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Öğe Adli tıbbi sürece yansıyan femur-humerus kırığı saptanan yenidoğanların retrospektif değerlendirilmesi: tanımlayıcı çalışma(Turkiye Klinikleri J Foren Sci Leg Med., 2022) Yıldız Silahlı,Nicel; Aslıyüksek, Hızır; Dikici,Fatih; Celkan, Tülin TirajeAmaç: Doğum travmaları, doğum eylemi esnasında mekanik etkiler ile ortaya çıkan fiziksel yaralanmalardır. Doğum sırasında karşılaşılan bu durum medikolegal sorunlara neden olmaktadır. Çalışmamızda, yenidoğan döneminde uzun kemik kırığı saptanarak medikolegal sürece konu olmuş olguların özelliklerinin tartışılması amaçlanmıştır. Gereç ve Yöntemler: Adli Tıp Kurumu 7. İhtisas Kurulunda 2018-2020 yılları arasında medikolegal değerlendirme yapılan uzun kemik (femur/humerus) kırığı geliştiği iddiası bulunan olgular (n=23) dâhil edildi. Olguların sosyodemografik ve klinik özellikleri ile kurul kararları retrospektif olarak incelendi. Çalışma izni 01 Aralık 2020 tarih ve 1114 sayılı Adli Tıp Kurumu Bilimsel Araştırma Komisyonundan alındı. Bulgular: Olguların kız/erkek oranı 8/15 idi. On sekiz (%78,2) raporda hekim şikâyeti vardı. Olgularda femur (n=14) ve humerus (n=9) kırıkları saptandı. Olguların gestasyon haftası ortalaması 33 idi. Olguların ortalama doğum tartıları 2.921 (750-5.140 g) g saptandı. Olguların, term/preterm oranı 18/5 saptandı. Humerus ve femur kırığı saptanan 23 olguda da konservatif tedavi uygulanmıştı. Yirmi iki olguda tıbbi uygulama hatası saptanmadı ve kırıklar komplikasyon olarak değerlendirildi. Sonuç: Medikolegal sürece yansıyan uzun kemik kırıklarının tanı ve takip sürecinin uygun şekilde yönetildiği görülmektedir. Süreçte hasta-hekim arasında yaşanan iletişim sorunlarının güven ilişkisini sarstığı görülmektedir. Tıbbi bakım ve tedavi sırasında meydana gelen ve direkt olarak hekimin hatalı bir eylemi sonucu oluşmayan bu durum doğum süreci açısından komplikasyon olarak tanımlanmaktadır.Öğe Can biomarkers predict myocardial iron overload in children with thalassemia major?(Cambridge Univ Press, 2023) Karakaş, Hasan; Eroğlu, Ayşe Güler; Akyel, Nazlı Gülsüm; Çiğ, Gülnaz; Adaletli, İbrahim; Özdemir, Gül Nihal; Türkkan, Emine; Celkan, Tülin TirajeAim:Beta-thalassemia major requires regular blood transfusions throughout life, which in turn leads to iron accumulation in the body. While cardiac T2* MRI is the gold standard in determining cardiac iron accumulation, it is not always feasible, which has led to the search for new biomarkers. Herein, the value of growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide in predicting cardiac iron accumulation is investigated in asymptomatic children with beta-thalassemia major. Materials and method:Forty-one patients aged 11-21 years and 41 age-, gender-, body mass index-matched healthy controls were included. Serum growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were compared between the patients and controls. Additionally, the relations of these biomarkers with cardiac and liver T2 * MRI were investigated in the patients. Results:In the patients, growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were higher than healthy controls (p < 0.001, p = 0.025, p < 0.001, respectively). There were no significant correlations of growth differentiation factor-15 and N-terminal pro-B-type natriuretic peptide levels with both cardiac and liver T2 * MRI measurements. While there was no significant correlation of serum galectin-3 with cardiac T2 * MRI measurements, a negative correlation was found with liver T2 * MRI measurements (p = 0.040, rho = -0.325). Conclusion:All three biomarkers investigated in this study failed to predict myocardial iron accumulation in asymptomatic children with beta-thalassemia major. However, a weak relation between serum galectin-3 level and hepatic iron accumulation was demonstrated.