Long-term outcome of LRBA deficiency in 76 patients after various treatment modalities as evaluated by the immune deficiency and dysregulation activity (IDDA) score

Background: Recent findings strongly support hematopoietic stem cell transplantation (HSCT) in patients with severe presentation of LPS-responsive beige-like anchor protein (LRBA) deficiency, but long-term follow-up and survival data beyond previous patient reports or meta-reviews are scarce for those patients who do not receive a transplant.Objective: This international retrospective study was conducted to elucidate the longitudinal clinical course of patients with LRBA deficiency who do and do not receive a transplant.Method: We assessed disease burden and treatment responses with a specially developed immune deficiency and dysregulation activity score, reflecting the sum and severity of organ involvement and infections, days of hospitalization, supportive care requirements, and performance indices.Results: Of 76 patients with LRBA deficiency from 29 centers (median follow-up, 10 years; range, 1-52), 24 underwent HSCT from 2005 to 2019. The overall survival rate after HSCT (median follow-up, 20 months) was 70.8% (17 of 24 patients); all deaths were due to nonspecific, early, transplant-related mortality. Currently, 82.7% of patients who did not receive a transplant (43 of 52; age range, 3-69 years) are alive. Of 17 HSCT survivors, 7 are in complete remission and 5 are in good partial remission without treatment (together, 12 of 17 [70.6%]). In contrast, only 5 of 43 patients who did not receive a transplant (11.6%) are without immunosuppression. Immune deficiency and dysregulation activity scores were significantly lower in patients who survived HSCT than in those receiving conventional treatment (P = .005) or in patients who received abatacept or sirolimus as compared with other therapies, and in patients with residual LRBA expression. Higher disease burden, longer duration before HSCT, and lung involvement were associated with poor outcome.Conclusion: The lifelong disease activity, implying a need for immunosuppression and risk of malignancy, must be weighed against the risks of HSCT.Transplantation and immunomodulatio

Linie purpurowej marchwi mające lepsze cechy technologiczne w Turcji

The purple carrot population, which is cultivated commonly in Konya region in Turkey for industrial purposes, was used. Different types of purple carrots were collected in order to have a gene pool, and they were subjected to selection according to the charac-ters studied. The experiment was carried out in a Randomized Blocks Trial Design in a 3-recurrent using 22 inbred lines of purple carrot during 2009–2012. As for average yield, Eregli 1 was the highest (11.54 t·ha-1) whereas Eregli 14 was the lowest (9.27 t·ha-1). A correlation between some physical and chemical quality characteristics was determined. In carrot samples, the amount of dry matter was 12.63–16.30%, total sugar content was 7.13–9.67 g·100 g-1, the amount of β-carotene was 117.17–249.55 μg·g-1, the amount of an-thocyanin was 272.0–596.2 μg·g-1, the amount of total phenolic 155.83–206.67 mg·10-2 g-1GAE, and antioxidant activity ranged from 31.85 to 44.20%. At the end of the study, the Eregli 9 and Eregli 10 lines were determined as hopeful lines, and Eregli 4 and Eregli 16 lines were determined as being cultivar candidate lines.Użyto populacji marchwi purpurowej, która jest powszechnie uprawiana w rejonie Konya w Turcji dla celów przemysłowych. Zebrano różne typy marchwi czerwonej dla puli genów. Poddano je selekcji ze względu na badane cechy. Doświadczenie przeprowadzono w latach 2009–2012 w układzie prób losowanych bloków w 3 powtórze-niach przy użyciu spokrewnionych linii marchwi purpurowej. Eregli 1 miał najwyższy (11,54 t·ha-1), a Eregli 14 najniższy plon (9,27 t·ha-1). Określono korelację między niektórymi cechami fizycznymi i chemicznymi. W próbkach marchwi ilość suchej masy wynosiła 12,3–16,30%, całkowita zwartość cukrów 7,13–9,67 g·100 g-1, ilość β-karotenu 117,17–249,55 μg·g-1, ilość antocyjaniny 272,0–596,2 μg·g-1, całkowita ilość fenoli 155,83–206,67 mg·10-2g-1 GAE, natomiast działanie antyoksydacyjne wynosiło od 31,85 do 44,20%. Przy końcu badania linie Eregli 9 i Eregli 10 określono jako linie dające nadzieję, natomiast Eregli 4 i Eregli 16 jako linie kandydujące na odmiany

Börülce (Vigna unguiculata (L.) Walp.) Çeşitlerinde Farklı Ekim Zamanlarının Sulu ve Kurak Koşullarda Verim ve Kalite Özelliklerine Etkisi

