Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.

A Comprehensive Assessment of Long-Term Complications in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

INTRODUCTION: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome are rare severe hypersensitivity reactions that lead to epithelial sloughing. Studies investigating the chronic multisystem effects of these syndromes and assessing patients in terms of quality of life (QOL), depression, and anxiety in the pediatric population are limited. In this study, we aimed to investigate the long-term effects of these diseases from a multisystem perspective. METHOD: Sixteen pediatric patients diagnosed with SJS, TEN, and SJS/TEN overlap syndrome were evaluated between September 2020 and March 2021. Physical and eye examinations were performed. To evaluate QOL and psychological status, Children's Dermatology Life Quality Index (CDLQI), Screen for Child Anxiety-Related Emotional Disorders (SCARED), and Children's Depression Inventory (CDI) were conducted. The patients' general characteristics, symptoms, and examination findings at their first admission were retrospectively obtained from the hospital's electronic records. RESULTS: Nineteen percent of the patients were female (n = 3). There were 7 patients (44%) with the diagnosis of SJS, 5 patients (31%) with TEN, and 4 patients (25%) with SJS/TEN overlap. The median follow-up time of the subjects was 6.5 years. The most common sequelae in the chronic period were skin changes (n = 13, 81%). Hyperpigmentation was the most common skin change (n = 9, 56%). In the last evaluation, 9 cases had eye involvement. In two cases, eye examination was normal in the acute phase, while ocular involvement was present in the chronic period. In 4 (50%) patients, there was height and/or weight percentile loss. Three patients' SCARED scores and 2 patients' CDI scores were high. According to the CDLQI survey, SJS, TEN, or SJS/TEN overlap syndrome had a small to moderate effect on the QOL in the 43% (n = 6) of the patients. The ANA values of 3 patients (60%) were positive at the follow-up and negative at the first admission. CONCLUSION: SJS, TEN, and SJS/TEN overlap syndrome may cause sequelae even after a long time of the onset of the disease. Patients' QOL and psychological status can be affected negatively. Ocular symptoms may develop in the follow-up, even without involvement in the acute period. Patients with SJS, TEN, and SJS/TEN overlap syndrome should be followed up in the chronic period and approached multidisciplinary.

Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. METHODS: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. RESULTS: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. CONCLUSIONS: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.

The effects of health literacy on disease control in adolescents with asthma.

Objective: Increased health literacy (HL) improves the management of chronic diseases. Data on the HL levels of adolescents with asthma are limited. In this study, we aimed to investigate the HL levels of adolescents with asthma and the effect of HL levels on asthma control.Methods: Our research included 81 adolescents with asthma and 47 age and sex-matched controls. The validated version of the European Health Literacy Survey Questionnaire (HLS-EU-Q16) was utilized to estimate the participants' health literacy levels. In addition, the Asthma Control Test (ACT) was used to determine the degree of asthma control.Results: No significant difference between the asthmatic adolescents (n = 45, 55.6%) and the control group (n = 28, 59.6%) has been established in terms of the number of participants who were considered to have adequate HL (p = 0.658). The difference between the patient and control groups in health care, disease prevention, health promotion, and overall HL scores was determined non-significant. According to the ACT scores, the overall median HL score was significantly higher in patients with controlled asthma {34.4 (14.6:50)} than in those with uncontrolled asthma {32.3 (16.7:48.9)} (p = 0.037). It was determined that there was a difference in the distribution of controlled asthma, uncontrolled asthma, and controls in HL subgroups (poor, problematic-limited, sufficient, and perfect HL) (p = 0.002).Conclusion: The level of HL is associated with asthma control. A significant proportion of asthmatic adolescents who participated in our research displayed low HL scores. Further studies should be conducted to increase the HL levels of adolescents to achieve better asthma control.

