Browsing by Author "Kiliç, M"

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    Traditional Uses of Medicinal Plants in Artuklu, Turkey
    Kiliç, M; Yildiz, K; Kiliç, FM
    We conducted an ethnobotanical field study of traditional medicinal plants in 91 villages of Artuklu District among four ethnic groups: Turkish, Kurdish, Arabic, and Assyrian. We interviewed 365 elderly informants and recorded traditional uses of 85 identified botanical taxa, including four endemic species previously unidentified as medicinal plants:Arum rupicolaBoiss.,Gundelia mesopotamicaFirat,Alkanna trichophilaHub.-Mor., andCrocus leichtlinii(Dewar) Bowles. We gathered data specifically on medicinal uses of these taxa to determine the relative importance of the species surveyed and calculate the informant consensus factor (FIC) in relation to medicinal plant use. The highest use values were recorded for the speciesTeucrium poliumL. (0.78),Urtica dioicaL. (0.60) andCrataegus azarolusL., (0.59), while the highest FIC was for gynecological diseases (0.93).
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    A palynological study of the genus Smyrnium (Umbelliferae) from Turkey
    Mungan, F; Yildiz, K; Minareci, E; Kiliç, M
    In this study, the pollen morphology and exine structure of six taxa of the genus Smyrnium L. (Umbelliferae) were investigated using light microscopy and scanning electron microscopy (SEM). Eight micromorphological characters (pollen shape, apocolpium, mesocolpium, polar length, equatorial width, exine thickness, colpus length and colpus width) of pollen grains of Smyrnium have been identified. The palynological observations revealed that pollen grains of all studied taxa of Smyrnium were prolate in shape and posses tricolporate aperture. Tectal surface sculpture was not a good criterion to identify particular taxa from Smyrnium. The pollen of which is characterized by rugulate, the pollen ornamentation was similar in all studied taxa. In addition the taxa could be included in sub-rectangular pollen type as classified by Cerceau - Larrival.
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    Increased vitamin D binding protein levels are associated with irritable bowel syndrome
    Börekci, E; Kiliç, M; Ozan, Z; Börekci, H; Yildirim, T; Göçmen, Y; Bas, H
    Objectives: There is no reliable and valid biomarker to identify Irritable bowel syndrome (IBS) and its subtypes. The aim of this study is to explore potential serum biomarkers that may be associated with IBS subtypes, particularly in the vitamin D pathway. Methods: The study population comprised 75 IBS patients and 79 controls. Patients divided into IBS subtypes. Routine biochemical parameters, 25-OH-vitamin D, vitamin D binding protein (VDBP) and vitamin D receptor (VDR) serum levels were compared between IBS subtypes and controls. Factors related to IBS subtypes were examined by multivariate logistic regression analysis. Results: Vitamin D levels were lower; VDBP and VDR were higher in all IBS patients than in controls (p<0.001; 0.047 and 0.029, respectively). According to logistic regression analysis, VDBP was a disease-related parameter as much as vitamin D in all IBS subtypes. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were higher especially in diarrhea-dominant IBS (IBS-D) (p=0.041; 0.046) and vitamin B12 were significantly lower in constipation-dominant IBS (IBS-C) (p=0.001). Conclusions: Increased VDBP levels were associated with all IBS subtypes. Patients, especially in IBS-D, had higher serum levels of VDBP, CRP and ESR. Vitamin B12 deficiency, which we consider as a result of the disease, was more common in IBS-C.
