A Successful Treatment Approach in 2 Patients With Type 1 Plasminogen Deficiency: Intratracheal Application of Fresh Frozen Plasma and Tissue Plasminogen Activator.

BACKGROUND: Respiratory system involvement is common in congenital plasminogen deficiency. Although many treatment approaches have been tried, there is still no definitive treatment for respiratory system involvement. OBSERVATIONS: We report 2 congenital plasminogen deficiency cases, who presented with severe respiratory symptoms, for whom a novel treatment modality was tried. After intravenous administration of FFP (fresh frozen plasma), tissue plasminogen activator and FFP were administered intratracheally, and respiratory system findings improved. CONCLUSIONS: Intratracheal administration of tissue plasminogen activator and FFP is an available treatment modality for patients with lung involvement. Fibrin plaques should be carefully removed and new lesion formation should be prevented.

Transepidermal water loss in allergic diseases.

Background: In recent years, the epithelial barrier hypothesis has been emphasized in the formation of allergic diseases. Transepidermal water loss (TEWL) occurs through diffusion and evaporation from the skin to the external environment. There are few studies on TEWL in allergic diseases. Objective: This study evaluated the relationship between patients with atopic diseases and healthy controls and hygiene habits in TEWL. Methods: The study was conducted on patients who were followed up for atopic disease (asthma, allergic rhinitis, immunoglobulin E mediated food allergy, and atopic dermatitis) and healthy children. TEWL measurement was in a room that was stable in terms of humidity and temperature by using a widely validated open room system. During the measurement, the participants reported their frequency of taking a shower and cleaning product use. Results: In the study group, TEWL was measured in 182 patients, and the median (min-max) TEWL was 21.3 g/hm² (7.8-101.3 g/hm²) in the disease group and 9.6 g/hm2 (3.9-30.3 g/hm²) in the control group (p < 0.001). The number of weekly baths was higher in the disease group (p < 0.001). The cutoff for atopic diseases was 13.2 g/hm² (sensitivity, 83.2%; specificity, 84.3%; p < 0.001). Conclusion: High TEWL in atopic diseases supports the epithelial barrier hypothesis associated with disease development. Further studies are necessary to determine the threshold between healthy controls and the patients in the disease group. The TEWL measurement can be an effective method to determine the risk groups. Moreover, further studies related to factors on TEWL and treatment methods to reduce this loss are necessary, too.

Does the severity of atopic dermatitis change with allergic sensitization? Is it real or a myth?

OBJECTIVE: Atopic dermatitis (AD) is a chronic inflammatory skin disease that can occur at any age. This study aimed to evaluate the impact of food allergy on disease severity as well as clinical/laboratory findings in children with AD. METHODS: Clinical and laboratory data of AD patients evaluated for food allergy between January 2021 and December 2022 were examined retrospectively. RESULTS: Of the 52 patients evaluated, 32 (61.5%) were males, with a median age of 6 months (2-118 months). Among them, 26 (50%) had food allergies (FA) and five (9.6%) had inhalant allergen sensitivity. No significant difference in AD severity was observed between patients with and without FA. However, the FA group showed higher serum lactate dehydrogenase (LDH) levels (343.3 ± 81.5 U/L vs 297.7 ± 77.4 U/L; P = 0.011) and lower red cell distribution width (RDW). Inhalant allergen sensitivity was associated with higher AD severity. CONCLUSION: While guidelines recommend investigating food allergies in moderate to severe AD, this study found no significant difference in the relationship between AD severity and the presence of FA. However, inhalant allergen sensitivity was linked to increased AD severity. Therefore, a comprehensive patient history should include an evaluation of food allergies in children with AD, regardless of disease severity. Elimination and provocation tests related to suspected food items should be performed, and allergenic foods should be removed from the diet if they are found to contribute to the allergy.

The importance of aeroallergen sensitivity in children with cystic fibrosis.

