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Surgical site infection (SSI) after elective orthopedic foot and ankle surgery is uncommon and may be higher in selected patient groups. Our main aim was to investigate the risk factors for SSI in elective orthopedic foot surgery and the microbiological results of SSI in diabetic and non-diabetic patients, in a tertiary foot center between 2014 and 2022. Overall, 6138 elective surgeries were performed with an SSI risk of 1.88%. The main independent associations with SSI in a multivariate logistic regression analysis were an ASA score of 3–4 points, odds ratio (OR) 1.87 (95% confidence interval (CI) 1.20–2.90), internal, OR 2.33 (95% CI 1.56–3.49), and external material, OR 3.08 (95% CI 1.56–6.07), and more than two previous surgeries, OR 2.86 (95% CI 1.93–4.22). Diabetes mellitus showed an increased risk in the univariate analysis, OR 3.94 (95% CI 2.59–5.99), and in the group comparisons (three-fold risk). In the subgroup of diabetic foot patients, a pre-existing diabetic foot ulcer increased the risk for SSI, OR 2.99 (95% CI 1.21–7.41), compared to non-ulcered diabetic patients. In general, gram-positive cocci were the predominant pathogens in SSI. In contrast, polymicrobial infections with gram-negative bacilli were more common in contaminated foot surgeries. In the latter group, the perioperative antibiotic prophylaxis by second-generation cephalosporins did not cover 31% of future SSI pathogens. Additionally, selected groups of patients revealed differences in the microbiology of the SSI. Prospective studies are required to determine the importance of these findings for optimal perioperative antibiotic prophylactic measures.

Celkan, Tulin Tiraje/0000-0001-7287-1276; Adaletli, Ibrahim/0000-0003-4531-6466; WOS:000466715800011 PubMed ID: 31205639 Objectives: To review a single center outcome of patients with Langerhans Cell Histiocytosis diagnosed at a tertiary referral hospital from Turkey. Methods: The files between 1989 and 2015 of 80 patients with LCH were retrospectively analyzed. Results: During the 25 years, 80 patients were diagnosed with LCH. The median age at diagnosis was 53 months (2-180 months) and the median follow-up time of patients was 10 years and 9 months (24 months-25 years). Bone was the most frequently affected organ (n:60, 75%). Initially, 43 patients (54%) had single system (SS) disease, 20 patients (25%) had multisystem (MS) disease without risk organ involvement (MS-RO-), and 17 patients (21%) had a multisystem disease with risk-organ involvement (MS-RO+). The overall survival (OS) rate was 91%, and event-free survival (EFS) rate was 67% at 10 years. 10-year OS rate was lower for patients with MS-RO+ (65%) when compared to those with, MS-RO-, and SS (100%, 97%, p value=

Objective: T-cell acute lymphoblastic leukemia (T-ALL) is associated with recurrent chromosomal aberrations andabnormal ectopic gene expression during T-cell development. In order to gain insight into the pathogenesis of T-ALLthis study aimed to measure the level of expression of 7 T-cell oncogenes (LMO2, LYL1, TAL1, TLX1, TLX3, BMI1, andCALM-AF10) in pediatric T-ALL patients Material and Methods: LMO2, LYL1, TLX1, TLX3, BMI1, TAL1, and CALM-AF10 expression was measured usingquantitative real-time PCR in 43 pediatric T-ALL patients. Results: A high level of expression of LMO2, LYL1, TAL1, and BMI1 genes was observed in a large group of T-ALL.Several gene expression signatures indicative of leukemic arrest at specific stages of normal thymocyte development(LYL1 and LMO2) were highly expressed during the cortical and mature stages of T-cell development. Furthermore,upregulated TAL1 and BMI1 expression was observed in all phenotypic subgroups. In all, 6 of the patients had TLX1and TLX3 proto-oncogene expression, which does not occur in normal cells, and none of the patients had CALM-AF10fusion gene transcription. Expression of LYL1 alone and LMO2-LYL1 co-expression were associated with mediastinalinvolvement; however, high-level oncogene expression was not predictive of outcome in the present pediatric T-ALLpatient group, but there was a trend towards a poor prognostic impact of TAL1 and/or LMO2 and/or LYL1 protooncogeneexpression. Conclusion: Poor prognostic impact of TAL1 and/or LMO2 and/or LYL1 proto-oncogene expression indicate the needfor extensive study on oncogenic rearrangement and immunophenotypic markers in T-ALL, and their relationship totreatment outcome. Conflict of interest:None declared.

Objective: To identify the well-known common translocations and FLT3 mutations in childhood acute myelogenousleukemia (AML) patients in Turkey. Material and Methods: The study included 50 newly diagnosed patients in which t(15;17), t(8;21), and inv(16)chromosomal translocations were identified using real-time PCR and FLT3 gene mutations were identified via direct PCR amplification PCR-RE analysis. Results: In all, t(15;17) chromosomal aberrations were observed in 4 patients (8.0%), t(8;21) chromosomal aberrationswere observed in 12 patients (24.0%), inv(16) chromosomal aberrations were observed in 3 patients (6.0%), and FLT3-ITD mutations were observed in 2 patients (4.0%); FLT3-D835 point mutation heterozygosity was observed in only 1patient (2.0%) patient. Conclusion: Despite of the known literature, a patient with FLT3-ITD and FLT3-D835 double mutation shows a bettersurvival and this might be due to the complementation effect of the t(15;17) translocation. The reportedmutation ratein this article (4%) of FLT3 gene seems to be one of the first results for Turkish population.

