Yazar "Toret, Ersin" seçeneğine göre listele
Listeleniyor 1 - 4 / 4
Sayfa Başına Sonuç
Sıralama seçenekleri
Öğe Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome(2019) Toret, Ersin; Ay, Yılmaz; Aksoylar, Serap; Karapınar, Tuba Hilkay; Oymak, YeşimType II Griscelli Syndrome (GS) is caused by a mutation in the RAB27A gene and usually manifests with silvery-gray hair, immune deficiency and the development of hemophagocytic lymphohistiocytosis (HLH). A hematopoietic stem cell transplantation is the curative treatment for HLH and reduced-intensity conditioning prevents the morbidity/mortality in the transplantation related to myeloablative conditioning. We report on a 21-month old boy with cerebral involvement of HLH related to GS.Öğe Evaluation of Bleeding Phenotype of Inherited Factor VII Deficiency in Children With a Bleeding Assessment Tool and Global Assays(Lippincott Williams & Wilkins, 2020) Toret, Ersin; Ay, Yilmaz; Karapinar, Tuba H.; Oymak, Yesim; Kavakli, Kaan; Vergin, Raziye C.Introduction:Inherited factor VII (FVII) deficiency is the most common of the rare bleeding disorders and shows a heterogenous distribution of bleeding phenotypes independent of factor activity level. the bleeding score (BS) evaluates the phenotype of patients with rare bleeding disorders. Thromboelastography (TEG) and thrombin generation assays (TGAs) are 2 methods to evaluate global hemostasis, and controversially both tests are useful for identifying different bleeding tendency phenotypes. the purpose of this study was to investigate the use of the BS and global assays (TEG and TGAs) to predict the bleeding phenotype of inherited FVII deficiency.Materials and Methods:A total of 27 patients with FVII deficiency were evaluated with the BS and global hemostasis assays.Results:The BS was compatible with disease severity according to the FVII activity level (P<0.05) but the BS and bleeding grade of patients did not show a statistically significant correlation with factor activity level (P>0.05). No significant correlation was observed between the factor activity level and any TEG parameter (P>0.05). the factor activity level was negatively correlated with the lag time of the TGA on the contrary positively correlated with the peak thrombin time of the TGA (P<0.05).Conclusions:The global assays do not successfully predict the bleeding phenotype. the BS is a more suitable tool than conventional and global assays for predicting the bleeding phenotype.Öğe Is Intravenous Iron Treatment in Pediatric Patients Safe and Effective Enough?(Galenos Publ House, 2022) Karadas, Nihal; Toret, Ersin; Karadas, UlasIntroduction: Iron deficiency anemia (IDA) is the most common hematological disease among infants and children. Although most of the children with IDA are treated with oral iron preparations, intravenous (IV) iron is an alternative for children with severe IDA who have difficulty in adhering to or absorbing oral iron. In this study, the reasons of IDA in patients treated with IV iron and the effectivity and safety of iron sucrose preparations in pediatric patients were evaluated. Materials and Methods: The medical records of children received IV iron sucrose at a pheripheral hospital between June 2014 and June 2017 were reviewed retrospectively. The IDA patients who are unresponsive to oral iron or whose hemoglobin (Hb) <7 g/dL and received IV iron treatment were evaluated for the reasons of anemia, the efficacy and safety of IV iron treatment. Before and after iron sucrose infusion laboratory results were compared. All records were reviewed for serious side effects and allergic reactions. Results: A total of 33 patients, aged between 3-18 years were enrolled into the study. While 58% of the patients who received IV iron treatment had inadequate consumption of iron, 24% had malabsorption. In the initial evaluation Hb levels of the patients were 3.2-7.8 (6.54 +/- 1.06) g/dL, (mean corpuscular volume) MCV was 59.18 +/- 6.66 fL and ferritin was 1.87 +/- 1.34 mu g/L, the mean post-treatment Hb was 11.39 +/- 1.51 g/dL, MCV was 76.06 +/- 7.59 fL and ferritin was 54.79 +/- 15.64 mu g/L. No serious side effects were seen. Conclusion: The use of IV iron sucrose in pediatric patients with IDA leads to significant increase in Hb and reduces erythrocyte suspension transfusion and is an effective and safe method for iron treatment.Öğe A Newborn with Giant Cell Tumor of the Occipital Bone: Case Report(2019) Toret, Ersin; Demirağ, Bengü; Çalkavur, Şebnem; Doğanavşargil, Başak; Tuncer, TurhanGiant cell tumors of bone (GCTB) are commonly benign neoplasms and characterized by regional progressive and destructive lesions. They have a malignant potential and the capacity to metastasis. Incidents of GCTB are reported in 20% of all benign and 5% of all malignant bone tumors and pediatric cases account for less than 5% of all them. the first line treatment strategy for GCTBs is surgical resection. A male baby presented at our hospital on his 10th day of life suffering from respiratory distress and persistent vomiting. His blood and urine panels were within normal parameters. CMRI was performed to evaluate his condition. the CMRI report noted a “suspected 4x3 cm contrasted bone-derived malignant-looking mass at the left posterior fossa of the cranium”. the biopsy confirmed: “A grade 1-2 giant cell tumor of bone”. Surgical resection was not possible because of the location of the mass and its proximity to blood vessels but chemotherapy was the one strategy available in this particular case. the chemotherapy regimen consisted of cisplatin 1 mg/kg/day (1-3 days) and doxorubicin 1 mg/kg/day (1,2 days) and was applied four times every month. Using CMRI, we noted a reduction in mass of more than 50% after two sessions and complete regression after four sessions. the patient was given regular follow-ups with no evidence of recurrence and co-morbidity were observed over the next 60 months. We recommend chemotherapy as a successful alternative strategy when surgical resection, radiotherapy, and other therapies are not applicable for GCTBs.
