Publications by authors named "Emel Ünal"

51 Publications

Evaluation of Genetic Diversity and Structure of Turkish Water Buffalo Population by Using 20 Microsatellite Markers.

Animals (Basel) 2021 Apr 9;11(4). Epub 2021 Apr 9.

Department of Animal Science, Tekirdağ Namık Kemal University, 59030 Tekirdağ, Turkey.

The present study was aimed to investigate the genetic diversity among 17 Turkish water buffalo populations. A total of 837 individuals from 17 provincial populations were genotyped, using 20 microsatellites markers. The microsatellite markers analyzed were highly polymorphic with a mean number of alleles of (7.28) ranging from 6 (ILSTS005) to 17 (ETH003). The mean observed and expected heterozygosity values across all polymorphic loci in all studied buffalo populations were 0.61 and 0.70, respectively. Observed heterozygosity varied from 0.55 (Bursa (BUR)) to 0.70 (Muş (MUS)). It was lower than expected heterozygosity in most of the populations indicating a deviation from Hardy-Weinberg equilibrium. The overall value for the polymorphic information content of noted microsatellite loci was 0.655, indicating their suitability for genetic diversity analysis in buffalo. The mean F value was 0.091 and all loci were observed significantly deviated from Hardy-Weinberg Equilibrium (HWE), most likely based on non-random breeding. The 17 buffalo populations were genetically less diverse as indicated by a small mean F value (0.032 ± 0.018). The analysis of molecular variance (AMOVA) analysis indicated that about 2% of the total genetic diversity was clarified by population distinctions and 88 percent corresponded to differences among individuals. The information produced by this study can be used to establish a base of national conservation and breeding strategy of water buffalo population in Turkey.
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http://dx.doi.org/10.3390/ani11041067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8070036PMC
April 2021

Allogeneic hematopoietic stem cell transplantation in leukocyte adhesion deficiency type I and III.

Blood Adv 2021 Jan;5(1):262-273

Pediatric Hematology, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, Ankara, Turkey.

Type I and III leukocyte adhesion deficiencies (LADs) are primary immunodeficiency disorders resulting in early death due to infections and additional bleeding tendency in LAD-III. The curative treatment of LAD-I and LAD-III is allogeneic hematopoietic stem cell transplantation (allo-HSCT). In this retrospective multicenter study, data were collected using the European Society for Blood and Marrow Transplantation registry; we analyzed data from 84 LAD patients from 33 centers, all receiving an allo-HSCT from 2007 to 2017. The 3-year overall survival estimate (95% confidence interval [CI]) was 83% (74-92) for the entire cohort: 84% (75-94) and 75% (50-100) for LAD-I and LAD-III, respectively. We observed cumulative incidences (95% CI) of graft failure (GF) at 3 years of 17% (9%-26%) and grade II to IV acute graft-versus-host disease (aGVHD) at 100 days of 24% (15%-34%). The estimate (95% CI) at 3 years for GF- and GVHD-II to IV-free survival as event-free survival (EFS) was 56% (46-69) for the entire cohort; 58% (46-72) and 56% (23-88) for LAD-I and LAD-III, respectively. Grade II to IV acute GVHD was a relevant risk factor for death (hazard ratio 3.6; 95% CI 1.4-9.1; P = .006). Patients' age at transplant ≥13 months, transplantation from a nonsibling donor, and any serological cytomegalovirus mismatch in donor-recipient pairs were significantly associated with severe acute GVHD and inferior EFS. The choice of busulfan- or treosulfan-based conditioning, type of GVHD prophylaxis, and serotherapy did not impact overall survival, EFS, or aGVHD. An intrinsic inflammatory component of LAD may contribute to inflammatory complications during allo-HSCT, thus providing the rationale for considering anti-inflammatory therapy pretreatment.
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http://dx.doi.org/10.1182/bloodadvances.2020002185DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7805328PMC
January 2021

Secondary intra-arterial chemotherapy and/or intravitreal chemotherapy as salvage treatment for retinoblastoma.

Eur J Ophthalmol 2020 Sep 22:1120672120957587. Epub 2020 Sep 22.

Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.

Purpose: To evaluate the results of secondary intra-arterial chemotherapy (IAC) and/or intravitreal chemotherapy (IVC) as a salvage treatment for retinoblastoma (RB).

Materials And Methods: The medical records of 31 (20 male, 11 female) cases (with 38 eyes) who underwent secondary IAC and/or IVC between February 2010 and June 2019 were retrospectively reviewed.

Results: Thirteen (41.9%) cases had unilateral and 18 (58.1%) had bilateral RB. According to the International Classification of RB, 6 (15.8%) eyes had group B, 9 (23.7%) eyes had group C, 16 (42.1%) eyes had group D, and 7 (18.4%) eyes had group E RB at diagnosis. All patients underwent six-cycle intravenous chemotherapy as primary treatment and 8 eyes received external radiotherapy before IAC/IVC. Secondary IAC was performed in 21 (55.3%) eyes, IVC in 10 (26.3%) eyes, and IAC + IVC in 7 (18.4%) eyes. External radiotherapy was applied in 2 (5.3%) eyes after IAC/IVC, one of which was later enucleated. In total, 17 (44.7%) eyes undergoing secondary IAC/IVC treatments were enucleated. Metastasis and death were not observed in any case during the mean follow-up period of 59.3 (median 61, range: 10-98) months.

Discussion: Although 60.5% of the eyes undergoing IAC/IVC consisted of groups D and E RB, globe salvage and survival rates were 55.3% and 100.0%, respectively. External radiotherapy was required in 5.3% of the eyes after IAC/IVC. In conclusion, IAC and IVC are safe and effective treatment methods in eyes with RB unresponsive to other eye-preserving treatments and those demonstrating recurrence.
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http://dx.doi.org/10.1177/1120672120957587DOI Listing
September 2020

Correction: A 20-year audit of retinoblastoma treatment outcomes.

Eye (Lond) 2020 10;34(10):1940

Şanlıurfa Balıklıgöl State Hospital, Şanlıurfa, Turkey.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.
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http://dx.doi.org/10.1038/s41433-020-1119-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7608207PMC
October 2020

Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives

Turk J Med Sci 2020 12 17;50(8):1916-1921. Epub 2020 Dec 17.

