Search

The number of studies evaluating teicoplanin lock therapy in coagulase-negative staphylococcus-associated catheter infection in pediatric malignancies is limited. The aim of this study was to evaluate the efficacy of teicoplanin lock therapy in pediatric cancer cases. Twenty-two patients with coagulase-negative staphylococcus-associated totally implantable venous access device infection, who had undergone teicoplanin closure treatment, were included in the study. Demographic data, number of lock treatment days, and treatment success data were obtained from the medical files of the patients. Fourteen of the patients (63.6%) had acute lymphocytic leukemia, 3 (13.6%) had acute myelocytic leukemia, and 5 (22.7%) had solid cancer. The median neutrophil count was 240×10 3 /μL (interquartile range: 0 to 1195×10 3 /μL). Between patients with and without catheter removal, no statistically significant difference was found in terms of baseline C-reactive protein, absolute neutrophil count, and the day of starting systemic teicoplanin treatment ( P0.05). The overall port survival rate of teicoplanin lock therapy was 72.7%. Within an average of 4 days, negative cultures of 16 (72.7%) patients whose catheters had not been removed were obtained. In conclusion, we suggest that teicoplanin lock therapy is an effective and safe treatment for catheter-related infections, caused by methicillin-resistant coagulase-negative staphylococcus.

Hepatic hemangiomas (HH) are the most common vascular tumors of the liver. It is important to distinguish hemangiomas from malignant liver tumors.The patients 0 to 1 years old, were diagnosed with HH and followed up in the oncology outpatient clinic between 2009 and 2020 were included in the study.A total of 127 patients with the diagnosis of HH were included in the study. Of the patients, 99 (78%) had focal, 20 (15.7%) had multifocal, and 8 (6.3%) had diffuse HHs. Surgery was performed and the diagnosis was confirmed histopathologically in 6 patients (4.7%). During the follow-up, 16 (12.5%) patients received medical treatment. Thirteen (10.2%) were treated with propranolol, 2 (1.5%) with corticosteroids, and 1 (0.8%) with propranolol and corticosteroids. Complete response was obtained in 9 (9/16) patients and partial response was obtained in 6 (6/16) patients with medical treatment.Although HH is a benign tumor, it is important to make its differential diagnosis with malignant tumors of the liver. Over the years, the need for histopathologic examination for diagnosis has decreased. The success rate of propranolol is high, and the need for other treatment options with a high side-effect profile has decreased significantly since 2008.

Neurofibromatosis type 1 (NF1) is caused by mutations in the NF1 gene. This retrospective study aims to evaluate the clinical manifestations and brain magnetic resonance images (MRI) analysis in 60 genetically confirmed NF1 patients. The results of next-generation sequencing (NGS), Sanger sequencing, and MLPA of NF1 gene were evaluated. A total of 54 different variants were identified. Fourteen out of them were novel variants (25.9%). Patients who complied with NIH criteria had most frequently frameshift variants (11/32 patients), and those with only CALMs had missense variants (9/28 patients). Neurofibromatosis type 1 bright objects (NBOs) on T2-weighted MRI were detected in 42 patients (42/56; 75%). These brain lesions were detected mostly in basal ganglia and in cerebellar vermis. NBOs were detected more in the patients who complied with NIH criteria (80.6%) compared to those who were only CALMs (68%). While frameshift variants (33.3%) were the most common type variants in the patients who had NBOs, the most common variants were splicing (35.7%) and missense (35.7%) variants in the patients whose MRIs were normal. Frameshift variants (11/28 patients; 39.3%) were the most common in the patients with more than one brain locus involvement. Therefore, we consider that frameshift variants may be associated with increased incidence of NBOs and involvement of more than one brain locus. In addition, NBOs may occur less frequently in the patients with splicing variants. To our knowledge, this is the first study evaluated the relationship between NF1 gene variants and NBOs. Future studies may help us understand the etiology of NBOs.

Vincristine (VCR), which is a key component of chemotherapy, is important for survival. VCR is associated with a well-known side effect, including neurotoxicity. The aim of this study was to evaluate the features of vincristine-induced peripheral neuropathy (VIPN) and the effectiveness of pyridoxine plus pyridostigmine therapy in children with acute lymphoblastic leukemia. The WHO and NCI CTCAE neurotoxicity scorings were used to evaluate VIPN at diagnosis, in the first month, and after the third month of the treatment. The clinical features of 23 patients having acute lymphoblastic leukemia with VIPN during the period of July 2013–February 2016 were prospectively evaluated. The mean age was 72.8 ± 51.6 months, and 26.1%, 56.5%, and 17.4% were in standard, moderate, and high-risk groups, respectively. Neuropathy frequently occurred at induction (82.6%) and reinduction (17.4%) of the protocol. Drop foot (82.6%), leg pain (82.6%), and difficulty in walking (82.6%) were observed. The mean total cumulative dose of neuropathy occurrence was 5.6 ± 2.03 mg/m2. Our study showed that both the WHO and NCI CTCAE scorings were significantly improved via pyridoxine plus pyridostigmine therapy. The WHO and NCI CTCAE scorings may be used for evaluating neuropathy at diagnosis and follow-up of neurotoxicity with treatment. Pyridoxine plus pyridostigmine therapy may be an effective option in the treatment of VIPN.