Pandemic influenza A infection (2009 H1N1) was associated with a worldwide outbreak of febrile respiratory infection. Although usually it results in a mild illness, certain patient groups are at increased risk for complications. The authors reviewed their experience in a pediatric hematology-oncology unit to determine the outcome of this disease in children with hematological conditions and solid tumors. During the second outbreak (1 November 2009 to 14 January 2010), a total of 187 children from pediatric clinic were tested for H1N1 influenza A by multiplex polymerase chain reaction (PCR), 63 of them were positive. Patients' signs and symptoms were recorded prospectively. Ten (35.7%) (5 children with solid tumors, 4 with leukemia, 1 with hereditary spherocytosis) of 28 tested children with hematological conditions were diagnosed with 2009 H1N1 influenza infection. Fever (100%) and cough (90%) were the most common symptoms. Five were neutropenic (neutrophil count

This study aimed to assess the demographic data and treatment results of children who were diagnosed with Burkitt lymphoma and treated according to the Berlin-Frankfurt-Münster-95 (BFM) protocol in a single institution. A total of 48 patients (37 boys, 77%) with a median age of 8 years (range 2 to 16 years) at diagnosis, were evaluated. Primary tumor sites were abdomen (70.8%), head and neck (22.9%), peripheral lymph node (2%), bone (2%), and testis (2%). The 5-year overall survival (OS) and event-free survival (EFS) were 78.1±4% and 76.6±6%, respectively. In univariate analysis, hemoglobin level less than 10 g/dL, cerebrospinal fluid (CSF) positivity and dialysis requirement at diagnosis were found to be important reverse predictor factors for EFS (P; 0.001, 0.001, 0.004, respectively). In multivariate analysis, hemoglobin level less than 10 g/dL and dialysis at diagnosis were found to be important reverse predictor factors for EFS (P; 0.0001). The EFS of our patients was lower than the values achieved with BFM-95 protocol in other centers. This study provides evidence that low hemoglobin level, CSF positivity and dialysis at diagnosis were important predictor factors for EFS in children with Burkitt lymphoma.

melikoglu, mustafa/0000-0001-9646-3787; Aydin, Burca/0000-0002-6013-6271 WOS: 000278808800001 PubMed: 20367260 Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients 2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

The NOTCH signaling pathway plays important role in the development of multicellular organisms, as it regulates cell proliferation, survival, and differentiation. In adults, it is essential for the T- or B-lymphocyte lineage commitment.NOTCH1and FBXW7 mutations both lead the activation of theNOTCH1pathway and are found in the majority of T-ALL patients. In this study, the mutation analysis ofNOTCH1andFBXW7genes was performed in 87 pediatric T-ALLs who were treated on the ALL-BFM protocols. In 19 patients (22%), activatingNOTCH1mutations were observed either in the heterodimerization domain or in the PEST domain and 7 cases (10%) demonstrated FBXW7 mutations (2 cases had bothNOTCH1andFBXW7mutations). We also analyzed the relationship of the mutation data between the clinical and biological data of the patients.NOTCH1andFBXW7,NOTCH1alone were found correlated with lower initial leucocyte counts which was independent from the sex and T- cell immunophenotype. However,NOTCH1andFBXW7mutations were not predictive of outcome in the overall cohort of pediatric T-ALLs.

The NOTCH signaling pathway plays important role in the development of multicellular organisms, as it regulates cell proliferation, survival, and differentiation. In adults, it is essential for the T- or B-lymphocyte lineage commitment. NOTCH1 and FBXW7 mutations both lead the activation of the NOTCH1 pathway and are found in the majority of T-ALL patients. In this study, the mutation analysis of NOTCH1 and FBXW7 genes was performed in 87 pediatric T-ALLs who were treated on the ALL-BFM protocols. In 19 patients (22%), activating NOTCH1 mutations were observed either in the heterodimerization domain or in the PEST domain and 7 cases (10%) demonstrated FBXW7 mutations (2 cases had both NOTCH1 and FBXW7 mutations). We also analyzed the relationship of the mutation data between the clinical and biological data of the patients. NOTCH1 and FBXW7, NOTCH1 alone were found correlated with lower initial leucocyte counts which was independent from the sex and T- cell immunophenotype. However, NOTCH1 and FBXW7 mutations were not predictive of outcome in the overall cohort of pediatric T-ALLs.

Polymorphisms have been identified in several DNA repair genes. These polymorphisms may effect DNA repair capacity and modulate cancer susceptibility. In this study, we aimed to determine the four polymorphisms in two DNA repair genes, xeroderma pigmentosum complementation group D (XPD) and X-ray repair cross-complementing group 1 (XRCC1), in a sample of Turkish patients with childhood acute lymphoblastic leukemia (ALL), and evaluate their association with childhood ALL development. We used polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP), to analyze XPD Asp312Asn, XPD Lys751Gln, XRCC1 Arg194Trp, and XRCC1 Arg399Gln polymorphisms in 70 patients with childhood ALL and in 75 disease-free controls, who were of a similar age. No significant differences were observed among the study groups with regard to the XPD codon 312, XPD codon 751, XRCC1 codon 194, and XRCC1 codon 399 polymorphisms. However, the combined XRCC1 Arg194Trp/Trp194Trp variant genotypes were associated with increased risk for ALL in females (OR = 5.47; 95% CI = 1.49–20.10; p = 0.008). This finding indicates that females carrying XRCC1 194Trp allele are at increased risk of developing childhood ALL. These results suggest that the risk of childhood ALL may be associated with DNA repair mechanisms, and understanding these mechanisms will help identify individuals at increased risk of developing childhood ALL, and also should be lead to improved treatment of ALL.