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    The increase of neuropathic symptoms in diabetic patients is related with osteopenia
    Börekci, E; Kara, A; Kiliç, M; Tanik, N; Ozan, ZT; Yildirim, T; Aral, Y
    Purpose To investigate the relationship between the intensity of neuropathic pain and the severity of osteopenia in type 2 diabetic patients with painful diabetic peripheral neuropathy (painful DPN). Methods In 220 patients with type 2 diabetes included in the screening, the presence of neuropathic pain was evaluated using the Douleur Neuropathique 4 Questions (DN4) scoring system. One hundred forty-five patients with painful DPN were identified and included in the study. Socio-demographic and laboratory evaluations were made and bone mineral density (BMD) of these patients was evaluated by the dual-energy x-ray absorptiometry (DEXA) method. Results There was a significant correlation between the neuropathic pain score and the total T scores of the lumbar spine and femur in patients with painful DPN. According to the regression analysis (standard coefficients), the DN4 score (0.498); the level of vitamin D (- 0.246) and the female sex (0.236) for the lumbar spine region; age (0.387); DN4 score (0.261); and vitamin D level (- 0.155) for the femur region were independently influencing factors on the development of osteoporosis. When osteoporosis (T score <= - 2.5) of the lumbar spine was analyzed by binary logistic regression, the risk of osteoporosis in women was 4.4 times higher, and the risk increased with increasing DN4 score. Conclusion The increase of neuropathic symptoms in patients with DPN is an effective and important factor in the development of diabetic osteopenia.
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    MORPHOLOGICAL, PALYNOLOGICAL AND PHYLOGENETIC RELATIONSHIPS OF GLAUCIUM MILL. IN TURKEY
    Kiliç, FM; Yildiz, K; Batir, MB; Kiliç, M; Büyük, I
    Glaucium taxa were investigated in terms of their morphological, palynological and phylogenetical characteristic. The results of this study show differences between the taxa in some of these characteristics, especially in micromorphology and formation of clades in phylogenetic trees based on the matK and ITS3-6 DNA sequence data. Based on the findings of the molecular analyses supported by morphological data (stem's trichomes), the genus Glaucium of Turkey was divided into subsections Glabrousae and Pubescentae.
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    Traditional uses of wild plants in Mardin central district and attached villages (Turkey)
    Kiliç, M; Yildiz, K; Kiliç, FM
    This research reveals traditional uses of wild plants found in Artuklu district in Mardin Province in the Southeastern Anatolia Region of Turkey. This study, conducted between 2017 and 2019, gathered information on the medicinal and other uses of plant species traditionally used in Artuklu and the local names of these plants. Face to face surveys were conducted with 183 people in 91 neighbourhoods. The ethnobotanical uses of 125 plants belonging to 35 families, which are traditionally underutilized by the local people, have been recorded. Our results showed that the highest Relative Frequency of Citation (RFC) was recorded for the species Lathyrus pseudocicera Pamp. (0.32). 1 taxon is from Stereocaulaceae of Fungi, 2 taxa are from Pteridophyta and the others 122 taxa are from Magnoliophyta. In the region, plants mostly belonging to the families Fabaceae (21 taxa), Lamiaceae (11 taxa), Asteraceae (10 taxa) and Brassicaceae (10 taxa) were determined. The recorded ethnobotanical uses are for: food (64), medicinal (40), fodder (12), children's toys (5) and various other purposes (4). Due to the fact that Turkish, Kurdish, Arabic and Assyrian people have lived together in the Artuklu district, this ethnic diversity increased the use of wild plants and added richness. Preserving the coexistence of different cultural and religious groups in the research zone is essential for the maintenance of the rich wild plant local heritage.
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    Evaluation of factors affecting bone mineral density in CF patients through CF registry of Turkey
    Soydas, SSA; Tugcu, GD; Gençoglu, MY; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Ünal, G; Yilmaz, A; Çaglar, HT; Damadoglu, E; Irmak, I; Demir, E; Öztürk, GK; Bingöl, A; Basaran, E; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Gayretli, ZG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Dogru, D
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    Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?