L-Asparaginase (L-Asp) is a critical component of chemotherapy for acute lymphoblastic leukemia (ALL). However, toxic effects associated with L-Asp, such as hepatic dysfunction, pancreatitis, hypercholesterolemia, and hyperglycemia, have occurred. In addition, acute pancreatitis is a significant life-threatening adverse event associated with ALL. We describe 2 patients with ALL who had L-Asp-associated pancreatitis (AAP), with one patient presenting with hyperglycemia and the other presenting with hypoglycemia during induction treatment. When octreotide was administered to both of these patients, the clinical findings and laboratory data were improved. AAP was not repeated after treatment with pegylated asparaginase. Although AAP has a high risk of mortality and morbidity in childhood, APP treatment with appropriate agents, such as octreotide, can be successful. (C) 2020 Elsevier Inc.

WOS: 000482629200002, PubMed ID: 30322830, Objective: The aim of this study is to determine the frequency of dental anomalies (DAs) (microdontia, hypodontia, hyperdontia, enamel defect, root malformation) in pediatric cancer patients at the ages

Background Acute viral respiratory infections are common causes of febrile episodes in children. There are still limited data about distribution of acute viral respiratory infections in children with cancer. Objective The first aim of this study was to evaluate the viral etiology and seasonality of acute viral respiratory infection in pediatric patients with cancer in a 3-year study. Our second aim was to evaluate the impact of viral infections on delaying the patients' chemotherapy or radiotherapy. Materials and methods This cross-sectional study was conducted from January 2014 to July 2017. Nasopharyngeal aspirates were analyzed in patients younger than 21 years with acute respiratory infections. Patients were treated in the Pediatric Hematology and Oncology Department of Dr. Behcet Uz Children's Hospital with real-time multiplex polymerase chain reaction. Data were analyzed to determine the frequency and seasonality of infections. The χ or the Fisher exact tests were used. Results A total of 219 samples of nasopharyngeal aspirates and blood were analyzed. The mean patient age was 76.8±59.3 months, with 46.3% female and 53.7% male children in a total of 108 patients. Of this total, 55% (60/108 cases) had multiple acute respiratory infections. Acute lymphoblastic leukemia (48.1%) was the most prevalent disease. The 3 most prevalent viruses were human rhinovirus (HRV) (33.1%), parainfluenza (PI) (18.7%), and coronavirus (CoV) (14.8%). In terms of the seasonal distribution of viruses, PI was most common in winter 2014, HRV in spring 2014, HRV in fall 2014, PI in winter 2015 and summer 2015, CoV in spring 2015, HRV in fall 2015, both influenza and HRV in winter 2016, both human metapneumovirus and bocavirus in spring 2016, HRV in summer 2016, both HRV and PI in fall 2016, both respiratory syncytial virus and influenza in winter 2017, HRV in spring 2017, and both HRV and adenovirus in summer 2017. The mean duration of neutropenia for patients with viral respiratory infection was 17.1±13.8 (range: 2 to 90) days. The mean duration of symptoms of viral respiratory infection was 6.8±4.2 (range: 2 to 31) days. A delay in chemotherapy treatment owing to viral respiratory infection was detected in 73 (33.3%) patients. The mean duration of delay in chemotherapy treatment was 9.6±5.4 (range: 3 to 31) days. Conclusions In conclusion, we report our 3-year experience about the frequency and seasonality of respiratory viruses in children with cancer.

BACKGROUND Candidemia and Candida-associated catheter-related bloodstream infections (CRBSIs) are the significant cause of mortality and morbidity in patients with malignancy. METHODS A retrospective analysis including all pediatric hematologic/oncologic malignancies patients with CRBSIs treated in Dr. Behcet Uz Children Diseases and Surgery Training and Research Hospital between the period of 2009 and 2020. RESULTS During the study period, 53 children with CRBSIs associated with Candida species were included. The most common malignancy was acute lymphoblastic leukemia (45.3%) and acute myeloid leukemia (15.1%). A total of 56 Candida isolates were present including non-albicans Candida species (80.4%) and Candida albicans (19.6%). The most common isolated Candida species was Candida parapsilosis (42.9%) and followed by C. albicans (19.6%). The ratio of azole prophylaxis was significantly higher in patients with the non-albicans Candida group (P=0.031). Candida-related endocarditis (vegetation) was present in 2 (3.8%) patients, and the overall rate of hepatosplenic candidiasis was 3.8%. Seven days Candida attributable mortality was 7.5% (4 patients) and 30 days Candida attributable mortality was 11.3% (6 patients). The Candida species responsible for the Candida-related deaths were as following: Candida tropicalis (n=3), C. parapsilosis (n=2), and C. lusitanae (n=1). CONCLUSION In pediatric cancer patients with Candida-associated CRBSIs, evaluation of the patient for organ involvement including liver and spleen ultrasonography and cardiac involvement with echocardiography are essential regardless of the patients' clinical picture.