Department of Pediatric Oncology, Faculty of Medicine, Ege University, İzmir, Turkey

Aim: 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.
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http://dx.doi.org/10.3906/sag-1911-193DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7775715PMC
December 2020

Genetic Characterization of Native Donkey () Populations of Turkey Using Microsatellite Markers.

Animals (Basel) 2020 Jun 24;10(6). Epub 2020 Jun 24.

Department of Animal Science, Tekirdağ Namık Kemal University, 59030 Tekirdağ, Turkey.

This study presents the first insights to the genetic diversity and structure of the Turkish donkey populations. The primary objectives were to detect the main structural features of Turkish donkeys by microsatellite markers. A panel of 17 microsatellite markers was applied for genotyping 314 donkeys from 16 locations of Turkey. One hundred and forty-two alleles were identified and the number of alleles per locus ranged from 4 to 12. The highest number of alleles was observed in AHT05 (12) and the lowest in ASB02 and HTG06 (4), while ASB17 was monomorphic. The mean in the Turkish donkey was estimated to be 0.677, while mean was 0.675. The polymorphic information content (PIC) was calculated for each locus and ranged from 0.36 (locus ASB02) to 0.98 (locus AHT05), which has the highest number of alleles per locus in the present study. The average PIC in our populations was 0.696. The average coefficient of gene differentiation (G) over the 17 loci was 0.020 ± 0.037 ( < 0.01). The G values for single loci ranged from -0.004 for LEX54 to 0.162 for COR082. Nei's gene diversity index (H) for loci ranged from 0.445 (ASB02) to 0.890 (AHT05), with an average of 0.696. A Bayesian clustering method, the Structure software, was used for clustering algorithms of multi-locus genotypes to identify the population structure and the pattern of admixture within the populations. When the number of ancestral populations varied from K = 1 to 20, the largest change in the log of the likelihood function (K) was when K = 2. The results for K = 2 indicate a clear separation between Clade I (KIR, CAT, KAR, MAR, SAN) and Clade II (MAL, MER, TOK, KAS, KUT, KON, ISP, ANT, MUG, AYD and KAH) populations.
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http://dx.doi.org/10.3390/ani10061093DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7341297PMC
June 2020

A 20-year audit of retinoblastoma treatment outcomes.

Eye (Lond) 2020 10 6;34(10):1916-1924. Epub 2020 May 6.

Şanlıurfa Balıklıgöl State Hospital, Şanlıurfa, Turkey.

Objectives: To evaluate the long-term treatment outcomes in intraocular retinoblastoma (RB) including the associated factors for eventual treatment with external beam radiotherapy (EBRT) and enucleation as well as to analyse the risk factors for metastasis and death in extraocular RB.

Methods: Retrospective analysis of 390 eyes from 256 (89.8%) intraocular RB and 29 (10.2%) extraocular RB cases diagnosed and treated between October 1998 and May 2018 at one of the largest tertiary care centers in Turkey.

Results: Of 351 intraocular RB eyes, 53.3% had group D/E disease at presentation. 75 (21.4%) of 351 eyes underwent primary enucleation. Of the remaining 276 eyes undergoing eye-conserving treatments, 201 (72.8%) were salvaged. Most of these eyes were treated using intravenous chemotherapy and/or focal treatments [transpupillary thermotherapy (TTT) and cryotherapy] initially. EBRT was eventually required in 48 (17.4%) eyes and secondary enucleation in 75 (27.2%) eyes. At mean follow-ups of 76.7 and 39.7 months for intraocular and extraocular RB cohorts, respectively, 180 (46.2%) eyes underwent primary/secondary enucleation and exenteration. Overall, 13 cases developed metastasis and 9 died. Two patients with trilateral RB also expired. Multivariable risk factors for enucleation were the presence of vitreous seeds (p < 0.001), absence of EBRT administration (p = 0.033), 5-9 TTT applications compared with no TTT (p = 0.031), and each 1 mm increase in tumour base diameter (p < 0.001). Univariate factors predictive of metastasis were the presence of extraocular RB detected by imaging methods (p < 0.001) and extrascleral/optic nerve cut end involvement at histopathological examination (p < 0.001).

Conclusions: In our series, 72.8% of the intraocular RB eyes undergoing eye-conserving treatments were saved. The globe salvage rate for all intraocular and extraocular RB eyes was 53.8% and the overall survival rate was 96.1%.
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http://dx.doi.org/10.1038/s41433-020-0898-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7608123PMC
October 2020

Muramyl Tripeptide Plus Chemotherapy Reduces Metastasis in Non-Metastatic Osteosarcoma: A Single-Center Experience.

Asian Pac J Cancer Prev 2020 Mar 1;21(3):715-720. Epub 2020 Mar 1.

Division of Pediatric Hematology and Oncology, Department of Pediatrics, Gülhane Training and Research Hospital, Ankara, Turkey.

Background: The immunomodulator mifamurtide plus a chemotherapy regimen has been shown to significantly improve the outcome in non-metastatic osteosarcoma patients. We report the results of the addition of mifamurtide to chemotherapy in newly diagnosed patients with osteosarcoma.

Methods: A total of 36 children with osteosarcoma without detectable metastasis were treated between November 2010 and April 2018 at the Ankara University Department of Pediatric Oncology. Mifamurtide was added to the chemotherapy regimen in 17 patients while the remaining 19 did not receive mifamurtide. The probabilities of metastasis and overall survival were compared between the groups.

Results: The 43-month survival rate was 87.5% and 89.9% in the patients who received and did not receive mifamurtide, respectively (p=0.65). Common side effects of mifamurtide were chills and fever. The addition of mifamurtide in the high-risk group with ≤95% necrosis tended to decrease the probability of distant metastasis (36.4% vs. 58.3%) (p=0.39). The time to metastasis in the group with positive surgical margins (4 months in one patient in the non-mifamurtide group, 7 and 20 months in the mifamurtide group) was also longer in the mifamurtide group. During the 43-month follow up period, median time to metastasis was longer in the mifamurtide group (20 vs. 5 months). In addition, mifamurtide plus chemotherapy decreased the risk of metastasis in the cases with primary site relapse.