Öğe Castleman disease: a multicenter case series from Turkey(2022) Gunduz, Eren; Kirkizlar, Hakki Onur; Umit, Elif Gulsum; Karaman Gulsaran, Sedanur; Ozkocaman, Vildan; Celkan, Tülin TirajeObjective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. Two main histological subtypes are hyaline vascular and plasma cell variant. It is classified as unicentric (UCD) or multicentric CD (MCD) according to the anatomical distribution of disease and the number of lymph nodes involved. The aim of this multicenter study is to evaluate all cases of CD identified to date in Turkey to set up a national registry aiming to improve CD early recognition, treatment and follow-up. Materials and methods: Both adult (n=130) and pediatric (n=10) patients with a lymph node or involved field biopsy result reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging studies, treatment strategies and clinical outcome were evaluated retrospectively. Results: 140 patients (69 male and 71 female) with a diagnosis of UCD (n = 73) and MCD (n = 67) were included. Mean age was 39 in UCD and 47 in MCD. Female patients were more common in UCD. The most common histologic subtype was hyaline vascular for both UCD and MCD. Asymptomatic patients were more common in UCD. Anemia, elevation of acute phase reactants and hypoalbuminemia were more common in MCD. Most commonly used treatment strategies in UCD were surgical excision, rituximab and radiotherapy respectively. All UCD patients were alive at a median 19.5 month follow up. Most commonly used treatment strategies in MCD were methyl prednisolone, R-CHOP, R-CVP and rituximab. Thirteen patients died at a median 34 month follow up. Conclusion: Our study is important due to representing the patient characteristics and treatment strategies from Turkey and have a potential of increasing awareness about CD. Treatment data may help making decision particularly in countries who can not get access to siltuximab. Larger prospective studies are needed to make definitive conclusions.Öğe Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives(Scientific and Technical Research Council of Turkey, 2020) İncesoy Özdemir, Sonay; Taçyıldız, Nurdan; Varan, Ali; Kebudi, Rejin; Zülfikar, Osman Bülent; Celkan, Tülin Tiraje; Şahin, Gürses; Çorapçıoğlu, Funda Vesile; Keskin Yıldırım, Zuhal; Pınarlı, Faruk Güçlü; Olgun, Hatice Nur; Sarı, Neriman; Dağdemir, Ayhan; Özyörük, Derya; Eren, Tuba; Çakır, Fatma Betül; Adaklı Aksoy, Başak; Bozkurt, Ceyhun; Güler, Elif; Özgüven, Ali Aykan; Erbey, Mehmet Fatih; Berber Hamamcı, Melda; Dinçaslan, Handan; Ünal, Emel; Kantar, MehmetAim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey. Material and methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires. Results: Only 1 of the 21 participating centers had a separate LTFU clinic. The remaining centers provided LTFU care for childhood cancer survivors at the pediatric oncology outpatient clinic. Of these centers, 17 (80.9%) reported difficulty in transition from the pediatric clinic to the adult clinic, 14 (66.6%) reported insufficient care providers, and 12 (57.1%) reported insufficient time and transportation problems. As neglected late effects, 16 (76.1%) centers reported psychosocial and getty job problems and 11 (52.3%) reported sexual and cognitive problems. None of the centers had their own LTFU guidelines for their daily LTFU practice Conclusion: This study was the first to gain an overview of the needs of POCs and the gaps in survivorship services in Turkey. The results from this study will help to develop a national health care system and national guidelines for pediatric cancer survivors.Öğe Effect of platelet count and platelet transfusion on fever duration in patients with febrile neutropenia(Taylor and Francis, 2022) Öztosun, Berrak; Pirdal, Betül Zehra; Özel Çınar, Simge; Gonca Kaçar, Ayşe; Apak, Hilmi; Celkan, Tülin TirajePlatelets play a role in hemostasis, thrombosis, and vascular integrity. They also play a major role in the development of inflammation and the activation of immune responses. They have phagocytic activity, stimulate the secretion of immune modulators, and activate other immune cells, which results in platelet-neutrophil aggregation, platelet-induced neutrophil degranulation, and the formation of neutrophil extracellular traps. Data on 124 febrile neutropenia attacks were retrospectively examined. Patients' age, sex, diagnosis, and relapse history were obtained. The complete blood count levels on the first and last febrile day of the febrile neutropenia attacks, duration of fever, and number, type, and timing of thrombocyte suspension transfusions were recorded. The patients were divided into three groups according to the day of fever when the platelet suspension was administered (1 day, 2-3 days, and >3 days); the duration of fever was compared between the three groups. The fever duration of those who were transfused with platelet suspension on the first day of fever was found to be significantly shorter (p = 0.03 and p < 0.001, respectively). When treating a patient with febrile neutropenia, if thrombocyte suspension transfusion is indicated, transfusing thrombocytes in the first days of fever shortens the fever duration and improves the prognosis of febrile neutropenia attack, supporting the hypothesis that not only neutrophils but also platelets may play a role in fighting against microorganisms.