The authors evaluated the possible effect of DNA repair genes, XPD (Xeroderma pigmentosum group D) codon (312 and 751) and XRCC1 (X-ray repair cross-complementing group 1) codon (194 and 399) SNPs (single-nucleotide polymorphisms) on the risk of childhood B-cell lymphoma.The polymorphisms were analyzed in 33 patients with BL cases and in 52 healthy, age-matched controls using PCR-RFLP method.The authors observed no association between variation in the XPD codon Asp312Asn, Lys751Gln, and XRCC1 codon Arg399Gln polymorphisms and B-cell lymphoma for any parameter. In contrast, tryptophan allele frequency in control and patient groups was 0.10 and 0.03 respectively (p = .04). The frequency of XRCC1 194Arg/Trp genotype in B-cell lymphoma was significantly lower than that in controls (p = .005). No significant relationship was found between genotypes and stage, lactate dehydrogenase, or bone marrow involvement.XRCC1 194Trp allele may be associated with a protective effect against development of childhood B-cell lymphoma. However, these results were based on a small number of case and further studies should be done.

The aim of our study was to show how the progression and severity of Familial Mediterranean Fever (FMF) is affected by procoagulant activity and alterations in the markers of thrombosis and fibrinolysis. The study cohort comprised 64 FMF patients who were classified as attack-free patients (Group 1; n = 34 patients, aged 3-19 years) and attack patients (Group 2; n = 30 patients, aged 3-21 years). All patients were on colchicine treatment with the exception the newly diagnosed patients in Group 2. A total of 14 healthy subjects between 5-12 years of age were enrolled as controls (Group 3). Laboratory tests, including leukocyte and thrombocyte counts, erythrocyte sedimentation rate, CRP, fibrinogen, PT, aPTT, Factor VIII, vW factor, D-dimer, P-selectin, tPA and PAI-1, were carried out on all patients. Inflammation continued both during the attack and attack-free period in FMF. The prolongation of PT was observed during attacks (PT = 13.6 s in Group 2, and PT = 12.6 s in Group 3; p = 0.002). tPA levels increased in FMF patients (tPA levels of group 1, 2 and 3 were 12.6, 13.2 and 9.7 ng/ml, respectively; p = 0.01). P-selectin was lower in both patient groups than in the control group. During attack periods PAI-1 levels increased (PAI-1 level of Group 1: 89.6 ng/ml and PAI-1 level of Group 2: 335.7 ng/ml, p = 0.000). Inflammation with increased acute phase reactants continued during both attack and attack-free periods in FMF patients. Prolongation of PT and differences in tPA and P-selectin levels suggest that hypercoagulability may have a role in the etiopathogenesis of FMF. It may be possible to use PAI-1 as a marker for the attacks of FMF.

Background:: There are a few published studies about prognostic markers of Epstein-B virus (EBV) related to outcomes in pediatric Hodgkin Lymphoma (HL). Objectives:: We aimed to investigate the prognostic value and effect of EBV on survival by using biopsy materials in children and adolescents diagnosed with HL. Patients and Methods:: EBV LMP-1 expression was examined using immunohistochemical methods in 58 tumor samples. Clinical features, overall survival (OS) and failure free survival time (FFS) were compared between EBV LMP-1 positive and negative patients. Results:: In 20 (35%) patients tumors were LMP-1 positive. When compared with patients above 10 years old, EBV LMP-1 was often positive in patients under 10 years old (30% vs. 70%, P = 0.02). In our most cases having B symptoms and advanced stage, EBV positiveness in Hodgkin Reed-Stenberg cells (H-RS) was not a significant determinant for survival (P = 0.78). Half of the past clinical trials in childhood HL reported longer survival rates in EBV LMP-1 positive patients. In some trials similar to our results there was no significant relationship between EBV and prognosis. Conclusions:: The reason of diminished EBV positiviness may be related to technical methods such as not using immunohistochemical and in situ hybridization for EBER antigen but in laboratory conditions painting of control tissues with EBV impair this probability. In addition, cases enrolled to our study were living in Istanbul where social and economical factors are improved rather than generally.

Immune thrombocytopenia (ITP) is an acute self-limited disease of childhood, mostly resolving within 6 months irrespective of whether therapy is given or not. Treatment options when indicated include corticosteroids, intravenous immune globulin (IVIG), and anti-RhD immunoglobulin. We reviewed our 32 years' experience for first-line therapy of acute ITP. Five hundred forty-one children (mean age: 5.3 years) diagnosed and treated for ITP were evaluated retrospectively. Among 491 acute ITP patients, IVIG was used in 27%, high-dose steroids in 27%, low-dose steroids in 20%, anti-D immunoglobulin G (IgG) in 2%, and no therapy in 22%. When the initial response (platelets >50 × 10(9)/L) to first-line treatment modalities were compared, 89%, 84%, and 78% patients treated by low-dose steroids, high-dose steroids, and IVIG responded to treatment, respectively (P > .05). Mean time to recovery of platelets was 16.8, 3.8, and 3.0 days in patients treated with low-dose steroids, high-dose steroids, and IVIG, respectively (P < .0001). Thrombocytopenia recurred in 23% of low-dose steroid, 39% of high-dose steroid, and in 36% of IVIG (P < .0001) treatment groups. Of 108 patients who were observed alone, 4 (3%) had a recurrence on follow-up and only 2 of these required treatment subsequently. Recurrence was significantly less in no therapy group compared with children treated with 1 of the 3 options of pharmacotherapy (P < .0001). Response rates were similar between patients treated by IVIG and low- and high-dose steroids; however, time to response was slower in patients treated with low-dose steroids compared with IVIG and high-dose steroids.