The Impact of the COVID-19 Pandemic in Adolescents with Asthma.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic is affecting people at any age and there is limited information about the effect of the COVID-19 pandemic on quality of life (QoL) in adolescents with asthma. In the present study, it was aimed to assess the attitudes of adolescents with asthma toward the COVID-19 pandemic and determine the effects of the pandemic on their QoL. METHODS: In total, 125 adolescents with asthma and 98 healthy adolescents participated in the present study. The questionnaire form consisted of three parts. In the first part, all the participants were asked whether they complied with the protective measures against COVID-19. The second part included questions for measuring the participants' level of concern about COVID-19, while the third part consisted of EUROHIS-QOL 8. RESULTS: The patient and control groups were similar in terms of the female/male ratio (55/70 and 48/50, respectively) and mean participant age (14.6 ± 2 and 15.1 ± 1.65 years, respectively) (P = 0.459 and P = 0.062, respectively). The prevalence of COVID-19 in the patients (n = 2, 1.6%) was lower than that in the controls (n = 6, 6.1%); however, the difference was not statistically significant (P = 0.142). The total EUROHIS-QOL score was significantly lower in the patients (31.2 ± 6.7) than in the controls (33.7 ± 4.4) (P < 0.001). The total QoL scores of asthmatic adolescents without other allergic disease (31.4 ± 6.7) was also lower than those of the controls (33.7 ± 4.4) (P = 0.009). Treatment disruption was significantly more common in patients who received subcutaneous immunotherapy (n = 20, 48.8%) than in those who did not (n = 8, 9.5%) (P < 0.001). Moreover, the patients had lower EUROHIS-QOL scores in the overall QoL, general health, finance, and home domains. CONCLUSION: Our results indicate that the mean QoL score of asthmatic adolescents during COVID-19 pandemic is lower than in the healthy population. Disruption in their treatment was most common in patients with asthma who were receiving subcutaneous immunotherapy.

Evaluation of pulmonary findings in patients with humoral immunodeficiency.

AIM: To determine the frequency of sinopulmonary infections, detect changes in the respiratory system, and measure functional capacity of the lungs in our patients with humoral immunodeficiency. MATERIAL AND METHODS: Fifty-six patients with humoral immunodeficiency were enrolled in this study. The clinical, laboratory, and radiologic data, and pulmonary function tests of the subjects were evaluated from their file records, retrospectively. RESULTS: The distribution of our patients was as follows: 25 patients had common variable immune deficiency, three patients had X-linked agammaglobulinemia, five patients had hyper immunoglobulin M syndrome, 19 patients had deficiency of immunoglobulin G subset, and four patients had selective immunoglobulin A deficiency. The most common symptom of the patients was chronic cough (n=47, 83.9%). The most common pathologies on high-resolution computed tomography of the chest were atelectasis and bronchiectasis (27.7%). The most common pathology in pulmonary function tests was the presence of moderate obstructive patterns along with restrictive patterns (n=6,12.5%). The FEV 1, FVC, and FEF 25-75 values were significantly lower in patients with common variable immunodeficiency compared with the patients who had IgG subset deficiencies (p=0.001, p=0.01, p=0.01). Among the patients who were treated with intravenous immunoglobulin, the age at the diagnosis of immunodeficiency was higher in patients with bronchiectasis (14.2±8.4 years) compared with those without bronchiectasis (10.1±11.4 years) (p=0.04). CONCLUSION: Clinical findings are not sufficient to monitor the structural and functional changes in the respiratory system, and patients should be evaluated using high-resolution computed tomography of the chest and pulmonary function tests.

Horseshoe lung associated with scimitar syndrome.

Horseshoe lung is a rare congenital anomaly and mostly accompanied by scimitar syndrome. Most aspects of this complex anomaly can be demonstrated via multidetector CT (MDCT). We present two baby girls who had horseshoe lung associated with right lung hypoplasia and scimitar vein. The chest roentgenograms showed displacement of the heart and mediastinum to the right with smaller right lung. Echocardiography revealed dextroposition, secundum atrial septal defect and bilateral slight peripheral pulmonary stenosis in the first case and dextroposition, severe pulmonary hypertension, secundum atrial septal defect and tricuspid regurgitation in the other one. On thoracic MDCT, the right lung and pulmonary artery were hypoplastic with cardiomediastinal shift to the right. There was an abnormal right pulmonary vein draining into the inferior vena cava on the lower zone of the right lung (scimitar vein). The posterobasal portions of the both lungs were fused through a midline isthmus behind the heart.

Health-care conditions in elementary schools and teachers' knowledge of childhood asthma.