    Uytun, S; Cinel, G; Polat, SE; Tabakçi, SO; Kiper, N; Yalçin, E; Tural, DA; Özsezen, B; Sen, V; Sen, HS; Altintas, DU; Çokugras, H; Kilinç, AA; Baskan, AK; Yazan, H; Çollak, A; Uzuner, S; Ünal, G; Yilmaz, AI; Çaglar, HT; Damadoglu, E; Irmak, I; Demir, E; Öztürk, GK; Bingöl, A; Basaran, E; Sapan, N; Canitez, Y; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Çobanoglu, N; Özcan, G; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Emiralioglu, N; Eyüboglu, TS; Pekcan, S; Çakir, E; Özçelik, U; Dogru, D
    BackgroundCystic 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. MethodsThis 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. ResultsIn 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. ConclusionsPatients 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.
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    Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey
    Çobanoglu, N; Özçelik, U; Çakir, E; Eyüboglu, TS; Pekcan, S; Cinel, G; Yalçin, E; Kiper, N; Emiralioglu, N; Sen, V; Sen, HS; Ercan, Ö; Çokugras, H; Kilinç, AA; Al Shadfan, LM; Yazan, H; Altintas, DU; Karagöz, D; Demir, E; Öztürk, GK; Bingöl, A; Basaran, AE; Sapan, N; Çekiç, S; Celebioglu, E; Aslan, AT; Gürsoy, TR; Tugcu, G; Özdemir, A; Harmanci, K; Yildirim, GK; Köse, M; Hangül, M; Tamay, Z; Süleyman, A; Yüksel, H; Yilmaz, O; Özcan, G; Topal, E; Can, D; Ekren, PK; Çaltepe, G; Kiliç, M; Özdogan, S; Dogru, D
    Background A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. Methods Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. Results Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). Conclusions The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.
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    Evaluation of respiratory function at 6 years of age in patients with cystic fibrosis with frequent pulmonary exacerbations in the first 2 years of life
    Tabakci, SO; Cinel, G; Uytun, S; Polat, SE; Yalçin, E; Kiper, N; Erdal, MA; Sen, V; Savas, S; Çelebi, Ö; Altintas, DU; Serbes, M; Kilinç, AA; Çokugras, H; Arslan, H; Yazan, H; Kafi, HM; Çollak, A; Ünal, G; Yilmaz, AI; Çaglar, HT; Irmak, I; Damadoglu, E; Öztürk, GK; Demir, E; Basaran, E; Bingöl, A; Canitez, Y; Çekiç, S; Asfuroglu, P; Aslan, AT; Harmanci, K; Kiliç, G; Köse, M; Ersoy, A; Hangül, M; Özdemir, A; Özcan, G; Çobanoglu, N; Aydin, ZGG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pecan, S; Çakir, E; Emiralioglu, N; Özçelik, U; Ersöz, DD
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    Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data
    Onay, ZR; Eyüboglu, TS; Aslan, AT; Gürsoy, TR; Yalçin, E; Kiper, N; Emiralioglu, N; Sen, HS; Sen, V; Ünal, G; Yilmaz, AI; Kilinç, AA; Çokugras, H; Baskan, AK; Yazan, H; Çollak, A; Uzuner, S; Sasihüseyinoglu, AS; Özcan, D; Altintas, DU; Öztürk, GK; Demir, E; Bingöl, A; Basaran, E; Çekiç, S; Sapan, N; Irmak, I; Damadoglu, E; Tugcu, GD; Polat, SE; Özdemir, A; Harmanci, K; Kiliç, G; Hangül, M; Köse, M; Tamay, Z; Yüksel, H; Özcan, G; Topal, E; Can, D; Korkmaz, P; Çaltepe, G; Kiliç, M; Özdogan, S; Çakir, E; Çobanoglu, N; Pekcan, S; Cinel, G; Özçelik, U; Dogru, D
    Background. We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Turkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. Methods. All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. Results. Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. Conclusions. There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.
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    The efficacy of inhaled hypertonic saline in children with cystic fibrosis who receive daily DNase.