An important life-threatening complication of intensive chemotherapy administered in children with leukemia is febrile neutropenia. The objective of this study was to evaluate the clinical features and consequences of febrile neutropenia attacks in children who were treated for acute lymphoblastic leukemia.Nighty-six children who received chemotherapy for acute lymphoblastic leukemia in our center between January 1995 and December 2010 were included in the study. The data related to demographic characteristics, treatment features, relapse and febrile neutropenia incidences, risk factors, culture results and prognosis were retrospectively evaluated from the patients' files.A total of two hundred-ninety nine febrile neutropenia attacks observed in the patients during initial treatment and relapse treatment were evaluated. When the incidence of febrile neutropenia was evaluated by years, it was observed that the patients treated after year 2000 had statistically significantly more febrile neutopenia attacks compared to the patients treated before year 2000. When the incidences of febrile neutropenia during initial treatment and during relapse treatment were compared, it was observed that more febrile neutropenia attacks occured during relapse treatment. Fifty-nine percent of all febrile neutropenia attacks were fever of unknown origin. Eighty microorganisms grew in cultures during febrile neutropenia throughout treatment in 75 patients; 86% were bacterial infections (50% gram positive and 50% gram negative), 8% were viral infections and 6% were fungal infections. Coagulase negative staphylococcus (n=17) was the most frequent gram positive pathogen; E. Coli (n=17) was the most commonly grown gram negative pathogen.In this study, it was found that an increase in the incidence of febrile neutropenia occured in years. Increments in treatment intensities increase the incidence of febrile neutropenia while improving survival. Evaluation of febrile neutropenia results by hematology-oncology units in years will be directive in early and successful treatment.

Anemia is a frequent manifestation and an important health problem. The aim of this study was to found the prevalence of anemia among primary schoolchildren and determine the risk factors associated with anemia. The study group was composed of 1531 students between 6 and 16 years old from 14 primary schools located in seven different regions of Istanbul. Data were collected via a questionnaire filled out by parents. For all students, a complete blood cell count detected by an automated cell counter and anemia prevalence was determined according to Dallman criteria. The overall prevalence of anemia was found to be 27.6 per cent. There was no significant relationship between the prevalence of anemia and the students' age, gender, parents' educational level and employment, and monthly family income by logistic regression analysis. Only the number of family members and malnutrition were risk factors for anemia. Today, anemia is a common problem among primary schoolchildren in Istanbul. Identification of the risk factors is essential for the prevention of anemia.

The objective of this study was to investigate the diphtheria-tetanus-pertussis and/or measles-mumps antibody titers before and after vaccination at various time points of acute lymphoblastic leukemia (ALL) therapy and to suggest an appropriate vaccination approach for ALL patients. The authors studied 37 ALL patients and 14 healthy control subjects, divided into three groups. In group 1 (newly diagnosed patients), baseline anti-diphtheria, anti-tetanus, and anti-pertussis titers were determined. Patients in group 2 (on maintenance chemotherapy) and group 3 (patients not receiving therapy for 3-6 months) were vaccinated with diphtheria-tetanus with or without acellular pertussis; group 3 and control subjects were also given measles-mumps-rubella vaccine. Preimmunization and 1-month postimmunization titers were drawn. Preimmunization anti-diphtheria and anti-tetanus antibody titers between the groups and the controls were statistically similar. The seropositivity rate for anti-measles antibody in group 3 was significantly lower than controls. After vaccination, all of the patients developed protective anti-diphtheria and anti-tetanus antibody titers. The seroconversion rates of group 3 and controls for anti-measles and anti-mumps antibodies were statistically similar. The results showed that patients on maintenance therapy and after cessation of therapy made good antibody responses to diphtheria and tetanus toxoids, but response to measles and mumps vaccines was not as sufficient as toxoid vaccines. Children with ALL can receive the appropriate vaccines during and after maintenance treatment.

The blue rubber bleb nevus syndrome (BRBNS) is a rare vascular malformation syndrome with cutaneous and visceral lesions frequently associated with serious, even fatal bleeding. No systemic therapy is currently available. We report here a case with disseminated skin and gastrointestinal venous malformations and findings of disseminated intravascular coagulation that was treated with interferon β. The disseminated intravascular coagulation manifestations were cleared with the treatment. The regression of the coagulopathy in our patient led us to think that the use of interferon β in BRBNS might be beneficial.

Infection-related mortality affects the overall survival rates of children who are receiving treatment for cancer. The leading cause of mortality is bacteremia and sepsis related to it in febrile neutropenic patients. All positive blood cultures of febrile neutropenic patients treated in the Department of Pediatric Hematology-Oncology, Cerrahpasa Medical School, between January 1995 and January 2001 were reviewed. Cultures grew 159 micro-organisms, 95 (60 per cent) of which were Gram-positive bacteria, 56 (35 per cent) were Gram-negative bacteria and eight (5 per cent) were fungi. Coagulase-negative staphylococci (63, 40 per cent) and S. aureus (8, 5 per cent) were the most frequent Gram-positive pathogens. Klebsiella, E. coli, Enterobacter and Pseudomonas infections were the primary Gram-negative pathogens. Twenty cases were lost because of sepsis: in 11 cases (55 per cent) Gram-negative bacteria, in eight cases (40 per cent) Gram-positive bacteria, and in only one case a fungus were the causative organisms. Although vancomycin was not included in the first-line treatment, the mortality rate of Gram-positive bacteremia was 8 per cent. In Gram-negative bacteremia it was 20 per cent. Gram-negative pathogens, which were resistant to multiple antibiotics, caused the mortality. Drug resistance and mortality due to micro-organisms must be taken into consideration while febrile neutropenia protocols are prepared.

Summary Sialoblastomas are very rare congenital tumors of the salivary gland that usually occur in parotid or submandibular glands. They can be benign or malignant histologically. The recommended treatment for sialoblastoma is the excision of tumor totally and widely. Alternative therapies for sialoblastoma treatment remains controversial, chemotherapy may be an alternative treatment for relapsed or metastatic sialoblastomas. In this report we describe a malignant and relapsed sialoblastoma at cheek region.

