Publications by authors named "Ali Aycicek"

38 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 Sep 22;38(6):555-563. 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
September 2021

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

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

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

Microorganisms isolated from cultures and infection focus and antibiotic treatments in febrile neutropenic children from Şanlıurfa, Turkey.

Turk J Pediatr 2016 ;58(1):47-53

Eskişehir State Hospital, Eskişehir, Turkey.

Chemotherapy induced febrile neutropenia predisposes patients to life threatening infections. We aimed to determine the causative microorganisms, infection focus and antibiotic treatment success in febrile neutropenic children with leukemia. A total of 136 febrile neutropenic episodes in 48 leukemic children were reviewed retrospectively from records. Among 136 febrile neutropenic episodes, 68 (50%) episodes were microbiologically documented. Methicillin sensitive coagulase (-) Staphylococcus aureus were the most common isolates from hemoculture (20.5%). The most frequently documented infection focus was mucositis (31.9%). Ceftazidime plus amikacin was the most commonly used antimicrobial treatment for the empirical therapy (52.9%). The overall response rates were 70.5%, 86.9%, and 66.6% of first line, second line and third line therapies, respectively. The spectrum of isolates among febrile neutropenic children in our hematology clinic appears to be gram positive pathogens which are the most common agents. Therefore the, documentation of the flora in each unit could help to decide appropriate empirical therapy which is life saving.
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http://dx.doi.org/10.24953/turkjped.2016.01.007DOI Listing
May 2017

Platelet Levels of High- and Mega-Dose Methylprednisolone Treatment in Acute Immune Thrombocytopenia.

Authors:
Ali Ayçiçek

Turk J Haematol 2015 Jun;32(2):186-7

Kanuni Sultan Süleyman Research and Education Hospital, Clinic of Pediatric Hematology/Oncology, İstanbul, Turkey E-mail:

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http://dx.doi.org/10.4274/tjh.2014.0436DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4451491PMC
June 2015

N-Acetylcysteine supplementation reduces oxidative stress and DNA damage in children with β-thalassemia.

Hemoglobin 2014 15;38(5):359-64. Epub 2014 Sep 15.

Pediatric Hematology Department, Harran University, Medical Faculty , Sanlıurfa , Turkey .

There are several reports that increased oxidative stress and DNA damage were found in β-thalassemia major (β-TM) patients. In this study, we aimed to evaluate the effects of N-acetylcysteine (NAC) and vitamin E on total oxidative stress and DNA damage in children with β-TM. Seventy-five children with transfusion-dependent β-thalassemia (β-thal) were randomly chosen to receive 10 mg/kg/day of NAC or 10 IU/kg/day of vitamin E or no supplementation; 28 healthy controls were also included in the study. Serum total oxidant status (TOS) and total antioxidant capacity (TAC) were measured, oxidative stress index (OSI) was calculated, and mononuclear DNA damage was assessed by alkaline comet assay; they were determined before treatment and after 3 months of treatment. Total oxydent status, OSI, and DNA damage levels were significantly higher and TAC levels were significantly lower in the thalassemic children than in the healthy controls (p < 0.001). In both supplemented groups, mean TOS and OSI levels were decreased; TAC and pre transfusion hemoglobin (Hb) levels were significantly increased after 3 months (p ≤ 0.002). In the NAC group, DNA damage score decreased (p = 0.001). N-Acetylcysteine and vitamin E may be effective in reducing serum oxidative stress and increase pre transfusion Hb levels in children with β-thal. N-Acetylcysteine also can reduce DNA damage.
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http://dx.doi.org/10.3109/03630269.2014.951890DOI Listing
June 2015

Efficacy of deferasirox in children with β-thalassemia: single-center 3 year experience.

Pediatr Int 2014 Aug 30;56(4):530-3. Epub 2014 May 30.

Pediatric Hematology/Oncology Clinic, Eskisehir State Hospital, Eskisehir, Turkey.

Background: Iron chelation therapy is an important component in the management of patients with β-thalassemia.

Methods: The study included 87 children with transfusion-dependent β-thalassemia aged 2-17 years (mean, 8.2 ± 4.1 years), 49 (56%) of whom were male. The patients received deferasirox 9-40 mg/kg per day as a single dose for 36 months. They were clinically and laboratory monitored.

