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Öğe Clinical and laboratory evaluation of Turkish children with IgG subclass deficiency(Elsevier, 2023) Kocaoğlu, Mehmet; Kocaoğlu, Burcu Ezgi; Aytekin, Selma Erol; Keskin, Doğukan Mustafa; Güner, Şükrü Nail; Keleş, Sevgi; Reisli, İsmailBackground: IgG subclass deficiency is a laboratory diagnosis and becomes important with recurrent infections. This study aimed to examine the demographic, clinical, and laboratory results of pediatric cases with IgG subclass deficiency and to improve the understanding of the clinical significance of IgG subclass deficiency. Methods: In this study, the clinical and laboratory features of 111 pediatric patients, with at least one whose serum IgG subclasses was measured as lower than 2 standard deviation of healthy aged-matched control values, were evaluated. The clinical and laboratory features of the cases with isolated IgG subclass deficiency (Group 1) and those with low serum levels of any of IgG, IgA, and IgM in addition to the IgG subclass deficiency (Group 2) were compared. Results: A total of 55 (49.54%) and 56 (50.45 %) patients were included in Groups 1 and 2, respectively. Among our studied cases, 20 (18. 1%) had a history of hospitalization in the neonatal period, 61 (54.95 %) had at least one hospitalization due to infection, and 55 (49.54%) had a history of recurrent infection. The frequencies of these three conditions were statistically significantly higher in Group 2 (p < 0.05). The frequencies of infections in the last year in Groups 1 and 2 were 4.4 +/- 1.2 and 5.4 +/- 1.9, respectively (p < 0.05). As a result of recurrent infections, 43.24% (n = 48) of our patients received antibiotic prophylaxis, and 21.62% (n = 24) had immunoglobulin replacement therapy. Furthermore, the numbers of pa-tients who needed these treatments were higher in Group 2 (p < 0.05).Conclusion: In cases with IgG subclass deficiencies, concomitant main-group immunoglobulin deficiencies may increase the number and severity of infections, leading to hospitalizations, antibiotic prophylaxis, and immunoglobulin therapy. More attention should be paid to cases of immunoglobulin main-group deficiencies in the follow-up of these cases.Copyright (c) 2022, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/ by-nc-nd/4.0/).Öğe Çocukluk çağında akut lenfoblastik lösemi: 10 yıllık tek merkez deneyimimiz(Necmettin Erbakan Üniversitesi Meram Tıp Fakültesi, 2016) Aytekin, Selma Erol; Tokgöz, HüseyinSt. Jude Total therapy XIIIB, BFM 2000 ve BFM 2009 protokolü alan hastalarda hastaların demografik özelliklerini, klinik ve laboratuar bulgularını, kemik iliğinin morfolojik, sitogenetik, immunfenotipik özelliklerini, kemoterapiye yanıtlarını, yaşam sürelerini, prognoza etki eden faktörleri, relapsları ve ölüm nedenlerini değerlendirmektir. Nisan 2006 –Nisan 2016 tarihleri arasında Necmettin Erbakan Üniversitesi Meram tıp Fakültesi Çocuk Hematoloji Anabilim Dalı' nda akut lenfoblastik lösemi tanısı ile takipli, St. Jude Total Therapi, BFM 2000, BFM 2009 protokolleri ile tedavi edilen toplam 167 hastanın dosyaları geriye dönük olarak incelendi. Hastaların tama yakınının kemik iliği örnekleri sitokimyasal boyalarla değerlendirilip, flow sitometri ile immunfenotiplendirme yapılmıştır. Hastaların büyük bir kısmında sitogenetik çalışma yapılmıştır. Tedavinin 8. Gününde periferik kan yayması ve tam kan sonuçları ile blast sayısı, 15. Ve 33. Günde kemik iliği aspirasyon ve biyopsisi ile kemik iliğinin remisyon durumu ve derecesi incelenmiştir. Hastalarımızın yaşları 9 ay ile 204 ay arasında değişiyordu. Median yaş 80 aydı. Hastaların %63,5' i erkek, %36,5' i kızdı. Kız erkek oranı K/E:1/1.7' dir. En sık başvuru sebebi ateşti. Hastaların yaklaşık yarısında hepatomegali, splenomegali ve lenfadenopati mevcuttu. Başvuru anında median lökosit sayısı 9750/mm³, median hemoglobin değeri 7,8 g/dl ve median trombosit sayısı 45000/mm³ idi. Morfolojik sınıflamaya göre L1, L2 ve L3 dağılımları sırasıyla %74,9, %24,6 ve % 0,6 idi. İmmunfenotiplendirme incelendiğinde prekursor B hücreli ALL %85,4 ve T hücreli ALL %12,6 idi. Sitogenetik incelemede hastaların %2,4' ünde t(9,22) sitoanomalisi, %2,4' ünde t(4,11) sitoanomalisi mevcuttu. Hastalarımızın %6,1' inde relaps gelişmiştir. Toplam 20 hastamız kaybedildi (%12). Genel sağ kalım oranımız %88, hastalıksız sağ kalım oranımız %85,6 bulundu. Çalışmanın sonuçları değerlendirildiğinde ürik asit yüksekliğ, 15. Gün kemik iliğinin remisyon durumu ve derecesi, relaps varlığı ve relaps türünün sağ kalım üzerine etkili olduğunu bulduk. Ürik asit ve LDH yüksekliği, 15. Gün kemik iliği değerlendirmesi ise olaysız sağ kalıma etkili bulundu. Bu bulgulara göre tedavi sonuçlarımız St. Jude protokolü, BFM 2000 ve 2009 protokolü uygulanan merkezlerle benzerdir.