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Öğe Clinical features and outcome of children with hereditary spherocytosis(Lippincott Williams and Wilkins, 2021) Kılıç, Mehmet A; Özdemir, Gül Nihal; Tahtakesen, Tuba Nur; Gökçe, Müge G.; Uysalol, Ezgi Paslı; Bayram, Cengiz; Ayçiçek, Ali; Aydo?an, GönülObjective: The aim of this study was to evaluate the demographics, clinical, and laboratory findings and treatment responses of patients with hereditary spherocytosis (HS). Materials and Methods: Data of children with HS were examined. Diagnosis was based on clinical history, physical examination, family history, presence of spherocytes on peripheral blood smear, and osmotic fragility test. Results: A total of 101 patients were included. The median (range) age at diagnosis was 38.0 (1 to 188) months. Mild, moderate, and severe forms of HS were present in 29 (28.7%), 15 (14.9%), and 57 (56.4%) patients, respectively. Family history was available in 73 patients and 56 of these (76.7%) had a positive family history for HS. Forty-five (44.5%) patients needed regular transfusions and all of these had severe disease. Although most patients did not require transfusion postsplenectomy, 2 of 45 (4.4%) patients continued to require transfusion. Transfusion dependence was significantly (P<0.001) higher in patients with severe spherocytosis. Conclusions: In HS, splenomegaly, pallor, and jaundice are the most common clinical features. Splenectomy dramatically reduces hemolysis in most cases and virtually abolishes further requirement for transfusion.Öğe Comparison of bone marrow biopsy and fluorodeoxyglucose-positron emission tomography for the assessment of bone marrow infiltration in children with Hodgkin's lymphoma(Georg Thieme Verlag Kg, 2023) Arslantas, Esra; Aycicek, Ali; Akici, Ferhan; Ozkara, Selvinaz; Yilmaz, Burcak; Ozdemir, Nihal; Bayram, CengizPurpose To compare bone marrow biopsy (BMB) with [18F]-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography (PET/CT) imaging in the demonstration of bone marrow involvement in children with Hodgkin's Lymphoma (HL) and to investigate the effectiveness of PET/CT imaging and thus the necessity for BMB at staging.Methods Pediatric patients with HL, who underwent both bilateral iliac BMB and PET/CT imaging at disease staging were retrospectively analyzed. In determining bone marrow involvement (BMinv), BMB and/or first/follow-up PET/CT imaging were eligible for review.Results Fifty-six patients were included. BMinv was detected by PET/CT imaging in 6/56 (10.7%), whereas the proportion was 3/56 (5.3%) in BMB specimens. Bone marrow biopsies and PET/CT images were concordant in 53/56 (94.6%) patients with BMB specimens missing three cases of BMinv detected by PET/CT. When diagnostic accuracy was calculated, sensitivity, specificity, positive predictive value and negative predictive values for PET/CT were 100%, 100%, 100%, 100%, respectively, and the same values for BMB were 50%, 100%, 100%, 94.3%, respectively.Conclusions The results of PET/CT and BMB for staging of pediatric HL patients were compatible, and PET/CT imaging was found to provide high diagnostic performance in determining BMinv. In keeping with earlier research, the current study showed that BMB may not be necessary in every patient at staging, and should be reserved for cases where PET/CT is inconclusive.Öğe A comparison of hypersensitivity reactions between intravenous and intramuscular applications of native E. coli asparaginase in children with acute lymphoblastic leukemia(Sage Publications Ltd, 2023) Al, Isik Odaman; Ozdemir, Nihal; Ersoy, Gizem Zengin; Bayram, Cengiz; Cilengiroglu, Ozgul Vupa; Arslantas, Esra; Uysalol, Ezgi PasliIntroduction Asparaginase is an indispensable drug in treating childhood acute lymphoblastic leukemia (ALL). Hypersensitivity reactions (HSR) are the most common side effects and interfere with the antineoplastic activity of the drug. This study aims to compare the intramuscular (IM) and intravenous (IV) administration routes of Native Escherichia coli Lasparaginase (L-ASNase) in terms of hypersensitive reactions. Methods L-ASNase was randomly administered IV or IM to newly diagnosed ALL patients and HSR was monitored in all patients for 1 h following the end of the IV infusion and for 2 h following the end of the IM administration of L-ASNase. Based on a retrospective review of clinical charts, reactions were identified. In order to determine the severity of each allergic reaction, we used the Common Terminology Criteria for Adverse Events (CTCAE) version 4.03 for allergic reactions. Results A total of 1032 doses of L-ASNase were administered to 85 patients (42 males and 43 females) during the study period. Among 85 patients, 30 reactions were recorded, which means that 35% of the patients reacted. According to the CTCAE, twenty-nine out of 30 reactions (97%) were grade 2, while one (3%) was grade 4. In terms of individual doses, there was a non-significant trend toward increased incidence of reactions with IV administration (3.8% versus 0.9%, p = 0.064). The rate of reactions was higher in patients who received all IV doses (n: 60) as compared to those who received all IM doses (n: 25) (31.7% vs. 3.5%; chi-square= 8.415, p value=0.04). Based on the risk groups and HSR incidence, it was found that high risk group (HRG) patients were significantly more likely to develop HSR compared to the standart risk group (SRG) and intermediate risk group (MRG) patients (chi-square p = 0.003, CI: 95%; odds ratio: 3.12 and 5.91, respectively). Conclusions In conclusion, IM administration of L-ASNase causes significantly less HSR to L-ASNase than the IV route. Patients with HRGALL have a higher risk of HSR. Since L-ASNase is still used in many developing countries and there are problems in the supply of Erwinia chrysanthemi ASNase (Erwinia), LASNase can be administered IM to reduce the frequency of HSR.Öğe Orak Hücreli Anemi: Geriye Dönük Tek Merkez Deneyimi(2023) Özdemir, Nihal; Kılıç, Mehmet Akif; Aydoğan, Gönül; Bayram, Cengiz; Akpınar Tekgündüz, Sibel; Hacısalihoğlu, Şadan; Terzi, ÖzlemAmaç: Orak hücreli aneminin klinik belirtileri yaşamın erken dönemlerinde ortaya çıkar ve çeşitli genetik ve çevresel faktörler sebebiyle değişkenlik gösterir. Çalışmamızda orak hücreli anemili çocuk hastaların demografik, klinik ve prognostik özelliklerini değerlendirmeyi amaçladık. Hastalar ve Yöntem: Orak hücreli anemili hastaların klinik ve laboratuvar özellikleri retrospektif olarak incelendi. Bulgular: Çalışmaya ortanca yaşı 12 (8-150) ay olan toplam 15 hasta dahil edildi. Hastaların çoğunluğu erkekti. Hastaların %60’ında aile öyküsü vardı. Tanı anında HbS medyan değeri %62.1 (37- 84.8) idi. Hastalarımızın komplikasyonlarının büyük kısmını ağrılı krizler oluşturuyordu. Sonuç: Gelecekteki klinik çalışmalar, orak hücreli anemili çocuklarda morbidite ve mortalite oranlarının azalmasına katkıda bulunacaktır.