Results: The treatment was generally well tolerated. Drug-related adverse events, including abdominal pain (14.9%) and nausea (5.8%), high alanine aminotransferase more than double the upper limit of normal (5.8%), and non-progressive rise in serum creatinine (2.3%), were generally mild to moderate, transient, and reduced in frequency over time. Two patients discontinued treatment due to severe abdominal pain and nausea. Mean deferasirox dose was calculated as 21.2 ± 8.6, 23.7 ± 8.1, 30.7 ± 8.2 and 32.4 ± 7.6 mg/kg per day at 0, 12, 24 and 36 months, respectively. Mean (median) serum ferritin level was found to increase progressively during the first 22 months of treatment, from 3.161 ± 1.683 ng/mL (2.760 ng/mL) to 3.679 ± 1.997 ng/mL (3.071 ng/mL; P < 0.001) and then decreased gradually to 2.907 ± 1.436 ng/mL (2.670 ng/mL; P = 0.023) at 36 months.

Conclusion: Deferasirox is safe and well tolerated; doses 21-24 mg/kg per day were not able to maintain stable iron balance, but ≥ 30 mg/kg per day was able to reduce iron in regularly transfused pediatric patients.
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http://dx.doi.org/10.1111/ped.12323DOI Listing
August 2014

Ferrous sulfate (Fe2+) had a faster effect than did ferric polymaltose (Fe3+) on increased oxidant status in children with iron-deficiency anemia.

J Pediatr Hematol Oncol 2014 Jan;36(1):57-61

Departments of *Pediatric Hematology ‡Clinical Biochemistry §Pediatrics, Harran University Medical Faculty, Sanliurfa †Department of Pediatric Hematology, Marmara University Medical Faculty, Istanbul, Turkey.

Objective: The purpose of this study was to compare the total oxidant and antioxidant effect of different oral iron preparations in children with iron-deficiency anemia (IDA).

Methods: A total of 65 children with IDA were randomized to receive 5 mg Fe/kg/d iron (II) sulfate (Fe(2+) group, n=33) or iron (III)-hydroxide polymaltose complex (Fe(3+) group, n=32); healthy controls (n=28) were also included in the study. Serum total thiol (-SH), total antioxidant capacity (TAC), total oxidant status (TOS), oxidative stress index (OSI), and hematological profile were evaluated at the baseline and on day 8 and day 30 of the therapy.

Results: Serum TOS and OSI levels were significantly higher and total -SH and total antioxidant capacity levels were significantly lower in the study groups at the beginning of therapy than in the controls (P>0.001). In multivariate analysis, after controlling for multiple confounding factors, on days 8 and 30, serum TOS and OSI levels were not different in the Fe(3+) group, whereas they were significantly reduced in the Fe(2+) group (P≤0.033).

Conclusions: Serum total oxidant status was significantly increased in children with IDA, and Fe(2+) was highly effective in correcting elevated oxidative status.
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http://dx.doi.org/10.1097/MPH.0b013e318299c91aDOI Listing
January 2014

Increased oxidant status in children with breath-holding spells.

Childs Nerv Syst 2013 Jun 19;29(6):1015-9. Epub 2012 Dec 19.

Pediatrician and Fellow of Pediatric Neurology, Harran University, Faculty of Medicine, Sanliurfa, Turkey.

Background: Breath-holding spells (BHS) are the most common form of non-epileptic paroxysmal events in infancy. The pathophysiology of BHS is not fully understood. Iron-deficiency anemia (IDA) may be a factor contributing to breath-holding spells. Although numerous reports have shown that elevated oxidative stress is implicated in the pathophysiology of neurodegenerative diseases and neurological conditions, such as epileptic seizures, brain damage, and neurotrauma, there are no data regarding the role of oxidative stress in the development of BHS. This study aimed to investigate oxidative stress in children with BHS.

Methods: This case-control study was conducted at the Department of Pediatric Neurology, Harran University School of Medicine, Sanliurfa, in Turkey. Blood samples from 31 patients (14 females, 17 males) with BHS which were taken at least 24 h after the BHS attack, and a control group of 35 healthy individuals (13 females, 22 males) were used for the measurement of the plasma total antioxidant capacity, total oxidant status, and oxidative stress index, hemoglobin concentration, serum iron, transferrin saturation and serum ferritin levels.

