Publications by authors named "Cengiz Bayram"

33 Publications

Glucose 6 phosphate dehydrogenase deficiency: A single-center experience.

Turk Arch Pediatr 2021 1;56(3):245-248. Epub 2021 May 1.

Pediatric Hematology and Oncology Clinic, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey.

Objective: This study aims to evaluate the demographic information, clinical and laboratory findings of patients with glucose 6 phosphate dehydrogenase deficiency.

Material And Methods: We collected data by reviewing files and electronic records of 65 patients with glucose 6 phosphate dehydrogenase deficiency under the age of 18 years who were followed up in our clinic between 2007 and 2019. Demographic, clinical, and laboratory features, family history, complications of the disease, and history of splenectomy and cholecystectomy were evaluated. Mean, standard deviation, and median values were used when descriptive analyses were presented.

Results: The age of diagnosis ranged between 1-192 months and the median age of diagnosis was two months. Fifty-nine patients (90.7%) were boys and six (9.2%) were girls. The mean value of glucose 6 phosphate dehydrogenase enzyme on admission was 1,9±1,4 U/g of hemoglobin (Hb). Family history was pesent in 40% of patients in whom information was avaliable. The most common presentation was prolonged jaundice and the most common physical finding was jaundice. Splenomegaly was detected in none of the patients. Cholelithiasis was present in one of 21 patients who were evaluated with ultrasonography. None of the patients required splenectomy, cholecystectomy, and regular erythrocyte transfusion during follow-up.

Conclusion: As G6PD variants with chronic hemolysis are not usually seen in Turkey, patients who required splenectomy, cholecystectomy, and regular erythrocyte transfusion were not detected. Although glucose 6 phosphate dehydrogenase deficiency is more common in males, it can also be seen in girls. In Turkey, glucose 6 phosphate dehydrogenase deficiency should be considered in patients presenting with prolonged jaundice.
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http://dx.doi.org/10.5152/TurkArchPediatr.2021.20042DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8152643PMC
May 2021

Clinical Features and Outcome of Children With Hereditary Spherocytosis.

J Pediatr Hematol Oncol 2021 May 31. Epub 2021 May 31.

Department of Pediatrics Division of Pediatric Hematology and Oncology, Kanuni Sultan Süleyman Training and Research Hospital Division of Pediatric Hematology and Oncology, Istinye University Medical Faculty, Istanbul, Turkey.

Objective: 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.
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http://dx.doi.org/10.1097/MPH.0000000000002211DOI Listing
May 2021

Diagnosis, Management, and Challenges in Synchronous Bilateral Wilms Tumor in a Horseshoe Kidney: A Case Report.

J Pediatr Hematol Oncol 2021 Apr 21. Epub 2021 Apr 21.

Departments of Pediatric Hematology and Oncology Pediatric Surgery Pediatric Radiology, Basaksehir Cam and Sakura City Hospital, Istanbul, Turkey.

Horseshoe kidney (HK) refers to a congenital malformation that results from fusion of both the kidneys at one pole, and is the most common renal fusion defect with an incidence of 1 in 400 to 600 individuals. Synchronous bilateral development of Wilms tumor (WT) in an HK is extremely rare. Here, we present a case of synchronous bilateral WT in an HK in an 18-month-old girl. The patient received 12 weeks of preoperative chemotherapy followed by 2-step surgical resection including nephron-sparing surgery (NSS) in both kidneys and left nephrectomy because of positive surgical margin and adjuvant chemotherapy. The patient is still in remission and asymptomatic 6 months after the completion of treatment. In this report, we discuss the treatment modalities of synchronous bilaterally located WT in HK. We conclude that successful outcomes can be obtained with preoperative chemotherapy and NSS in such cases even in the presence of advanced disease and complex anatomic conditions. In addition, the deepest tumor point can be reached during NSS, but total nephrectomy should be considered regarding the possibility of microscopic residue.
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http://dx.doi.org/10.1097/MPH.0000000000002165DOI Listing
April 2021

Safety and efficacy of deferasirox in patients with transfusion-dependent thalassemia: A 4-year single-center experience.

Pediatr Hematol Oncol 2021 Mar 22:1-8. Epub 2021 Mar 22.

Pediatric Hematology Oncology, Kanuni Sultan Suleyman Training and Research Hospital, Istanbul, Turkey.

