Publications by authors named "Gulcihan Ozek"

19 Publications

  • Page 1 of 1

Metabolic syndrome and risk factors after hematopoietic stem cell transplantation in children and adolescents.

J Pediatr Endocrinol Metab 2021 Apr 25;34(4):485-493. Epub 2021 Feb 25.

Department of Pediatric Hematology & Oncology and Bone Marrow Transplantation, Medical School of Ege University, Izmir, Turkey.

Objectives: The early and late complications after hematopoietic stem cell transplantation (HSCT) determine the patients' prognosis and life quality. We aim to determine the metabolic syndrome development frequency after HSCT in children to find out the risk factors and compare them with healthy adolescents.

Methods: Thirty-six children who underwent HSCT at least two years ago were analyzed prospectively and cross-sectionally. Our study included 18 healthy children between the ages of 11 and 17 as a control group. All of the cases were assessed in terms of metabolic syndrome (MS) through the use of Modified WHO Criteria.

Results: The patients' median age was 10.6 (5.1-17) years, the median time of follow-up after HCST was 4.1 (2-13.5) years and 70% were male. Two cases were diagnosed with MS (5.6%). When considered in terms of the sub-components of MS, 2 cases (5.6%) were found to have obesity, 17 cases (47%) abnormal glucose tolerance, 11 cases (30.7%) dyslipidemia, and 3 cases (8.6%) hypertension. The MS rate was not different when compared with the 11-17 year-old healthy control group (0 vs. 11%, p=0.48). Myeloablative conditioning regimen (65 vs. 20%) and the increased age at which HSCT was performed were considered to be risk factors in terms of insulin resistance (p=0.025 and 0.002).

Conclusions: Age and conditioning regimens were found to be the risk factors for insulin resistance development. The long-term follow-up of the cases who had undergone HSCT in childhood in terms of MS and its sub-components is important in order to increase life quality.
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http://dx.doi.org/10.1515/jpem-2020-0584DOI Listing
April 2021

Severe Rare Bleeding Disorders: A Single-center Experience.

J Pediatr Hematol Oncol 2020 Nov 23. Epub 2020 Nov 23.

Department of Pediatric Hematology, Children's State Hospital of Sanliurfa, Sanliurfa, Turkey.

Although rare bleeding disorders (RBDs) are not common diseases, they are important for life-threatening bleedings and prophylaxis approaches, especially in severe forms. In this retrospective study, the authors have analyzed data from children with severe RBDs who were examined at the center over a period of 10 years in order to describe the distribution, clinical features, treatment patterns, and outcome of severe RBDs in patients. Data from all children (age under 18 y) with RBDs who were examined in the center between 2005 and 2015 were retrospectively reviewed. In total, 12 patients were included in the study. Four of the cases had factor (F) VII (33.3%), 6 had FX (50%), 1 had FXIII (8.3%), and 1 had fibrinogen deficiency (8.3%). Of the 12 children with severe RBDs, 8 (67%) experienced at least 1 major bleeding. Prophylaxis was applied to 10 patients. In conclusion, RBDs are more common in our country because of the high parental consanguinity rates. So, it is necessary to raise public awareness about the risks of consanguineous marriages and increase access to genetic counseling and testing facilities. Delayed diagnosis and lack of adequate prophylactic replacement therapy are the most important risk factors that increase life-threatening bleeding.
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http://dx.doi.org/10.1097/MPH.0000000000002019DOI Listing
November 2020

Non-typhoidal bacteraemia in paediatric leukaemia patients.

Contemp Oncol (Pozn) 2018 14;22(2):105-107. Epub 2018 Jun 14.

Department of Paediatric Haematology, Dr. Behçet Uz Children's Hospital, İzmir, Turkey.

Non-typhoidal (NTS) is an important pathogen that causes gastroenteritis, bacteraemia, and focal infections. Herein, we present our experience with bloodstream infections caused by in paediatric leukaemia patients, which has been reported for the first time in both Europe and the US. According to our research, NTS might be a cause of serious infections in paediatric haematology-oncology patients. Following a low bacterial diet and increasing the hygiene of both the children and their surroundings would be beneficial in preventing these infections.
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http://dx.doi.org/10.5114/wo.2018.76446DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6103228PMC
June 2018

LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY.