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 expiratory 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 ≤ 0.01). However, no similar difference was found in other allergen sensitivities. MSK scores (P = 0.001) and predictive FEV1 values (P = 0.005) of 25 (49%) patients with bacterial colonization were significantly lower than those without colonization. CONCLUSION: Aeroallergen sensitivity was detected in approximately one-third of CF patients. Although it has been emphasized in studies that environmental factors may have an impact on lung functions and clinical conditions in CF, the effect of allergens other than AF sensitivity may be less important compared to other environmental factors, such as the presence of bacterial colonization.

Is There a Clinical Significance of Very Low Serum Immunoglobulin E Level?

PURPOSE: High serum immunoglobulin (Ig) E levels are associated with allergies, parasitic infections, and some immune deficiencies; however, the potential effects and clinical implications of low IgE levels on the human immune system are not well-known. This study aims to determine the disorders accompanying very low IgE levels in children and adults. METHODS: The patients whose IgE levels were determined between January 2015 and September 2020 were analyzed, and the patients with an IgE level < 2 IU/mL were included in this study. Demographic data, immunoglobulin levels, autoantibody results, and the diagnoses of the patients were noted from the electronic recording system of the hospital. RESULT: The IgE levels were measured in 34,809 patients (21,875 children, 12,934 adults), and 130 patients had IgE levels < 2 IU/mL. Fifty-seven patients were children (0.26%); 73 were adults (0.56%). There was a malignant disease in 34 (9 of them children) (26%), autoimmune diseases in 20 (3 of them children) (15.4%), and immunodeficiency in 17 (14 of them children) (13.1%) of the patients. The most common reasons were other diseases, immunodeficiency and malignancy in children, and malignancy, autoimmune disorders, and other diseases in the adults, in rank order. The IgE level did not show any correlation with the levels of other immunoglobulins. CONCLUSION: Although rare, a low IgE level has been shown to accompany malignancies, autoimmune disorders, and immune deficiencies. Patients with very low IgE levels should be carefully monitored for systemic disorders.

The comparison of physical fitness and anaerobic capacity in asthmatic and non-asthmatic children.

INTRODUCTION AND OBJECTIVE: Asthma, is the most common chronic inflammatory disease in childhood period. It can affect the daily life to an advanced level and may become vital. The purpose of this study is to compare physical fitness and anaerobic capacity in asthmatic children (AC) and non-asthmatic children (NC). MATERIALS AND METHODS: A total of 47 children participated in the study; 25 individuals with mild to moderate asthma and 22 healthy children were assessed. The assessed variables consist pulmonary function and peripheral muscle strength (PMS). Additionally, physical fitness was evaluated by using fitnessgram test battery, which includes body composition, modified shuttle walk test (MSWT), curl-up test, push-up test, and sit and reach test variables. Anaerobic capacity was measured with wingate anaerobic capacity test (WAnT) and counter-movement jump (CMJ) using a tri-axial accelerometer. RESULTS: FEV1/FVC ratio (p = 0.01), MSWT (p = 0.001), push-up test (p = 0.01), and WAnT peak power (p = 0.05) were measured significantly to be found reduced in AC compared with that of NC. Between the two groups, PMS, curl-up test, sit and reach test, and CMJ were not significantly different (p = 0.05). High to moderate positive correlation was found among WAnT, CMJ parameters and FEV1, fat-free body mass (FFM), dominant handgrip, and quadriceps strengths (p = 0.05). CONCLUSIONS: Physical fitness level and anaerobic capacity were lower in AC compared with that of NC. Physical fitness parameters and anaerobic exercise capacity should be evaluated on the physiotherapy and rehabilitation program in AC.

Allergic bronchopulmonary Aspergillosis in children.