Conclusions: The addition of mifamurtide to chemotherapy might improve event-free survival by decreasing the probability of distant metastasis in bad histologic responders, and also by increasing the time to distant metastasis in the surgical margin positive group. Additional clinical studies are necessary to determine the long-term effects of mifamurtide on metastatic disease.
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http://dx.doi.org/10.31557/APJCP.2020.21.3.715DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7437342PMC
March 2020

Global Retinoblastoma Presentation and Analysis by National Income Level.

JAMA Oncol 2020 05;6(5):685-695

Imam Hussein Cancer Center, Karbala, Iraq.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.

Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis.

Design, Setting, And Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017.

Main Outcomes And Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis.

Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]).

Conclusions And Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.
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http://dx.doi.org/10.1001/jamaoncol.2019.6716DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7047856PMC
May 2020

A targeted salvage therapy with Brentuximab vedotin in heavily treated refractory or relapsed pediatric Hodgkin lymphoma patients before and after stem cell transplantation.

Turk J Pediatr 2019 ;61(5):671-676

Divisions of Pediatric Oncology, Ankara University Faculty of Medicine, Ankara, Turkey.

Taçyıldız N, Tanyıldız HG, Ünal E, Dinçaslan H, Asarcıklı F, Adaklı Aksoy B, Vatansever G, Yavuz G. A targeted salvage therapy with Brentuximab vedotin in heavily treated refractory or relapsed pediatric Hodgkin lymphoma patients before and after stem cell transplantation. Turk J Pediatr 2019; 61: 671-676. Hodgkin`s lymphoma (HL) is highly curable disease in its early stages, but in advanced stages, it presents a dilemma when it becomes refractory or relapses after several rounds of chemotherapy. Brentuximab vedotin (BV) is an antibody-drug conjugate that targets the tumor necrosis receptor family protein member CD30 positive malignancies via an anti-CD30 monoclonal antibody linked to monomethyl auristatin-E. In adult and pediatric studies, it has been shown to be an effective salvage therapy for primary refractory HL or relapse after autologous stem cell transplant (ASCT). Between July 2012 and August 2017, we administered BV (1.8 mg/m2 every three weeks; 12 cycles totally) with doxorubucin, vinblastin, dacarbazine (AVD), rituximab + ifosfamide + carboplatin + etoposide (RICE), or bendamustine combination treatment in pediatric HL patients, who were previosuly treated for refractory or relapsed advanced stage HL before (seven patients) or after (one patient) ASCT in our center. After eight BV courses, one patient was able to undergo match unrelated donor (MUD) SCT. Another seven pediatric HL patients, who were not able to go into remission with any other classical HL chemotherapy protocols, received 4-6 courses of BV-AVD and/or RICE/bendamustine. All were able to undergo ASCT after negative positron emission tomography (PET) imaging results. After ASCT, we switched to BV as consolidation therapy until a total of 12 cycles was completed. Patients went into remission after a median 34 (range: 12-42) months from the start of BV treatment. BV is an encouraging, well- tolerated, and effective targeted therapy especially when combined with AVD or when alternated with another targeted therapy combination, including RICE, when needed.
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http://dx.doi.org/10.24953/turkjped.2019.05.005DOI Listing
July 2020

Comparison of the efficiency of transgelin, smooth muscle myosin, and CD31 antibodies for the assessment of vascular tumor invasion and free tumor deposits in gastric, pancreatic, and colorectal adenocarcinomas.

Indian J Pathol Microbiol 2020 Jan-Mar;63(1):25-31

Department of Pathology, Faculty of Medicine, Gazi University, Ankara, Turkey.

Background: This study aimed to compare CD31, smooth muscle myosin (SMM), and transgelin antibodies for their efficiency in detecting venous invasion (VI) and the nature of free tumor deposits (TDs) in gastric, pancreatic, and colorectal adenocarcinomas.

Materials And Methods: Eleven Whipple, 5 gastrectomy, and 3 colectomy specimens and 1 low anterior resection specimen were reviewed and examined, revealing 254 probable foci. Foci were reviewed and divided into 3 types: Type A, the "orphan artery" pattern; Type F, free TDs in the periorgan adipose and connective tissue without an unaccompanied artery; and Type X, a focus that could be detected only with the immunohistochemical procedures mentioned.

Results: No foci were positive for CD31. Transgelin staining was more sensitive than SMM staining in all focus types, Type A only and Type F only (P < 0.001, P = 0.001, and P = 0.10, respectively). In free TDs (Type F), 35.7% of the samples were negative for all four stains, and 64.2% of the samples were positive for SMM and transgelin. We did not make the distinction between a metastatic lymph node and VI in positive foci.

Conclusion: We conclude that hematoxylin and eosin (H and E) staining is inadequate and that smooth muscle markers, such as transgelin and/or SMM, are more effective than endothelial markers, such as CD31, in revealing VI and lymph node/large extramural invasion.
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http://dx.doi.org/10.4103/IJPM.IJPM_109_19DOI Listing
October 2020

Bloodstream infections in pediatric hematology/oncology patients: Six years’ experience of a single center in Turkey

Turk J Med Sci 2019 08 8;49(4):1157-1164. Epub 2019 Aug 8.

Department of Pediatric Infectious Diseases, Ankara University Faculty of Medicine, Ankara, Turkey

Background/aim: Bloodstream infections are the major cause of morbidity, increased cost, prolonged hospitalization, and mortality in pediatric patients. Identifying the predominant microorganisms and antimicrobial susceptibilities in centers helps to select effective empirical antimicrobials which leads to positive clinical outcomes. We aimed to identify the causative microorganisms and their antimicrobial susceptibilities in patients with bloodstream infections.

Materials And Methods: Data belonging to patients with hematological and/or oncological diseases admitted to our hospital with fever between January 2010 and November 2015 were analyzed.

Results: In total, 71 patients who had 111 bloodstream infection episodes were included. Responsible pathogens were detected as follows: 35.1% gram-positive microorganisms, 60.5% gram-negative bacteria, and 4.4% fungi. The most common causative gram-negative pathogen was Escherichia coli and the most commonly isolated gram-positive microorganism was coagulase-negative staphylococci.