Rev Paul Pediatr 2017 Jan-Mar;35(1):115-119

Ataturk Research and Training Hospital, Esmirna, Turquia.

Objective: To report a case of late-onset self-healing Langerhans cell histiocytosis.

Case Description: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later.

Comments: Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy.
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http://dx.doi.org/10.1590/1984-0462/;2017;35;1;00015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5417814PMC
June 2018

Varicella-Zoster Virus Infections in Pediatric Malignancy Patients: A Seven-Year Analysis.

Turk J Haematol 2016 Dec 18;33(4):346-348. Epub 2016 Oct 18.

Dr. Behçet Uz Children Training and Research Hospital, Clinic of Pediatric Infectious Diseases, İzmir, Turkey, Phone: +90 232 489 56 56, E-mail:

Primary varicella-zoster virus (VZV) infection is a benign self-limited disease. In this study, we review our experience in focusing on the outcome and treatment of VZV infection in pediatric malignancy patients. During the study period, a total of 41 patients with pediatric malignancy had been hospitalized with the diagnosis of VZV infection. All the patients were treated with intravenous acyclovir for a median of 7 days (ranging from 5 to 21 days). The calculated attributable delay of chemotherapy due to VZV infections was 8 days (ranging from 2 to 60 days). VZV-related complications were observed in 3 of 41 patients (7%) who suffered from acute respiratory distress syndrome, and one of them with hemophagocytic lymphohistiocytosis died due to respiratory failure despite acyclovir and broad-spectrum antimicrobial treatment plus supportive treatment. VZV infections are still important contagious diseases in pediatric cancer patients, because they cause not only significant mortality but also a delay in chemotherapy.
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http://dx.doi.org/10.4274/tjh.2016.0046DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5204192PMC
December 2016

Evaluation of the glucocorticoid, mineralocorticoid, and adrenal androgen secretion dynamics in a large cohort of patients aged 6-18 years with transfusion-dependent β-thalassemia major, with an emphasis on the impact of cardiac iron load.

Endocrine 2016 Jul 2;53(1):240-8. Epub 2016 Feb 2.

Department of Adolescent Medicine, Istanbul School of Medicine, Istanbul University, Istanbul, Turkey.

The variable presence of adrenal insufficiency (AI) due to hypocortisolemia (HC) in patients with thalassemia is well established; however, the prevalence of adrenocortical hypofunction (ACH) in the zona glomerulosa and zona reticularis of the adrenal cortex is unknown. To establish the prevalence of ACH, we examined the cortisol response to 1-µg and 250-µg ACTH tests, plasma aldosterone (A)/plasma renin activity (PRA) ratio, and serum dehydroepiandrosterone sulfate (DHEAS) levels in a large cohort of patients with thalassemia, and to investigate the impact of total body iron load (TBIL) on adrenocortical function. The setting used was University hospital and government-based tertiary care center. One hundred twenty-one (52 females) patients with β-thalassemia major (β-TM) and 72 healthy peers (38 females) were enrolled. The patients underwent a 250-µg cosyntropin test if their peak cortisol was <500 nmol/L in a 1-µg cosyntropin test. Magnetic resonance imaging (MRI) was performed to assess the MRI-based liver iron content and cardiac MRI T2* iron. The associations between ACH and TBIL were investigated. The patients with thalassemia had lower ACTH, cortisol, DHEAS, and A/PRA values compared with the controls (p < 0.001). Thirty-nine patients (32.2 %) had HC [primary (n = 1), central (n = 36), combined (n = 2)], and 47 (38.8 %) patients had reduced DHEAS levels; 29 (24.0 %) patients had reduced A/PRA ratios. Forty-six (38.0 %) patients had hypofunction in one of the adrenal zones, 26 (21.5 %) had hypofunction in two adrenal zones, and 9 (7.4 %) had hypofunction in all three zones. Patient age and TBIL surrogates were significant independent parameters associated with ACH. Cardiac MRI T2* iron was the only significant parameter that predicted the severity of ACH at a cut-off of 20.6 ms, with 81 % sensitivity and 78 % specificity. Patients with thalassemia have a high prevalence of AI due to HC and zona glomerulosa and zona reticularis hypofunction. TBIL surrogates can predict ACH, but cardiac iron was the only surrogate that was adequately sensitive to predict the severity of ACH.
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http://dx.doi.org/10.1007/s12020-016-0872-2DOI Listing
July 2016

Pediatric langerhans cell histiocytosis: single center experience over a 17-year period.