Results: The plasma total antioxidant capacity values were markedly lower and total oxidant status and oxidative stress index values in the BHS group were significantly higher than that in the controls (P ≤ 0.01).

Conclusion: Our data suggest that the value of oxidative stress was significantly higher in patients with BHS than in the controls. Conditions associated with increased oxidative stress such as IDA may be a risk factor for the development of BHS.
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http://dx.doi.org/10.1007/s00381-012-2001-8DOI Listing
June 2013

Outcome of modified St Jude total therapy 13A for childhood acute lymphoblastic leukemia in the southeast region of Turkey.

J Pediatr Hematol Oncol 2013 Jan;35(1):36-41

Pediatric Hematology Department, Medical Faculty, Harran University, Sanliurfa, Turkey.

Objective: To fill the gap in the current data on childhood acute lymphoblastic leukemia (ALL) in low-income and middle-income countries.

Methods: This study included 106 children between the ages of 1 and 17 years with newly diagnosed ALL monitored between 1999 and 2010. All the patients were treated with the modified St Jude Total 13A treatment plan at the Pediatric Hematology Clinic at Harran University.

Results: Sixty-eight (64.2%) patients were boys and 38 (35.8%) were girls. The median age at diagnosis was 5.9 ± 3.7 years. Thirty-eight (35.8%) children were classified as standard risk, 53 (39.3%) were intermediate risk, and 15 (14.2%) were high risk. Thirteen (12.3%) children died in induction before the remission date (43 d of remission induction). Of all the 93 (100%) patients who completed remission induction therapy and whose bone marrow were in remission, 5 (4.7%) had a bone marrow relapse, 1 (0.9%) had a retinal relapse, and 5 (4.7%) had secondary acute myeloid leukemia. At a median follow-up of 44 months (range, 0.36 to 135.5 mo), the estimated 5-year overall survival and event-free survival were 77.4 ± 5% and 68.9 ± 6.5%, respectively. The estimated 5-year overall survival for boys and girls was 76.5 ± 6% and 65.8 ± 8%, respectively (P = 0.182).

Conclusions: St Jude Total 13A treatment protocols to treat childhood ALL can be successfully adapted, which suggests that such an approach may be useful in low socioeconomic regions; however, it should be noted that secondary leukemia can occur at a high rate.
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http://dx.doi.org/10.1097/MPH.0b013e318271f43fDOI Listing
January 2013

Beta-globin gene mutations in children with beta-thalassemia major from Şanlıurfa province, Turkey.

Turk J Haematol 2011 Dec;28(4):264-8

Department of Pediatric Hematology, Faculty of Medicine, Harran University, Şanlıurfa, Turkey Phone: +90 414 318 30 00 E-mail:

Objective: The prevalence of β-thalassemia in Şanlıurfa province, Turkey is reported to be 2.6%-3.7%, whereas nation-wide the frequency of β-thalassemia is 2%. This study aimed to identify the most frequent β-thalassemia mutations in Şanlıurfa province.

Methods: In total, 22 mutations were investigated in 115 pediatric patients with β-thalassemia using a commercially available reverse dot blot platform.

Results: The study included 60 male and 55 female patients with a mean age of 7.3±4.6 years (range: 1-17 years). In total, 76% of the patients had consanguineous parents. In all, 16 different mutations were observed in the 115 patients. IVS-1-110 (G-A) (29.1%), IVS-1-1 (G-A) (13.9%), codon 39 (C>T) (10.4%), and codon 8 (-AA) (9.1%) accounted for 62.5% of all the β-thalassemia mutations, and 6% of the patients had 2 different thalassemia mutations. According to the present results, IVS-1-110 (G>A) was the most frequent mutation observed in the patients from Şanlıurfa province, as in other geographical regions of Turkey. In addition, the following 34 compound heterozygote mutant alleles were observed; IVS-1-1 (G>A)/IVS 2.848 (n=4), codon 39 (C>T)/codon 8 (-AA) (n=2), codon 6 (-A)/IVS 1.5 (G>C) (n=2), IVS-1-110 (G>A)/IVS-1-1 (G>A) (n=2), IVS-1-110 (G>A)/codon 8 (-AA) (n=1), IVS-1-110 (G>A)/codon 39 (C>T) (n=1), IVS-1-110 (G>A)/IVS-1-6 (T>C) (n=1), IVS-1-110 (G>A)/IVS-1-5 (G>C) (n=1), IVS-1-110 (G>A]/codon 8/9 (+G) (n=1), IVS-1-1 (G>A)/codon 39 (C>T) (n=1), and codon 8 (-AA)/IVS-1-5 (G>C) (n=1). The following β-globin gene promoter mutations were not observed; -101 (C>T), -87(C>T), -30 (T>A), codon 15 (TTG>TGA), codon 27 (G>T) Knossos, and IVS-1-116 (G>C). In all, 5 of the 115 patients (4.3%) had an unidentified mutation.