Objective: Anthracyclines which are the main drug of chemotherapy protocols had cardiotoxicity as the most frequent and well-known side effect. We aimed to evaluate prospectively the heart rate variability with 24-hour Holter electrocardiography (ECG) in children with cancer who treated with anthracycline drugs. Material and Methods: The 24-hour Holter ECG monitoring was performed at the baseline, at time of 120 mg/m2 and ≥240 mg/m2 of cumulative anthracycline dose in patients with cancer who treated with anthracycline. The time-domain and frequency-domain measurements of heart rate variability (HRV) were obtained. The patients were classified into three groups as Group1:at baseline (n=54), Group 2:≥120mg/m2 (n=54), Group 3:≥240mg/m2 (n=54).Results: The median age was 48 months (range 9-192 months). All types of cancer were 38 patients (70.4%) of acute leukemia, two patients (3.8%) of T lymphoblastic lymphoma, and 14patients (25.8%) of other childhood cancer who treated with anthracycline. However, all heart rate variability parameters were decreased after each increased cumulative anthracycline dose, especially time-domain parameters such as nSDNN index, rMSSD, pNN50, frequency parameters such as LF, HF, and Total power were significantly altered among Group1 and Group 3. LF/HF ratio was also statistically significantly increased in Group 3. According the heart rate parameters, the mean average heart rate, mean minimum heart rate and mean RR were statistically significantly prolonged from Group1 to Group 3.Conclusion: Heart rate variability parameters are a noninvasive technique to demonstrate cardiac autonomic neural dysfunction and early myocardial injury. The 24-hour Holter ECG may be used for detecting early cardiac dysautonomia effect during anthracycline treatment with each elevated 120mg/m2 anthracycline of cumulative dose., Amaç: Kemoterapi protokollerinin temel ilacı olan antrasiklinler en sık kardiyotoksisite yan etkisine sahiptir. Antrasiklin ile tedavi edilen kanserli çocuklarda kalp hızı değişkenliğini 24 saatlik Holter elektrokardiyografi (EKG) ile prospektif olarak değerlendirmeyi amaçladık.Gereç ve Yöntemler: Antrasiklin ile tedavi edilen kanserli hastalarda hiç tedavi almadan, 120mg/m2 ve ≥240 mg/m2 kümülatif antrasiklin dozunda 24 saatlik Holter EKG monitörizasyonu yapıldı. Kalp atış hızı değişkenliğinin (HRV) zaman alanı ve frekans alanı ölçümleri elde edildi. Hastalar üç gruba ayrıldı: hiç tedavi almadan Grup 1 (n = 54), Grup 2 :≥120 mg/m2 (n = 54), Grup 3: ≥240 mg/m2 (n = 54) seklinde ayrıldı.Bulgular: Ortanca yaş 48 aydı (aralık 9-192 ay). 38 (% 70.4) hasta akut lösemi, iki hasta T lenfoblastik lenfoma (% 3.8) ve 14 hasta (% 25.8) diğer çocukluk çağı kanserleridir. Bununla birlikte, tüm kalp hızı değişkenleri, nSDNN indeksi, rMSSD, pNN50 gibi zaman parametreleri ve LF, HF ve Toplam güç gibi frekans parametreleri gibi her artan kümülatif antrasiklin dozu ile azalma görüldüğü belirlendi. LF/HF oranı da Grup 3’te istatistiksel olarak anlamlı şekilde artış gösterdiği saptandı. Kalp hızı parametrelerine göre, ortalama kalp hızı, ortalama minimum kalp hızı ve ortalama RR, kümülatif doz artıkça istatistiksel olarak anlamlı şekilde uzadığı belirlendi.Sonuç: Kalp hızı değişkenleri, kardiyak otonomik nöral disfonksiyonu ve erken miyokardiyal hasarın gösterilmesi için invazif olmayan bir tekniktir. 24 saatlik Holter EKG’si, her artan 120 mg/m2 antrasiklin kümilatif dozu ile antrasiklin tedavisi sırasında erken kardiyak dysautonomia etkisini saptamak için kullanılabilir.

EgeUn###, Hepatoblastoma (HB) is the most common liver malignancy in children. The prognosis changes according to the histologic subtypes of HB. In the present study, we aimed to characterize the expression level of selected microRNAs (miRNAs) in HB as well as in histologic subtypes, and to consider the association with the prognosis. A total of 22 HB tumor samples, subtyped as fetal (n= 16) and embryonal (n= 6), and 10 nontumorous surrounding liver samples were evaluated in this study. Expressions of miR-17, miR-146a, miR-302d, and miR-19b were analyzed in 22 HB tumor samples and 10 nontumorous surrounding liver samples by quantitative real-time polymerase chain reaction. Lower miRNA-17 expression levels were obtained in tumor samples in comparison with nontumorous surrounding liver samples (P= 0.028). Lower miRNA-17 expression was significant for predicting prognosis in HB patients (area under receiver-operator characteristic curve= 0.875, P= 0.044). A higher-level of miR-19b was found in embryonal samples (P= 0.008). Overall and event-free survival was not found to correlate with miRNA expression levels (P> 0.05). This research finds miRNA-17 and miRNA-19b expression levels can provide important data on diagnosis and prognosis in HB showing different clinical behaviors.