BACKGROUND: Allergic bronchopulmonary aspergillus (ABPA) is a lung disease caused by hypersensitivity from Aspergillus fumigatus. Diagnostic criteria, staging systems and treatment methods for ABPA disease have been reported in studies evaluating populations, the majority of which are adult patients. Our study aimed to discuss the use of ABPA diagnostic criteria in children, the success of other alternative regimens to oral corticosteroids in the treatment of ABPA, and the changes that occur during treatment, in the light of the literature. METHODS: Between January 2017 and 2020, patients diagnosed with ABPA at the Dokuz Eylül University Child Allergy and Immunology clinic were identified; demographic characteristics, clinical and laboratory findings, diagnostic scores and stages, and treatment protocols were analyzed retrospectively. RESULTS: The mean age of patients diagnosed with ABPA was 14.33 ± 1.96. At the time of ABPA diagnosis, the median total IgE level was 1033 IU/mL (1004-6129), and the median AF specific IgE was 10.64 (2.59-49.70) kU/L. Bronchiectasis was detected in HRCT of 5 cases. We detected significant improvement in spirometric analysis with omalizumab treatment in our patient with steroid-related complications. DISCUSSION: Today, although risk factors have been investigated for ABPA, it has not been revealed clearly. Both diagnostic criteria and treatment regimens have been described in research studies, mostly adults. In pediatric patients; clarification of diagnosis and treatment algorithms is necessary to prevent irreversible lung tissue damage and possible drug side effects.

Is There an Optimal Training Interval to Improve the Correct Use of Adrenaline Auto-Injectors?

BACKGROUND: Accurate use of adrenaline auto-injectors (AAIs) for anaphylaxis is critical to decrease mortality and morbidity. OBJECTIVE: In this study, we aimed to assess user knowledge of AAIs and evaluate the factors that affect their correct use. METHODS: The study involved caregivers of pediatric patients diagnosed with anaphylaxis who were trained with trainer injectors up to 24 months ago. The demographics of the caregivers, anaphylaxis history of the patients, usage of AAIs in the case of anaphylaxis, and the reasons for not using AAIs in anaphylaxis were evaluated. Users were asked to demonstrate the use of Penepin® with a trainer injector. RESULTS: Fifty-nine caregivers were enrolled in the study. Forty-seven (79.7%) users stated that they always carry AAIs with them. Forty-one (69.5%) of the users demonstrated all steps of the use of AAIs. The time from the last AAI training was the most significant parameter affecting the ability to use AAIs correctly (OR 0.678, 95% CI 0.546-0.841, p < 0.0001). AAI training every 6 months results in the proper usage of AAIs, with 96% probability. Thirty (50.8%) caregivers stated that anaphylactic reactions occurred in their children after the last AAI training. Of these, 16 (53.3%) users stated that they did not use an AAI for the anaphylactic reaction. The most common reason (50%) was not carrying an AAI on their person. CONCLUSION: Training users at least every 6 months is associated with the proper application of AAIs. Although regular training increases the frequency of AAI use in anaphylaxis, awareness of carrying AAIs is the most important factor for usage of AAI in anaphylaxis.

Pulmonary Alveolar Proteinosis in Hereditary and Autoimmune Forms With 2 Cases.

Pulmonary alveolar proteinosis (PAP) is a respiratory pathology characterized by the accumulation and increase of surfactant-derived material in the lungs. In clinical practice, PAP may present as the primary form, which includes autoimmune and hereditary PAP, or as the secondary form. Diffuse alveolar radiopacities on chest x-ray and the crazy-paving pattern on high-resolution computed tomography are important, although not specific findings for PAP. Bronchoalveolar lavage biopsy is a diagnostic method, and whole-lung lavage remains the criterion standard for the treatment of PAP. Evidence is required regarding treatment with exogenous anti-granulocyte/macrophage colony-stimulating factor.Here, we present a 13-year-old male patient with hereditary PAP and a 15-year-old female patient with autoimmune PAP who presented with complaints of easy fatigability and weakness to emphasize the importance of keeping in mind PAP as a differential diagnosis in patients with respiratory failure findings.

[Evaluation of SARS-CoV-2 PCR Positive Cases in the Pediatric Emergency Department]. / Çocuk Acil Serviste SARS-CoV-2 PCR Pozitif Saptanan Olgularin Degerlendirilmesi.