Öğe Evaluation of left ventricular function and myocardial deformation in children with beta-thalassemia major by real-time three-dimensional (four-dimensional) and speckle tracking echocardiography(John Wiley and Sons Inc, 2022) Eroğlu, Ayşe Güler; Uluğ, Nujin; Karakaş, Hasan; Yüksel Karabıyık, Esra; Akyel, Nazlı Gülsüm; Gülnaz, C. I.G.; Adaletli, İbrahim; Özdemir, Gül Nihal; Türkkan, Emine; Celkan, Tülin TirajeAim: This study aimed to evaluate the role of real-time three-dimensional (four-dimensional) and speckle tracking echocardiography for early detection of left ventricular systolic dysfunction and also for the relationship between myocardial deformation parameters and myocardial iron load which is measured by cardiac magnetic resonance relaxation time T2* values in asymptomatic children with beta-thalassemia major. Material and Methods: This multicenter cross-sectional study included 40 patients (mean age 15.4 ± 2.9, 42.1% male) and 40 healthy children whose age, gender, and body mass index-matched with patients. Each participant underwent conventional echocardiography and tissue Doppler imaging. Left ventricular ejection fraction; global longitudinal, circumferential, radial strains; twist; and torsion were measured by real-time three-dimensional and speckle tracking echocardiography. Cardiac magnetic resonance imaging T2* was measured in patients. Results: Left ventricular global longitudinal, circumferential, and radial strains were decreased despite preserved global ventricular function in patients compared to healthy children (p = p =.029, p = p <.001, p =.003, respectively). There were no statistically significant differences between patients with T2* ? 20 ms and patients with T2* < 20 ms for all echocardiographic parameters. Also, there were no significant correlations between all echocardiographic parameters and T2* values in all patients, those with T2* ? 20 ms, and T2* < 20 ms. Conclusion: We found that even in asymptomatic children with beta-thalassemia major, left ventricular longitudinal, circumferential and, radial functions were impaired by real-time three-dimensional (four-dimensional) and speckle tracking echocardiography. This novel echocardiographic method might be an important tool for detecting subclinical left ventricular systolic dysfunction irrespective of T2* values.Öğe Hemophagocytic lymphohistiocytosis(Gelanos, 2022) Kaçar, Ayşe Gonca; Celkan, Tülin TirajeHemophagocytic lymphohistiocytosis (HLH) is an aggressive life-threatening disease that consists of uncontrolled activated lymphocytes and macrophages that secrete excessive cytokines. Symptoms and laboratory findings of HLH include prolonged fever, cytopenia, hepatosplenomegaly, liver dysfunction, hypertriglyceridemia, hyperferritinemia, increased soluble interleukin-2 receptor, low fibrinogen, and neurological problems. HLH has two forms: primary (familial autosomal recessive) or secondary (related to infections, malignancy, autoimmune and metabolic disorders, transplantations, chimeric antigen receptor T-cell therapies, etc.) form. As underlying conditions in HLH varied, clinical findings are nonspecific and disease diagnosis is challenging. Furthermore, patients diagnosed with primary HLH can have a secondary triggering agent, such as infection. Thus, there is no clear-cut distinction between these two forms. Abnormal immune response and a low number or absence of natural killer cells and cytotoxic T-lymphocytes are hallmarks of HLH. Despite the early and aggressive treatment, HLH is a deadly disease. Urgent immunosuppressive therapy is necessary to control hyperinflammation. Hematopoietic stem cell transplantation is a curative treatment in familial forms. Targeted therapy with emapalumab was also recently reported to be effective.