Immune thrombocytopenic purpura (ITP) results from accelerated platelet destruction mediated by autoantibodies to platelet glycoproteins. Some patients with chronic ITP are refractory to all therapies [steroids, intravenous immunoglobulin (IVIG), anti-D and immunosuppresive drugs] and have chronic low platelet counts and episodic bleeding. We retrospectively evaluated the efficacy and safety of rituximab treatment and splenectomy in paediatric patients diagnosed with chronic and refractory ITP who were unresponsive to steroids, IVIG, cyclosporine and mycophenolate mofetil. Records of patients with chronic and refractory ITP in 459 patients with primary ITP who were followed up in our hospital from January 2005 to December 2014 were reviewed. Fifteen of patients received rituximab and/or applied splenectomy. Fifteen chronic ITP patients (10 boys, five girls) with a mean age of 10 years were enrolled in the study. Two of these patients were suffering from Evans syndrome. The median time since diagnosis of ITP was 10 years. The median follow-up duration after starting Rituximab and splenectomy were 13 and 9.5 months, respectively. None of the seven patients who were treated with rituximab achieved a response. A splenectomy was performed in six of the seven patients who had been treated with rituximab. Complete and partial responses were achieved in 67 and 33% of the patients, respectively. We evaluated the clinical characteristics and responses of chronic ITP patients who did not receive rituximab therapy and underwent a splenectomy. The success rate was 100% in the eight patients with chronic and refractory ITP. Rituximab therapy might not be beneficial for some children with severe chronic ITP who are refractory to standard agents. A splenectomy might be useful and preferable to rituximab. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

Thymomas are the most common masses located in the anterior mediastinum, and they are often associated with autoimmune disorders including myasthenia gravis, polymyositis, and aplastic anemia (AA). Autoreactive T-cell clones generated by the thymoma may lead to autoimmune disorders. We report the case of a 14-year-old boy who was examined for AA, and the underlying cause was determined to be an immune-mediated complication of thymoma. He had no matched sibling donors. He underwent thymectomy, and 3 months later he was treated with immunosuppressive therapy (IST), consisting of antithymocyte globulin and cyclosporine A. The duration of the IST was determined to be a period of 12 months. He has recently been in complete response condition for 6 months since IST stopped. IST is a successful treatment choice for thymomas associated with AA in childhood.

Background The aim of this study was to evaluate the diagnostic utility of serum galactomannan (GM) test by investigating the impact of positivity according to different threshold levels and consecutiveness in terms of invasive aspergillosis (IA) in pediatric hematology-oncology patients. Methods Positive GM test results between January 2015 and August 2017 were reviewed, retrospectively. The children with hematological malignancies and GM positivity were included in the study and grouped according to the presence of IA. Impact of single and consecutive (3-day interval) GM positivity on IA diagnosis were evaluated according to different galactomannan index (GMI) threshold values of >0.5, >0.7, >1.0, and >1.5. Results There were 104 positive GM results from 70 patients. Forty-one patients (58.6%) had no clinical evidence of IA and categorized as the non-IA group. Invasive aspergillosis diagnosis was identified in 29 (41.4%) of the patients; 2 of them were proven and 27 were probable. Demographic characteristics and clinical findings of the patients were reviewed in Tables 1 and 2. According to different cutoff GMI values, the number of positive results was 104 for >0.5, 76 for >0.7, 57 for >1.0 and 32 for >1.5. The PPVs were low at a single GMI of >0.5 (39.4%) and reached to 50.0% with single GMI of >1.0. There was not a statistically significant difference between IA and non-IA groups in terms of different thresholds of a single GM positivity (P > 0.05) (Table 3). The number of two consecutive positive results was 34 for GMI of >0.5, 20 for GMI of >0.7, 13 for GMI of >1.0 and 4 for GMI of >1.5. In the IA group, GM positivity of consecutive results was significantly higher than non-IA group (P < 0.05). The PPVs of two consecutive positive results for GMI >0.5, GMI >0.7, GMI >1.0, and GMI >1.5 were 58.8%, 65.0%, 84.6%, and 100.0%, respectively. The effect of the GMI increase between two consecutive GM results on IA diagnosis (GM2-GM1 >0.5) was also evaluated and the PPV was found 53.8% without a statistical significance between two groups (Table 4). Conclusion When evaluated with consecutive GM positivity, the GM assay would have higher PPVs independently from the GMI cutoff value chosen. Since it may be more effective on IA diagnosis, consecutive sampling should be performed in pediatric patients at high risk. Disclosures All authors: No reported disclosures.

