Determining the Role of Immunoglobulin Levels in Children with Febrile Seizures.

Background: Febrile seizures are the most common form of convulsive disorder in childhood. The mechanisms underlying the pathogenesis of febrile seizure remain unclear. Objective: The aim of this study was to assess the immunoglobulin (IG) sub-group levels in children with febrile seizures. Methods and Material: The patients with a diagnosis of febrile seizure with an age range of 1-7 years who attended the clinic were included in the study. Neurologically normal and age- and sex-matched children with no history of febrile seizures were considered as controls. Results: A total of 64 patients and 100 control subjects participated in this study. There were no significant inter-group differences in terms of sex and age of the participants (p >.05). There was no statistical difference between case and control groups for serum lymphocyte count, IgA, IgG, IgM, IgE levels, and anti-HB response (p >.05). Conclusion: Our study demonstrated that there is no difference in humoral immunity between children with febrile seizures and control subjects with the same age range.

Prevalence of Viruses in Acute Asthma Exacerbations in Childhood in a Hospital in West Part of Turkey.

Acute asthma exacerbations (AAE) are episodes characterized by potentially life-threatening and rapidly deteriorating asthma symptoms. Viral respiratory infections are one of the major triggers in the pathophysiology of childhood asthma exacerbations. In this study, we aimed to determine the distribution of viral agents among pediatric AAE patients. One hundred and three AAE patients, aged 5 or older, hospitalized between from February 2017 through February 2020 at Pediatric Immunology and Allergic Diseases Unit were included in this study. Fifty patients (48.5%) were female, and the mean age of the patients was 108.2 months. Viruses were detected in 58 (%56.3) of the patients, in 5 of whom more than one virus type was detected. The most commonly detected virus was human rhinovirus (n=43, 67.1%). Other types included respiratory syncytial virus (n=8; 12.5%), influenza (n=6; 9.3%), human metapneumovirus (n=4; 6.2%), adenovirus (n=1; 1.5%), enterovirus (n=1; 1.5%), and parainfluenza (n=1; 1.5%). Viral agents were detected in 29 out of the 47 patients with allergic asthma, with human rhinoviruses comprising the majority (18 patients). The mean length of hospital stay was 7.89 days. Human rhinovirus is the most common virus that triggers AAE, with similar distributions in allergic and non-allergic asthma. We found no correlation between virus type and the length of hospital stay.

Do Platelet Count and mean Platelet Volume have a Predictive Role as a Marker in Children with Atopic Dermatitis?

Background: It is known that platelets play an important role in inflammatory diseases. Atopic dermatitis (AD) is a chronic, itchy, recurrent inflammatory skin disease that affects 2%-30% of the population, especially in childhood. Aims: We investigated the role of platelet count and mean platelet volume (MPV) as biomarkers in children with AD. Methods: This cross-sectional retrospective study examined the medical reports of patients who were referred to the Pediatric Allergy and Immunology Outpatient Clinic of the Medical Faculty Hospital, Istanbul Biruni University and the Pediatric Immunology and Allergy Diseases Outpatient Clinics of the Izmir S.B.U Tepecik Training and Research Hospital, for AD. A total of 167 children with AD and 170 healthy children were included in the study. Results: Among all participants, 36.5% (n = 61) and 31.8% (n = 54) were female in the patient and control groups, respectively. The mean age was 2.8 ± 2.8 and 3.3 ± 2.5 years in the patient and control groups, respectively. MPV was statistically significantly higher in the patient group than in the control group (P =0.003). Mean platelet to neutrophil ratio and mean absolute lymphocyte count values were significantly higher in the patient group (P <.0001 for both values). However, the mean absolute neutrophil count was lower in the patient group than in the control group and it was considered statistically significant (P <.0001). Conclusion: In conclusion, we found significantly higher platelet counts in patients with AD. The decrease in the neutrophil to lymphocyte ratio rate was remarkable. However, there was no significant difference in the MPV values between the patient and control groups.

22q11.2 deletion syndrome: 20 years of experience from two pediatric immunology units and review of clues for diagnosis and disease management.