BACKGROUND: For the adequate control of asthma in school-age children, it is recommended that teachers, school health personnel and administrators should have sufficient knowledge of how to manage asthma during school hours. AIM: To investigate asthma health care in elementary schools, and teachers' knowledge of childhood asthma and its management. METHODS: The extent of knowledge of childhood asthma in 2779 teachers in 141 elementary schools (children aged 6-14, grades 1-8) in Bursa, the fourth largest city in Turkey, was evaluated. Section I comprised questions about asthma health-care in schools, Section II teachers' knowledge of the main characteristics of asthma and Section III (Likert Scale) teachers' detailed knowledge of the signs, triggering factors, treatment and general knowledge of asthma. RESULTS: The findings of Section I demonstrated that the organisation of health-care for asthma in schools was insufficient. Of the teachers questioned, 14·7% were not even aware and only 1% and 9·6% of the teachers had been made aware by school health personnel and school records, respectively, of asthmatic children. Only 27·3% of the teachers stated that they were responsible for the health of an asthmatic child. The majority of teachers (70%) said that asthmatic children could use the medication (e.g. inhalers) themselves. In Section II, there were between 44·1% and 75·5% correct answers, while in Section III this figure ranged from 3·3% to 78·4%. The correct answer rate was 60·4% for Sections II and III combined. The results of Sections II and III showed that the teachers' knowledge of asthma was poor in many respects. Teachers who stated that they had asthma or had first-degree relatives with asthma, or those with 10 or more years' experience provided significantly more correct answers in Sections II and III combined than did those without these characteristics (P<0·001). CONCLUSIONS: There is a need to improve and standardise health care for asthma (asthma management policies) in schools. The implementation of asthma education programmes for teachers and other staff responsible for pupils' health should result in better control of this common disease.

Evaluation of the patients diagnosed with Stevens Johnson syndrome and toxic epidermal necrolysis: a single center experience.

AIM: Stevens Johnson syndrome and toxic epidermal necrolysis are severe acute mucocutaneous diseases. In this study, we evaluated the clinical aspects of Steven Johnson syndrome, toxic epidermal necrolysis and Stevens-Johnson syndrome/toxic epidermal necrolysis overlap patients who admitted to our clinics in the last five years. MATERIAL AND METHODS: Eleven patients diagnosed as Stevens-Johnson syndrome, toxic epidermal necrolysis and Stevens-Johnson syndrome/toxic epidermal necrolysis overlap in Department of Pediatric Allergy in Uludag University School of Medicine were included in this study. Clinical findings, laboratory tests and response to treatments were evaluated via electronic files. RESULTS: Two of the patients had Stevens-Johnson syndrome, four had Stevens-Johnson syndrome/toxic epidermal necrolysis overlap, and five had toxic epidermal necrolysis. The median period for drug usage was 10 days (2-44 days). Herpes simpleks virus IgM antibody was detected two patients. The median healing time was 38 days 26-94 days). Maculopapular eruptions and oral mucositis were seen in all patients. Vesicul or bullae, epidermal detachment and ocular involvement in 10 of patients. Wound care, H1 antihistamine and methyl prednisolon were used in all patients, intravenous immunoglobulin were used in 7 patients and cyclosporine in 1 patient. Sequel lesions developed in 2 of the patients and there was no death. CONCLUSION: Anticonvulsants, antibiotics and non steroid anti-inflammatory drugs play a major role in the etiology of Stevens-Johnson syndrome and toxic epidermal necrolysis. Anticonvulsants are associated with severe disease. The patients with proper wound care and treatment with immunosuppressive drugs can be recovered without or with minimal sequelae.

Value of serum and bronchoalveolar fluid lavage pro- and anti-inflammatory cytokine levels for predicting bronchopulmonary dysplasia in premature infants.

OBJECTIVE: The aim of this study was to determine the value of pro- and anti-inflammatory cytokine levels in both blood and tracheal aspirate (TA) samples that were obtained within 24 h after birth for predicting bronchopulmonary dysplasia (BPD) development in premature infants. MATERIAL AND METHODS: Premature infants, who were born before 32 weeks of gestation, weighing less than 1,500 g, and admitted with respiratory distress between September 2009 and December 2010, were enrolled. Tracheal aspirate samples and serum were obtained from all infants on the first day of admittance for evaluation of pro- and anti-inflammatory cytokine levels using ELISA. RESULTS: The study included 102 premature babies of whom 31 (30%) had BPD diagnosed in the follow-up. Mild, moderate and severe BPD was diagnosed in 10 (32%), 14 (45%) and seven (23%) infants, respectively. Both serum and TA sample pro-inflammatory cytokine (TNF-α, IL-1ß, IL-6) levels were significantly higher, and anti-inflammatory cytokine (IL-10) levels were significantly lower in infants who developed BPD compared with those who had no BPD. No significant differences were detected in either serum or TA sample pro- and anti-inflammatory cytokine concentrations in preterm infants with BPD in terms of BPD severity. Cut-off values of both serum and TA sample pro- and anti-inflammatory cytokine concentrations for predicting BPD were also determined. CONCLUSION: It is suggested that higher serum and TA pro-inflammatory cytokine (TNF-α, IL-1ß, IL-6) concentrations, along with lower anti-inflammatory cytokine (IL-10) concentrations, might be used for predicting the development of BPD in premature infants with respiratory distress at birth.