Conclusion: The present results illustrate the heterogeneity of β-thalassemia mutations in Şanlıurfa Province. The present findings may be of value for genetic counseling, and premarital and prenatal diagnosis in Şanlıurfa province.
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http://dx.doi.org/10.5152/tjh.2011.86DOI Listing
December 2011

Increased oxidative stress in preschool children exposed to passive smoking.

J Pediatr (Rio J) 2011 Nov-Dec;87(6):523-8. Epub 2011 Nov 14.

Sanliurfa Children’s Hospital, Pediatrics Clinic, Sanliurfa, Turquia.

Objectives: To study the effect of passive cigarette smoking on plasma oxidative and antioxidative status in passive smoking preschool children and to compare them with controls.

Methods: Thirty-four passive smoking (five to 50 cigarettes per day) preschool children (study group) and 32 controls who had never been exposed to cigarette smoke were randomly chosen from children aged from 4 to 6 years. Urinary cotinine and plasma indicators of oxidative and antioxidative status, i.e., total oxidant status (TOS), total antioxidant capacity (TAC), and oxidative stress index (OSI), were determined.

Results: Mean environmental cigarette consumption was 22±13 cigarettes per day in passive smoking children. Mean urinary cotinine levels were 77.6±41.4 ng/mL and 11.9±2.3 ng/mL in the study and control groups, respectively (p < 0.001). Mean plasma TAC levels were 0.95±0.13 mmol Trolox equivalent/L and 1.01±0.09 mmol Trolox equivalent/L, respectively (p = 0.039). Mean plasma TOS levels were 28.6±7.9 µmol H2O2 equivalent/L and 18.5±6.3 µmol H2O2 equivalent/L, respectively (p < 0.001). Mean OSI levels were 3.08±0.98 arbitrary units and 1.84±0.64 arbitrary units, respectively (p < 0.001). A small amount of cigarette smoke (five to 10 cigarettes per day) causes considerable oxidative stress. There were significant correlations between number of cigarettes consumed and oxidant status and OSI levels.

Conclusions: Passive smoke is a potent oxidant in preschool children. Its deleterious effects are not limited just to heavy passive smoking, but also occur with exposure to small amounts of smoke.
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http://dx.doi.org/10.2223/JPED.2139DOI Listing
July 2012

Vitamin B12 treatment reduces mononuclear DNA damage.

Pediatr Int 2011 Dec 30;53(6):1023-7. Epub 2011 Oct 30.

Department of Pediatrics, Eskisehir Sakarya Hospital, Eskisehir, Turkey.

Background: DNA damage effects of vitamin B(12) deficiency were performed in vitro and in adults.

Methods: The study group included 32 children (13 girls, 19 boys) with vitamin B(12) deficiency (mean age 44 ± 58 months) and their 27 mothers (mean age 30.4 ± 5.3 years). The control group contained 30 healthy children and 25 mothers. DNA strand breaks in peripheral blood mononuclear leukocytes were assayed by single-cell alkaline gel electrophoresis (comet assay) before and 8 days after the first injection of vitamin B(12).

Results: Mean DNA damage scores in children with vitamin B(12) deficiency and their mothers were significantly higher before treatment than those after treatment. The DNA damage scores of children after treatment were still significantly higher than controls. There were significant negative correlations between the children and their mothers in terms of vitamin B(12) levels and DNA damage scores (r = 0.3, P= 0.02; r = 0.58, P= 0.002, respectively). There were correlations between the children's and their mothers' DNA damage and the severity of vitamin B(12) deficiency, suggesting that the children and their mothers may play a role in the scarcity of nutritional vitamin B(12).

