Evaluation of the current disease severity scores in paediatric FMF: Is it necessary to develop a new one?

Objectives: Modified adult disease severity scoring systems are being used for childhood FMF. We aim to test the clinical consistency of two common severity scoring systems and to evaluate the correlation of scores with the type of FMF mutations in paediatric FMF patients since certain mutations are prone to severe disease.Methods. Two hundred and fifty-eight children with FMF were cross-sectionally studied. Assessment of the disease severity was performed by using the modified scoring systems of Mor et al. and Pras et al. Genetic analysis was performed using PCR and restriction endonuclease digestion methods for the presence of 15 FMF gene mutations. FMF mutations were grouped into three based on well-known genotypic-phenotypic associations. Correlation between the mutation groups and the severity scoring systems was assessed. The consistency of the severity scoring systems was evaluated.Results. The results of two scoring systems were not statistically consistent with each other (κ = 0.171). This inconsistency persisted even in a more homogeneous subgroup of patients with only homozygote mutations of M694V, M680I and M694I (κ = 0.125). There was no correlation between the mutation groups and either of the scoring systems (P = 0.002, r = 0,196 for scoring systems of Mor et al.; P = 0.009, r = 0.162 for Pras et al.).Conclusions. The inconsistency of the two scoring systems and lack of correlation between the scoring systems and mutation groups raises concerns about the reliability of these scoring systems in children. There is a need to develop a scoring system in children based on a prospective registry. © The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved

Clinical and demographic characteristics of children with familial mediterranean fever in Central Anatolia

PubMe

Behçet disease: evaluation of clinical manifestations in Turkish children

PubMe

Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever - a randomized controlled noninferiority trial

Background: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF). Methods: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage. Clinical and laboratory characteristics and medication side effects were recorded and compared between groups. The study was carried out in compliance with Good Clinical Practice and the Consolidated Standards for Reporting of Trials (CONSORT) statement. Results: A total of 92 patients were selected, and 79 patients completed the study. There were 42 patients in the once-daily dosage group and 37 in the twice-daily dosage group. The results indicated that the once-daily dosage was not inferior to the twice-daily dosage regarding decrease in attack frequency and duration as well as improvement in clinical findings and Mor severity scores. Alterations in laboratory findings indicating inflammation, such as erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A, were similar in both groups. The rates of drug side effects were similar between the once- and twice-daily dosage groups, implying comparable safety of colchicine, with the exception of diarrhea, which was slightly higher in the once-daily dosage group. Conclusions: Using colchicine with either a once- or twice-daily dosage provides similar clinical and laboratory improvements. Considering both efficacy and safety, colchicine can be prescribed with a once-daily dosage. Trial Registration ID: ClinicalTrials.gov identifier NCT02602028. Registered 5 November 2015

The measures which are to be taken against ınformel economy within the context of awareness of people’s

Farklı kavramlarla ifade edilen kayıt dışı ekonomi Türkiye’nin karşı karşıya kaldığı en önemli sorunlardan birisini oluşturmaktadır. Kayıt dışı ekonominin verdiği zararlar sayılamayacak kadar çok olsa da en önemlilerini şu şekilde saymak olanaklıdır: Kayıt dışı ekonomi, AB’ye tam üyelik yolunda engel oluşturmakta, GSYH hesaplarını bozarak ve vergi gelirlerini azaltarak kalkınmanın sağlanmasını olanaksız veya oldukça güç hale getirmektedir. Yine, kayıt dışı ekonomi, işçi, işveren ve devlet bakımından önemli sakıncalar doğurmakta, kayıt dışı ekonominin bir türünü oluşturan suç faaliyetleri ülke ve toplumu çökertme noktasına getirmekte, söz konusu ekonominin kentlerde yoğunlaşması kent istihdamı ve çevresel açılardan sorunlara yol açmaktadır. Hangi tür önlemler alınırsa alınsın kayıt dışı ekonominin zararları konunda yeterli bir toplumsal bilinç uyandırılmazsa kayıt dışı ekonomiye karşı başarı kazanabilmek olanaksız değilse bile oldukça güçtür. Okuma alışkanlığının son derece zayıf, buna karşın televizyon seyretme alışkanlığının oldukça güçlü olduğu Türkiye’de televizyonlarda izlettirilecek kamu spotlarıyla kayıt dışı ekonominin zararları konusunda halkta bilinç oluşturulması, kayıt dışı ekonomiyle mücadelede başarı için yaşamsal bir önem taşımaktadır.Described different concepts, underground economy is one of the biggest problems that Turkey faces to it. Although informal economy gives immeasurable damages, one can enumerate the most important: The informal economy creates an obstacle on the way to full membership to EU, it makes the development impossible or very difficult by reducing tax revenue and disrupting GDP accounts. Again, informal economy gives significant damages for the employee, employer, and the state. Criminal activities, which constitute a kind of informal economy, bring the country and society to the point of collapse, the concentration of the aforementioned economy in cities causes problems in terms of urban employment and environmental issues. It is thought that a significant success will be gained against the uderground economy by creating public awareness about the damages of it. Inasmuch as it does not matter what kind of measures taken against it, without arousing public conscious on the damages of underground economy; the success against underground economy is very difficult if not impossible. In Turkey, the habitual of reading books is very thin, on the other hand, the habitual of watching television is very strong so public spots on the damages of underground economy must have been spectacled on television programs for the society. Establishing public conscious by public spots against the underground economy is a life and death matter to gain victory on it

Analysis of Nurmuhammet's Baba Ruşen, Zeynel Arap ve Kıssa-yı Firgun folk stories