Bu araştırma, Isparta ekolojik koşullarında börülce yetiştiriciliğinde en uygun tohum ekim tarihi, çeşidi ile sulu ve kuru koşullardaki verim ve verim öğeleri farkının saptanması amacıyla yapılmıştır. Kuru ve sulu koşullarda üç börülce çeşidi (Akkız, Karnıkara ve Sarıgöbek), beş farklı ekim zamanı (15 Mayıs, 30 Mayıs, 15 Haziran, 30 Haziran ve 15 Temmuz) kullanılmıştır. Araştırma faktöryel düzenlemede tesadüf blokları deneme desenine göre 4 tekerrürlü ve her tekerrürde 30 bitki olacak şekilde yapılmıştır. Son ekim zamanında çeşitler vejetasyon sürelerini tamamlayamadıkları için tane hasadı yapılamamıştır. Araştırma sonucunda en yüksek dekara tane verimi (213.0 kg/da) sulu koşullarda Sarıgöbek çeşidinde 30 Mayıs ekim tarihinden elde edilmiştir. Dekara biyolojik verimde ise farklı uygulamalara göre 132.7-396.4 kg/da arasında değişim saptanmıştır. Uygulamalara göre bitkideki bakla sayısı 3.8-33.4 adet; bakladaki tane sayısı ise 5.9-11.1 adet/bakla arasında değişmiştir. Sırasıyla bakla uzunluğu, bakla eni ve 1000 tane ağırlıkları uygulamalara göre 10.97-18.47 cm, 5.05-8.78 mm, 125.54-215.25 g arasında tespit edilmiştir. Bitki gövde çapları farklı uygulamalara göre 5.9-10.8 mm arasında değişim gösterirken; bitkideki dal sayısı 6.4-11.1 adet/bitki arasında değişmiştir. Araştırmada tanedeki % protein oranı 29.32-41.79 arasında olduğu saptanmıştır

Określenie chemicznych i sensorycznych cech niektórych odmian marchwi uprawianej w Turcji

This study was conducted in Konya region where 60% of the carrot production of Turkey is realized. Eight different-colored carrot varieties were used in the study and their sensory and chemical quality characteristics were determined. It was determined that the orange-colored root varieties were preferred more than those with other colored root varieties in terms of outer and inner color, juiciness, taste and bitterness. A correlation between some sensory and chemical quality characteristics was determined. The most phenolic matter content was determined in Purple carrot (163.52 mg 10-2 g -1 GAE) and Bolero (orange) (155.90 mg 10-2 g -1 GAE) varieties. The most antioxidant activity (45.91% inhibition), dry matter content (16.77%), total sugar content (8.27 g 10-2 g -1) and β carotene (183.01 µg g-1) content were determined in the Maestro (orange) variety. It was determined that the orange colored was preferred more than the other colored varieties in terms of sensory features.Badanie przeprowadzono w rejonie Konya, gdzie produkuje się 60% tureckiej marchwi. W badaniu zastosowano osiem odmian marchwi o różnej barwie, określając ich cechy chemiczne i sensoryczne. Stwierdzono, że odmiany o pomarańczowym korzeniu są preferowane w porównaniu z odmianami o innej barwie jeśli chodzi o barwę, soczystość, smak i gorzkawy posmak. Określono korelację między niektórymi cechami chemicznymi i sensorycznymi. Największą zawartość fenoli stwierdzono w marchwi Purple (163,52 mg 10-2 g-1 GAE) i Bolero (pomarańczowa) (155,90 mg 10-2 g-1 GAE). W odmianie Maestro (pomarańczowa) stwierdzono największe działanie antyoksydacyjne (45,91% inhibicji), zawartość suchej masy (16,77%), całkowitą zawartość cukrów (8,27 g 10-2 g-1) i β karotenu (183,01µg g-1). Stwierdzono, że w kategoriach cech sensorycznych odmiany pomarańczowe były lepiej oceniane od odmian o innej barwie

The Effects of Different Mycorrhizae Species on Quality Properties in Organic Carrot Growing