Conclusion: Gram-negative microorganisms were predominant pathogens in bloodstream infections. Escherichia coli and coagulase-negative staphylococci were the most commonly isolated responsible pathogens. Beta-lactam/lactamase inhibitors were suitable for empirical treatment. However, in critical cases, colistin could have been used for empirical treatment until the culture results were available. Routine glycopeptide use was not required. By identifying the causative microorganisms and their antimicrobial resistance patterns, it will be possible to obtain positive clinical results.
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http://dx.doi.org/10.3906/sag-1812-101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7018311PMC
August 2019

Is antibiotic lock therapy effective for the implantable longterm catheter-related bloodstream infections in children?

Turk J Pediatr 2019 ;61(6):895-904

Departments of Pediatric Infectious Diseases, Ankara University Faculty of Medicine, Ankara, Turkey.

Tural Kara T, Özdemir H, Erat T, Yahşi A, Aysev AD, Taçyıldız N, Ünal E, İleri T, İnce E, Haskoloğlu Ş, Çiftçi E, İnce E. Is antibiotic lock therapy effective for the implantable long-term catheter-related bloodstream infections in children? Turk J Pediatr 2019; 61: 895-904. Catheter-related bloodstream infections (CRBSIs) are an important problem in pediatric patients with central venous catheters. This study aimed to determine the incidence of CRBSIs, responsible pathogens and outcomes of antibiotic lock treatment (ALT) in pediatric patients. Between January 2010 and November 2015 all hospitalized pediatric hematology, oncology and immunology patients diagnosed with CRBSIs were retrospectively analyzed. Seventy-eight CRBSI episodes were detected in 60 pediatric patients. The incidence of CRBSIs was 4.20/1000 catheter days. The most frequently detected pathogen was methicillin-resistant coagulase-negative Staphylococcus. Pseudomonas aeruginosa, Klebsiella spp., and Escherichia coli were other commonly isolated microorganisms. ALT was administered in 42 patients. The success rate of ALT was 81% (34/42). Catheter was removed without ALT in 36 episodes. Common reasons for catheter removal were sepsis and causative microorganisms which had high probability of biofilm formation. CRBSIs are an important cause of morbidity and mortality in pediatric patients. ALT is safe and effective. It is possible to obtain satisfactory results when ALT is used with intravenous systemic antibiotics for CRBSIs, though in some cases catheter removal is necessary. ALT helps to prevent unnecessary catheter removal in pediatric patients.
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http://dx.doi.org/10.24953/turkjped.2019.06.011DOI Listing
August 2020

The Efficiency and Toxicity of Mifamurtide in Childhood Osteosarcoma.

J Pediatr Hematol Oncol 2018 08;40(6):e373-e376

Department of Pediatrics, Division of Pediatric Oncology, Faculty of Medicine, Ankara University.

The aim of the present study was to evaluate the efficiency and side effects of mifamurtide in childhood osteosarcoma (OS). In total, 477 doses of 2 mg/m intravenous (IV) mifamurtide, along with paracetamol as a premedication, were given to 15 patients with primary nonmetastatic OS after complete surgical resection and to 3 patients with progressive OS. The most common side effects encountered in the patients were chills and fever (17/18). These reactions were observed in 4 patients during the administration of each dose, in a single patient during the last administration, and in the remaining 12 patients during the first or initial 2 administrations. Headache, myalgia, and arthralgia were observed in 2 patients during each infusion. Headache was observed in 1 patient with additional hearing loss during the first 2 infusions. One patient had back pain occuring within the first infusion. Of the 15 patients with primary nonmetastatic OS and treated with the addition of mifamurtide to chemotherapy, 13 showed a complete remission, and 2 patients were still under treatment with a complete remission. Of 3 patients with progressive disease, 2 died while the disease progressed further in the third case over a 51-month period. The 3-year overall survival and event-free survival distributions were 87.5% (mean follow-up time, 46.12; 95% confidence interval, 37.79-52.45 mo) and 75.6% (mean follow-up time, 31.30; 95% confidence interval, 26.54-36.06 mo), respectively. We consider that mifamurtide therapy is a safe and well-tolerated agent in childhood OS.
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http://dx.doi.org/10.1097/MPH.0000000000001236DOI Listing
August 2018

New Insights on Water Buffalo Genomic Diversity and Post-Domestication Migration Routes From Medium Density SNP Chip Data.

Front Genet 2018 2;9:53. Epub 2018 Mar 2.

Department of Animal Science, Faculty of Agriculture, Namik Kemal University, Tekirdag, Turkey.

The domestic water buffalo is native to the Asian continent but through historical migrations and recent importations, nowadays has a worldwide distribution. The two types of water buffalo, i.e., river and swamp, display distinct morphological and behavioral traits, different karyotypes and also have different purposes and geographical distributions. River buffaloes from Pakistan, Iran, Turkey, Egypt, Romania, Bulgaria, Italy, Mozambique, Brazil and Colombia, and swamp buffaloes from China, Thailand, Philippines, Indonesia and Brazil were genotyped with a species-specific medium-density 90K SNP panel. We estimated the levels of molecular diversity and described population structure, which revealed historical relationships between populations and migration events. Three distinct gene pools were identified in pure river as well as in pure swamp buffalo populations. Genomic admixture was seen in the Philippines and in Brazil, resulting from importations of animals for breed improvement. Our results were largely consistent with previous archeological, historical and molecular-based evidence for two independent domestication events for river- and swamp-type buffaloes, which occurred in the Indo-Pakistani region and close to the China/Indochina border, respectively. Based on a geographical analysis of the distribution of diversity, our evidence also indicated that the water buffalo spread out of the domestication centers followed two major divergent migration directions: river buffaloes migrated west from the Indian sub-continent while swamp buffaloes migrated from northern Indochina via an east-south-eastern route. These data suggest that the current distribution of water buffalo diversity has been shaped by the combined effects of multiple migration events occurred at different stages of the post-domestication history of the species.
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http://dx.doi.org/10.3389/fgene.2018.00053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5841121PMC
March 2018

The prognostic importance of TGF-β, TGF-β receptor, and fascin in childhood solid tumors.