In December 2019, a previously unknown type of coronavirus was detected in China and named as "severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)". The World Health Organization has named the SARS-CoV-2 related as coronavirus disease-2019 (COVID-19) and declared it as a pandemic. There is a limited data about the COVID-19 disease for the pediatric patients. In this study, it was aimed to evaluate the epidemiological, clinical, laboratory and radiologic findings, treatment and clinical outcomes of patients admitted to the pediatric emergency department with the suspicion of COVID-19. Between March 11 and June 16, 2020, patients aged between 1 month-18 years admitted to the pediatric emergency department and who have an indication for sampling for the polymerase chain reaction (PCR) method with the suspicion of COVID-19 according to the current guidelines published by the Ministry of Health were included in the study. The demographic characteristics, symptoms, durations and the history of contact with the suspected/definite COVID-19 cases were questioned in the patients with positive results. Physical examination, laboratory and imaging data of the patients were recorded. According to clinical severity, patients were divided into five groups. Treatment methods, ward/intensive care unit admission, length of stay at hospital, and prognosis were recorded. Of the 237 patients included in the study, 45 (18.9%) of the samples were positive and 192 (81.1%) were negative. There was a history of contact with COVID-19 positive case in 38 (85.6%) of COVID-19 PCR positive patients. The mean time for onset of symptoms after contact was 3.5 ± 1.7 days. Twenty-one of the patients (46.6%) were asymptomatic and the most common symptom was fever (34.1%) and cough (27.3%). Of the patients whose laboratory tests were requested, lymphopenia wasdetected in 50% and 52.3% of procalcitonin, 23.5% of C-reactive protein and 64.7% of D-dimer values were found to be high. Chest radiography was obtained from 45.4% of the patients; 90.0% were evaluated as normal, bronchovascular change, pleural effusion and consolidation were detected in one of each (5.0%) patient. Thorax computed tomography (CT) was obtained from 4 (9.0%) patients. One patient had normal CT findings, two patients had consolidation, one patient had peripheral ground-glass appearance and one patient had pleural effusion. Antibiotics were started in 38.6% of the patients and the most commonly used antibiotic was azithromycin (34.1%). Oseltamivir was started in one (2.3%) patient, and 10 (24.7%) patients were treated with hydroxychloroquine. There were no serious and critical cases according to the clinical severity. Pediatric patients constitute a small part of COVID-19 individuals in the community, and a significant part of them are asymptomatic, and patients who are symptomatic present with a mild clinic. In our study, most of the patients had a history of contact with COVID-19 positive cases, therefore, it should be questioned when evaluating a pediatric patient. There were no specific findings for COVID-19 positive patients in terms of laboratory and radiology.

Outcomes of Baked Milk and Egg Challenge in Cow's Milk and Hen's Egg Allergy: Can Tolerance Be Predicted with Allergen-Specific IgE and Prick-to-Prick Test?

BACKGROUND: Adding baked food into the diets of patients with cow's milk allergy (MA) and hen's egg allergy (EA) has several benefits. OBJECTIVE: We aimed to determine baked and unbaked food tolerance and evaluate the effectiveness of laboratory findings on the prediction of baked and unbaked food tolerance in patients with MA and EA. METHODS: Clinical outcomes of the patients with MA and EA who had been exposed to oral food challenge with baked food were retrospectively analyzed. RESULTS: Ninety-one patients were evaluated. The median age of the study group was 22 months. Forty-nine and 42 patients had IgE-mediated MA and EA, respectively. While all patients with EA tolerated baked egg, 24.5% patients with MA could not tolerate baked cow's milk (BM). In patients with MA, BM tolerance showed negative association with milk-specific IgE, skin prick test (SPT), and prick-to-prick test (PTP), and the PTP was the most significant parameter (sensitivity 83.8%, specificity 91.7% for PTP ≤7 mm). Negative association was seen between milk-specific IgE, SPT, PTP, and unbaked milk (UBM) tolerance, and PTP was the most significant parameter (sensitivity 100%, specificity 55% for PTP ≤4 mm). In patients with EA, at the end of 6 months of baked hen's egg (BE) consumption, scrambled egg tolerance showed negative association with egg white-specific IgE level, egg white SPT and PTP. Egg white PTP was the most significant parameter (sensitivity 82.4%, specificity 96.0% for PTP ≤5 mm). CONCLUSION: Specific-IgE, SPT, and PTP should be kept in mind as parameters that can be used to predict tolerance to BM and BE for patients with MA and EA.