This study was organized to determine the efficacy and safety of deferasirox (DFX) in reducing the SF of patients with transfusion-dependent thalassemia (TDT). This is a retrospective, descriptive study of 101 transfusion- dependent patients with thalassemia major who were followed for 48 months. Twenty-nine patients who used an alternative chelator either alone or combined, who were not compliant to the treatment, changed the drug due to adverse reactions, and had multiple transfusions and did not complete 4 years of DFX use were excluded. A total 72 out of 101 patients completed the study. SF decreases were noted for the 6-12 and >18-year age groups, from a median of 1532 ng/mL to 1190 ng/mL, and from 1386 ng/mL to 1165 ng/mL, respectively (p > 0.05). The proportion of patients with SF concentrations >2000 ng/mL is decreased (29% at baseline decreased to 15% at the end of the study) during the 48 months. The median SF of those who used <30 mg/kg/day (n = 38) increased from 767 ng/mL to 1006 ng/mL, whereas the >30 mg/kg/day (n = 34) group's SF concentrations decreased from a median of 1575 ng/mL to 1209 ng/mL (p = 0.029). The decrease of median SF values for Syrian patients was statistically significant (p = 0.043). Most common adverse events were gastric irritation symptoms (19.4%). The total DFX discontinuation ratio was calculated as 9.7%. Although dosages between 25-30 mg/kg/day are adequate to stabilize SF concentrations higher dosages are needed to achieve a statistically significant decrease.
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http://dx.doi.org/10.1080/08880018.2021.1901809DOI Listing
March 2021

The Use of Social Network in Daily Pediatric Practice and Education: Turkish Pediatric Atelier.

Int J Pediatr 2020 21;2020:7301309. Epub 2020 Sep 21.

Memorial Ataşehir Hospital, Dept. of Pediatrics, Vedat Günyol Cd. No. 28, 34758 Ataşehir, İstanbul, Turkey.

Using social media applications in pediatric education is not outdated, and its effectiveness has not been tested yet. For this reason, we shared the first results of the Pediatric Atelier experience that we realized through telegram application. We make an online survey to investigate the needs, requirements, pleasure, and suggestions of members through a web-based questionnaire. This cross-sectional survey study was delivered only to participants who were members of the workshop via their email addresses. Online questionnaires organized using Google Forms were sent to pediatric workshop members between March and June 2019. The questionnaire consisted of questions that measured the participants' basic demographic data, the use of the workshop, and the overall impact of the workshop on their professional behavior. While the institutions and positions of the participants were recorded, no other personal data (such as address and telephone) were collected. Among the 997 members, 417 (42%) of them answered the questionnaire. Respondents included 300 (72%) pediatrician, 21 (5%) pediatric subspeciality fellows, and 75 (18%) pediatric subspecialists. Of the 417 respondents, 217 (52%) were working in Istanbul, and 200 (48%) were working in other cities of Turkey. Among the responders, 233 (56%) were working in private hospitals or doctor offices. A total of 520 cases were consulted in 241 days of study period. Most consultations ( = 309, %59) were made from the Istanbul metropolitan area, and 203 (40%) consultations were from other cities of Turkey. The most frequently consulted departments were Pediatric infectious diseases: 166 (32%), Pediatric hematology and oncology: 56 (11%), and Neonatology: 43 (8%). Of the 520 consulted cases, 44 (8%) were related to life-threatening events, and 25 of them were hospitalized in the intensive care units, and 6 of them were required surgical operations. Of the 94% of responders thought this platform was useful and 82% of them stated that the case counseling part of the atelier was the most useful part. We think that the development of technology and artificial intelligence may lead to the usage of on-line platforms or systems in clinical medical practice. . Registry name, registration number, web link to study on registry, and data sharing statement.
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http://dx.doi.org/10.1155/2020/7301309DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7528139PMC
September 2020

Flow Cytometry-based Absolute Blast Count on Day 8: Reliable, Fast, and Inexpensive Method.

J Pediatr Hematol Oncol 2021 01;43(1):33

Clinic of Pediatric Hematology/Oncology Istanbul Kanuni Sultan Suleyman Research and Education Hospital, Istanbul, Turkey.

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http://dx.doi.org/10.1097/MPH.0000000000001838DOI Listing
January 2021

Thiol Disulfide Homeostasis and Ischemia-modified Albumin Level in Children With Beta-Thalassemia.

J Pediatr Hematol Oncol 2019 10;41(7):e463-e466

Department of Clinical Biochemistry, Faculty of Medicine, Yildirim Beyazit University, Ankara, Turkey.

Objective: It is well known that increased oxidative stress leads to tissue damage in beta-thalassemia (β-thal) patients. Thiols are one of the most important antioxidant agents, and thiol/disulfide (SH/SS) homeostasis is a novel oxidative stress marker. This study aimed to investigate the relationship of thiol levels, SH/SS homeostasis, and ischemia-modified albumin (IMA) in patients with β-thal.

Materials And Methods: A hundred transfusion-dependent β-thal patients and 41 healthy controls were included in the study.

Results: Native thiol, total thiol, disulfide, catalase, and IMA levels were significantly higher in the β-thal group compared with the control group (P<0.02). There were no correlation between serum ferritin level and SH/SS homeostasis, and weak positive correlations were found between serum ferritin and IMA (r=0.242, P=0.022).