Pediatr Hematol Oncol 2017 May 24;34(4):238-253. Epub 2017 Oct 24.

a Department of Pediatric Oncology , Ankara University School of Medicine , Ankara , Turkey.

Fascin plays a role in tumor metastasis under the influence of TGF-β, each potentiating the effect of the other. We retrospectively investigated whether there was a prognostic relationship between TGF-β and fascin, and disease stage, local recurrence, metastasis tendency, and response to treatment. Twelve neuroblastomas, 17 osteosarcomas, 14 Ewing's sarcomas, 15 rhabdomyosarcoma cases, and 8 rare solid tumors were included. Serum TGF-β levels were high at the time of diagnosis in all groups (p = .015) and decreased significantly during remission (p = .008). Serum TGF-β values in the relapse period rarely reached high levels at the time of diagnosis and even stayed under the control group values (p = .017). When TGF-β receptor expression in tumor tissues was evaluated, the association of TGF-β receptor positivity with metastatic disease and advanced stage was striking. We found that 88% of rhabdomyosarcoma cases with alveolar histopathology expressed the TGF-β receptor, and the association between TGF-β receptor positivity and alveolar histopathology seemed to be a negative prognostic marker. When fascin levels were evaluated in childhood solid tumor tissue, the risk of relapse increased when the fascin total score at diagnosis was >4. This is one of the few studies including prognostic markers such as serum TGF-β, tissue TGF-β, TGF-β receptor, and fascin in pediatric solid tumors. Considering the poor prognosis of advanced stage pediatric solid tumors and the need for biomarkers to predict which patient might need more intensive therapy or warrant closer follow-up afterward, we think that TGF-β, TGF-β receptor, and fascin expression have an important prognostic role.
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http://dx.doi.org/10.1080/08880018.2017.1363838DOI Listing
May 2017

Treatment of high-risk neuroblastoma: National protocol results of the Turkish Pediatric Oncology Group.

J Cancer Res Ther 2017 Apr-Jun;13(2):284-290

Department of Pediatric Oncology, Dokuz Eylul University Institute of Oncology, Izmir, Turkey.

Background: The national protocol aimed to improve the outcome of the high risk neuroblastoma patients by high-dose chemotherapy and stem cell rescue with intensive multimodal therapy.

Materials And Methods: After the 6 induction chemotherapy cycles, patients without disease progression were nonrandomly (by physicians' and/or parent's choices) allocated into two treatment arms, which were designed to continue the conventional chemotherapy (CCT), or myeloablative therapy with autologous stem cell rescue (ASCR).

Results: Fifty-six percent (272 patients) of patients was evaluated as high risk. Response rate to induction chemotherapy was 71%. Overall event-free survival (EFS) and overall survival (OS) at 5 years were 28% and 36%, respectively. "As treated" analysis documented postinduction EFS of 41% in CCT arm (n = 138) and 29% in ASCR group (n = 47) (P = 0.042); whereas, OS was 45% and 39%, respectively (P = 0.05). Thirty-one patients (11%) died of treatment-related complications.

Conclusion: Survival rates of high-risk neuroblastoma have improved in Turkey. Myeloablative chemotherapy with ASCR has not augmented the therapeutic end point in our country's circumstances. The adequate supportive care and the higher patients' compliance are attained, the better survival rates might be obtained in high-risk neuroblastoma patients received myeloablative chemotherapy and ASCR.
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http://dx.doi.org/10.4103/0973-1482.183205DOI Listing
April 2018

An unusual case of neuroblastoma: a 17-year-old adolescent presented with bilateral diffuse lung metastasis at initial diagnosis.

Turk J Pediatr 2016 ;58(1):86-89

Divisions of Pediatric Oncology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.

Neuroblastoma (NB) is the most frequently diagnosed neoplasm during infancy and its incidence declines within the first 3-5 years of life. It can be rarely diagnosed in adolescents and young adults. Adolescents have advanced stage of disease, higher frequency of uncommon metastatic sites such as lungs, and worse outcomes. Herein, we describe an unusual case of NB in a 17-year-old adolescent presented with lung metastasis at diagnosis. The patient was diagnosed with stage IV NB. Thorax high-resolution computed tomography (HRCT) scan revealed irregular septal thickening with ground glass opacity consistent with pulmonary parenchymal metastases. After the first cycle of chemotherapy he developed pulmonary hemorrhage and respiratory distress. He required ventilation support and mechanical ventilation was started. Metastatic nodules were determined on second thorax HRCT. We lost the patient due to septic shock and multiple organ failure 2 months after diagnosis. In conclusion, adolescents with NB have unfavorable prognosis. These patients may have lung metastases at diagnosis. Therefore, detailed chest imaging at initial diagnosis is crucial.
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http://dx.doi.org/10.24953/turkjped.2016.01.012DOI Listing
June 2017

Chemotherapy in Retinoblastoma: Current Approaches.

Turk J Ophthalmol 2015 Dec 5;45(6):259-267. Epub 2015 Dec 5.

Ankara University Faculty of Medicine, Department of Pediatrics, Ankara, Turkey.

Retinoblastoma (RB) is the most common childhood malignant intraocular tumor. Although enucleation and external beam radiotherapy have been historically used, today the most commonly used eye-sparing approach is chemotherapy. Chemotherapy can be used in both intraocular and extraocular RB cases. Chemotherapeutic agents may be applied in different ways, including systemic, subconjunctival, intra-arterial and intravitreal routes. The main purposes of application of systemic therapy are to reduce the tumor size for local treatment (chemoreduction), or to reduce the risk of metastasis after enucleation surgery (adjuvant therapy). Intra-arterial chemotherapy with the current name "super-selective intra-arterial infusion therapy" could be applied as primary therapy in tumors confined to the retina or as a secondary method in tumor recurrence. The most important advantage of intra-arterial therapy is the prevention of systemic chemotherapy complications. Intravitreal chemotherapy is administered in the presence of persistent or recurrent vitreous seeding. The term "extraocular RB" includes orbital invasion and metastatic disease. Current treatment for orbital invasion is neoadjuvant chemotherapy followed by surgical enucleation and adjuvant chemotherapy and radiotherapy after surgery. In metastatic disease, regional lymph node involvement, distant metastases, and/or central nervous system (CNS) involvement may occur. Among them, CNS involvement has the worst prognosis, remaining at almost 100% mortality. In metastatic disease, high-dose salvage chemotherapy and autologous hematopoietic stem cell rescue therapy are the possible treatment options; radiotherapy could also be added to the protocol according to the side of involvement.
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http://dx.doi.org/10.4274/tjo.06888DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5082265PMC
December 2015

Lymphoma Secondary to Congenital and Acquired Immunodeficiency Syndromes at a Turkish Pediatric Oncology Center.