Turk J Pediatr 2016 ;58(4):349-355

Department of Pediatric Hematology-Oncology Clinic, Dokuz Eylül University Institute of Oncology, İzmir.

This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.
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http://dx.doi.org/10.24953/turkjped.2016.04.001DOI Listing
July 2017

A 7-year study of the distribution of nosocomial candidemia in children with cancer.

Turk J Pediatr 2015 May-Jun;57(3):225-9

Division of Pediatric Infectious Diseases Dr. Behçet Uz Children's Hospital, İzmir, Turkey.

Candidemia is an important cause of morbidity and mortality in cancer patients. The incidence of candidemia has been reported to have shifted toward nonalbicans species. The aim of this study was to determine the distribution of Candida species resulting in bloodstream infections or catheter-related blood stream infections (CRBSIs) in pediatric hematology-oncology (PHO) patients over a 7-year-period. Medical and computerized microbiology laboratory records of all positive blood fungal cultures during the study period were analyzed retrospectively. The ratio of non-albicans Candida species (81.4%) was nearly four times higher than that of C. albicans candidemia (18.5%). Overall, C. parapsilosis caused the majority (61.4%) of candidemia episodes, followed by C. tropicalis (14.8%), C. famata (2.9%), C. ciferrii (1.4%) and C. glabrata (0.7%). The rate of CRBSIs was significantly higher in C. Parapsilosis candidemia. The overall rate of 30-day mortality in 135 candidemia episodes was 4.44%. Nearly half of the C. parapsilosis candidemia was associated with CRBSIs, suggesting its importance in PHO, in which several types of central venous catheters have been used.
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August 2016

Successful immune tolerance induction with low-dose coagulation factor VIII in a patient with hemophilia A from a developing country.

Blood Coagul Fibrinolysis 2016 Sep;27(6):729-31

aDepartment of Pediatric Hematology-Oncology, Dr Behcet Uz Children's Hospital, Izmir bDepartment of Pediatric Hematology-Oncology, Marmara University Faculty of Medicine, Istanbul, Turkey.

Inhibitor development is the most frequent and serious complication of the treatment in patients with hemophilia. Immune tolerance induction (ITI) is the only option of treatment for the eradication of factor VIII (FVIII) inhibitor. We would like to present our case with hemophilia whose FVIII inhibitor eradication was done by a low-dose ITI regimen. Our patient has been applied on-demand therapy until 8 years of age. Secondary prophylaxis was began because of having hemophilic arthropathy. A low titer of FVIII inhibitor (4.2 BU/ml) was detected in the fifth month of the prophylaxis. The peak inhibitor titer of patient was 4.6 BU/ml, and there was no decrease in inhibitor titer in the follow-up duration. The low-dose ITI (50 IU/kg, 3 days a week) was started. His inhibitor level was detected negative and the recovery test was ameliorated in the 15th of the ITI therapy. High-dose regimen ITI could not be given particularly in developing countries such as Turkey in view of the high cost of treatment. Patients who had good risk factors might be successfully treated by using low-dose ITI regimen as effective as high-dose ITI regimen.
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http://dx.doi.org/10.1097/MBC.0000000000000366DOI Listing
September 2016

Half-and-half nails in a pediatric patient after chemotherapy.

Cutan Ocul Toxicol 2015 19;34(4):350-1. Epub 2015 Jan 19.

b Department of Hematology , Dr. Behcet Uz Children's Hospital , Izmir , Turkey.

Half-and-half nail, characterized by a reddish brown distal band with a sharply demarcated white proximal band, is a specific manifestation of chronic kidney disease, but it is unusual to occur after chemotherapy. We report a seven-year-old girl who developed half-and-half nails in her fingers one month after treatment with modified Berlin-Frankfurt-Munster protocol followed by maintenance therapy with oral methotrexate and 6-mercaptopurine for pre-B acute lymphoblastic leukemia.
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http://dx.doi.org/10.3109/15569527.2014.999861DOI Listing
September 2016

Acute generalized exanthematous pustulosis induced by ceftriaxone use.

Postepy Dermatol Alergol 2014 Aug 8;31(4):269-71. Epub 2014 Sep 8.