Conclusions: Our study results suggest that antioxidant systems try to compensate for peroxidative damage in the patients' group and serum IMA level was found increased because of increased oxidative status. To the best of our knowledge, there has been no report evaluating plasma dynamic SH/SS homeostasis in β-thal patients.
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http://dx.doi.org/10.1097/MPH.0000000000001535DOI Listing
October 2019

Variant Guillain-Barré syndrome in a patient with Hodgkin lymphoma: AMSAN.

Turk Pediatri Ars 2018 Dec 1;53(4):263-266. Epub 2018 Dec 1.

Pediatric Hematology and Oncology Clinic, Kanuni Sultan Süleyman Training and Research Hospital, İstanbul, Turkey.

Lymphomas are solid tumors characterized by the malignant proliferation of lymphoid cells. Neurologic signs encountered in patients with Hodgkin's lymphoma can be due to the direct spread of tumor to the nervous system, secondary to chemotherapy or radiation, secondary to tumor mass compression, infectious causes and paraneoplastic syndromes. Paraneoplastic neurologic syndromes are rarely encountered in patients with Hodgkin's lymphoma and non-Hodgkin's lymphoma. Except for paraneoplastic cerebellar degeneration in Hodgkin's lymphoma and dermato/polymyositis in both Hodgkin's lymphoma and non-Hodgkin's lymphoma, other paraneoplastic syndromes are uncommon and have only been reported as isolated case reports or short series. Here, we present a patient with Hodgkin's lymphoma with symptoms of bilateral lower extremity weakness and loss of sensation before the start of therapy, who was eventually diagnosed as having motor and sensory axonal neuropathy.
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http://dx.doi.org/10.5152/TurkPediatriArs.2018.4763DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6408190PMC
December 2018

Refugee children with beta-thalassemia in Turkey: Overview of demographic, socioeconomic, and medical characteristics.

Pediatr Blood Cancer 2019 05 1;66(5):e27636. Epub 2019 Feb 1.

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

Aim: Since the beginning of the Syrian civil war, more than 3.5 million Syrians have been under temporary protection status in Turkey. Because beta-thalassemia (BT) is a prevalent disorder in the Mediterranean countries, we decided to estimate the prevalence of and make an overview of the demographic, socioeconomic, medical characteristics, and healthcare problems of refugee children with BT.

Patients: Eighteen Turkish Pediatric Hematology Oncology Centers (PHOC) with 318 refugee children from 235 families participated in the study. The mean age of the patients was 8.1 ± 4.8 years (0.5-21 years). The mean time after immigration to Turkey was 2.5 ± 1.5 years (range, 0.1-7 years). Seventy-two (22.6%) of them were born and diagnosed with BT in Turkey. On physical examination, 82 patients (26%) were underweight and 121 patients (38%) were stunted. The appearance of a thalassemic face was reported for 207 patients (65.1%). Hepatomegaly and splenomegaly were reported in 217 (68.2%) and 168 (52.8%) patients, respectively. The median ferritin level was 2508 ng/mL (range, 17-21 000 ng/mL) at the first admission, and 2841 ng/mL (range, 26-12 981 ng/mL) at the last visit after two years of follow-up in a PHOC (P > 0.05). The most frequently encountered mutation was IVSI-110 (G>A) (31%). Before immigration, only 177 patients (55.6%) reported the use of chelators; after immigration it increased to 268 (84.3%).

Conclusion: Difficulties in communication, finding a competent translator capable in medical terminology, nonregular use of medications, and insensitivity to prenatal diagnosis were preliminary problems. The current extent of migration poses emerging socioeconomic and humanitarian challenges for refugee patients with BT.
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http://dx.doi.org/10.1002/pbc.27636DOI Listing
May 2019

Causes of Hypochromic Microcytic Anemia in Children and Evaluation of Laboratory Parameters in the Differentiation.

J Pediatr Hematol Oncol 2019 May;41(4):e221-e223

Kanuni Sultan Suleyman Training and Research Hospital, Pediatric Hematology Oncology, Istanbul, Turkey.

Most common causes of microcytic anemia in children are iron deficiency anemia (IDA) and thalassemia. Differentiation of these and detection of coexistence is essential for genetic counseling and to set a treatment plan. Aim is to characterize the frequency of IDA and thalassemia trait (TT) in children presenting with hypochromic, microcytic anemia and to define the significance of blood count parameters in differential diagnosis. Of the 200 enrolled, 107 were male (53.5%). In total 154 had IDA (77%), 27 had β-TT (13.5%), and in 11 (5.5%) both conditions coexisted. Eight patients had α-thalassemia gene mutations, 3 of these also had IDA. RBC, MCV, Mentzer index, serum iron, TIBC, ferritin were significantly different between IDA and β-TT patients (P<0.001); however, RDW was not different between the 2 groups (P>0.05). Sensitivity and specificity of Mentzer index for the detection of β-TT were 100% and 69.4%, respectively. The positive and negative predictive values of Mentzer index in diagnosing β-TT were 36.6% and 100%, respectively. Differential diagnosis of microcytic anemia is important in children, especially in regions where IDA and thalassemia are both prevalent. We found that 7% of children referred to our clinic for hypochromic, microcytic anemia had both TT and IDA. Our data showed that serum iron, ferritin, TIBC, MCV, and Mentzer index were all valuable markers in diagnosing IDA and were significantly different compared with β-TT patients; RDW was not different between the 2 groups.
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http://dx.doi.org/10.1097/MPH.0000000000001382DOI Listing
May 2019

Burkitt Leukemia With Precursor B-Cell Immunophenotype and Dual Translocation of t(14;18) and t(8;14) in a Child: Case Report and Review of the Literature.

