Esen Demir

Oral and dental manifestations of young asthmatics related to medication, severity and duration of condition

Background: The aim of this study was to investigate the caries risk of asthmatics in relation to dental plaque indices, salivary flow rate, pH and buffer capacity, saliva composition and salivary levels of Streptococcus mutans compared with healthy subjects and also to evaluate these parameters within different groups of asthmatics according to their medication, duration and severity of the disease.

Eponym. Scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of scimitar syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of scimitar syndrome.

Association of interleukin-1beta and interleukin-1 receptor antagonist gene polymorphisms in Turkish children with atopic asthma.

Asthma is a complex genetic disease. Genetic and functional characteristics of interleukin (IL)-1 support a role as an asthma locus for the IL-1 family on chromosome 2q12-21. This study was performed to investigate the relationship between polymorphisms of IL-1beta promoter region -511C/T and IL-1 receptor antagonist (IL-1Ra) gene (IL-RN) and bronchial asthma in Turkish children. Children were divided into two groups: (1) bronchial asthma (n=328) and (2) healthy control (n=246). Polymerase chain reaction was used to resolve the IL-1beta -511C/T and the IL-1Ra intron 2 polymorphisms. Plasma IgE concentrations were measured by immunoassays, and skin-prick tests were done in children with atopic diseases. The number of genotype CC and C allele in the control groups in IL-1beta -511C/T polymorphisms increased. The number of genotype 1/1 in the asthma groups and genotypes 1/2 and 5/5 and 5 allele in the control groups in IL-1Ra intron 2 gene polymorphism increased. Serum spIgE level increased in the 2/2 genotype in the asthma groups in IL-1Ra intron 2 gene polymorphism. Based on these results, we conclude that there was an association of pediatric asthma with the IL-1beta -511C/T and IL-1Ra intron 2 gene polymorphism. Based on these findings, it has been proposed that IL-1beta -511C/T and IL-Ra intron 2 gene polymorphism are useful markers for prediction of asthma.

Childhood asthma and atmospheric conditions.

Bronchial asthma is the most common chronic respiratory illness in childhood. It is characterized by paroxysmal bronchospastic periods. There are many studies giving reasons to explain the bronchospasm periods. One of the reasons, atmospheric conditions, is effective in creating a clinical picture of asthmatic patients. In the present study, the correlation between atmospheric conditions and asthmatic symptoms in children was investigated using peak expiratory flow rate (PEFR) as the respiratory function test. Twenty‐one children with bronchial asthma were monitored in the study. They were followed as outpatients of the Ege University Medical Faculty, Department of Pediatric Allergy and Pneumotology, between November 1993 and June 1994. Atmospheric conditions were recorded from the local meteorology center. Complaints and the PEFR of children were compared with the meteorological data. Asthmatic symptoms were increased by low temperatures in all asthmatic children. An increase was detected in the extrinsic group by relative humidity and ratio of cloud, but in the intrinsic group only by relative humidity.

A multicenter survey of childhood asthma in Turkey. II: Utilization of asthma drugs, control levels and their determinants.

Many surveys worldwide have consistently demonstrated a low level of asthma control and under‐utilization of preventive asthma drugs. However, these studies have been frequently criticized for using population‐based samples, which include many patients with no or irregular follow‐ups. Our aim, in this study, was to define the extent of asthma drug utilization, control levels, and their determinants among children with asthma attending to pediatric asthma centers in Turkey. Asthmatic children (age range: 6–18 yr) with at least 1‐yr follow‐up seen at 12 asthma outpatient clinics during a 1‐month period with scheduled or unscheduled visits were included and were surveyed with a questionnaire‐guided interview. Files from the previous year were evaluated retrospectively to document control levels and their determinants. From 618 children allocated, most were mild asthmatics (85.6%). Almost 30% and 15% of children reported current use of emergency service and hospitalization, respectively; and 51.4% and 53.1% of children with persistent and intermittent disease, respectively, were on daily preventive therapy, including inhaled corticosteroids. Disease severity [odds ratio: 12.6 (95% confidence intervals: 5.3–29.8)], hospitalization within the last year [3.4 (1.4–8.2)], no use of inhaled steroids [2.9 (1.1– 7.3)], and female gender [2.3 (1.1–5.4)] were major predictors of poor asthma control as defined by their physicians. In this national pediatric asthma study, we found a low level of disease control and discrepancies between preventive drug usage and disease severity, which shows that the expectations of guidelines have not been met even in facilitated centers, thus indicating the need to revise the severity‐based approach of asthma guidelines. Efforts to implement the control‐based approach of new guidelines (Global Initiative for Asthma 2006) would be worthwhile.