Background: The port catheters are used to give long-term total parenteral nutrition, intravenous fluids, blood and blood products, or to be preferred for oncology and hematological diseases. In this study, we sought to review our results concerning the subcutaneous port catheter appliance in childhood malignancies. Methods: Two hundred ten subcutaneous port catheters applied to 192 patients between November 2010 and October 2015 were examined retrospectively in a cross- sectional study. Information such as demographic data, primary diagnoses, port types and implantation durations were recorded. Localization of the intervention, surgical technique, early and late complications and causes of port removal were evaluated. Results: Mean age of patients was 6.4 ± 4.9 years (1 month - 17 years), 77 (40.1%) were female and 115 (59.9%) male. One hundred twenty nine ports were inserted from the right internal jugular vein, 59 to right subclavian vein, 14 to left subclavian vein and 8 to left internal jugular vein. Total duration of implantation was 55,492 days and median duration was 289 days. Early complications were: 1 (0.5%) hemothorax, 1 (0.5%) pneumothorax, 1 (0.5%) carotid artery injury, 1 (0.5%) arrhythmia and in 2 (1%) cases port mal-positioning. Late complications were: 9 (4.3%) infection and 8 (3.8%) mechanical problems, 7 (3.3%) thrombosis, and in 1 (0.5%) port fracture was detected. Port catheter was removed in 18 (8.6%) cases. No significant difference was found when jugular and subclavian vein implantation was compared in terms of all complications, especially infection and thrombosis. Conclusion: Use of port catheter is a safe option in children who are receiving a long-term chemotherapy. Education of the medical team will diminish the complication rate in port catheter insertions.

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.Primer varisella zoster virüs (VZV) enfeksiyonu benign, kendi kendini sınırlayan bir hastalıktır. Bu çalışmada pediatrik malignitesi olan hastalarda VZV enfeksiyonu ve tedavisine odaklı tecrübelerimizi gözden geçirmeyi amaçladık. Çalışma süresi boyunca; VZV enfeksiyonu tanısı alan pediatrik maligniteli toplam 41 hasta hastaneye yatırıldı. Tüm hastalar ortalama 7 gün (5 ila 21 gün arasında değişen) intravenöz asiklovir ile tedavi edildi. VZV enfeksiyonlarına bağlı olarak hesaplanan atfedilebilir kemoterapi gecikmesi ortalama 8 gündü (2 ile 60 gün arasında değişen). VZV enfeksiyonuna bağlı komplikasyonlar 41 hastadan 3’ünde (%7) akut solunum distres sendromu olarak görüldü ve bu hastalardan hemofagositik lenfohistiyositozu olan bir tanesi asiklovir, geniş spektrumlu antibiyotik ve destekleyici tedaviye rağmen solunum yetmezliği nedeniyle kaybedildi. VZV enfeksiyonları, pediatrik malignite hastalarında hala önemli bulaşıcı hastalıklardan biridir, çünkü sadece ciddi mortaliteye sebep olmakla kalmayıp kemoterapi başlangıcını da geciktirmektedir.

WOS: 000439064100006 PubMed ID: 30150887 Non-typhoidal Salmonella (NTS) is an important pathogen that causes gastroenteritis, bacteraemia, and focal infections. Herein, we present our experience with bloodstream infections caused by Salmonella 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.

Introduction:Invasive fungal infections, including central nervous system fungal infections (CNSFI), cause significant morbidity and mortality in children with leukemia,necessitating early diagnosis and treatment. Material and Method: In this retrospective study, the demographic features, diagnostic approach, treatment and outcome of CNSFI in 32 children with leukemia treated in 12 centers in Turkey were evaluated. Results:Of the 32 patients,12 were female,20 were male.The diagnosis was ALL in 23, recurrent ALL in 5, CML in 1, AML in 1; two others had underwent allogeneic bone marrow transplantation. Most (22/32-68,7%) CNSFI episodes occurred during the remission induction phase of the treatment. All recieved broad-spectrum antibiotics for prolonged neutropenic fever. Twenty two had seizures. Six patient had headache, two patient had double vision and headache, one patient had loss of vision and one patient had fascial paralysis. Radiologic imaging was performed for neurologic symptoms and/or prolonged fever in patients with febrile neutropenia after a median of seven days (1-22). Twenty three had multiple and 9 single brain lesions in magnetic resonance imaging. In 15/23 patients, invasive pulmonary fungal infection; in two patients, hepatosplenic fungal infection were detected concurrently. On blood cultures, one patient had geotrichum capitatum fungemia and one had candida albicans fungemia.Galactomannan was positive in 15 (46%) patients. Fourteen patients underwent surgery, in ten microbiological evidence of fungi was confirmed; four aspergillus flavus, one aspergillus niger, one candida, one mucormycosis and one trichoderma longibrachiatum. Hemophagocytosis was observed in three patients. Nine received granulocyte transfusions. Fifteen were transferred to the ICU. Extracorporeal Membrane Oxygenation (ECMO) was used in one patient. Eleven patients (34%) died due to CNSFI at a median of 30 (15-42) days from the beginning of the episode. Out of 21 survivors, seven have permanent neurological sequela. Eighteen patients were recieving antifungal prophylaxis (Fluconazole (16), voriconazole(1) and itraconazole(1), before the CNSFI diagnosis. Thirteen patients received a single antifungal agent, 19 were treated with a combination of antifungals. Median duration of antifungal treatment was 186 (20-730)days. Liposomal amphoterisin-B and voriconazole were the two mostly used agents. Six of the 12 centers participating in the study had a construction going on near their ward at the time of diagnosis. Conclusion: CNSFI is a major cause of mortality and morbidity in children with leukemia and prolonged neutropenic fever. The evaluation of children with leukemia and prolonged neutropenic fever for invasive fungal infections and the addition of empirical antifungal treatment to antimicrobial treatment is important. Cranial imaging in children with leukemia and prolonged neutropenic fever, even in the absence of local sign and symptoms may lead to earlier diagnosis of CNSFI. Disclosures No relevant conflicts of interest to declare.