Genel anlamda kapsayıcı bir başlık olarak "Türk Edebiyatı"nı edebiyat disiplini alt başlıklarını da hesaba katarak incelemek, birçok şeyi dışarıda bırakmaya sebep olacaktır. Zira tarihi veya coğrafi kapsamı düşünüldüğünde bu kadar geniş bir uzamda ve böyle büyük bir kronolojide çok büyük bir kültür yaratımı önümüze çıkacaktır. Hiçbir detayın atlanmadan ve olması gerektiği gibi incelenmesi ancak tarihi veya coğrafi bölümlemelerle ve kendisini benzerlerinden ayıran spesifik özellikleriyle daha küçük "Türk Edebiyatları"nın araştırılmasıyla mümkündür. Dolayısıyla her bir Türk sahası kendisine mahsus kültür kodlarıyla oluşturduğu yaratı geleneği ile diğerlerinden genelde benzerlik gösterse de özelde ayrıdır. Bu haliyle de ayrıca incelenmesi ve araştırılması gerekir. Özbek, Kırgız, Kazak, Türkmen, Azerbaycan, İran, Kıbrıs veya Anadolu'da ortak bir "Türk Edebiyatı" oluşurken bu sayılan her saha diğer sahalardan farklı olarak kendisine ait özellikleri, kültür kodlarını o sahanın eserlerine ve o eserleri vücuda getiren sanatkârlarına yüklemiştir. Bu amaçla çalışma konuları seçilirken genelden daha özele doğru araştırma ve incelemeler aynı zamanda bütün "Türk Edebiyatı" için büyük önem arz etmektedir. Bu büyük geleneğin bir parçası olan Türkmen edebiyatı, Türk kültürü ve edebiyatı içerisinde gerek sözlü gerekse yazılı ürünleriyle önemli bir yere sahiptir. Türkmen halk hikâyeciliğinin ve hikâyelerinin Türkiye'de az tanınan bir gelenek olması ve Türkmenlerin çok mühim şairlerinden Nurmuhammet Andalıp'ın Türkiye'de fazla bilinmemesi bu çalışmanın hazırlanmasına zemin oluşturmuştur. Bu bağlamda da "Nurmuhammet Andalıp'ın Baba Ruşen, Zeynel Arap, Kıssa-yı Fırgun isimli halk hikâyelerinin incelenmesi" adlı çalışma konu olarak seçilmiştir. Çalışmada Nurmuhammet Andalıp'ın Hayatı, Edebî Kişiliği ve Eserleri, Türkmen Destancılık Geleneği ve Andalıp'ın Hikâyeleri, Andalıp'ın Eserleri Üzerine Türkiye'de Yapılan Çalışmalar kısaca tanıtılıp ve değerlendirildikten sonra Baba Ruşen, Zeynel Arap ve Kıssa-yı Fırgun Hikâyelerinin epizot yapısı ortaya çıkarılmış, bu epizotlara bağlı motifler Stith Thomopson'un "The Motif Index of Folk Literature" (Halk Edebiyatının Motif İndeksi) adlı çalışması temel alınarak incelenmiştir. Anahtar Kelimeler: halk hikâyeleri, yazarlar, gelenek, motifler, epizotlarIn general, analyzing "Turkish Literature" as an inclusive heading when sub-headings of the literary discipline would lead to the exclusion of many details, since a very comprehensive cultural heritage would be revealed, given the scale of the history and geography of this broad space and long chronology. An elaborate study, without overlooking any details, can only be possible by analyzing smaller-scaled "Turkish Literatures" based on historical or geographical classifications and that have their own distinctive qualities. Therefore, even though each location occupied by Turks resembles each other and creates a common tradition through similar, specific cultural codes; in reality, each location is different from each other. Thus, each location has to be separately analyzed and studied. Especially, the Uzbek, Kirghiz, Kazakh, Turkmen, Azerbaijani, Iranian, Cypriot and Anatolian; all together create the common heading, the "Turkish literature," yet, all such locations have their own unique properties and cultural codes, which sprung to life in their respective local works and that were reflected by the craftsmen and artists who gave life to these works. As a result, it is crucial for researches and studies to assume a deductive approach, especially at the stage of research topic selection, for the whole "Turkish Literature." The Turkmen literature, which is a part of this tradition, has a crucial place in both oral and written Turkish culture and literature. The fact that Turkmen folktales, storytelling, and Nurmuhammet Andalıp, one of the most recognized and important poets of the Turkmens are not widely known and recognized in Turkey, established the basis for this study. In this respect, the "Study of folk stories, 'Baba Ruşen,' 'Zeynel Arap,' and 'Kıssa-yı Fırgun' by Nurmuhammet Andalıp'" has been selected as the research topic for this study. In this study, the life of Nurmuhammet Andalıp, his literary identity and his works, the Turkmen storytelling tradition and stories of Andalıp, and studies on Andalıp's works that were conducted in Turkey will be shortly introduced and examined, then the episode structure of Baba Ruşen, Zeynel Arap, Kıssa-yı Fırgun will be introduced and analyzed on the basis of Stith Thomopson's "The Motif Index of Folk Literature." Keywords: folk stories, writers, tradition, motifs, episod

Investigation of the Diagnostic Value of Platelet Indices for the Diagnosis of Acute Bronchiolitis

Objective: We explored whether the platelet large cell ratio (PLCR), mean platelet volume (MPV), immature granulocyte number, and other complete blood count indices were diagnostic of acute bronchiolitis in children