INTRODUCTION AND OBJECTIVES: The purpose of this study was to evaluate patients diagnosed with 22q11.2 deletion syndrome and determine the clues directing to diagnosis and evaluation of immunological findings for excellent management of the disease. MATERIAL AND METHODS: Thirty-three pediatric patients with 22q11.2 deletion syndrome diagnosed between 1998 and 2019 at Pediatric Immunology Division of Ege University Faculty of Medicine and SBU Izmir Dr Behcet Uz Children's Education and Research Hospital were evaluated. RESULTS: This study includes the largest case series reported from Turkey. Congenital cardiac anomalies were the most common pathology associated with the syndrome (90.9%). Hypocalcemic symptoms were observed in 13 patients (40%). Twenty-two of the 33 (66.6%) patients were diagnosed before two years of age. Autoimmune diseases, dysmorphic facial findings, recurrent infections, growth retardation, and speech impairment were other clues for diagnosis in older patients. Clinical spectrum and immunological abnormalities of this syndrome are quite variable. All T-cell subset counts were less than 5th percentile below median by age in one patient (3%) and 10 patients had normal all T-cell subset counts (30.3%). Overall, 69.6% of the patients had normal IgG, IgA, and IgM levels and two patients had panhypogammaglobulinemia. Recurrent infections were revealed in 75.7% of the patients during follow-up. CONCLUSIONS: Presence of cardiac anomaly is more helpful in the diagnosis, especially under two years of age. Patients with immunologically high or standard risk did not show any difference in terms of numbers and severity of infections and autoimmunity.

Risk Factors Influencing Tolerance and Clinical Features of Food Protein-induced Allergic Proctocolitis.

OBJECTIVE: Continued progress in our understanding of the food protein-induced allergic proctocolitis (FPIAP) will provide the development of diagnostic tests and treatments. We aimed to identify precisely the clinical features and natural course of the disease in a large group of patients. Also, we investigated the predicting risk factors for persistent course since influencing parameters has not yet been established. METHODS: Infants who were admitted with rectal bleeding and had a diagnosis of food protein-induced allergic proctocolitis in 5 different allergy or gastroenterology outpatient clinics were enrolled. Clinical features, laboratory tests, and prognosis were evaluated. Risk factors for persistent course were determined by logistic regression analyses. RESULTS: Among the 257 infants, 50.2% (n = 129) were girls and cow's milk (99.2%) was the most common trigger. Twenty-four percent of the patients had multiple food allergies and had more common antibiotic use (41.9% vs 11.8%), atopic dermatitis (21% vs 10.2%), wheezing (11.3% vs 1.5%), colic (33.8% vs 11.2%), and IgE sensitization (50% vs 13.5%) compared to the single-food allergic group (P < 0.001, P = 0.025, P = 0.003, P < 0.001, respectively). In multivariate logistic regression analysis, presence of colic (odds ratio [OR]: 5.128, 95% confidence interval [CI]: 1.926-13.655, P = 0.001), IgE sensitization (OR: 3.964, 95% CI: 1.424-11.034, P = 0.008), and having allergy to multiple foods (OR: 3.679, 95% CI: 1.278-10.593, P = 0.001] were found to be risk factors for continuing disease after 1 year of age. CONCLUSION: Although most children achieve tolerance at 1 year of age, IgE sensitization, allergy to multiple foods, and presence of colic were risk factors for persistent course and late tolerance. In this context, these children may require more close and extended follow-up.

CD137 deficiency causes immune dysregulation with predisposition to lymphomagenesis.

Dysregulated immune responses are essential underlying causes of a plethora of pathologies including cancer, autoimmunity, and immunodeficiency. We here investigated 4 patients from unrelated families presenting with immunodeficiency, autoimmunity, and malignancy. We identified 4 distinct homozygous mutations in TNFRSF9 encoding the tumor necrosis factor receptor superfamily member CD137/4-1BB, leading to reduced, or loss of, protein expression. Lymphocytic responses crucial for immune surveillance, including activation, proliferation, and differentiation, were impaired. Genetic reconstitution of CD137 reversed these defects. CD137 deficiency is a novel inborn error of human immunity characterized by lymphocytic defects with early-onset Epstein-Barr virus (EBV)-associated lymphoma. Our findings elucidate a functional role and relevance of CD137 in human immune homeostasis and antitumor responses.