17th Congress of the European-Pediatric-Surgeons-Association (EUPSA) -- 2016 -- Milanno, ITALY, WOS: 000463135800003, PubMed ID: 30859760, Radiological nodule behavior (RNB) means the course of nodule appearance on consecutive CT scans. In this study, we aimed to discuss the effects of RNB on prognosis of patients with pulmonary metastasis. Retrospective analysis of patients who underwent pulmonary metastasectomy (PM) between 2005-2015 was performed. RNBs were grouped as stable, enlarging or new rising nodules. The effects of RNBs were analyzed on recurrence, 1- and 3-year event free survival (EFS) and mortality. Twenty-seven patients (18 male) underwent PM with a median age of 15 years (3-18 years). The diagnoses were listed as osteosarcoma (13), Wilms' tumor (7), Ewing's sarcoma (3), synovial sarcoma (2), rabdomyosarcoma (1) and mixed germ cell tumor of testis (1). RNBs were new rising in 15 patients (55%), enlarging in seven patients (26%) and stable in five patients (19%). Vital tumor metastasis was detected in all of the patients with enlarging nodules (100%); in 10 of the 15 patients with new rising nodules (66%) and none of the patients with stable nodules. None of the patients with stable nodules developed recurrence or died after PM (p, European Pediat Surg Assoc

Ince D, Demirag B, Ataseven E, Oymak Y, Tuhan H, Karakus OZ, Hazan F, Abaci A, Ozer E, Mutafoglu K, Olgun N. Child with RET proto-oncogene codon 634 mutation. Turk J Pediatr 2017; 59: 590-593. Herein we reported a 7-year-old child with RET proto-oncogene c634 mutation. Her mother had been diagnosed with medullary thyroid carcinoma (MTC), and treated six years ago. Heterozygous mutation of the RET proto-oncogene at c634 had been detected in her mother. Genetic analysis showed the presence of the same mutation in our patient. Thyroid functions were normal. Serum calcitonin level was found mildly elevated. Parathormone (PTH) and carcinoembrionic antigen (CEA) levels were normal. Prophylactic thyroidectomy and sampling of cervical lymph nodes were performed. Histopathologic examination revealed hyperplasia in thyroid C cells, and reactive lymphadenopathy. The risk of MTC has been reported 100% through the life of patients with RET proto-oncogene mutation. It has been reported that particularly patients with c634 mutation have more risk of occurence of metastatic and progressive/recurrent MTC. Prophylactic `thyroidectomy, cervical lymph node dissection` before 5-years-of-age should be considered for these patients.

WOS: 000410406300006, PubMed ID: 28387647, Objective: The management of childhood thyroid nodules is still a big challenge for clinicians. In this study, we aimed to present our surgical and endocrinological experience in more than one hundred pediatric cases. Methods: A retrospective analysis of patients admitted with a thyroid nodule between 2006 and 2014 was performed. Detailed ultrasonography and fine-needle aspiration biopsy (FNAB) were the cornerstones of the diagnostic approach. Results: One hundred-three children (72 female, 31 male) with a mean age of 13.1 +/- 3.6 years (3-18 years) were admitted to our center. Management strategy was surgery in 58 patients and follow-up in 45 patients. Mean nodule size was 17 +/- 12.7 mm (2-45 mm). The diagnoses were listed as benign solitary nodule (48 patients), thyroid carcinoma (26 patients), multinodular goiter (23 patients), Hashimoto thyroiditis (4 patients), and Graves' disease (2 patients). Surgical procedures were nodulectomy/lobectomy (32 patients), total thyroidectomy (TT) (13 patients), or TT+ neck dissection (13 patients). The rate of malignancy was 25% in the total group and 44% in the surgery group. The malignancy rate was higher in patients younger than 12 years compared to older children (41% vs. 17%, p = 0.040). Metastasis was seen in 38% of the malignant nodules. Postoperative complications were transient hypocalcemia (8%), permanent hypocalcemia (1.7%), and unilateral vocal cord paralysis (1.7%). Recurrence or mortality was not encountered in the 5.4 +/- 1.2-year follow-up period. Conclusion: Thyroid nodule in a child requires an aggressive diagnostic approach due to increased risk of malignancy and metastasis. Intraoperative frozen section examination must be done as a useful adjunct to determine the surgical strategy. Incidence of complications is small in thyroid surgery when performed by experienced surgeons.