Having read the article “Oral valacyclovir as prophylaxis against herpes simplex virus reactivation during high dose chemotherapy for leukemia” by Orlowski et al. [1], we wanted to write about our ...

Amaç: Eritrosit ortalama hacmi (MCV) özellikle anemilerin s›n›fland›r›lmas›nda kullan›lan, otomatik kan say›m cihazlar›n›n günlük kullan›ma girmesinden sonra kolay de¤erlendirilebilen bir kan de¤iflkenidir. Folik asit antagonistleri, DNA sentez inhibitörleri ve siklofosfamid içeren protokollerin kullan›ld›¤› kemoterapi yönteminde eritrosit ortalama hacmi’nin yükseldi¤i bilinmektedir. Lösemili hastalar›m›zda kemoterapi etkisi ile MCV de¤ifliminin ve tedavi sonras› de¤erlerinin seyir ve nüks ile iliflkisini incelemeyi hedefledik. Gereç ve Yöntem: Ocak 1995 sonras›nda Cerrahpafla T›p Fakültesi, Çocuk Hematoloji-Onkoloji Bilim Dal›’nda tan› ve tedavi alm›fl 125 akut lenfoblastik lösemi hastas›n›n dosyas› geriye dönük olarak incelendi. Olgular›n (yafllar› 1-15 ortalama 5,99+ 3,94 olan 42 k›z, 83 erkek) tan› s›ras›nda, yo¤un kemoterapi bitiminde ve idame tedavi sonras›nda MCV de¤erlerine bak›ld›. Bulgular: Hastalar›n tan› s›ras›nda, yo¤un kemoterapi bitiminde ve idame tedavi sonras›nda MCV de¤erleri s›ras› ile 78,5+3,9 fL; 89,1+6,8 fL ve 95,6+3,8 fL olarak bulundu. Tedavi sonras›nda MCV de¤erlerinin ortalama ayda 1 fL geriledi¤ini saptand›. Bir talasemi minör olan olgumuzun antimetabolitlere ra¤men MCV de¤erlerinin maksimum 73 fL oldu¤unu gördük. Nüks saptanan 15 olgunun verileri 110 nüks saptanmayan olgu ile karfl›laflt›r›ld›. Aralar›nda MCV de¤erleri aç›s›ndan anlaml› fark saptanmad› (p>0,05). Çıkarımlar: Eritrosit ortalama hacmi’nin lösemi izleminde seyir ve nüks aç›s›ndan önemi gösterilememifltir., nbsp

Malignant rhabdoid tumor is a rare and malignant tumor of childhood. Generally it originates from the kidney and central nervous system, but occasionally it may arise from the orbit. Differential diagnosis from other childhood soft tissue sarcomas should be done. We report here an 8-week-old female infant with malignant rhabdoid tumor of the orbit who was treated with chemotherapy and surgery. The 8 week-old girl was referred to our hospital with a history of right proptosis first noted at birth. Physical and laboratory evaluation of the patient was normal except for right proptosis. The mass was removed surgically. Histopathologic examination and immunohistochemical findings of the specimen were evaluated as malignant rhabdoid tumor. Chemotherapy was administered. While in clinical remission, she succumbed during a febrile episode. Malignant rhabdoid tumor can rarely originate from the orbit. Malignant rhabdoid tumor should be kept in mind in the differential diagnosis of orbital masses, and surgery, chemotherapy and local radiotherapy should be used as combined therapy due to the poor prognosis.

B-lineage acute lymphoblastic leukemia (B-ALL) is a common subtype of acute leukemia in children. PAX5 plays a central role in B-cell development and differentiation. In this study, we analyzed PAX5 expression levels, transactivation domain mutations/deletions in B-ALL patients (n=115) and healthy controls (n=10). Relative PAX5 mRNA levels were significantly increased in B-ALL patients (p

We report a fatal case of toxic epidermal necrolysis (TEN) resulting from a high dose of cytosine arabinoside (ARA-C). A 13-year-old girl with acute lymphocytic leukemia was treated according to the protocol of the BFM Group (BFM-95, HRG). On the fifth day after administration of a high dose of ARA-C (2 g/m2 intravenously every 12 hours), she developed bullous lesions on the hands and soles that disseminated, evolving to necrosis, sepsis, and death on the 22nd day. ARA-C is frequently associated with dermatologic toxicity, but this is only the second case of toxic epidermal necrolysis described in connection with this drug.

Background It is well known that survivors of acute lymphoblastic leukemia (ALL) show a tendency to become overweight. Cranial irradiation (CRT), is considered to be the primary risk factor for development of obesity. Aims The aim of our study was to evaluate body mass index (BMI) and serum leptin levels in survivors of childhood ALL. Methods Subjects (Group I) consisted of 93 survivors of childhood ALL (53 males > or = 9 years old, 40 females > or = 8 years old) diagnosed between January 1975 and December 2002 in the Hematology-Oncology Division in Cerrahpasa Medical Faculty, Istanbul University after a follow up 10.21 +/- 4.90 (mean +/- SD) years. Fifty healthy individuals of similar chronological age were taken as controls (29 males, 21 females). Seventy-four subjects had received radiotherapy (Group IA) and 19 had not (Group I B). Results In Group I, BMI was significantly higher than in Group II (21.65 +/- 4.02 vs 20.31 +/- 3.49, p = 0.04). However, BMI was significantly higher only in Group I A (21.83 +/- 4.27) than in Group II (p = 0.032). Leptin levels were significantly higher in Group I A females than in Group II females. There was a significant correlation between BMI-SDS and serum leptin levels in group IA females. Conclusions Leukemia treatment leads to obesity. Higher leptin levels in girls may suggest that sex may be a differentiating factor for this late effect.