J Pediatr Hematol Oncol 2020 01;42(1):e61-e63

Department of Pediatric Hematology and Oncology, Kanuni Sultan Suleyman Education and Research Hospital.

Background: Burkitt leukemia (BL) with the precursor B-cell immunophenotype is a rarely reported condition. The prognosis of such patients is similar to that of classic BL. However, the combination of chromosomal translocations associated with bcl-2 and c-myc rearrangement has a poor prognosis.

Observations: An 11-year-old child presented with fever and weakness. Bone marrow aspiration showed morphologically L1 type blasts and flow cytometry analysis was compatible with precursor B-cell immunophenotype. Cytogenetic analysis revealed a combination of t(8;14) and t(14;l8).

Conclusions: The combination of t(8;14) and t(14;l8) can exhibit different immunophenotypical and morphologic features in leukemias.
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http://dx.doi.org/10.1097/MPH.0000000000001354DOI Listing
January 2020

Hemophagocytic Lymphohistiocytosis Associated With Visceral Leishmaniasis.

J Pediatr Hematol Oncol 2018 07;40(5):395

Pediatric Hematology and Oncology Department, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey.

This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol. Repeated bone marrow aspiration showed amastigotes on follow-up. In endemic countries, visceral leishmaniasis should be considered in the differential diagnosis to avoid chemotherapy toxicity.
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http://dx.doi.org/10.1097/MPH.0000000000001178DOI Listing
July 2018

A Rare Cause of Paraplegia: Myeloid Sarcoma

Turk J Haematol 2018 08 2;35(3):206-207. Epub 2018 Feb 2.

University of Health Sciences, Kanuni Sultan Süleyman Traning and Research Hospital, Clinic of Pediatric Hematology and Oncology, İstanbul, Turkey

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http://dx.doi.org/10.4274/tjh.2017.0423DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6110446PMC
August 2018

Congenital Factor Deficiencies in Children: A Report of a Single-Center Experience.

Clin Appl Thromb Hemost 2018 Sep 19;24(6):901-907. Epub 2017 Oct 19.

2 Department of Hematology-Internal Medicine, Cerrahpaşa Medical School, İstanbul University, İstanbul, Turkey.

Congenital factor deficiencies (CFDs) refer to inherited deficiency of coagulation factors in the blood. A total of 481 patients with CFDs, who were diagnosed and followed at our Pediatric Hematology and Oncology Clinic between 1990 and 2015, were retrospectively evaluated. Of the 481 cases, 134 (27.8%) were hemophilia A, 38 (7.9%) were hemophilia B, 57 (11.8%) were von Willebrand disease (vWD), and 252 (52.3%) were rare bleeding disorders (RBDs). The median age of the patients at the time of diagnosis and at the time of the study was 4.1 years (range: 2 months to 20.4 years) and 13.4 years (range: 7 months to 31.3 years), respectively. The median duration of the follow-up time was 6.8 years (range: 2.5 months to 24.8 years). One hundred nineteen (47.2%) of 252 patients with RBDs were asymptomatic, 49 (41.1%) of whom diagnosed by family histories, 65 (54.6%) through preoperative laboratory studies, and 5 (4.2%) after prolonged bleeding during surgeries. Consanguinity rate for the RBDs was 47.2%. Prophylactic treatment was initiated in 80 patients, 58 of whom were hemophilia A, 7 were hemophilia B, 13 were RBDs, and 2 were vWD. Significant advances have been achieved during the past 2 decades in the treatment of patients with CFDs, particularly in patients with hemophilias. The rarity and clinical heterogeneity of RBDs lead to significant diagnostic challenges and improper management. In this regard, multinational collaborative efforts are needed with the hope that can improve the management of patients with RBDs.
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http://dx.doi.org/10.1177/1076029617731596DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6714728PMC
September 2018

Evaluation of endocrine and metabolic dysfunctions after hematopoietic stem cell transplantation in children: a study from Turkey.

J Pediatr Endocrinol Metab 2017 May;30(6):683-691

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Background: Endocrine organs are highly susceptible to effects of high-dose chemotherapy. The objective of the study was to evaluate endocrine and metabolic complications after hematopoietic stem cell transplantation (HSCT) in children.

Methods: The patients who underwent HSCT in our center from April 2010 to October 2014 with at least 1 year follow-up were analyzed retrospectively.