    Gencoglu, MY; Tugcu, GD; Soydas, SSA; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Ünal, G; Yilmaz, A; Çaglar, HT; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Bingöl, A; Basaran, E; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Gayretli, ZG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Dogru, D
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    Early Pseudomonas aeruginosa colonization in cystic fibrosis patients
    Ocak, E; Tugcu, GD; Polat, SE; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Ünal, G; Yilmaz, AI; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Basaran, AE; Bingöl, A; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Aydin, ZGG; Keskin, AÖ; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Ersöz, DD
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    One-Year Follow-Up of Cystic Fibrosis Patients Who Cannot Reach the Modulator Therapies Although Having Indications: What are Their Losses?
    Uytun, S; Polat, SE; Tabakçi, SÖ; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Çollak, A; Uzuner, S; Ünal, G; Yilmaz, A; Çelik, S; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Basaran, E; Bingöl, A; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Gayretli, ZG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Süleyman, A; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Dogru, D
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    Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study
    Emiralioglu, N; Çakir, B; Sertçelik, A; Yalçin, E; Kiper, N; Sen, V; Altintas, DU; Serbes, M; Çokugras, H; Kilinç, AA; Baskan, AK; Hepkaya, E; Yazan, H; Türel, O; Kafi, HM; Yilmaz, AI; Ünal, G; Çaglar, T; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Bingöl, A; Basaran, E; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Tugcu, G; Polat, SE; Özcan, G; Gayretli, ZG; Keskin, O; Bilgic, S; Yüksel, H; Özdogan, S; Topal, E; Caltepe, G; Can, D; Ekren, PK; Kiliç, M; Süleyman, A; Eyüboglu, TS; Cinel, G; Pekcan, S; Çobanoglu, N; Çakir, E; Özcelik, U; Dogru, D
    Background: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. Methods: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. Results: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 >= 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 >= 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. Conclusions: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.
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    The reliability and validity of Turkish version of Childhood Asthma Control Test
    Sekerel, BE; Soyer, OU; Keskin, O; Uzuner, N; Yazicioglu, M; Kiliç, M; Artaç, H; Ozmen, S; Can, D; Zeyrek, D; Cokugras, H; Canitez, Y; Aydogan, M; Kuyucu, S; Inal, A; Gurkan, F; Orhan, F; Yilmaz, O; Boz, AB; Tahan, F; Cevit, O
    Introduction The reliability and validity of Turkish version of Childhood Asthma Control Test (C-ACT). Purpose The management of asthma is an important as well as difficult issue of physician's daily practice particularly in busy clinical settings. C-ACT was created to identify asthma control levels in children aged 4-11 years. Our aim was to evaluate the reliability, validity and responsiveness of C-ACT in a Turkish sample of children with asthma. Method In this multicenter study, 368 children were enrolled. C-ACT was completed every month by parents and patients who were evaluated in 3 visits within 2 month intervals. At each visit, physicians interpret the control level and decided for the treatment step as established in GINA guidelines. Results The internal consistency reliability of the Turkish version of C-ACT (C-ACT1 to C-ACT5) was found to be 0.82, 0.83, 0.82, 0.82 and 0.80, respectively (reliability statistics, Cronbach's alpha). Test-retest reliability was 0.71. There was significant correlation between C-ACT and physician's assessment of asthma control at visit 1 (r = 0.65, P < 0.001). Conclusions Turkish version of C-ACT is an accurate and reliable tool to evaluate asthma control in children aged 4-11 years. Its widespread use may facilitate appropriate assessment of asthma control and may lead to decrease the number of uncontrolled patients.
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    Anaphylaxis: Turkish National Guideline 2018
    Orhan, F; Civelek, E; Sahiner, ÜM; Arga, M; Can, D; Çalikaner, AZ; Çetinkaya, F; Ercan Sariçoban, H; Erkoçoglu, M; Gelincik, A; Güner, SN; Igde, M; Kartal, Ö; Kiliç, M; Kocabas, CN; Musabak, UH; Topal, E; Toyran, M; Tamay, ZÜ; Yilmaz, Ö
    Anaphylaxis is an acute and potentially fatal systemic reaction that sould be recognized and managed by all helthcare professionals. This is the first national guideline of Turkish National Society of Allergy and Clinical Immunology on diagnosis and management of anaphylaxis. The guideline is prepared by Anaphylaxis Interest Group with the incorporation of 20 allergy/immunology specialists and the epidemiology, etiology, pathophysiology, symptoms and signs, diagnosis/differentials, and acute and long-term management of anaphylaxis are reviewed in detail.