The aim of this study was to evaluate the incidence of anemia detected in familial Mediterranean fever (FMF) and the effect of disease activity and colchicine therapy along with interleukins to laboratory tests, including serum transferrin receptor (TfR), in the diagnosis of anemia seen in FMF in children. After detecting anemia in 63.4% of 172 FMF patients followed up by our rheumatology outpatient polyclinics, it was decided to study 3 groups of patients: group 1, 17 newly diagnosed FMF patients; group 2, 36 FMF patients on colchicine therapy; and group 3, 17 healthy children as control for the symptom of anemia. All 3 groups of patients were investigated for their hematological parameters, iron status, including soluble transferrin receptor (sTFR) concentrations and sTFR index, and IL-6 levels. Anemia ratio was 9/17, 53%; 11/36, 31%; and 1/17, 5% in the groups 1, 2 and 3, respectively. There was a significant difference between hemoglobin (Hb) values in the first group and the second (patients who were on colchicine therapy). Furthermore, in the second group there was a significant difference between the Hb concentrations at the time of diagnosis and after colchicine therapy (p = .003). There was a positive correlation between Hb and plasma iron and transferrin saturation in group 1 and disease beginning age, iron, transferrin saturation, and erythrocyte sedimentation rate (ESR) in the second group. In the first group the anemic patients' iron and transferrin saturation were significantly lower than normal, while ferritin levels were higher. In the second group, a good correlation was found with ESR and Hb levels; the higher ESR values were detected in patients with lower Hb values. Of the anemic and nonanemic patients of the first and second groups, values for interleukin 6 and iron parameters, including sTFR, were found similar. Anemia detected in FMF patients was found related to iron status more than interleukins. Colchicine therapy had a positive effect on anemia as well as on disease activity. Resolution of symptoms of FMF occurred with correction of the anemia, if the patient ESR values also decreased on colchicine therapy.

WOS: 000223843200001, PubMed ID: 15626013, This study analyzes the data of thrombotic children who were followed up in different pediatric referral centers of Turkey, to obtain more general data on the diagnosis, risk factors, management, and outcome of thrombosis in Turkish children. A simple two-page questionnaire was distributed among contact people from each center to standardize data collection. Thirteen pediatric referral centers responded to the invitation and the total number of cases was 271. All children were diagnosed with thromboembolic disease between January 1995 and October 2001. Median age at time of first thrombotic event was 7.0 years. Of the children 4% of the cases were neonates, 12% were infants less than 1 year old, and 17% were adolescents. Thromboembolic event was mostly located in the cerebral vascular system (32%), deep venous system of the limbs, femoral and iliac veins (24%), portal veins (10%), and intracardiac region (9%). Acquired risk factors were present in 86% of the children. Infection was the most common underlying risk factor. Inherited risk factors were present in 30% of the children. FVL was the most common inherited risk factor. Acquired and inherited risk factors were present simultaneously in 19% of the patients. Eleven children had a history of familial thrombosis. Due to the local treatment preferences, the treatment of the children varied greatly. Outcome of the 142 patients (52%) was reported: 88 (62%) patients had complete resolution, 47 (33%) had complications, 12 (9%) had recurrent thrombosis, and 34 (24%) died. Three children (2.1%) died as a direct consequence of their thromboembolic disease. The significant morbidity and mortality found in this study supports the need for multicentric prospective clinical trials to obtain more generalizable data on management and outcome of thrombosis in Turkish children.

Background NAD(P)H:quinone oxidoreductase1 (NQO1) is a two-electron reductase that detoxifies quinones derived from the oxidation of phenolic metabolites of benzene. Exposure to benzene metabolites increases the risk of hematotoxicity and leukemia. NQO1 enzyme activity protects the cells against metabolites of benzene. C to T base substitution at nucleotide 609 of NQO1 cDNA (C609T) results in loss of enzyme activity. Low NQO1 activity may play a role in etiology of acute leukemia. Procedure We analyzed NQO1 C609T gene polymorphism using the PCR-RFLP method in 273 patients with de novo acute leukemia (189 acute lymphoblastic leukemia (ALL), and 84 acute myeloid leukemia (AML) and 286 healthy volunteers to investigate the role of NQO1 polymorphism in the etiology of acute leukemia. Results and Conclusions The frequency of homozygosity for NOQ1 C609T polymorphism was 3.5% in the healthy control population and 2.5% in pediatric acute leukemia. The NQO1 C609T allele frequency was not statistically different in the children with acute leukemia in comparison to the controls (odds ratio (OR), 0.76; 95% confidence interval (CI), 0.58–1.01; P = 0.06). The distribution of NQO1 genotypes among children with acute leukemia was not statistically different from the control group (P = 0.13). These findings do not support the role of NQO1 C609T polymorphism in the etiology of de novo pediatric acute leukemia. © 2004 Wiley-Liss, Inc.

Purpose: The t(12;21) translocation is the most common reciprocal chromosomal rearrangement in pediatric acute lymphoblastic leukemia (ALL). This translocation fuses two genes, TEL and AML1, and results in the production of the TEL-AML1 fusion protein. The authors investigated the incidence and prognostic significance of the TEL-AML1 fusion gene in patients with ALL in Turkey. Methods: The authors analyzed 219 children with ALL using the reverse transcription-polymerase chain reaction. Results: The TEL-AML1 fusion transcript was detected in 20.1% (44/219) of newly diagnosed children with ALL. TEL-AML1-positive patients had precursor B-cell ALL and were 3 to 10 years old at diagnosis. TEL-AML1-positive patients had a significantly lower rate of relapse compared with TEL-AML1-negative patients. TEL-AML1-positive patients have a higher overall survival rate than TEL-AML1-negative patients. Conclusions: These data support that the presence of TEL-AML1 at diagnosis is an independent favorable prognostic indicator in patients with ALL in Turkey.