Department of Pediatric Allergy, Dr Behcet Uz Children's Hospital, Izmir, Turkey. Head of Department: Demet Can Assoc. Prof., MD.

Acute generalized exanthematous pustulosis (AGEP) is a rare cutaneous rash characterized by the abrupt onset of a generalized pustular rash often accompanied by fever. There is a history of drug use in 90% of the cases. Here we have reported a 15-year-old male patient with sickle cell anemia who developed AGEP after the use of ceftriaxone. Our patient was hospitalized because of vaso-occlusive crisis and on the third day of ceftriaxone treatment, erythematous pustular lesions accompanied with fever were observed on the body and extremities. Resolution of symptoms followed discontinuation of ceftriaxone. Sensitivity to ceftriaxone was shown with a patch test. The AGEP was considered due to clinical and histopathological findings. This is the first pediatric case of AGEP due to ceftriaxone.
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http://dx.doi.org/10.5114/pdia.2014.40938DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4171671PMC
August 2014

Congenital amegakaryocytic thrombocytopenia: three case reports from patients with different clinical diagnoses and somatic abnormalities.

Blood Coagul Fibrinolysis 2015 Apr;26(3):337-41

aDepartment of Pediatric Hematology, Faculty of Medicine,Celal Bayar University, Manisa bDepartment of Pediatric Hematology, Dr Behcet Uz Children's Hospital, Izmir cDepartment of Pediatric Hematology, Faculty of Medicine, Sütçü İmam University, Kahramanmaraş dDepartment of Pediatric Hematology, Faculty of Medicine, AkdenizUniversity, Antalya, Turkey.

The congenital amegakaryocytic thrombocytopenia (CAMT) is a syndrome characterized by preservation of granulocytic and erythroid cells during genesis, with a gradual or progressive decrease in the number of megakaryocytic series of cells in the bone marrow. At later times, most patients develop aplastic anemia. It is important to rule out specific causes of thrombocytopenia that develop in the early stages of CAMT. Typically, there are no specific somatic abnormalities that accompany this deadly disease. Here we present three CAMT cases that presented with different clinical diagnoses, with various physical anomalies in two of those cases. The first patient was examined because of a cytomegalovirus infection. The second patient had been referred with a suspected neonatal alloimmune thrombocytopenia, whereas the third patient presented with chronic immune thrombocytopenic purpura. Subsequently, all three patients were diagnosed with CAMT. Two of the patients had physical anomalies. In particular, the first patient had a duplex urinary system. To our knowledge, this is the first patient with CAMT to have a duplicated collecting sysem. The second patient had a secundum atrial septal defect, an atypical facial appearance, and growth retardation. Since CAMT could also be observed outside the neonatal period, the differential diagnosis for thrombocytopenia should be considered for all age groups. Moreover, it should be considered that CAMT may also be accompanied with somatic abnormalities.
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http://dx.doi.org/10.1097/MBC.0000000000000192DOI Listing
April 2015

The effectiveness of tools for monitoring hemophilic arthropathy.

J Pediatr Hematol Oncol 2015 Mar;37(2):e80-5

*Clinic of Hematology ‡Clinic of Physical Medicine and Rehabilitation, Dr Behcet Uz Children's Hospital †Alsancak Bilrad Magnetic Resonance Imaging Center, Izmir, Turkey.

Introduction: Hemophilic arthropathy is the most important cause of morbidity in patients with hemophilia. The earliest alterations that occur during the development of hemophilic arthropathy can be shown using magnetic resonance imaging (MRI). In addition, various tools have been developed to monitor joint health.

Aim: : The purpose of this study was to determine the correlation between these tools when used to assess hemophilia patients.

Methods: This cross-sectional study enrolled 38 hemophilia patients between 2 and 18 years of age. Hemophilia Joint Health Score (HJHS) and radiologic scores (Pettersson and Arnold-Hilgartner) were used to evaluate the joints of the patients (n=236). Magnetic resonance imaging (MRI) was performed on 46 joints that were pathologic according to the HJHS. These joints were imaged bilaterally; therefore, 14 normal joints were imaged. In addition, the Functional Independence Score in Hemophilia (FISH) was used to evaluate the joints of 33 patients.

Results: The HJHS scores were correlated with the MRI and FISH scores. The annual bleeding rate was not correlated with any scores; however, the number of painful joints was correlated with the MRI scores. The radiologic scores were correlated weakly with progressive score and HJHS.