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    Eligibility of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies: cohort of cystic fibrosis registry of Türkiye
    Erdal, MA; Büyüksahin, HN; Sen, V; Kilinç, AA; Çokugras, H; Dogan, G; Yilmaz, AI; Ünal, G; Serbes, M; Altintas, DU; Arik, E; Keskin,Ö; Özaslan, MM; Karcioglu, O; Köse, M; Basaran, AE; Çakir, EP; Canitez, Y; Özdemir, A; Harmanci, K; Uytun, S; Polat, SE; Hangül, M; Yüksel, H; Özcan, G; Korkmaz, P; Kiliç, M; Aydin, ZGG; Çaltepe, G; Can, D; Dogru, S; Öztürk, GK; Süleyman, A; Topal, E; Özsezen, B; Hizal, M; Demirdögen, E; Ogun, H; Börekçi, S; Yazan, H; Sen, HS; Demir, AD; Çakir, E; Eyüboglu, TS; Emiralioglu, N; Pekcan, S; Özçelik, U; Dogru, D
    Background. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) variants are essential for determining eligibility for CFTR modulator drugs (CFTRms). In contrast to Europe and the USA, the treatment eligibility profile of cystic fibrosis (CF) patients in T & uuml;rkiye is not known. In this study we aimed to determine the eligibility of CF patients in T & uuml;rkiye for the CFTRms. Methods. The Cystic Fibrosis Registry of T & uuml;rkiye (CFrT) data was used to determine the age of patients in the year 2021 and the genetic variants they were carrying. Age- and CFTR-variant appropriate modulator therapies were determined using the Vertex (R) algorithm. Results. Among a total of 1930 registered patients, CTFR gene analysis was performed on a total of 1841 (95.4%) patients. Mutations were detected in one allele in 10.7% (198 patients), and in both alleles in 79% (1455 patients) of patients. A total of 855 patients (51.7% for whom at least 1 mutation was detected) were eligible for the drugs. The most appropriate drug among genotyped patients was found to be elexacaftor/tezacaftor/ivacaftor for 486 patients (26.4%), followed by ivacaftor for 327 patients (17.7%) and lumacaftor/ivacaftor for 42 patients (2%). Conclusions. Only half of patients registered in CFrT were eligible for CFTRms, which is a significant difference from the CFTR variant profile seen in USA and Europe. However, access to treatment is hampered for some patients whose genes are not analysed. Further studies in CF populations, where rare mutations are relatively more common, will contribute to the field of CFTR modulator treatments for such rare mutations.
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    Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators
    Büyüksahin, HN; Emiralioglu, N; Yalçin, E; Sen, V; Sen, HS; Arslan, H; Baskan, AK; Çakir, FB; Koray, CF; Yilmaz, AI; Ercan, F; Altintas, DU; Serbes, M; Keskin, Ö; Arik, E; Gülen, F; Barlik, M; Karcioglu, O; Damadoglu, E; Köse, M; Ersoy, A; Bingöl, A; Basaran, E; Çakir, EP; Aslan, AT; Canitez, Y; Korkmaz, M; Özdemir, A; Harmanci, K; Soydas, SS; Hangül, M; Yüksel, H; Özcan, G; Korkmaz, P; Kiliç, M; Aydin, ZGG; Çaltepe, G; Can, D; Dogru, S; Öztürk, GK; Süleyman, A; Topal, E; Özsezen, B; Hizal, M; Demirdögen, E; Ogun, H; Börekçi, S; Yazan, H; Çakir, E; Eyüboglu, TS; Çobanoglu, N; Cinel, G; Pekcan, S; Özçelik, U; Dogru, D
    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 (R) 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.