Efficacy of allergen-blocker mechanical barrier gel on symptoms and quality of life in patients with allergic rhinitis.

PROPOSE: Allergic rhinitis (AR) is a very common, chronic and global health problem. In the last two decades, the efficiency of barrier-enforcing measures in AR has been investigated. In this study, we aimed to evaluate the effect of allergen-blocker mechanical barrier gel (MBG) (AlerjiSTOP®) treatment on symptoms and quality of life score (QoLS) in patients with seasonal and perennial allergic rhinitis. METHODS: A single-center, prospective study was conducted between January 2017 and May 2018. Patients diagnosed with allergic rhinitis with a visual analogue scale (VAS) of 5 or higher (moderate/severe) were enrolled in the study. Patients were evaluated in terms of VAS, nasal symptom score (NSS), ocular symptom score (OSS), total symptom score (TSS) and QoLS at baseline, 1 week and 1 month of MBG treatment. RESULTS: A total of 83 patients with AR were enrolled in the study. Clinical and laboratory examinations showed that 50 (60.2%) patients were mono-sensitized. Allergen-blocker mechanical barrier gel treatment was performed as monotherapy in 22 (26.5%) patients. Median VAS, NSS, OSS and TSS decreased from 7 to 4, 8 to 3, 4 to 0 and 12 to 4, respectively (p < 0.0001). Correlation analysis revealed positive correlations between lower pediatric rhinoconjunctivitis quality of life questionnaire scores for patients under 12 years of age and decrease in VAS, NSS and TSS (r = 0.380, p = 0.008; r = 0.544, p < 0.0001; r = 0.543, p < 0.0001). Positive correlations were detected between lower rhinoconjunctivitis quality of life questionnaire (self-administered) scores for patients ≥ 12 years of age and decrease in VAS, NSS, OSS and TSS (r = 0.703, p < 0.0001; r = 0.465, p = 0.005; r = 0.526, p = 0.001; r = 0.624, p < 0.0001). CONCLUSION: In conclusion, we found significant decrease in all symptom scores and improvement in QoLS of patients treated with MBG as monotherapy and combination therapy.

Local and systemic reactions to subcutaneous allergen immunotherapy: Ten years' experience in a pediatric clinic.

BACKGROUND: Local and especially systemic reactions are important problems in subcutaneous immunotherapy (SCIT). Local and systemic reactions develop in 0.7% to 4% and 0.2% of all injections, respectively. OBJECTIVE: To evaluate the frequency of and risk factors for reactions developing in pediatric patients undergoing SCIT. METHODS: Local and systemic reactions developing after 14,308 injections between 2003 and 2013 were retrospectively evaluated in the current study using the Subcutaneous Immunotherapy Systemic Reaction Grading System, as recommended by the World Allergy Organization. The type of allergic disease, allergens producing a sensitivity, allergen immunotherapy content, adjuvant content, and the effects of treatment phase on the frequency of adverse effects were investigated. RESULTS: Of 319 patients, local reactions occurred in 11.9%, wide local reactions occurred in 5%, and systemic reactions occurred in 4.7%. A local reaction was observed in 0.38% of all injections, whereas a systemic reaction was observed in 0.1% of all injections. Local reactions were most frequent in the build-up phase, and systemic reactions were most frequent in the maintenance phase (P = .01). Side reactions were more common in patients undergoing SCIT with multiple allergens (P = .002) and house dust mite (P = .001). No statistically significant difference was found between adjuvant content and adverse effect frequency (P = .32). CONCLUSIONS: The frequencies of local and wide local reactions during SCIT were lower than expected. Although systemic reactions were frequently seen, no fatal reaction was observed in the current study. House dust mite SCIT and multiple allergen use increased the risk of reaction.

Cyclosporin treatment improves skin findings in omenn syndrome.