Results: One-hundred children (M/F:59/41; mean age 8.9±4.8 years, mean follow-up time 3.4±1.2 years) were included in the study. Female hypogonadism was the most common endocrine dysfunction (35.7%), followed by growth impairment (29.4%), malnutrition (27.4%), dyslipidemia (26%), low bone mineral density (BMD) (25%), hypothyroidism (13%) and insulin resistance (12%). Patients who underwent HSCT >10 years of age were significantly at risk for hypogonadism, metabolic syndrome, growth impairment and malnutrition (p<0.05).

Conclusions: Endocrine or metabolic dysfunctions are more prevalent in children who are older than 10 years of age at HSCT. Children who underwent HSCT should be followed-up by a multidisciplinary team during puberty and adolescence.
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http://dx.doi.org/10.1515/jpem-2016-0306DOI Listing
May 2017

A Rare Late Complication of Port Catheter Implantation: Embolization of the Catheter.

Turk J Haematol 2018 May 26;35(2):142-143. Epub 2017 Apr 26.

University of Health Sciences, İstanbul Kanuni Sultan Süleyman Training and Research Hospital, Clinic of Pediatric Hematology and Oncology, İstanbul, Turkey.

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http://dx.doi.org/10.4274/tjh.2017.0134DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5972342PMC
May 2018

Evaluation of Endocrine Late Complications in Childhood Acute Lymphoblastic Leukemia Survivors: A Report of a Single-Center Experience and Review of the Literature

Turk J Haematol 2017 03 18;34(1):40-45. Epub 2016 Apr 18.

Ankara Children’s Hematology and Oncology Hospital, Clinic of Pediatric Hematology, Ankara, Turkey Phone: +90 505 839 60 92 E-mail:

Introduction: Improvement in long-term survival in patients with acute lymphoblastic leukemia (ALL) in childhood has led to the need for monitorization of treatment-related morbidity and mortality. In the current study, we aimed to evaluate endocrine side effects of treatment in ALL survivors who were in remission for at least 2 years.

Methods: Sixty patients diagnosed with ALL, who were in remission for at least 2 years, were cross-sectionally evaluated for long-term endocrine complications.

Results: The median age of the patients at the time of diagnosis, at the time of chemotherapy completion, and at the time of the study was 5 years (minimum-maximum: 1.7-13), 8 years (minimum-maximum: 4.25-16), and 11.7 years (minimum-maximum: 7-22), respectively, and median follow-up time was 4 years (minimum-maximum: 2-10.1). At least one complication was observed in 81.6% of patients. Vitamin D insufficiency/deficiency (46.6%), overweight/obesity (33.3%), and dyslipidemia (23.3%) were the three most frequent endocrine complications. Other complications seen in our patients were hyperparathyroidism secondary to vitamin D deficiency (15%), insulin resistance (11.7%), hypertension (8.3%), short stature (6.7%), thyroid function abnormality (5%), precocious puberty (3.3%), and decreased bone mineral density (1.7%). There were no statistically significant correlations between endocrine complications and age, sex, and radiotherapy, except vitamin D insufficiency/deficiency, which was significantly more frequent in pubertal ALL survivors compared to prepubertal ALL survivors (57.5% and 25%, respectively, p=0.011).

Conclusion: A high frequency of endocrine complications was observed in the current study. The high frequency of late effects necessitates long-term surveillance of this population to better understand the incidence of late-occurring events and the defining of high-risk features that can facilitate developing intervention strategies for early detection and prevention.
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http://dx.doi.org/10.4274/tjh.2015.0332DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5451687PMC
March 2017

Candida Associated Bloodstream Infections in Pediatric Hematology Patients: A Single Center Experience.

Mediterr J Hematol Infect Dis 2016 1;8(1):e2016018. Epub 2016 Mar 1.

Ankara Children's Hematology and Oncology Hospital, Department of Pediatric Hematology, Ankara, Turkey.

Background And Objectives: Candida-associated bloodstream infections are frequent and potentially life-threatening conditions in hematology patients. The aim of this study is to evaluate the characteristics, risk factors, and outcome of Candida-associated bloodstream infections in children with hematological diseases.

Methods: The medical records of the patients with hematological diseases and hematopoietic stem cell transplantation (HSCT) recipients who were diagnosed as Candida-associated bloodstream infection between February 2010 and February 2014 were reviewed retrospectively.

Results: Thirty episodes of candidemia involving 26 patients (38% female, and 62% male) with a median age of 7-year (range; 1 to 17) were noted. The incidence of candidemia in our study was 5.2 per 1000 hospital admissions. Infections with non-albicans Candida spp. occurred more frequently (63%) and C. krusei was the predominant microorganism among non-albicans Candida spp. (37%). Candida albicans was isolated from 11 of the 30 episodes (37%). Twenty-six of the episodes (88%) patients had a central venous catheter (CVC) prior to candidemia, and they were removed in 16 (62%). Thirty-day mortality rate was 20%. Isolated Candida spp, underlying disease and its status, presence of mucositis, neutropenia, using of broad spectrum antibiotics, corticosteroids or total parenteral nutrition were not identified as predictors of outcome. Multivariate analysis revealed that CVCs kept in place was the only significant factor associated with mortality (OR, 0.07; 95% CI, 0.006-0.716).