Primary cardiac tumor is uncommon in childhood, with an incidence of 0.06-0.32%, and intrapericardial teratoma represents an exceptional rarity among these entities. Germ cell tumors (GCT) are rare, representing only 1-3% of childhood tumors. Twenty per cent of GCT are malignant and are associated with age and location. Extragonadal involvement accounts for nearly half of the cases. Anterior mediastinum is a common location of malignant germ cell tumors, yet pericardial and aortic adventitia involvement have been rarely reported. Here we report the case of a preterm twin baby boy with intrapericardial mixed germ cell tumor who presented with hydrops fetalis and pericardial effusion.

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.

Objective The aim of this study was to analyse the genetic and non genetic risk factors for cisplatin ototoxicity. Methods This study was conducted on 72 children who received cisplatin based chemotherapy. Brock and Muenster classifications were used to evaluate ototoxicity seen in these children. 6 single nucleotide polymorphisms (SNP); ERCC1 rs 11615, GSTP1 rs1138272, GSTP1 rs1695, LRP2 rs 2075252, TPMT rs 12201199, COMT rs 9332377, were evaluated as genetic factors by real time PCR. Non genetic factors such as cranial irradiation, cumulative doses of cisplatin, age, gender, administration of other ototoxic drugs were analysed as well. By using Chi-square test, risk factors were matched with the ototoxicity classifications. Significant risk factors were reevaluated using logistic regression modelling. Results According to univariate analyses, male gender, co-treatment with aminoglycosides and mutant genotype of GSTP1 rs1695 were significantly related with cisplatin ototoxicity. Logistic regression modelling analyses also showed that male gender, co-treatment with aminoglycosides were found to be significantly related with cisplatin ototoxicity. Mutant genotype of GSTP1 rs1695 was not found to be significant, but close to the level of statistical significance. Conclusion Male gender, co-treatment with aminoglycosides are significant risk factors for cisplatin ototoxicity in pediatric patients. Mutant genotype of GSTP1 rs1695 seems to be a genetic risk factor in univariate analyses, although not confirmed by multivariate analyses. Therefore, GSTP1 rs1695 SNP needs to be studied in larger series.

WOS: 000401520200012, PubMed ID: 28169428, The genetic basis of haemophagocytic lymphohistiocytosis (HLH) has not been elucidated in 10% of affected patients. In this study, we report four HLH episodes in three patients with HAX1 gene mutations. We screened the mutations associated with congenital neutropenia (CN) because the neutropenia persisted following HLH treatment. There were homozygous HAX1 mutations detected in all patients. This is the first case series of patients with CN caused by HAX1 mutation who presented with HLH. We hypothesize that severe neutropenia persists after an HLH episode in children without HLH mutations (especially infants) because these patients have CN caused by HAX1 mutations.

OBJECTIVE Neutrophil gelatinase-associated lipocalin (NGAL) and Kidney injury molecule-1 (KIM-1) play important roles in both immunity and cell proliferation. It was reported previously that they are overexpressed in various human cancers. The present study was undertaken to examine the expressions of NGAL and KIM-1 in Wilms Tumors. MATERIAL AND METHOD Tissue samples of 50 Wilms Tumors were evaluated and underwent immunhistochemical staining for NGAL and KIM-1 protein expressions. The correlations between them, and some clinical prognostic factors such as tumor weight, stage and histological features were also evaluated. RESULTS Twenty-three (46%) of the cases were male while 27 (54%) were female. The mean age was found to be 3.26±2 years. The average tumor size was 9.16 ± 2.9 cm in diameter and the average weight of the kidney was 478±312 gr. Thirteen (26%) cases were stage I, 18 (36%) cases were stage II, 7 (14%) cases were stage III, and 6 (12%) cases were stage IV. Thirty-nine cases were alive (78%), while 11 cases (22%) were deceased. Mean overall survival time was 68.2±39.5 (2-148) months. NGAL expression was negative in all tumors except the neutrophils within the tumors. KIM-1 expression was positive in 37 tumors (74%), while it was absent in 13 tumors (26%). Using Mann-Whitney U Analysis, KIM-1 expression was found to be associated with the stage of the tumor (p=0.027). CONCLUSION The preliminary data indicates that KIM-1 expression may be associated with stage in Wilms Tumor. However, further studies are needed to validate these pilot observations and to clarify the functional and mechanistic significance of this relevance.