Characteristics and prognosis of childhood atopic dermatitis: a multicenter study in Turkey.

BACKGROUND: Childhood atopic dermatitis (AD) is classically accepted as initial finding of atopic march; however, nonatopic cases do not follow this course. The aim of this study was to determine the characteristics and prognosis of AD in childhood in Turkey. METHODS: The study included 531 children with AD that presented to pediatric allergy departments in 11 different regions of Turkey. Age at diagnosis, total serum and inhalant-specific immunoglobulin E (IgE) levels and allergen skin prick test results were recorded retrospectively. Clinical characteristics like additional allergic diseases at presentation or during follow-up were recorded as well as duration of follow-up. RESULTS: Mean age at diagnosis was 37.8 +/- 36.2 months. Mean IgE level was 318.3 +/- 677.8 IU/ml (median 100 IU/ml). Skin prick tests yielded positive results in 47% of children. At presentation, 31.6% of children reported additional allergic disease, while 11.7% developed allergic disease during follow-up. Among all, 46.6% had additional allergic disease at any point. IgE levels were significantly higher in children with additional allergic diseases (p = 0.001). Allergen skin prick test positivity and family history of allergic diseases increased the risk of additional allergic diseases significantly (OR = 3.90, 95% CI = 2.3-6.6 and OR = 1.89, 95% CI = 1.3-2.8, respectively). CONCLUSIONS: Allergic sensitization is not present in all cases of AD. Coexistence of additional allergic diseases is not as high as expected but more common in children who have been demonstrated to have atopic sensitization with high IgE levels and allergen skin prick test positivity.

Airborne pollen in European and Asian parts of Istanbul.

Pollen concentrations in the atmosphere of Istanbul, a city located between two continents, has been monitored for 1 year as part of a larger research program. The sampling sites were located in two different continents: the Asian part (AS) and the European part (EP). The sampling was performed in AS and EP of the city by using Hirst type volumetric method, and pollen grains of 58 and 62 taxa were identified in the two parts, respectively. The pollen spectrum reflected the floristic diversity of the region. The main pollen producers at the sites were characterized by some allergenic pollen and were identified as Cupressaceae/Taxaceae, Urticaceae, Pistacia sp., Quercus sp., Platanus sp., Fraxinus sp., and Xanthium sp. These pollen types contributed to the total pollen sum with a percentage of more than 80% at both monitoring sites. The highest amount of pollen grains was recorded in April. The greatest number of species was recorded in May, when 42 types (AS) and 44 types (EP) were present.

An aerobiological study on pollen grains in the atmosphere of North-West Turkey.

A continuous aerobiological survey of the atmosphere of Bursa was carried out from 1st January 2003 to 31st December 2004 by means of the volumetric method using a Lanzoni trap. During 2 years, a total of 57,124 pollen grains/m(3), which belonged to 66 taxa and 869 unidentified pollen grains, were recorded. In the region investigated, Pinus sp., Olea sp., Platanus sp., Cupressaceae/Taxaceae, Quercus sp., Poaceae, Moraceae, Urticaceae, and Castanea sp. were responsible for the greatest amounts of pollen. During the study period, the pollen concentration reached the highest level in May. A correlation analyses was made between the daily fluctuations of the main pollen types and meteorological parameters.

Risk factors for wheezing in primary school children in Bursa, Turkey.