Conclusions: Candida-associated bloodstream infections were common in children with hematological diseases and HSCT recipients, particularly in patients with CVCs. In addition to appropriate antifungal therapy, CVC removal improves the outcome of candidemia in children with hematological disease.
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http://dx.doi.org/10.4084/MJHID.2016.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4771141PMC
March 2016

The successful non-invasive management of pulmonary thromboembolism in a child with acute lymphoblastic leukemia.

Arch Argent Pediatr 2016 Feb;114(1):e17-20

Department of Pediatric Hematology, Ankara Çocuk Sağlığı Hastalıkları Hemotoloji Onkoloji Eğitim Araştırma Hastanesi, Ankara, 06110, Turkey.

In childhood, pulmonary thromboembolism (PTE) is an uncommonbut potentially life-threatening disease. The greater numbers of venous thromboembolism (VTE) are complications of underlying risk factors such as malignancies, chemotherapy (L-asparaginase), and central venous catheter. We report a patient with acute lymphoblastic leukemia and PTE, who presented with near-syncope, and was successfully treated with low molecular weight heparin and calcium channel blockers.
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http://dx.doi.org/10.5546/aap.2016.eng.e17DOI Listing
February 2016

Comparison of prophylactic use of intravenous immunoglobulin versus Pentaglobin® in pediatric patients after hematopoietic stem cell transplantation.

Pediatr Transplant 2016 Mar 28;20(2):276-83. Epub 2015 Nov 28.

Department of Pediatric Hematology and Oncology, Ankara Children's Hematology and Oncology Hospital, Ankara, Turkey.

There are few studies evaluating the use of IgM-enriched IVIG (Pentaglobin(®) ) in HSCT recipients. This study aimed to compare the efficacy of prophylactic use of IVIG versus prophylactic use of Pentaglobin(®) within the first 100 days after allogeneic HSCT. We performed a prospective, randomized study of the use of prophylactic IVIG versus prophylactic use of Pentaglobin(®) in patients after allogeneic HSCT. The first dose of IVIG or Pentaglobin(®) was given before conditioning regimen and after transplant was given on day +1, +8, +15, and +22. And then, it was given if IgG level was below 400 mg/dL. Twenty-seven patients in IVIG group and 32 patients in Pentaglobin(®) group were included in the study. There were no significant differences in the duration of neutropenia, hospitalization, fever, and in the number of pyrexial episode, septicemia, bacteremia, local infection, CMV infection, acute GVHD, VOD, and adverse events between the IVIG group and Pentaglobin(®) group. Randomized placebo-controlled trials are needed to conclude that utilization of IVIG or Pentaglobin(®) has no beneficial effect in HSCT.
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http://dx.doi.org/10.1111/petr.12636DOI Listing
March 2016

Evaluation of cardiotoxicity by tissue Doppler imaging in childhood leukemia survivors treated with low-dose anthracycline.

Pediatr Cardiol 2015 Apr 11;36(4):862-6. Epub 2015 Jan 11.

Department of Pediatric Hematology, Ankara Children's Hematology and Oncology Hospital, Ankara, Turkey,

Improvement in long-term survival in patients with acute childhood leukemia has led to the need for monitorization of chemotherapy-related morbidity and mortality. This study included 60 patients with acute lymphoblastic leukemia that were in remission for at least 2 years and 30 healthy controls. Systolic and diastolic function of myocardium was evaluated using conventional echocardiography and tissue Doppler imaging of the left ventricle, interventricular septum and right ventricle. Median age of patients was 11.7 years (range 10-14.9 years), and the median duration of remission was 4 years (range 2.5-5 years). All patients were treated with a low cumulative dose of adriamycin (100 mg/m(2)) according to the St. Jude Total-XIIIA protocol. The ejection fraction (EF) and fractional shortening were normal in the patient and control groups, even though EF values were significantly lower in the patients (69.5 ± 2.3 vs. 72.7 ± 3 %, P < 0.01). Myocardial systole (S m), early diastole (E m) and late diastole (A m) velocities in all segments of the myocardium were significantly lower in the patient group (P < 0.01 for all segments). Cardiotoxicity was noted in all segments of the myocardium in the patient group, despite the fact that they were all treated with a low cumulative dose of adriamycin. Based on these findings, we think that there is no safe dose for anthracyclines and periodic echocardiographic evaluation of both the left and right ventricles must be performed in all patients treated with anthracyclines, even at low doses.
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http://dx.doi.org/10.1007/s00246-015-1096-6DOI Listing
April 2015

Secondary antifungal prophylaxis in pediatric hematopoietic stem cell transplants.