MicroRNA expression profiling in children with different asthma phenotypes

An improved understanding of the molecular mechanisms in asthma through exploring the role of microRNAs may offer promise to reveal new approaches for primary prevention and identification of new therapeutic targets in childhood asthma. The primary goal of this study is to identify the microRNAs that play a role in the pathogenesis of asthma in pediatric age group. The secondary goal is to analyze these microRNAs according to the asthma phenotype, atopic status, and severity of the disease exacerbation. To our knowledge, this is the first research project in the literature which studies the relationship between microRNA expression and the severity of childhood asthma. One hundred children between 6 and 18 years old with a diagnosis of asthma, and 100 age‐matched healthy children were enrolled in this study, and the analyses of microRNA expression profiles were performed in the Medical Genetics Laboratories of Ege University between November 2009 and June 2010. The expression of 10 microRNAs were shown to be higher in patients with more severe asthma, and the expression of these microRNAs were also found to be higher in patients who present with more severe acute asthma exacerbation symptoms (P < 0.001). Also, five microRNAs were found to be expressed more than twofold in allergic patients when compared to non‐allergic participants (P <0.001). Asthma is one of the best examples of complex genetic diseases, and further studies, which will investigate the relationship between these microRNAs and their target genes, are needed to learn more about the specific roles of microRNAs in respiratory diseases. Pediatr Pulmonol. 2016;51:582–587.

Genotype-phenotype correlation in three homozygotes and nine compound heterozygotes for the cystic fibrosis mutation 2183AA→G shows a severe phenotype

Editor—Cystic fibrosis (CF) is the most common lethal childhood disorder in white populations and occurs at a frequency of about 1/2500 with regional variations. Over 1000 mutations in the CF transmembrane conductance regulator ( CFTR ) gene accounting for the disease have been identified so far and the most common gene mutation is ΔF508.1 The frameshift mutation 2183AA→G in exon 13 was first described in three Canadian CF patients2 and later was shown to have a significant frequency in patients from mid and southern Europe. The frequency among CF patients is 9.3% in north east Italy,3 2.4% in the Tyrol,4 1-2.1% in Belgium,3 1.8% in Greece,5 1% in Bavaria, Bulgaria, and France,3 and 0.4% in mid and northern Germany.6 We identified three homozygotes among 120 Turkish patients (2.5%), two born to first cousin parents, three compound heterozygotes among 185 Bulgarian patients (0.8%), and seven compound heterozygotes among 650 Spanish patients (0.5%).7 The mutation was detected by denaturing gradient gel electrophoresis or single strand conformational analysis followed by DNA sequence analysis. We report here the genotype-phenotype correlation in 12 patients with CF with the mutation 2183AA→G (three homozygous and nine compound heterozygous for 2183AA→G and other mutations). The anamnestic, clinical, and laboratory data are summarised in table 1. Pancreatic insufficiency (PI) was assessed by the fat content of stools and requirement of pancreatic enzyme replacement therapy. Gastrointestinal symptoms (GI) are abdominal cramps and …

Scimitar syndrome associated with partial anomalous pulmonary venous draining into superior vena cava.

Scimitar syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder.

FcγRIIIa‐V/F 158 polymorphism in Turkish children with asthma bronchiale and allergic rhinitis

Fc receptors (FcR) play an important role in immune regulation. This might be linked to the variability in immune response, therefore relating to the pathogenesis of atopic diseases. The aim of the present study was to evaluate the FcγRIIIa gene polymorphism in Turkish children with asthma and allergic rhinitis. The study included 364 atopic children (184 bronchial asthma, 180 allergic rhinitis) and 234 healthy subjects as the control group, aged between 5 to 16 years. Patients were recruited from outpatient clinics of allergy and general pediatric care. Plasma IgE concentrations were measured by immunoassays and skin prick test was done in children with atopic diseases. The FcγRIIIa gene polymorphism was determined using the polymerase chain reaction method. Distribution of V158V genotype was significantly different among patient groups compared to controls (for asthmatic children OR: 5.33, 95% CI: 2.80–10.23, p < 0.001; for allergic rhinitis OR: 3.25, 95% CI: 1.75–6.07, p = 0.001). Distribution of 158 V allele was significantly different among asthmatic children (OR: 2.20, 95% CI: 1.65–2.92, p < 0.001) and allergic rhinitis patients (OR: 1.77, 95% CI: 1.32–2.35, p < 0.001) compared to healthy controls. Our study shows that the V158V genotype in FcγRIIIa gene polymorphism may be a genetic risk factor for the development of atopic diseases.

Efficacy of pollen immunotherapy in seasonal allergic rhinitis

Background: The efficacy of subcutaneous pollen immunotherapy has been documented in published double‐blind, placebo‐controlled studies related to treatment of seasonal allergic rhinitis. In the present study, subjective (symptom scores) and objective (nasal peak inspiratory flow, nasal smear, nasal biopsy) parameters were used to study the efficacy of pollen immunotherapy.