A 7-year-old boy with pica presented with a diagnosis of leukemia. He did not have hepatosplenomegaly, lymphadenopathy, or infection. Laboratory results were as follows: hemoglobin (Hb): 6.9 g/dL; mean corpuscular volume (MCV): 66 fL; red cell distribution width (RDW): 20.3%. Iron studies results were consistent with iron deficiency, as follows: serum iron: 13 μg/dL; total iron binding capacity: 416 μg/dL; ferritin level: 10 ng/mL. The patient’s white blood cell (WBC) count was 2.9×103/μL (1.5×103/μL neutrophils) and platelet count was 28×103/μL. Bone marrow aspiration showed a normal number of megakaryocytes, low-level stored iron, and no blasts. The patient was diagnosed as IDA. On d 5 of iron treatment the patient’s findings were as follows: Hb: 7.4 g/dL; MCV: 68 fL; RDW: 25.7%; WBC: 5.6×103/μL; platelet count: 430×103/μL.

Son 30 40 yılda çocuk kanserlerinde büyük aşamalar kaydedilmiş ve çok yüksek sağkalım oranlarına ulaşılmıştır Ergenlik döneminde görülen nbsp; kanserlerin kendine has özellikleri vardır Bu dönemde görülen kanser tipleri farklı olduğu gibi elde edilen sağkalım oranları da farklıdır Bunda ergenin fiziksel yapısı kadar psikolojik etmenler de rol oynamaktadır Çocukluk yaşına göre daha çok sayıda ergen kanser tanısı almaktadır Ergende lösemi ve sarkomların iyileşme oranları çocuklara göre daha düşüktür Bu yaş grubunda Hodgkin hastalığı ve kemik tümörleri de daha sık gözlenmektedir Ergene konusunda deneyimli psikolojik destek ve eğitim sağlayacak bir ekibin tedavisi başarıyı artırmaktadır Türk Ped Arş 2011; 46 Özel Sayı: 68 9, Great progresses were achieved in chilhood cancers in the last 30 40 years and high survival rates are obtained Cancer in adolescents have special features and survival rates This is because of adolescent rsquo;s physical status and psychological needs More adolescents are diagnoseed with cancer in comparison to children The overall survival and cure rates of leukemias and sarcomas in the adolescent period are much lower than childhood Hodgkin rsquo;s disease and bone tumors are more frequent in adolescents Success in the treatment of adolescent cancers will increase with the treatment of these cases in specialised centers with good medical psychologic and educative staff Turk Arch Ped nbsp; 2011; 46 Suppl: 68 9

Aim: Overall survival from leukemia is less in low and middle-income countries than in high-income countries. The aim of this study is to review febrile neutropenia (FN) episodes in children with acute lymphoblastic leukemia (ALL) treated with BFM (Berlin-Franfurt-Münich) protocols at a single center in Turkey. Methods: Retrospective data were extracted from medical records of children (1-18 years old) with newly diagnosed and relapsed ALL and who were treated between 1995-2010 at Istanbul University, Cerrahpasa Medical Faculty, Pediatric Hematology Oncology Dept. Results: Two hundred -forty-five FN episodes were detected in 96 evaluable children (mean age: 5.9±3.7 years; M/F: 50/46). According to BFM risk groups; 32 were SRG (standard risk group), 56 were MRG (medium risk group) and 8 were HRG (high risk group) patients. Fifteen patients had relapsed on follow-up and 2 of them had a second relapse. The mean number of FN episodes was 2.5 (±1.58; 0-8) per newly diagnosed patient and 3.6 (±1.88; 1-8) per relapsed patient. Febrile neutropenia episodes were more frequent at high risk patients compared to standart risk patients (p≤0.05). Patients diagnosed before year 2000 had less FN episodes compared to patients diagnosed after 2000 (p≤0.05). Fifty-seven of the FN episodes were fever of unknown origin, 16% were clinically documented infection and 27% were microbiologically documented infection. Of the microbiologically documented infections, 44% of them were caused by gram positive bacteria and 43% were by gram negative bacteria. Eight of 96 patients developed invasive fungal infection during initial treatment and 4 out of 15 patients who relapsed. Of 9 patients who expired on follow-up, the cause of death was fungal infections in 4 and FN in 1. Conclusion: The high rate of infectious deaths in leukemia compared to that reported in high-income countries, suggests that improvements in infection care and prevention are necessary to improve survival in patients with leukemia. We showed that over the years FN episodes increased at our center in contrast to better quality of patient care. This increase in infections may be atrributed to more intensive treatment regimens. We also showed that high risk and relapsed patients who are treated more aggresively have increased infection rate. The most common cause of death was fungal infections. Disclosures No relevant conflicts of interest to declare.