J Pediatr Hematol Oncol 2015 01;37(1):e19-22

*Pediatric Hematology Unit ‡Pediatric Infectious Disease Unit, Ankara Hematology Oncology Children's Training and Research Hospital †Gazi University Faculty of Medicine Pediatric Infectious Diseases Unit, Ankara, Turkey.

Invasive fungal infections (IFIs) constitute a leading cause of morbidity and infection-related mortality among hematopoietic stem cell transplant (HSCT) recipients. With the use of secondary prophylaxis, a history of IFI is not an absolute contraindication to allo-HSCT. However, still, IFI recurrence remains a risk factor for transplant-related mortality. In this study, of the 105 children undergoing HSCT between April 2010 and February 2013, 10 patients who had IFI history before transplantation and had undergone allo-HSCT were evaluated retrospectively to investigate results of secondary prophylaxis. In conclusion, our study shows that amphotericin B and caspofungin was successful as secondary antifungal prophylaxis agents with no relapse of IFI. In addition, after engraftment, secondary prophylaxis was continued with voriconazole orally in 4 patients that yielded good results.
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http://dx.doi.org/10.1097/MPH.0000000000000175DOI Listing
January 2015

Homozygosity for HBA1: c.179G > A: Hb Adana in an infant.

Hemoglobin 2014 24;38(6):449-50. Epub 2014 Oct 24.

Department of Hematology, Ankara Children's Hematology and Oncology Research Hospital , Ankara , Turkey.

Hb Adana (HBA1: c.179G > A) is a very rare, unstable form of α-globin variant that results from deficient synthesis of functional α chains. We present a 2-month-old boy with hypochromic microcytic anemia, and remarkable anisocytosis, target cells and basophilic stippling on his peripheral blood smear. α-Globin gene analysis of the patient determined homozygosity for HBA1: c.179G > A, a mutation known as Hb Adana. On his follow-up visit, hemoglobin (Hb) levels were stable at 9.0-9.5 g/dL and mean corpuscular volume (MCV) was 62.2-62.5 fL without the need for a blood transfusion. Clinical and hematological findings of our case were comparable to Hb H (β4) or β-thalassemia intermedia (β-TI)-like phenotypes, despite the fact that he carried an α1 gene mutation. Heterozygosity for the HBA1: c.179G > A mutation may also lead to microcytosis only as seen in his parents. According to our current knowledge, this is the first described case with homozygosity for the Hb Adana mutation, carried on the α1 gene. The relatively mild presentation of the case highlights the milder phenotypic consequences of nondeletional α mutations in the α1 vs. the α2 gene.
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http://dx.doi.org/10.3109/03630269.2014.969373DOI Listing
July 2015

Neurological complications after allogeneic hematopoietic stem cell transplantation in children, a single center experience.

Pediatr Transplant 2014 Jun;18(4):405-11

Department of Pediatric Hematology Oncology, Ankara Children`s Hematology Oncology Hospital, Ankara, Turkey.

In this study, we retrospectively examined the data of children who underwent allo-HSCT from HLA-matched family donors. We analyzed the incidence, etiological factors, clinical characteristics, possible reasons, risk factors, and follow-up of neurologic complications. BU-based conditioning regimens were used in most of the cases (n = 62). The median duration of follow-up for the 89 patients was 20 months (range 1-41 months). Eleven percent of transplanted children developed one or more neurological symptoms after HSCT with a median observation time of two months (range -6 days to 18 months). The median age of the four girls and six boys with neurological complication was 13 yr (range 5.3-17.6 yr). Cylosporine A neurotoxicity was diagnosed in five children, four of them were PRES. The rest of complications were BU and lorazepam toxicity, an intracranial hemorrhage, a sinovenous thrombosis, and a transient ischemic attack during extracorpereal photopheresis. No difference was found between groups of neurological complication according to age, gender, diagnosis, hospitalization time, neutrophil and platelet engraftment time, stem cell source, and conditioning regimen, acute and chronic GVHD or VOD. Neurological complication was the cause of death in one patient (1.1%).
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http://dx.doi.org/10.1111/petr.12265DOI Listing
June 2014

Hepatitis B vaccine-associated atypical hemolytic uremic syndrome.

Turk J Haematol 2013 Dec 5;30(4):418-9. Epub 2013 Dec 5.

Dr. Sami Ulus Research and Training Hospital of Women's and Children's Health and Diseases, Ankara, Turkey.

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http://dx.doi.org/10.4274/Tjh-2013.0226DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3874978PMC
December 2013

Beta-globin Gene Mutations in Turkish Children with Beta-Thalassemia: Results from a Single Center Study.

Mediterr J Hematol Infect Dis 2013 2;5(1):e2013055. Epub 2013 Sep 2.

