (VWF) replacement products used in the treatment of acute bleeding episodes or as prophylaxis. Patients who receive multiple transfusions have an increased risk of developing antibodies against these products. These antibodies can lead to life-threatening anaphylactic reactions. Previous studies have identified immune complex formation and complement activation as the trigger for anaphylaxis, rather than IgE. IgE-mediated anaphylaxis against VWF concentrates has not yet been published. Case: A 4-year-old female patient with type 3 VWD was referred to our hospital because of an anaphylactic reaction during FVIII/VWF concentrate (Immunate®) infusion. She had previously received FVIII/VWF concentrate (Haemate P®) infusions 8 times without any complications. She did not have antibodies against VWF and FVIII, and serum IgA level was normal. Since she needed factor replacement therapy as a result of a growing hematoma on her scalp, we performed skin prick and intradermal tests 2 days after the reaction. The prick test, with FVIII/VWF (Immunate), was negative, but the intradermal test was positive. We administered a 12-step desensitization protocol with FVIII/VWF concentrate (Immunate) successfully without any reactions. Conclusion: Anaphylactic reaction to factor replacement products is a major problem for patients with VWD, especially type 3 VWD requiring multiple factor infusions. We achieved a successful desensitization with FVIII/VWF concentrate in a patient who had an anaphylactic reaction during the infusion of this product. Our patient is important since she represents the first case of IgE-mediated anaphylaxis against VWF concentrate reported in the literature.

Maternal anxiety and internet-based food elimination in suspected food allergy.

BACKGROUND: Food allergy is associated with stress, anxiety, and impairment in quality of life in both children and families. This study aimed to assess the anxiety of mothers in the case of suspected food allergy in their children and the influence of Internet usage on their levels of anxiety and food elimination. METHODS: Ninety-two patients aged 0-2 years presented at a pediatric allergy department following either a physicians' presumptive diagnosis or family's suspicion of food allergy (Group 1) and 99 healthy controls (Group 2) were evaluated. The clinical diagnosis of food allergy was confirmed or excluded by oral food challenge (OFC). The validated Turkish version of Spielberger State-Trait Anxiety Inventory (STAI) was applied to the mothers of both groups on admission and to Group 1 3 months after OFC. RESULTS: The state and trait anxiety (STAI-S and STAI-T) scores were significantly higher in Group 1 than Group 2 (P < 0.001, z = 5.410, and P = 0.021, z = 2.304, respectively). Seventy-nine mothers (85.9%) had been eliminating foods on admission. Thirty-seven (40.2%) mothers had eliminated foods on their own. The performance of food allergy-related Web searches was significantly higher among mothers who eliminated foods by themselves than those who did not (P = 0.004, χ2  = 8.391, df = 1). The second STAI-S score was significantly lower among mothers whose children's food allergy diagnosis was excluded than confirmed (effect size = 1.06 (95% CI: 0.56-1.57), P = 0.001, z = 3.442). CONCLUSION: It is important to make a correct food allergy diagnosis with a detailed allergy work-up, including OFC, to avoid anxiety and not cause unnecessary food elimination.