2021 Cilt 19 Sayı 2

Permanent URI for this collectionhttps://hdl.handle.net/11452/24568

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Browsing by Subject "Çocuk"

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    Clinical characteristics and treatment outcomes of patients with malignant extracranial germ cell tumors: A 20-year single-center experience
    (Galenos Yayıncılık, 2021-04-02) Küpesiz, Funda Tayfun; Tüysüz, Gülen; Akınel, Ayşe Nur; Tekneci, Ayşegül; Sivrice, Ayşe Çiğdem; Melikoğlu, Mustafa; Pestereli, Hadice Elif; Küpesiz, Osman Alphan; Güler, Elif
    Introduction: Germ cell tumors account for 2–3% of all pediatric tumors. The aim of this study was to evaluate the clinical features and treatment outcomes of pediatric patients treated and followed up for extragonadal MGCTs in our center. Materials and Methods: A total of 41 patients diagnosed with MGCTs in the pediatric oncology department of Akdeniz University between June 1999 and June 2019 were evaluated retrospectively. Results: Twenty-nine (71%) of the patients were girls and female dominance (p<0.001). The median age was 3.22 (0–18) years. The most patients in the ≤ 5year age group (p<0.001). Nineteen (44%) of the tumors were gonadal and 22 (54%) were extragonadal. The most common histolology of MGCTs were yolk sac tumor (36%), mixed GCTs (29%), immature teratoma (20%), and dysgerminoma (15%). Twenty-five (61%) patients presented with advanced stage disease and 37 patients (90%) were treated with chemotherapy. The patients with stage I testicular and stage I ovarian germ cell tumors underwent complete tumor resection followed by a watch-and-wait approach with alpha fetoprotein monitoring without chemotherapy. Of six patients with relapse/refractory disease, two patients survived. Two patients who underwent autologous stem cell transplantation showed complete response but later died due to infection. The median follow-up period of the patients was 34.9 (4–190.6) months and the 10-year overall and disease-free survival rates were 77.1±6.8% 77.1±6.8%. Two relapsed refractory patients who underwent autologous transplantation survived at a mean of 33.21 months. Conclusions: The clinical features and treatment outcomes of the patients in our study were consistent with the literature. The fact that most of our patients were symptomatic at presentation and had advanced stage disease when diagnosed highlights the importance of detailed evaluation and examination. Although good outcomes are achieved in patients with early stage disease, new treatment approaches are needed for patients with advanced and relapsing disease
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    Evaluation of 36 patients with rare factor deficiency
    (Galenos Yayıncılık, 2021-06-01) Üzel, Veysiye Hülya; Yılmaz, Kamil; Öncel, Kahraman; Tekin, Suat; Söker, Murat
    Introduction: Rare factor deficiencies are predominantly autosomal recessively inherited disorders with a frequency of approximately 1: 500000 to 1: 2000000 in the general population. They account for 3-5% of all inherited coagulation disorders. In this study, we aimed to evaluate the demographic features and clinical findings of 36 patients who were followed up and treated with the diagnosis of rare factor deficiency. Materials and Methods: A total of 36 patients aged between 0-16 years diagnosed with rare coagulation deficiencies were evaluated in terms of demographic, physical examination, clinical follow-up, and laboratory findings at the Dicle University Pediatric Hematology Unit. Ethics committee approval was obtained from Dicle University for the study on 16.03.2018 with decision no 115. Results: Rare factor deficiencies were diagnosed in 36 (35 %) of 103 patients who were followed up with coagulation disorders. Hemophilia a, hemophilia b, and von Willebrand disease constituted 67 of our patients. Familial consanguinity was present in 75, 6 %, and positive family history was found at 16.6% of the patients. 11 (32.4%) of our patients were diagnosed under the age of one year. Most of our patients diagnosed with factor X deficiency (38.8%). The most common symptoms were mucocutaneous bleeding (50%). Intracranial hemorrhage was detected in 7 (%19,5) patients. Conclusions: Early diagnosis and treatment are very important in the case of rare factor deficiency since severe bleeding complications such as intracranial hemorrhage may develop. Rare factor deficiencies are seen more frequently in places where consanguineous marriage is more common than the general population. Families should be informed about this issue, and family screening should be done early.