BACKGROUND: Wheezing is the most common symptom of childhood respiratory tract illnesses. It is important not only for its associated acute morbidity, but also for the fact that early childhood wheezing confers a high risk for asthma. Epidemiological studies from various countries show that 10-15% of children <1 year of age and 25% of children <5 years of age have wheezing-associated respiratory tract illness, and one-third of these children develop asthma later in life. METHODS: In this retrospective study, we evaluated the association between a history of wheezing and prenatal, postnatal, familial, and environmental risk factors in 858 7-year-old children, randomly selected from seven primary schools in Bursa, Turkey, by means of an easy-to-understand questionnaire form. Among these children, 12.4% had a history of early transient wheezing, 7.1% had persistent wheezing, and 7.7% had late onset wheezing; 72.8% had no wheezing symptoms and 33.3% of children who experienced wheezing during the first 3 years of life had physician-diagnosed asthma. RESULTS: Notable risk factors associated with wheezing were as follows: male gender, lower socioeconomic status, premature birth, maternal smoking during pregnancy, bottle-feeding before 2 months of age, dampness and mold at home, hospitalization due to any respiratory illness in infancy, history of croup between 6 months and 5 years of age, frequent upper respiratory infections during the first 3 years of life, allergic eczema in the child, and any allergic disease in the mother or siblings. CONCLUSION: This study shows that the high rates of reported wheezing in the 858 primary school children in Bursa are clearly attributable to important risk factors that have long been recognized and discussed by researchers worldwide, and this suggests that all efforts at primary prevention may be insufficient.

The first camps in Turkey for asthmatic children: six years' experience.

The first asthma camp in Turkey was organized for one week in Iznik in September 1996. The camps were continued annually around the same time of the year in 1997, 1998, 2000, and in the consecutive years thereafter. The camp includes educational, sports and social activities. Children's knowledge about asthma and their attitudes towards physical and social activities were evaluated by a questionnaire. Pulmonary function tests were performed on the first and last day of the camp. There was no statistically significant difference between the mean spirometric values of the first and last day of the camp in spite of a vigorous physical and social program. Children enhanced their skills and knowledge about asthma and gained self-confidence in participating in sportive and social activities during the camp program. They did not experience any emergency room visit in the following year. In conclusion, a summer camping experience is very beneficial for asthmatic children in terms of both self-education and social and physical participation.

Airborne pollen grains in Bursa, Turkey, 1999-2000,.

In this study, pollen grains were sampled by using a Lanzoni trap (Lanzoni VPPS 2000) in atmosphere of Bursa in 1999 and 2000. During two years. a total of 13,991 pollen grains/m3 which belonged to 59 taxa and unidentified pollen grains were recorded. A total of 7.768 pollen grains were identified in 1999 and a total of 6.223 in 2000. From these taxa, 36 belong to arboreal and 23 taxa to non-arboreal plants. Total pollen grains consist of 78.61% arboreal. 20.37% non-arboreal plants and 1.03% unidentified pollen grains. In the region investigated, Pinus sp., Olea sp., Platanus sp., Gramineae, Cupressaceae/Taxaceae, Quercus sp., Acer sp.. Morus sp. Xanthium sp., Castanea sp., Chenopodiaceae/Amaranthaceae, Corvlus sp., Artemisia sp., Urtica sp.and Fraxinus sp. were responsible for the greatest amounts of pollen. During the study period the pollen concentration reached its highest level in April.

Evaluation of the need for routine preoperative latex allergy tests in children.

BACKGROUND: Recently, there was a great increase in allergic reactions to latex and this brought relatively more concern to the latex allergy. In this prospective study we aimed to identify the frequency of latex allergy in preoperative patients, and tried to clarify whether it is necessary to perform latex allergy tests routinely in the preoperative period or not. METHODS: A total of 188 children, aged 1-14, who were admitted for various operations, were randomly included in this study and of them, 181 completed the study. Latex specific history was taken from all patients. Latex skin prick tests, challenge tests with latex gloves, total IgE and latex specific IgE measurements were performed. RESULTS: Of 181 children, two (1.1%) had positive latex skin prick tests. Latex challenge tests were negative in all children. Latex specific IgE was positive in 12 children (6.6%) as class II or higher, but no patient had allergic reactions in operations. History of repeated operations was a risk factor for latex sensitization. The risk was higher in the presence of both history of repeated operations and history of allergic disease. However, the risk was not higher in patients with the history of only allergic disease, compared to ones who had a history of neither repeated operations nor allergic disease. CONCLUSION: We conclude that routine preoperative latex allergy tests seem to be not necessary because of no allergic reactions during operation in spite of the sensitization of 6.6% detected by latex specific IgE. However, this should be investigated in larger studies.