Öğe Evaluation of immunological abnormalities in patients with rare syndromes(Termedia Publishing House Ltd, 2022) Gul, Yahya; Kapakli, Hasan; Aytekin, Selma Erol; Guner, Sukru Nail; Keles, Sevgi; Zamani, Ayse Gul; Yildirim, Mahmut SelmanIntroduction: Recurrent infections are important problems in syndromic patients. This study aimed to evaluate immunological abnormalities in patients who presented with recurrent infections and were diagnosed with rare syndromes. Material and methods: This retrospective analysis included 14 patients with complaints of recurrent infections, all of whom were diagnosed with a rare syndrome. Results: The study group consisted of patients with Aicardi syndrome, Brugada syndrome, Phelan-McDermid syndrome, trichothiodystrophy, LEOPARD syndrome, Prader-Willi syndrome, Seckel syndrome, trisomy 18 (Edwards' syndrome), Wiedemann-Steiner syndrome, West syndrome, Williams syndrome, 47,XYY syndrome, 16p13 deletion syndrome, and 13q1.3 deletion syndrome. Seven patients (50%) were girls and seven (50%) were boys (mean age, 56.7 +/- 32.9 months; median [range] age: 45.5 [27-153] months). There were high rates of consanguinity (50%), cesarean section delivery (71%), and hospitalization in the intensive care unit (78.5%). No patients had a family history of immunodeficiency. On admission, all patients exhibited humoral and/or cellular immune system abnormalities. During the follow-up period, all T-cell abnormalities were improved after immunoglobulin replacement therapy (IGRT), while B-cell abnormalities persisted. These findings suggested that the patients predominantly had antibody deficiencies associated with mild T- cell abnormalities because of recurrent infections. The rates of infections and hospitalizations were significantly reduced after IGRT (p < 0.001); the rate of intensive care unit admission also significantly decreased (from 78.5% to 21.4%). Two of the three oxygen-dependent patients exhibited improvement therein. IGRT was discontinued in two patients with significant clinical improvement during follow-up. Conclusions: An immunological evaluation should be considered in pediatric patients with rare syndromes and recurrent infections. IGRT may help to improve the prognoses of these patients.Öğe Retrospective analysis of patients with severe combined immunodeficiency and alternative diagnostic criteria: A 20-year single centre experience(Wiley, 2023) Korkmaz, Sevim Busra; Karaselek, Mehmet Ali; Aytekin, Selma Erol; Tokgoz, Huseyin; Reisli, Ismail; Guner, Sukru; Keles, SevgiSevere combined immunodeficiency (SCID) is an inborn errors of immunity (IEI) disorder characterized by impairment in the development and function of lymphocytes and could be fatal if not treated with hematopoietic stem cell transplant in the first 2 years of life. There are various diagnostic criteria for SCID among different primary immunodeficiency societies. We retrospectively evaluated clinical and laboratory findings of 59 patients followed up with the diagnosis of SCID at our clinic over the past 20 years in order to develop an algorithm that would help diagnosis of SCID for the countries where a high ratio of consanguineous marriage is present because these countries have not launched TREC assay in their newborn screening programs. The mean age at diagnosis was 5.80 +/- 4.90 months, and the delay was 3.29 +/- 3.99 months. The most common complaint and physical examination findings were cough (29.05%), eczematous rash (63%) and organomegaly (61%). ADA (17%), Artemis (14%), RAG1/2 (15%), MHC Class II (12%) and IL-2R (12%) deficiencies were the most common genetic defects. Lymphopenia (87.5%) was the most frequent abnormal laboratory finding and below 3000/mm(3) in 95% of the patients. The CD3(+) T cell count was 300/mm(3) and below in 83% of the patients. As a result, a combination of low lymphocyte count and CD3 lymphopenia for SCID diagnosis would be more reliable for countries with high rate of consanguineous marriage. Physicians should consider diagnosis of SCID in a patient presenting with severe infections and lymphocyte counts below 3000/mm(3) under 2 years of age.