Aim: To investigate the clinical features, diagnostic and treatment results and long-survival of children with HL at a single center in Turkey. Methods: Retrospective data were extracted from medical records of children ( Results: Twohundred-fortyeight patients (M/F: 1.8) were identified for analysis. Mean age at diagnosis was 10 years. Four-percent were stage 1, 43% were stage 2, 34% were stage 3 and 17% were stage 4 at diagnosis. B symptoms were present in 105 children (42%). Mixed cellular was the most common subtype (50%) followed by nodular sclerosis. MOPP regimen was primarily used until 1989 along with extended field radiotherapy (30-40 Gy) after 1989 ABVD with involved field RT (20-25 Gy) was the treatment of choice. Onehundred-fortyseven patients (42%) had radiotherapy. Median follow-up time was 14.3 years (range: 5 months-38 years). PET was used for assessment in 45 cases. Ten year overall survival rate was 99%. Ten year event free survival rate (EFS) was 90%. Univariate analysis showed that stage, histological subtype, presence of B symptoms and hematological parameters were not related to OS while treatment regimen correlated. Twenty-six patients relapsed and 17 patients expired on follow-up. Ten patients who relapsed were treated with autologous stem cell transplantation, 3 of them relapsed again and were treated with brentixumab. Secondary tumours developed in 4 patients (1.6%). One had AML, 1 osteosarcoma, 1 thyroid papillary cancer and 1 had craniopharyngioma. Conclusion: Ethnic and regional differences in the epidemiology and pathological aspects of HL may be associated with differences in clinical features and prognosis however we found that clinical features and treatment outcomes are similiar in Turkish patients. Our results showed that hematological parameters and B symptoms were not significant prognostic parameters. Disclosures No relevant conflicts of interest to declare.

Oral cavity is a region where various mucosal lesions and infections occur. Saliva has an important role in the protection of oral cavity. Cancer and its treatment lead to several changes in oral cavity including the changes observed in saliva composition. These can affect the healing process of the oral wounds and the incidence of tooth decay. Thromboplastin (tissue factor, FIII) is a membrane bound glycoprotein and it is

BACKGROUND The aim was to evaluate the characteristics of Wilms' tumor and the results of combined modality treatment obtained in our center in Turkey. METHODS From January 1978 to December 1996, 106 patients with Wilms' tumor were diagnosed. Of these 106 patients, 61 were male and 45 were female (M/F = 1,35); the median age at diagnosis was 39 months. The distribution of the 106 patients according to clinical stage was stage I 10%, stage II 42%, stage III 35%, stage IV 9% and stage V 4%. Histologically, 102 of the cases could be evaluated: favorable histology was diagnosed in 88.2% and unfavorable histology in 11.8% of the patients. Ninety-eight patients were treated according to NWTS and eight patients according to SIOP protocols. RESULTS The EFS and overall survival rates at 2 years were 74.2 and 79.5% respectively, and at 5 years 72.4 and 76.6% respectively. CONCLUSION As a developing country we evaluated our survival rates and report an improvement in treatment in recent years.

Background: We aimed to assess the potency and efficacy of tropisetron, a 5-HT3 receptor antagonist, in the prevention of nausea and emesis observed in the pediatric patient population taking various chemotherapy protocols. Methods: Tropisetron (Navoban'[) was given to 100 children (62 boys and 38 girls aged 6 months to 15 years) with various malignancies. Patients received tropisetron during one or more courses of emetogenic chemotherapy for a total of 350 courses administered intravenously or intravenously and intrathecally. Tropisetron (0.2 mg/kg/day, maximum: 5 mg/day) was adminis­ tered as a single intravenous dose slowly, before the start of chemotherapy on day 1 and intravenously or by mouth on subsequent days (median treatment duration: 5 days). Results: The patients receiving cytotoxic chemotherapy had a 70% complete response rate and a 24% partial response rate during the first 24 h period of the first course. We observed headache (five courses), diarrhea (three courses) and loss of appetite (one course) as side-effects (2.5%). Conclusion: Tropisetron is safe, effective, easy to use, has no serious side-effects and can be recommended for pediatric patients. The efficacy of tropisetron may be enhanced by the addition of corticosteroids in patients receiving highly emetogenic cancer chemotherapy.

Aim The aim of this study is to determine whether iron deficiency is associated with zinc deficiency. Methods Ninety-eight patients with ID and/or IDA and 100 healthy controls who were examined at Cerrahpasa Medical Faculty, Pediatric and Child Health Department outpatient clinic were included prospectively in this study. Blood samples were collected from cases and controls for complete blood count, iron, TIBC, ferritin and zinc levels. The association between zinc levels and hematological and biochemical parameters were examined. Results There was significant difference (p 0.05) between zinc levels in relation to the absence or presence of anemia. There was no correlation between zinc levels and Hb, Htc, MCV, ferritin or TIBC (p>0.05). However there was a positive correlation beween zinc levels and iron and TSI. In the control group, there was no statistical relation between zinc levels and Hb, Htc, MCV, iron, TIBC, TSI and ferritin levels (p>0.05). Conclusion In our study, we detected significant lower zinc levels in children with iron deficiency before the onset of anemia. Therefore, we propose that the use of preparations containing both iron and zinc might be more effective in improvement of clinical signs of iron and zinc deficiency.

Intensive multiagent chemotherapy and central nervous system prophylaxis has resulted in remarkably improved event-free survival (EFS) approaching 80% in children with acute lymphoblastic leukemia (ALL) [1, 2, 3]. In contrast, prognosis of childhood ALL has remained poor with conventional treatment in our department with an overall long-term survival of 13% and an event-free interval (EFI) of 12%. Similar results have been reported from other centers in Turkey [4]. Besides the quality of chemotherapy; supportive care measures, access to drugs, patient incompliance and socioeconomic conditions are important factors in developing countries [4, 5], leading to reluctance in the institution of intensive regimens.

Fifty-three patients in the pediatric age group diagnosed as having small non-cleaved follicular center cell (FCC) lymphoma (40 Burkitt's and 13 non-Burkitt's type) have been re-evaluated. Although the histopathologic distinction between the two subgroups is fairly easy to determine there is a considerable degree of clinical overlap in regard to age, sex, anatomic presentation of the disease, the incidence of bone marrow and central nervous system involvement, and the survival. The most important determinant of survival in both subgroups is the stage of the disease. Cancer 59:925-932, 1987.