Ankara Children's Hematology and Oncology Research Hospital, Department of Hematology. Ankara, Turkey.

Introduction: The beta thalassemias are common genetic disorders in Turkey and in this retrospective study our aim was to evaluate β-globin chain mutations and the phenotypic severity of β-thalassemia patients followed-up in our hospital, a tertiary center which serves patients from all regions of Turkey.

Materials And Methods: 106 pediatric patients were analysed for β-globin gene mutations by using DNA analysis. Patients were classified as having β-thalassemia major or β-thalassemia intermedia based on age at diagnosis, transfusion frequency and lowest hemoglobin concentration in between transfusions.

Results: There were 106 patients (52.8% female and 47.2% male) with a mean age of 11.2±5 years (1.6 - 22.3 years). Eighty-four (79.2%) patients had β-thalassemia major, whereas the remaining 22 patients (20.8%) were identified as having β-thalassemia intermedia. Overall, 18 different mutations were detected on 212 alleles. The most frequently encountered mutation was IVS I.110 (G>A) (35.3%), followed by Codon 8 del-AA (10.4%), IVS II.1 (G>A) (8%), IVS I.1 (G>A) (7.5%), Codon 39 (C>T) (7.1%) and Codon 5 (-CT) (6.6%), which made up 79.4% of observed mutations. According to present results, IVS I.110 (G>AA) was the most frequent mutation observed in this study, as in other results from Turkey. Evaluation of β-thalassemia mutations in 106 patients with 212 alleles, revealed the presence of homozygous mutation in 85 patients (80.2%) and compound heterozygous mutation in 21 patients (19.8%). The mutations detected in patients with homozygous mutation were IVS I.110 (G>A) (38.8%), Codon 8 del -AA (11.8%), IVS II.1 (G>A) (8.2%) and IVS I.1 (G>A) (8.2%). Observed mutations in the compound heterozygotes were Codon 39 (C>T)/Codon 41-42 (-CTTT) (14.3%), IVS I.110 (G>A)/Codon 39(C>T) (14.3%), IVS I.110 (G>A)/Codon 44(-C) (14.3%), and IVS II.745 (C>G)/5'UTR + 22 (G>A) (9.5%).

Conclusion: Our hospital is a tertiary referral center that provides care to patients from all over the country, and thus the distribution of mutations observed in the current study is significant in term of representing that of the country as a whole.
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http://dx.doi.org/10.4084/MJHID.2013.055DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787702PMC
October 2013

Proton pump inhibitor therapy in chemotherapy-induced pancreatitis.

J Pediatr Hematol Oncol 2014 Nov;36(8):660-1

Departments of *Pediatric Hematology and Oncology †Pediatric Hematology ‡Pediatric Gastroenterology, Ankara Children's Hematology and Oncology Hospital, Ankara, Turkey.

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http://dx.doi.org/10.1097/MPH.0b013e318290bf31DOI Listing
November 2014

Adjuvant corticosteroid therapy in hepatosplenic candidiasis-related iris.

Mediterr J Hematol Infect Dis 2012 13;4(1):e2012018. Epub 2012 Mar 13.

Department of Pediatric Hematology, Ankara Children's Hematology and Oncology Hospital, Ankara, Turkey.

Candida infections are the most frequent infections in neutropenic patients. Hepatosplenic candidiasis (HSC) is a part of disseminated Candida infection that occurs most commonly in patients with hematologic malignancies treated with chemotherapy and requires protracted antifungal therapy. During invasive mycosis with rapid resolution of immunosuppression, immune reconstitution inflammatory syndrome (IRIS) which mimics treatment failure, drug toxicity or breakthrough infections may occur. Manifestation period, histopathologic findings and favorable effect of steroids to its inflammatory symptoms strongly suggest that HSC belongs to the invasive fungal infection induced IRIS. We present a child with B cell-acute lymphoblastic leukemia who developed HSC and addition of corticosteroid therapy to antifungal treatment achieved rapid resolution of the clinical symptoms and laboratory findings.
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http://dx.doi.org/10.4084/MJHID.2012.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3340993PMC
October 2012

Surgical management of recurrent malignant schwannoma of the scalp.

J Craniofac Surg 2011 May;22(3):1120-2

Department of Neurosurgery, Dr Abdurrahman Yurtaslan Oncology Training and Research Hospital, Ankara, Turkey.

Localization of malignant schwannoma in the head and neck region is rare. Malignant schwannoma is a highly aggressive, slowly growing, encapsulated tumor of nerve sheath origin. The most common presentation of this tumor is a subcutaneous progressively enlarging mass with or without neurologic symptoms. These tumors arise from neurofibromas with or without von Recklinghausen disease, and most of the remains develop from peripheral nerve trunks. We report a case with recurrent malignant schwannoma of the scalp that is an uncommon presentation and discuss the clinicopathologic properties of this tumor.
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http://dx.doi.org/10.1097/SCS.0b013e3182108f69DOI Listing
May 2011