Conclusion: The agreement between the HJHS scores and the MRI scores suggests that the HJHS may be used safely as a first-line tool. We recommend that the FISH should be used in the routine follow-up of hemophilia patients as a functional evaluation tool. Painful joints may be useful in deciding to apply MRI, whereas the bleeding frequency may not be useful.
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http://dx.doi.org/10.1097/MPH.0000000000000210DOI Listing
March 2015

The investigation of relationship between joint findings and serum angiogenic and inflammatory factor levels in severe hemophilia A patients.

Blood Coagul Fibrinolysis 2014 Oct;25(7):703-8

aDepartment of Pediatric Hematology, Dokuz Eylül University Faculty of Medicine bDepartment of Pediatric Hematology-Oncology, Ege University Faculty of Medicine cDepartment of Pediatric Hematology-Oncology, Dr Behçet Uz Training and Research Hospital dDepartment of Pediatric Hematology-Oncology, Tepecik Training and Research Hospital eDokuz Eylül University Faculty of Medicine, Hematology Laboratory, Izmir, Turkey.

Despite the use of primary prophylactic Factor VIII replacement in severe hemophilia A patients, bleeding into joints cannot be prevented completely and early diagnosis and treatment of the joint bleedings are important for prevention of permanent joint damage. Recent studies have shown that neoangiogenesis plays important role in development of synovitis after recurrent joint bleedings. This study aimed to investigate the relationship between joint findings and levels of serum angiogenic and inflammatory factors in severe hemophilia A patients.The patient groups consisted of 10 severe hemophilia A patients with acute joint bleeding and 25 severe hemophilia A patients without acute joint bleeding. They were all inhibitor negative. The control group consisted of 22 healthy male children. Complete blood cell count analysis, C-reactive protein (CRP), serum ferritin, lactic acid, and ELISA-based detection of vascular endothelial growth factor (VEGF), intercellular adhesion molecule-1, thrombomodulin, macrophage migration inhibitory factor (MIF), and endostatin were performed from peripheral blood of patient and the control groups. CRP and MIF levels were detected significantly higher in hemophilia patients with acute joint bleeding than patients without acute joint bleeding. There was a positive correlation between serum thrombomodulin, VEGF, and MIF levels. In this study, we demonstrated that serum CRP and MIF levels increases in acute bleeding period regardless of the presence of previous joint damage in children with severe hemophilia. CRP elevation may be a useful and rapid marker for acute bleeding in these patients.
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http://dx.doi.org/10.1097/MBC.0000000000000131DOI Listing
October 2014

Spontaneous epidural and subdural hematoma in a child with afibrinogenemia and postoperative management.

Blood Coagul Fibrinolysis 2014 Jun;25(4):398-400

aDepartment of Neurosurgery bDepartment of Pediatric Hematology-Oncology cDepartment of Pediatric Intensive Care Unit dDepartment of Pediatric Emergency, İzmir Behcet Uz Children's Training and Research Hospital, Izmir, Turkey.

Congenital afibrinogenemia is a rare coagulation disorder that exhibits recessive inheritance. The prevalence of this disease is around 1 per 1 000 000, but it is increased in countries where consanguineous marriages are common. Umbilical cord bleeding during the neonatal period is generally the first manifestation of the disease, but a later age of onset is not uncommon. This disease may also be manifested by gastrointestinal, genitourinary, mucosal, muscular, articular, and intracranial bleeding during childhood. Intracranial hemorrhage is a rare condition, but it is the leading cause of death in patients with afibrinogenemia. In this report, we present the case of a 13-year-old female patient with afibrinogenemia who underwent an operation for spontaneous massive extradural and subdural hematoma.
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http://dx.doi.org/10.1097/MBC.0000000000000049DOI Listing
June 2014

A single center's experience with Candida parapsilosis related long-term central venous access device infections: the port removal decision and its outcomes.

Pediatr Hematol Oncol 2014 Aug 2;31(5):435-41. Epub 2014 Jan 2.

1Division of Pediatric Infectious Disease, Dr. Behçet Uz Children's Hospital, İzmir, Turkey.