J Clin Immunol 2016 10 4;36(7):667-76. Epub 2016 Aug 4.

Department of Pediatric Oncology, Ankara University Faculty of Medicine, Ankara, Turkey.

The prevalence of lymphoma in primary immunodeficiency cases and autoimmune diseases, as well as on a background of immunodeficiency following organ transplants, is increasing. The lymphoma treatment success rate is known to be a low prognosis. Our study aimed to emphasize the low survival rates in immunodeficient vs. immunocompetent lymphoma patients and also to investigate the effect of rituximab in patients with ataxia telangiectasia and other immunodeficiencies. We summarized the clinical characteristics and treatment results of 17 cases with primary immunodeficiency that developed non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) retrospectively. Seven patients were diagnosed with ataxia-telangiectasia, two with common variable immunodeficiency, two with selective IgA deficiency, one with X-related lymphoproliferative syndrome, one with Wiskott-Aldrich syndrome, one with Epstein-Barr virus-related lymphoproliferative syndrome, one with interleukin-2-inducible T-cell kinase (ITK) deficiency, and one with lymphoma developing after autoimmune lymphoproliferative syndrome (ALPS). One patient underwent a renal transplant. Of the nine males and eight females (aged 3-12 years, median = 7) that developed lymphoma, seven were diagnosed with HL and ten with NHL (seven B-cell, three T-cell). The NHL patients were started on the Berlin-Frankfurt-Münster, POG9317, LMB-96, or R-CHOP treatment protocols with reduced chemotherapy dosages. HL cases were started on the doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) and/or cyclophosphamide, vincristine, procarbazine, and prednisone (COPP) protocol, also with modified dosages. Importantly, all seven cases of HL are alive and in remission, while six of the ten NHL patients have died. Primary immunodeficiency is a strong predisposing factor for developing lymphoma. Low treatment success rates relative to other lymphomas and difficulties encountered during treatment indicate that new treatment agents are needed. While some success has been achieved by combining rituximab with lymphoma treatment protocols in B-NHL cases with primary immunodeficiency, the need for new treatment approaches for these patients remains critical.
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http://dx.doi.org/10.1007/s10875-016-0324-zDOI Listing
October 2016

Can Fluorine-18-Fluorodeoxyglucose Positron Emission Tomography Be Used As a Useful Method to Evaluate the Treatment Response to Neoadjuvant Therapy Combined With Sorafenib and Anti-VEGF in Children Diagnosed With Metastatical Bone Sarcoma?

Iran J Pediatr 2016 Apr 5;26(2):e4008. Epub 2016 Mar 5.

Department of Orthopedics, School of Medicine, Ankara University, Ankara, Turkey.

Background: The prognosis is still poor for patients with a metastatic bone tumor and new treatment approaches (anti-VEGF and tyrosine kinase inhibitors vs) are therefore needed.

Objectives: The aim of our study was to evaluate how the primary and metastatic lesions of our patients with a bone tumor were affected by these treatments and to determine the importance of the 18F-FDG PET method.

Patients And Methods: Twenty metastatic bone tumor cases were included. Sorafenib and anti-VEGF were added to the standard treatment in cases with widespread metastatic disease at diagnosis or after neoadjuvant chemotherapy showing less than 90% tumor necrosis in the surgical sample. Positron emission tomography (PET) imaging was performed at diagnosis, the preoperative period following neoadjuvant chemotherapy, during postoperative follow-up, and when treatment was discontinued.

Results: The primary treatment region median SUVmax level decreased from 7.35 to 2.5 in the living patients (n = 16) while there was no significant decrease in the patients who succumbed to the disease (P < 0.001). Comparison of the pre- and post-treatment metastasis region median SUVmax levels in patients with metastatic involvement showed a decrease from 2.1 to 0 in the surviving patients but only from 4.8 to 3.2 in the deceased patients (P < 0.01). Survival results indicated that 28.6% of the patients receiving classical treatment only died while all the patients receiving additional sorafenib and anti-VEGF survived.

Conclusions: 18F-PET may be a useful technique before and during the follow-up of neoadjuvant treatment in pediatric metastatic bone tumor patients. The addition of sorafenib and anti-VEGF to classical treatment has a favorable contribution to the response and therefore the survival duration.
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http://dx.doi.org/10.5812/ijp.4008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4904339PMC
April 2016

Prognostic Factors and a New Prognostic Index Model for Children and Adolescents with Hodgkin's Lymphoma Who Underwent Autologous Hematopoietic Stem Cell Transplantation: A Multicenter Study of the Turkish Pediatric Bone Marrow Transplantation Study Group.

Turk J Haematol 2016 Dec 18;33(4):265-272. Epub 2016 Apr 18.

Gülhane Training and Research Hospital Clinic of Pediatric Oncology, Ankara, Turkey Phone: +90 312 304 43 94 E-mail:

Objective: The prognostic factors and a new childhood prognostic index after autologous hematopoietic stem cell transplantation (AHSCT) in patients with relapsed/refractory Hodgkin's lymphoma (HL) were evaluated.

Materials And Methods: The prognostic factors of 61 patients who underwent AHSCT between January 1990 and December 2014 were evaluated. In addition, the Age-Adjusted International Prognostic Index and the Childhood International Prognostic Index (CIPI) were evaluated for their impact on prognosis.