Öğe Is hypoxia a cause or a consequence? the medico-legal considerations of neonatal encephalopathy and cerebral palsy(ROMANIAN LEGAL MED SOC, 2022) Silahlı, Nicel Yıldız; Akçakaya, Nihan Hande; Aslıyüksek, Hızır; Yılmaz, Muhammed Emre; Madazlı, Rıza; Celkan, Tülin TirajeNeonatal encephalopathy (NE) occurs in the first month after birth of infants and progresses with seizures, respiratory depression and hypotonia. NE is multifactorial, however hypoxia is the first considered cause. Our study aims to discuss medicolegal processes behind the etiological and clinical features of NE and cerebral palsy (CP) over cases evaluated by the specialized board of malpractice of the Council of Forensic Medicine. The 130 cases in the judicial process claimed to be from lack of oxygen at birth, were evaluated retrospectively. The cases were categorized into two groups as evidenced with hypoxic-ischemic encephalopathy (HIE) and not evaluated evidence with HIE, by using blood gas evaluations and brain MRI findings. All risk factors related to labor, resuscitation, intubation, and referral to other NICUs, and presence of seizure were documented in both groups. We found no evidence of intrapartum hypoxia in about 70% of NE and it is suggesting that the causes of NE may have started in the antepartum period. It is necessary to focus on other inherent diseases that play a role in the etiology in medicolegal process, as well as investigate the presence of hypoxia and whether the correct intervention is made.Öğe A peculiar disease of a young woman who wants to get pregnant(Gelenos, 2021) Celkan, Tülin Tiraje; Fenercioğlu, Şeyma; Kaçar, Ayşe GoncaPlasminogen has an important role in intravascular and extravascular fibrinolysis (1-3). Severe hypoplasminogenemia is associated with ligneous conjuctivitis, but ligneous lesions can also occur in many different mucosal membranes like cervix and endometrium (4,5). Despite numerous clinical evaluations, biopsies, and laboratory tests, these kind of patients' diagnosis remained elusive for many years (6). By presenting this case, we want to increase the awareness of this peculiar disease, for its proper diagnosis.Öğe PTEN and AKT1 variations in childhood T-Cell acute lymphoblastic leukemia(Galenos Yayincilik, 2020) Küçükcankurt, Fulya; Erbilgin, Yücel; Fırtına, Sinem; Ng, Özden Hatırnaz; Karakaş, Zeynep; Celkan, Tülin Tiraje; Ünüvar, Ayşegül; Özbek, Uğur; Sayitoglu, MugeObjective: PTEN/AKT pathway deregulations have been reported to be associated with treatment response in acute leukemia. This study examined pediatric T-cell acute lymphoblastic leukemia (T-ALL) samples for PTEN and AKT1 gene variations and evaluated the clinical findings. Materials and Methods: Fifty diagnostic bone marrow samples of childhood T-ALL cases were investigated for the hotspot regions of the PTEN and AKT1 genes by targeted next-generation sequencing. Results: A total of five PTEN variations were found in three of the 50 T-ALL cases (6%). Three of the PTEN variations were first reported in this study. Furthermore, one patient clearly had two different mutant clones for PTEN. Two intronic single-nucleotide variations were found in AKT1 and none of the patients carried pathogenic AKT1 variations. Conclusion: Targeted deep sequencing allowed us to detect both low-level variations and clonal diversity. Low-level PTEN/AKT1 variation frequency makes it harder to investigate the clinical associations of the variants. On the other hand, characterization of the PTEN/AKT signaling members is important for improving case-specific therapeutic strategies.Öğe Zinc finger protein 384 ( ZNF384) impact on childhood mixed phenotype acute leukemia and B-cell precursor acute lymphoblastic leukemia(Taylor and Francis, 2022) Sudutan, Tuğce; Erbilgin, Yücel; Hatırnaz, Özden; Karaman, Serap; Karakaş, Zeynep; Küçükcankurt, Fulya; Celkan, Tülin Tiraje; Timur, Çetin; Özdemir, Gül Nihal; Hacısalihoglu, Sadan; Aylan Gelen, Sema; Sayitoğlu, MügeB-cell precursor acute lymphoblastic leukemia (BCP-ALL) is a heterogeneous malignancy and consists of several genetic abnormalities. Some of these abnormalities are used in clinics for risk calculation and treatment decisions. Patients with ZNF384 rearrangements had a distinct expression profile regardless of their diagnosis, BCP-ALL or mixed phenotype acute leukemia (MPAL) and defined as a new subtype of ALL. In this study, we screened 42 MPAL and 91 BCP-ALL patients for the most common ZNF384 fusions; ZNF384::TCF3, ZNF384::EP300 and ZNF384::TAF15 by using PCR. We identified ZNF384 fusions in 9.5% of MPAL and 7.6% of BCP-ALL. A novel breakpoint was identified in ZNF384::TCF3 fusion in one BCP-ALL patient. T-myeloid MPAL patients showed significantly lower ZNF384 expression compared to lymphoid groups. Patients with ZNF384r had intermediate survival rates based on other subtypes. Prognostic and patient-specific treatment evaluation of ZNF384 fusions in both ALL and MPAL might help to improve risk characterization of patients.