Conclusion: DNA damage is increased in children with vitamin B(12) deficiency and in their mothers. DNA damage scores were significantly improved through vitamin B(12) therapy 8 days after the first injection, however, they were still significantly higher than those of controls.
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http://dx.doi.org/10.1111/j.1442-200X.2011.03448.xDOI Listing
December 2011

Maternal active or passive smoking causes oxidative stress in placental tissue.

Eur J Pediatr 2011 May 28;170(5):645-51. Epub 2010 Oct 28.

Medical Faculty Pediatric Hematology Department, Harran University, 63200 Sanliurfa, Turkey.

The aim of this study was to assess the influence of active and passive maternal smoking on placenta total oxidant/antioxidant status in term infants. The levels of cord blood total antioxidant capacity (TAC), total oxidant status (TOS), and oxidative stress index (OSI) were measured in samples of fetal placental tissue, cord blood, and the maternal peripheral blood serum and from 19 mothers who were active smokers, 19 who were passive smokers, and 22 who were nonsmokers (not exposed to active or passive smoking). The pregnancies were between 37 and 40 weeks' gestation, were uncomplicated, and the infants were delivered vaginally. Birth weight and head circumference in the active smokers were significantly (P < 0.001) lower than those in the controls. Placenta, cord blood, and the maternal peripheral TAC levels were significantly lower in the active smokers compared with the controls (P < 0.001), while TOS and OSI levels were significantly higher in the active and passive smokers than in the controls (P < 0.001). A positive significant correlation was found between active maternal smoking and placenta TOS and OSI levels (P < 0.016), and a significant negative correlation was found between number of cigarettes exposed to and birthweight and head circumference (P < 0.05). In conclusion, active or passive maternal smoking is associated with important alterations in oxidant and antioxidant balance in fetal placental tissue and causes potent oxidative stress.
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http://dx.doi.org/10.1007/s00431-010-1338-9DOI Listing
May 2011

Pseudotumor cerebri secondary to subacute sclerosing panencephalitis.

Pediatr Neurol 2009 May;40(5):371-6

Department of Pediatrics, Medical Faculty, Research Hospital, Harran University, Sanliurfa, Turkey.

Unusual presentations are not rare in subacute sclerosing panencephalitis. Five patients initially diagnosed with pseudotumor cerebri were ultimately determined to have pseudotumor cerebri secondary to subacute sclerosing panencephalitis. The present study retrospectively reviewed 56 cases history, neurologic symptoms, and clinical and laboratory data, as well as the outcomes. On admission, five patients (group 1) presenting with pseudotumor cerebri exhibited bilateral papilledema, and in each of them cranial magnetic resonance imaging revealed small lateral ventricles, effacement of the subarachnoid space, and no mass lesion. On admission, 51 patients (group 2) had no pseudotumor cerebri findings. The year of original measles infection, the interval between measles and onset of subacute sclerosing panencephalitis, and initial neurologic symptoms were similar, but length of symptoms before diagnosis of subacute sclerosing panencephalitis was shorter in group 1, and the clinical stage of subacute sclerosing panencephalitis on admission was more advanced in group 2. Cerebrospinal fluid mean open pressure was 378 +/- 22 H(2)O in group 1 and 146 +/- 28 H(2)O in group 2; cerebrospinal fluid antibody was 2038 +/- 768 U/L in group 1 and was 664 +/- 214 U/L in group 2. Only three of the five patients with pseudotumor cerebri had typical periodic discharges on electroencephalographic examination. These findings suggest that subacute sclerosing panencephalitis can cause pseudotumor cerebri.
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http://dx.doi.org/10.1016/j.pediatrneurol.2008.12.003DOI Listing
May 2009

Phototherapy causes DNA damage in peripheral mononuclear leukocytes in term infants.

J Pediatr (Rio J) 2008 Mar-Apr;84(2):141-6. Epub 2008 Mar 18.

Pediatrics Department, Medical Faculty, Harran University, Sanliurfa, Turkey.

Objective: Our aim was to determine whether endogenous mononuclear leukocyte DNA strand is a target of phototherapy.

Methods: The study included 65 term infants aged between 3-10 days that had been exposed to intensive (n = 23) or conventional (n = 23) phototherapy for at least 48 hours due to neonatal jaundice, and a control group (n = 19). DNA damage was assayed by single-cell alkaline gel electrophoresis (comet assay). Plasma total antioxidant capacity and total oxidant status levels were also measured, and correlation between DNA damage and oxidative stress was investigated.