Pediatric cancer patients have an increased risk of potentially life-threatening fungal infections such as Candida parapsilosis, associated with long-term CVADs. The Infectious Diseases Society of America (IDSA) guidelines on Candida catheter-related bloodstream infections recommend systemic antifungal therapy and catheter removal. In this study, we focused on our experience with antifungal failure due to totally implanted catheter-associated C. parapsilosis bloodstream infections. We investigated cases leading to port removal in pediatric malignancy patients and the associated patient outcomes. In the first phase of the study, a retrospective chart review was performed to collect patient information, including primary disease; time from hospitalization to port-related candidemia; antifungal drug choice; and the time at which port removal occurred. During the second phase, antifungal susceptibility tests for C. parapsilosis were performed in our microbiology laboratory. All patients had fevers and were neutropenic at the time of candidemia diagnosis. The mean duration between the first isolation of Candida parapsilosis from the port samples to the port removal was 9.75 ± 5.29 days for 11 patients. Patient fevers lasted for a mean time of 16.22 ± 6.51 days. The median recovery duration from fever after CVC removal was four days (range 2-12 days). The median duration for achieving negative blood cultures, following antifungal treatment was 18 days (range 10-27 days). Our data favored the removal of catheters in the presence of ongoing fever, as suggested by the guidelines, independent of the chosen antifungal treatment. Future studies with large samples are needed to evaluate the effects of catheter removal on mortality rates and patient outcomes.
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http://dx.doi.org/10.3109/08880018.2013.862587DOI Listing
August 2014

Analysis of fatal cases of pandemic influenza A (H1N1) virus infections in pediatric patients with leukemia.

Pediatr Hematol Oncol 2013 Aug 22;30(5):437-44. Epub 2013 May 22.

Division of Pediatric Hematology, Dr. Behçet Uz Children's Hospital, İzmir, Turkey.

Background: Pandemic influenza A/H1N1/2009 virus usually causes mild illness in healthy children. Chronic medical conditions are recognized as increasing the risk for complications of influenza virus infection. Although most studies including children with acute leukemia and H1N1 virus have reported no deaths, some anectodal reports with low patient numbers have reported mortality rates as high as 28.5%. Here, we report patients with leukemia and H1N1 virus and review the literature.

Methods: Medical records of all children with leukemia and H1N1 virus in our institution were reviewed for demographic, clinical, and laboratory data. We also carried out a systematic review of the English-language literature. Among the 24 articles found, only patients with leukemia and pandemic H1N1 infections were reviewed by two independent reviewers.

Results: Eight of 98 children who received chemotherapy for leukemia were diagnosed with pandemic H1N1 infection. One developed pneumonia and acute respiratory distress syndrome (ARDS) and died. Another one developed hemophagocytic lymphohistiocytosis (HLH) and died due to secondary infection during the 6th week of treatment for HLH. In our study, 2 of 8 patients had a fatal course (25%), compared with an overall mortality of 2.5% in the studies retrieved from PubMed (6/232).

Conclusion: Pandemic H1N1 influenza virus caused mortality in patients with ARDS or HLH; an unexpected finding for pandemic H1N1 (2009) influenza virus. Thus, for children with leukemia and infected with H1N1 virus, short- and long-term complications should be kept in mind during evaluation.
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http://dx.doi.org/10.3109/08880018.2013.796025DOI Listing
August 2013

Higher expression of the novel gene upregulated gene 4 in two acute lymphoblastic leukemia patients with poor prednisolone response.

Acta Haematol 2012 6;128(2):73-6. Epub 2012 Jun 6.

Department of Pediatric Hematology, Harran University, Sanliurfa, Turkey.

Elucidation of the molecular mechanisms of leukemogenesis is important for a better understanding of the prognosis of acute lymphoblastic leukemia (ALL). Studies have shown that the expression of upregulated gene 4 (URG4), which promotes cell growth and survival, is increased in different types of carcinomas including hepatocellular carcinoma, gastric cancer and osteosarcoma. Similarly, higher expression of URG4 and cyclin D1 gene might promote proliferation of the blast cells by causing escape from the G1 checkpoint and entry into the S phase. This study reports the high expression level of URG4 in 2 high-risk ALL patients for the first time in the literature. In conclusion, the higher expression of URG4 in our 2 patients suggests that URG4 might be involved in leukemogenesis. Future studies with a large number of high-risk ALL patients and cell culture studies are needed to demonstrate the exact role of URG4 in leukemogenesis.
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http://dx.doi.org/10.1159/000338220DOI Listing
October 2012