Results: The median age of the 61 patients was 14.8 years (minimum-maximum: 5-20 years) at the time of AHSCT. There were single relapses in 28 patients, ≥2 relapses in eight patients, and refractory disease in 25 patients. The chemosensitivity/chemorefractory ratio was 36/25. No pretransplant radiotherapy, no remission at the time of transplantation, posttransplant white blood cell count over 10x103/µL, posttransplant positron emission tomography positivity at day 100, and serum albumin of <2.5 g/dL at diagnosis were correlated with progression-free survival. No remission at the time of transplantation, bone marrow positivity at diagnosis, and relapse after AHSCT were significant parameters for overall survival.

Conclusion: The major factors affecting the progression-free and overall survival were clearly demonstrated. A CIPI that uses a lactate dehydrogenase level of 500 IU/L worked well for estimating the prognosis. We recommend AHSCT at first complete remission for relapsed cases, and it should also be taken into consideration for patients with high prognostic scores at diagnosis.
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http://dx.doi.org/10.4274/tjh.2015.0280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5204179PMC
December 2016

Herlyn-Werner-Wunderlich Syndrome: A Rare Cause of Pelvic Pain and High CA 19-9 Levels in an Adolescent Girl.

APSP J Case Rep 2016 Jan-Apr;7(1). Epub 2016 Jan 1.

Department of Radiology, Ankara University School of Medicine, Ankara, Turkey.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare developmental anomaly that includes uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. A 13-year-old girl presented with chronic abdominal pain. Magnetic resonance imaging revealed uterus didelphys, hematometrocolpos and renal agenesis on the right side with imperforate hymen. Subsequently the patient was found to have Mullerian duct anomalies. CA 19-9 level was high. At laparoscopy combined with vaginoscopy hematocolpos was drained following which she improved clinically and CA 19-9 level returned to normal.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4715886PMC
January 2016

Serum Basic Fibroblastic Growth Factor Levels in Children with Infantile Hemangioma.

Indian J Pediatr 2016 Sep 27;83(9):937-40. Epub 2016 Jan 27.

Division of Biostatistics, Cebeci Medical Faculty Ankara, Ankara University, Ankara, Turkey.

Objectives: To determine serum levels of basic fibroblastic growth factor (b-FGF) in hemangioma patients under 2 y of age.

Methods: The study group consisted of 43 children with infantile hemangioma and b-FGF levels were analyzed using ELISA.

Results: The serum b-FGF levels were higher in hemangioma patients than in healthy control individuals (p 0.01). There were no differences between the lesion size, number of lesions, patient age and serum b-FGF levels.

Conclusions: Thus, b-FGF is an important growth factor that plays a central role in hemangioma, but determining b-FGF serum levels was not helpful in distinguishing between patients who require treatment and those who do not.
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http://dx.doi.org/10.1007/s12098-016-2041-2DOI Listing
September 2016

Secondary Neoplasms in Children with Hodgkin's Lymphoma Receiving C-MOPP and Radiotherapy: Presentation of Four Cases.

Turk J Haematol 2016 Mar 6;33(1):66-70. Epub 2015 Aug 6.

Kemer Sk. No: 21/78 Büyükesat, Ankara, Turkey. E-mail:

Patients who survive Hodgkin lymphoma (HL) are at increased risk of secondary neoplasms (SNs). A wide variety of SNs have been reported, including leukemias, non-Hodgkin's lymphomas, and solid tumors, specifically breast and thyroid cancers. Herein we report subsequent neoplasms in four patients with HL receiving chemoradiotherapy. It is interesting that three SNs, fibrosarcoma, thyroid carcinoma, and retrobulbar meningioma, were observed in the radiation area in one of our patients. A hypopharyngeal epithelioid malignant peripheral nerve sheath tumor as an unusual secondary malignant neoplasm developed in another patient, while a benign thyroid nodule and invasive ductal breast carcinoma were observed at different times in the female patient. Follicular adenoma of the thyroid gland developed in one of our patients.
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http://dx.doi.org/10.4274/tjh.2015.0027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4805347PMC
March 2016

Outcome of autologous hematopoietic stem cell transplantation in children and adolescents with relapsed or refractory Hodgkin's lymphoma.

Pediatr Transplant 2015 Nov 8;19(7):745-52. Epub 2015 Sep 8.

Pediatric BMT Units, Bahcesehir University Faculty of Medicine Medical Park Goztepe Hospital, Istanbul, Turkey.

This study evaluates the outcome of 66 pediatric patients with rrHL who underwent autoHSCT. Twenty-nine patients experienced early relapse, and 19 patients experienced late relapse. Of 18 newly diagnosed with HL, 13 were primary refractory disease and five had late responsive disease. At the time of transplantation, only 68% of the patients were chemosensitive. The majority of patients received BCNU + etoposide + ara-C + melphalan for conditioning (45/66), and peripheral blood (56/66) was used as a source of stem cells. After a median follow-up period of 39 months, 46 patients were alive. At five yr, the probabilities of OS, EFS, the relapse rate, and the non-relapse mortality rate were 63.1%, 54.3%, 36.4%, and 9.1%, respectively. The probability of EFS in chemosensitive and chemoresistant patients at five yr was 72.3% and 19%, respectively (p < 0.001). Multivariate analysis showed that chemoresistant disease at the time of transplantation was the only factor predicting limited both OS (hazard ratio = 4.073) and EFS (hazard ratio = 4.599). AutoHSCT plays an important role for the treatment of rrHL in children and adolescents, and survival rates are better for patients with chemosensitive disease at the time of transplantation.
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http://dx.doi.org/10.1111/petr.12573DOI Listing
November 2015

Challenges and differences in external radiation therapy for retinoblastoma: from standard techniques to new developments.

Tumori 2017 Sep 28;103(5):438-442. Epub 2015 Aug 28.

Department of Radiation Oncology, Ankara University School of Medicine, Cebeci Hospital, Ankara - Turkey.

Aims: The purpose of this study is to calculate the treatment plans and to compare the dose distributions and dose-volume histograms (DVH) for 6 external radiotherapy techniques for the treatment of retinoblastoma as well as intensity-modulated radiotherapy (IMRT) and fractionated stereotactic radiotherapy (Cyberknife).