Omenn syndrome is a combined immunodeficiency characterized by a generalized erythematous skin rash, enlarged lymph nodes, hepatosplenomegaly, severe susceptibility to infections, eosinophilia, and hyperimmunoglobulinemia E. A 3-month-old girl was admitted to our hospital with a history of recurrent sepsis. Physical examination revealed severe erythroderma, hepatosplenomegaly, lymphadenopathy, and failure to thrive. Laboratory findings revealed leukocytosis, lymphocytosis with high CD3 T-cells, a high CD4:CD8 ratio, absence of CD19 B-cells, high eosinophil count, and low immunoglobulin levels. A heterozygote RAG1 gene mutation was found. She had itchy, scaling, ichthyosiform erythroderma and protracted diarrhea. Cyclosporin treatment up to 10 mg/kg effectively resolved erythroderma and lowered total eosinophil counts, and she gained weight during treatment. Since extensive erythroderma with generalized itching causes patient discomfort in Omenn syndrome, cyclosporin treatment can be considered while waiting for treatment with hematopoietic stem cell transplantation.

Evaluation of risk factors for recurrence of cutaneous adverse reactions due to anti-seizure medications in children: A retrospective study.

Background: Cutaneous adverse reactions (CARs) are one of the most important reasons for anti-seizure medication (ASM) discontinuation in epilepsy. However, such discontinuations can cause an increase in seizures. This study investigates the risk factors for ASM-related rash recurrence in children. Methods: This retrospective case-control study consisted of the patient group with a single rash due to ASMs (group 1), the patient group with rash recurrence (group 2), and the control group. While the demographic and clinical features of group 1 and the control group were compared in terms of a single rash, group 1 and group 2 were compared for rash recurrence. Results: Group 1, group 2, and control group consisted of 112, 33, and 166 patients, respectively. Female gender was a risk factor for a single rash (P < 0.001) but not for recurrence (P = 0.439). Presence of atopic disease [odds ratio (OR): 9.5, 95% confidence interval (CI): 3.8-23.1, P < 0.001], family history of drug allergy (OR: 26.3, 95% CI: 9.6-72.1, P < 0.001), and polytherapy (OR: 23.5, 95% CI: 8.7-62.9, P < 0.001) were risk factors for rash recurrence. Aromatic nature of both the ASMs associated with the first rash (OR: 14.4, 95% CI: 3.2-63.2, P < 0.001) and rash recurrence (OR: 11.3, 95% CI: 4.6-27.5, P < 0.001) were determined as risk factors separately. Conclusion: Careful use of aromatic drugs may prevent recurrence of ASM-related CAR in children, particularly in cases of personal history of allergic disease and family history of drug allergy.

Perceptions of parents and physicians concerning the Childhood Asthma Control Test.

BACKGROUND: The Childhood Asthma Control Test (C-ACT) has been proposed to be a simple, patient-based test that is able to reflect the multidimensional nature of asthma control. In this analysis, the aim was to evaluate the perceptions of physicians and caregivers concerning C-ACT and its predictive value for future asthma-related events. METHOD: In a multicenter prospective design, 368 children aged 4-11 years with asthma who were either well- or not well-controlled were included in the study. The study participants were evaluated during three visits made at 2-month intervals and the Turkish version of C-ACT was completed each month. Parents completed questionnaires concerning their perception of asthma (before and after the study) and the C-ACT (after the study). Physicians completed a survey about their perception of a control-based approach and the C-ACT. RESULTS: The C-ACT scores increased from visit 1 to visit 3, with improvement seen in all domains of the test. At the end of the study period, the parents more strongly agreed that asthma could be controlled completely and that asthma attacks and nocturnal awakenings due to asthma were preventable (p < .05). Most of the parents reported that the C-ACT helped them to determine asthma treatment goals for their children and also that the C-ACT improved communication with their physicians. The physicians indicated that a control-centered approach was more convenient (95%) and simpler (94.5%) than a severity-centered approach and provided better disease control (93.4%). A higher C-ACT score was associated with a decreased risk of asthma attack and emergency department admittance in the 2 months following the administration of C-ACT. Conclusion. Our findings indicated that the C-ACT improved both parental outlook on asthma control and the communication between the physician and parents. There was a good correlation between the C-ACT score and the level of asthma control achieved, as described by the physician. Additionally the C-ACT score was predictive of future asthma-related events. These findings suggest that the C-ACT may have an important role in asthma management in the future.