Results: Mean values of DNA damage scores in both the intensive and conventional phototherapy groups were significantly higher than those in the control group (p < 0.001). Mean values and standard deviation were 32 (9), 28 (9), 21 (7) arbitrary unit, respectively. Total oxidant status levels in both the intensive and conventional phototherapy groups were significantly higher than those in the control group (p = 0.005). Mean (standard deviation) values were 18.1 (4.2), 16.9 (4.4), 13.5 (4.2) micromol H2O2 equivalent/L, respectively. Similarly, oxidative stress index levels in both the intensive and conventional phototherapy groups were significantly higher than those in the control group (p = 0.041). Plasma total antioxidant capacity and total bilirubin levels did not differ between the groups (p > 0.05). There were no significant correlations between DNA damage scores and bilirubin, total oxidant status and oxidative stress levels in either phototherapy group (p > 0.05).

Conclusions: Both conventional phototherapy and intensive phototherapy cause endogenous mononuclear leukocyte DNA damage in jaundiced term infants.
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http://dx.doi.org/10.2223/JPED.1765DOI Listing
September 2008

Oxidant and antioxidant parameters in the treatment of meningitis.

Pediatr Neurol 2007 Aug;37(2):117-20

Pediatric Service, Sanliurfa Children's Hospital, Medical Faculty, Harran University, Sanliurfa, Turkey.

The aim of this study was to assess the effects of meningitis treatment on the serum and cerebrospinal-fluid oxidant and antioxidant status in children with bacterial meningitis. Forty children with bacterial meningitis, at ages ranging from 4 months to 12 years (mean age, 4 years), were enrolled in the study. Within 8 hours after admission (before treatment) and 10 days after clinical and laboratory indications of recovery (after treatment), cerebrospinal fluid and venous blood were collected. Thirty-seven healthy children (mean age, 4 years) were enrolled as control subjects, and only venous blood was collected. Serum total oxidant status, lipid hydroperoxide, oxidative stress index, uric acid, albumin, and ceruloplasmin levels were lower in the patient group after treatment (P<0.05). Serum total antioxidant capacity levels, vitamin C, total bilirubin, and catalase concentrations were not significantly altered by treatment (P>0.05). However, cerebrospinal fluid total oxidant status, lipid hydroperoxide, and oxidative stress index levels were higher, and cerebrospinal fluid total antioxidant capacity levels were lower after treatment than before treatment (P<0.05). In conclusion, we demonstrated that serum oxidative stress was lower, and cerebrospinal fluid oxidative stress was higher, after rather than before treatment in children with bacterial meningitis.
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http://dx.doi.org/10.1016/j.pediatrneurol.2007.04.002DOI Listing
August 2007

Total oxidant/antioxidant status in jaundiced newborns before and after phototherapy.

J Pediatr (Rio J) 2007 Jul-Aug;83(4):319-22. Epub 2007 Jul 11.

Children's Hospital at Sanliurfa, Sanliurfa, Turkey.

Objective: To assess the effect of phototherapy on serum oxidant and antioxidant status in hyperbilirubinemic full-term newborns.

Method: Thirty-four full-term infants from 3 to 10 days of age exposed to phototherapy were studied. The serum antioxidant status was assessed by measuring the total antioxidant capacity (TAC) and individual antioxidant components: vitamin C, uric acid, albumin, thiol contents and total bilirubin. The oxidant status was assessed by determining the total oxidant status (TOS), oxidative stress index (OSI) and individual oxidant components: malondialdehyde (MDA), and lipid hydroperoxide levels.

Results: Vitamin C, uric acid, total bilirubin and MDA concentration were significantly lower, whereas serum TOS, lipid hydroperoxide and OSI levels were significantly higher after phototherapy (p < 0.05). There were significant positive correlations between serum total bilirubin and MDA (r = 0.434, p = 0.001).

Conclusions: Although the MDA level was reduced after phototherapy, phototherapy has a negative impact on numerous parts of the oxidant/antioxidant defense system in jaundiced full-term newborns, exposing them to potential oxidative stress.
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http://dx.doi.org/10.2223/JPED.1645DOI Listing
February 2008
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