Methods: Treatment plans were developed using 6 techniques, including an en face electron technique (ET), an anterior and lateral wedge photon technique (LFT), a 3D conformal (6 fields) technique (CRT), an inverse plan IMRT, tomotherapy, and conventional focal stereotactic external beam radiotherapy with Cyberknife (SBRT). Dose volume analyses were carried out for each technique.

Results: All techniques except electron provided similar target coverage. When comparing conformal plan with IMRT and SBRT, there was no significant difference in planning target volume dose distribution. The mean volume of ipsilateral bony orbit received more than 20 Gy, a suggested threshold for bone growth inhibition. The V20 Gy was 73% for the ET, 57% for the LFT, 87% for the CRT, 65% for the IMRT, 66% for the tomotherapy, and 2.7% for the SBRT.

Conclusions: This work supports the potential use of IMRT and SBRT to spare normal tissues in these patients.
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http://dx.doi.org/10.5301/tj.5000406DOI Listing
September 2017

The seroprevalence of Kaposi's sarcoma associated herpes virus and human herpes virus-6 in pediatric patients with cancer and healthy children in a Turkish pediatric oncology center.

Indian J Med Paediatr Oncol 2014 Jul;35(3):221-5

Department of Biostatistics, Ankara University Medical School, Ankara, Turkey.

Background: Many studies have tried to be establish a pathogenic role for human herpesvirus-6 and -8 (HHV-6, HHV-8) in malignant diseases, but whether these viruses plays a role in these pathologies remains unclear. HHV-6 and HHV-8 seropositivity were shown in a healthy population. There is no published data in Turkey about seroprevalence of these viruses. We aimed to determine the seroprevalence of HHV-6 and HHV-8 in pediatric cancer patients and to compare with healthy Turkish children's viral seroprevalence.

Patients And Methods: Ninety-three pediatric cancer patients and 43 age-matched healthy children were included in the study. All sera were screened for antibodies to HHV-6 and HHV-8 by ELISA.

Results: HHV-8 immunoglobulin G (IgG) was positive in 3.3% of lymphoma patients, in 4.8% of acute lymphoblastic leukemia (ALL) patients, in 4.8% of retinoblastoma patients and in 7% of healthy children. There was no significant difference in HHV-8 seroprevelance between these groups. HHV-6 seroprevalence was 81% in ALL patients, 70% in lymphoma group, 81% in retinoblastoma patients and 69.8% in healthy children. Although there was no significant difference in HHV-6 prevalence between healthy children and pediatric cancer patients, HHV-6 seropositivity tended to be higher in retinoblastoma patients under age of 4 years (odds ratio: 2.925).

Conclusion: HHV-6 seroprevalence was higher than HHV-8 seropositivity in our study. Viral studies related HHV-6 seroprevelance in retinoblastoma patients would be useful to clarify if there is any etiological association between HHV-6 and retinoblastoma.
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http://dx.doi.org/10.4103/0971-5851.142039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4202619PMC
July 2014

The evaluation of thyroid carcinoma in childhood and concomitance of autoimmune thyroid disorders.

J Pediatr Endocrinol Metab 2014 Sep;27(9-10):901-8

Autoimmune thyroiditis has been suggested as a precancerous condition in some adult studies, but there is still controversy. The importance of autoimmune thyroiditis in childhood thyroid cancer is not yet completely clear. We aimed to evaluate in this study the characteristics of childhood thyroid cancer in patients particularly in terms of coexisting factors including autoimmune thyroid disorders (ATD). Twenty patients diagnosed with primary thyroid cancer were evaluated retrospectively in a Pediatric Endocrinology clinic for 10 years. Patients were followed up for 57.22±11 months. Concomitant conditions (thyroidal and/or extra thyroidal) were determined. Most of the patients (80%) had a coexisting factor. ATDs are the most frequently encountered among them (40%). The ages at the time of diagnosis were older; and the tumor sizes were smaller in patients with concomitant ATDs than without autoimmune thyroid disorders. The follow-up characteristics were similar in both groups. In conclusion, ATDs are frequently encountered in association with thyroid cancer during childhood and adolescence. A thyroid autoimmunity may facilitate the development of a malignant thyroid tumor; on the other hand, increased attention to the thyroid gland may facilitate frequent diagnosis of thyroid cancer. A close follow-up of ATD patients should also include the evaluation of the development of thyroid malignancy.
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http://dx.doi.org/10.1515/jpem-2013-0273DOI Listing
September 2014

Rare childhood tumors in a Turkish pediatric oncology center.

Indian J Med Paediatr Oncol 2013 Oct;34(4):264-9

Department of Pediatric Endocrinology, Ankara University, Ankara, Turkey.

Background: It has been estimated that rare tumor rate is about 15% of all childhood cancer in United States. According to Turkish Pediatric Oncology Group (TPOG) datas, 8889 children were diagnosed between 2002 and 2008 in our country and 3.7% of them were diagnosed as rare tumors.

Aim: To investigate the frequency and clinical features of rare tumors in our pediatric oncology center.

Materials And Methods: A total of 43 cases that have diagnosed as rare tumor in 574 cancer patients between the yaer 2002 and 2012 were reviewed retrospectively. All cases definitive diagnosis were established by histopathological and immunohistochemical studies.

Results: Frequency of rare tumors was 7.4% in our center. Benign and border line rare tumors were 27 (62.7%) cases, malignant rare tumor were 16 (37.2%) cases. Median follow-up period was 48 months (between 1 and 110 months). Six of the malignant rare tumors were died with progressive disease (synovial sarcoma, mixed malignant mesenchymal tumor, undifferentiated sarcoma, plexus choroideus carcinoma, renal peripheral primitive neuroectodermal tumor, adrenocortical carcinoma). Malignant rare tumor mortality rate was found 37.5% in our clinic.

Conclusion: We have found that our rare tumor rate (7.4%) was higher than Turkish rare tumor rate (3.7%) according to TPOG's datas. However, it was still lower than rare tumor rates of western countries (15%), probably due to difficulties of diagnosis and referral problems.
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http://dx.doi.org/10.4103/0971-5851.125241DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3932593PMC
October 2013