Autoimmune/autoinflammatory syndrome induced by adjuvants after multi-component meningococcal serogroup B vaccination in a 7-year-old girl: a case report.

BACKGROUND: Vaccines, which make it possible to be protected from many life-threatening infectious diseases, have been used safely and effectively for years. Most vaccines used today contain a variety of adjuvants and exogenous proteins. Severe reactions, in addition to transient and self-limiting mild reactions, mostly caused by these components, have been reported. The effects of vaccine adjuvants on the pathogenesis of immunemediated diseases are still under investigation. The syndrome called Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been defined in the literature. CASE: We found a novel mutation of autoinflammatory diseases in the genetic analysis of our patient. The patient developed symptoms of prolonged fever, rash, arthritis and serositis after multicomponent serogroup B meningococcal (Bexsero®) vaccination, without a previously known rheumatic disease. In the presence of clinical findings in our patient, the diagnostic criteria of ASIA syndrome were met. CONCLUSION: To the best of our knowledge, this is the first case report of a patient diagnosed with the autoinflammatory disease with a novel mutation after Bexsero® vaccination. We consider that genetic examinations will be useful in patients with a systemic vaccine reaction in the presence of ASIA when diagnostic criteria are met.

Three-axis accelerometer system for comparison of gait parameters in children with cystic fibrosis and healthy peers.

BACKGROUND: Cystic Fibrosis (CF), affecting functional exercise capacity generally measured by submaximal exercise test such as 6min walk test, is a progressive, autosomal recessive and metabolic disorder. Three-axis accelerometers, which are used during gait, are an easy way to assess gait parameters in patients and healthy individuals. Gait parameters were significantly associated with clinical outcomes of COPD. However, the association between gait parameters and clinical outcomes in children with CF is unclear. RESEARCH QUESTION: Do clinical outcomes in CF have an important role in determining gait parameters?. METHODS: Twenty-one CF and 21 healthy subjects participated in this case-control study. Body composition was evaluated using Tanita-BC 418. Respiratory and knee extension muscle strengths were assessed. Functional exercise capacity was evaluated using the 6-min walk test (6MWT). Spatiotemporal gait parameters were evaluated using a validated wireless inertial sensing device (G-Sensor, BTS Bioengineering S.p.A., Italy) during the 6MWT and 7-meter gait test. RESULTS: MIP, the distance of 6MWT, and stride length were significantly lower in the CF group compare to healthy children (p<0.05). Gait speed and functional exercise capacity, cadence and functional exercise capacity, quadriceps muscle strength, FEV1, fat-free mass were found to be correlated in CF patients (p<0.05). SIGNIFICANCE: The aerobic capacity and gait parameters were affected in CF patients with mild disease severity in our study. Clinical outcomes were associated with gait parameters in CF patients. This is the first study to use the 3-axis accelerometer to evaluate functional exercise capacity and gait parameters of CF and healthy children. A three-axis accelerometer can be used to assess functional exercise capacity and gait parameters in CF patients at the clinics.

[Pneumomediastinum and subcutaneous emphysema during asthmatic attacks in three patients]. / Uç olguda astim atagi sirasinda görülen subkütan amfizem ve pnömomediastinum.

Pneumomediastinum is defined as free air or other gases contained within the mediastinum. In children it is an uncommon clinical condition with good prognosis. It most frequently occurs with exacerbations of asthma but also may occur after cough, vomiting, excessive valsalva maneuver and after the first wheezing attack as well. The first-line treatment for pneumomediastinum is to relieve the inciting factor. Otherwise, no specific therapy is recommended for uncomplicated cases, three boys, with the ages of 4, 8 and 13, presented in the emergency department, each after a few days of shortness of breath and respiratory distress. Chest radiography revealed pneumomediastinum and subcutaneous emphysema which had occured after severe asthmatic attacks. All of these patients have improved spontaneously with conservative treatment.