Bu araştırma; farklı içerikli mikrobiyal gübrelerin, farklı doz uygulamalarının havucun kalite özellikleri üzerine etkisini araştırmak amacıyla gerçekleştirilmiştir. Denemede toplam fenolik madde, antioksidan aktivite, ? karoten, toplam şeker, kuru madde, suda çözünebilir kuru madde ve pH içerikleri incelenmiştir. Araştırma sonucunda toplam fenolik madde, antioksidan aktivite, ? karoten, toplam şeker, kuru madde, suda çözünebilir kuru madde ve pH içerikleri mikrobiyal gübre dozlarından önemli derecede etkilenmiştir. En yüksek toplam fenolik madde (85.02 mg GAE/100 g), antioksidan aktivite (%42.33), toplam şeker (7.38 g/100 g) ve suda çözünebilir kuru madde %12.54 içeriği Vitormone 150 ml uygulamasında tespit edilmiştir.? karoten içeriği 130.94a207.34 µg/gr arasında değişmiştir. Kuru madde içeriği %10.07a14.50 arasında ve pH değeri ise 6.20a6.68 arasında değişim gösterdiği belirlenmiştir. Farklı dozda mikrobiyal gübre uygulamalarının havucun kalite özellikleri üzerine pozitif yönde etki ettiği tespit edilmiştir.This study was carried out to determine effects of different dosing in different ingredient microbial fertilizer applications on the quality characteristics of carrot. In the experiment, the total phenolic content, antioxidant activity, ß-carotene, total sugar, dry matter, soluble solid content and pH contents was examined. At the end of the study, the total phenolic content, antioxidant activity, ß-carotene, total sugar, dry matter, soluble solid content and pH contents were significantly affected by varying microbial fertilizer doses. The highest total compounds of phenolic (85.02 mg GAE/100 g), antioxidant activity (42.33%), total sugar (7.38 g/100 g) and soluble solid content (12.54%) was determined in Vitormone 150 ml application. The amount of ß caroten content varied between 130.94-207.34 ng/gr. The dry matter content 10.07 to 14.50% and the pH value of the change in 6.20-6.68 were determined. The applications of different in dose microbial fertilizer have been identified as a positive impact contributed on the quality characteristics of carrot

Skład chemiczny i działanie przeciwutleniające pustynnika okazałego (Eremurus spectabilis)

Eremurus spectabilis is used as a vegetable in Turkey, especially in Eastern Anatolia region. In this study, eight E. spectabilis from different growing areas have been analyzed for its nutrition value and antioxidant properties. The results showed that there were significant differences among samples in terms of all above parameters. The mean values of the parameters investigated were 86.62–91.35% for water content, 4.78–5.15 for pH; 0.42–0.70% for acidity, and 0.61–1.11% for ash content. The antioxidant activity tests evaluated by using 2-diphenyl-1-picryhydrazyl (DPPH) free radical scavenging and β-carotene/linoleic acid assays indicated that the extracts of E. spectabilis samples had high antioxidant capacity. In the DPPH and β-carotene/linoleic acid systems, average values were 73.69 ȝg extract . ml⁻¹ and 94.56%, respectively. The average amount of total phenolics in samples was 223 mg GAE . 100 g⁻¹ FW. Protein, K, Ca, Mg, Fe and Cu contents of E. spectabilis species were found higher than in some other commonly used vegetables. The results suggest that E. spectabilis could be a valuable source of antioxidants, phenolics and minerals.Eremurus spectabilis używany jest w Turcji jako warzywo, zwłaszcza w regionie Wschodniej Anatolii. W niniejszej pracy przeanalizowano wartość odżywczą i właściwości przeciwutleniających ośmiu roślin E. spectabilis z różnych obszarów uprawowych. Wyniki wykazały, że pomiędzy próbkami wystąpiły znaczące różnice we wszystkich powyższych parametrach. Średnie wartości badanych parametrów wynosiły: 86,62–91,35% dla zawartości wody, 4,78–5,15 dla pH; 0,42–0,70% dla kwasowości oraz 0,61–1,11% dla zawartości popiołu. Badania właściwości przeciwutleniających z wykorzystaniem strącania oczyszczającego wolnych rodników 2-difenylo-1-pikryhydrazylem (DPPH) oraz próby z β-karotenem/kwasem linoleinowym wykazały, że wyciągi z E. spectabilis mają silne zdolności przeciwutleniające. W systemach DPPH i β-karotenowych/z kwasem linoleinowym wartości średnie wynosiły odpowiednio 73.69 ȝg wyciągu. ml⁻¹ i 94,56%. Średnia ogólna zawartość fenoli w próbkach wynosiła 223 mg GAE . 100 g⁻¹ św.m. Stwierdzono, że zawartość białka, K, Ca, Mg, Fe i Cu w roślinach gatunku E. spectabilis jest wyższa niż w innych powszechnie wykorzystywanych warzywach. Wyniki sugerują, że E. spectabilis może być cennym źródłem przeciwutleniaczy, fenoli i związków mineralnych