İnce D, Demirağ B, Özek G, Erbay A, Ortaç R, Oymak Y, Kamer S, Yaman Y, Kundak S, Vergin C. Pediatric langerhans cell histiocytosis: single center experience over a 17-year period. Turk J Pediatr 2016; 58: 349-355.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.

Although central venous catheters (CVCs) have become an integral element of the care of patients undergoing hematopoietic stem cell transplantation (HSCT), the associated complications can lead to treatment delays and negatively affect the child and family’s quality of life. The aim of this study was to identify the types, rate of, and risk factors for the complications relating to CVCs used in children undergoing HSCT in Turkey. One hundred children were followed prospectively to track catheter-related complications in the transplantation unit. Overall, 186 complications were documented. The most frequent complications were catheter-related infections and malfunctions. Children who had nonhematological disease had a higher rate of complications than those with hematological disease. HSCT nurses should follow and apply evidence-based clinical practice guidelines to support the management of catheters. Nurse staffing ratios and workload may be an issue in developing countries. The authors believe that proper maintenance of CVCs contributes to the reduction of catheter-related complications despite these issues and that problems can be overcome by hiring experienced staff, appropriate nursing education, and by organizing and improving home care programs for HSCT patients.

The aim of this study was to assess the prognostic value of MIB-1 and p53 in the pediatric medulloblastoma group. Forty-two pediatric medulloblastoma cases diagnosed in a single institution during the past 10 years were evaluated. Follow-up data were available for 35 patients. The immunoreactivity of MIB-1 ranged from 10% to 95%; p53 immunoreactivity was found in five cases. Of the 35 patients with follow-up, 34 patients received a combination of chemotherapy and radiotherapy, while one received chemotherapy alone. The follow-up period ranged from 5 to 64 months. Of the 35 patients, 21 were alive without any evidence of recurrent disease, three were alive with evidence of recurrent disease and 11 died of disease during follow-up. The mean survival for these 11 patients was 21.9 ± 10.4 months. Of the 35 cases, 16 had MIB-1 value of 25% or lower and 19 had a value of 26% or more. Of the 16 cases with low MIB-1 value, six died of disease; of the 19 cases with high MIB-1 value, five died of disease. The statistical difference between MIB-1 and prognosis was not significant. Three of the 35 (8.5%) cases were found to be positive with p53 protein; no correlation was observed between p53 immunoreactivity and prognosis. It appears that the MIB-1 value and p53 immunoreactivity have no relation with prognosis in pediatric medulloblastomas. However, it is convenient to support these findings with large series.

Purpose To evaluate the results of radiotherapy and the prognostic factors affecting local control in nonextremity Ewing sarcomas. Methods Between 1995 and 2011, 44 patients with nonextremity Ewing sarcomas were treated with radiotherapy. Tumor localizations were pelvis in 23, spine in 13, thoracic region in 5, and cranium in 3 patients. Tumor size was ≥8 cm in 56.8% of patients. Distant metastases were present in 19 of the patients at the time of diagnosis (43.1%). All patients were treated with 12 weeks of neoadjuvant chemotherapy followed by surgery and radiotherapy (45-54 Gy) or radiotherapy alone (54-64.8 Gy). Radiotherapy was applied due to microscopic residue (R1) in 5 patients after the operation and macroscopic tumor in 39 patients (macroscopic residue [R2] and nonresectable tumor). Results Median follow-up was 49 months (range 9-195). Local failures developed in 7 patients (15.9%) and local control at 5 years was 81.4%. Local recurrence was detected in 6 patients (6/38) who did not have residual tumor after RT. Progression was detected in 1 patient (1/6) who had residual tumor. All those patients with local failure experienced further distant metastases. Possible prognostic factors such as age (≤17 vs >17), tumor localization, tumor volume (≤8 cm vs >8 cm), and M status at diagnosis (0 vs 1) were not related to local control. Conclusions Radiotherapy, either alone or adjuvant to surgery, provides local control in 80% of nonextremity Ewing sarcomas and plays an important role in treatment.

Objective: Neuroblastoma (NB) is the most common extracranial solid cancer in childhood and the most common cancer in infancy. Nearly half of neuroblastoma cases occur in children younger than two years. It is a neuroendocrine tumor, arising from neural crest. It most frequently originates from one of the adrenal glands, but can also develop in neural tissues in the neck, chest, abdomen, or pelvis. In this study, neuroblastoma cases were reviewed and classified occording to the Shimada classification.

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.

VRE species are an increasingly important and universal problem in intensive care units and hematology-oncology departments due to the spread of glycopeptide resistance. Rapid and accurate identification of VRE is therefore crucial. The intent of this study was to compare the diagnostic performance of a real-time PCR test, the BD GeneOhm VanR assay (GeneXpert vanA/ vanB, Cepheid, USA), with conventional cultures for screening hospitalized immunocompromised hematology-oncology patients for VRE. Three hundred and six duplicate rectal swab specimens were obtained from 120 pediatric hematology-oncology patients. PCR and conventional culture-based studies were performed. One hundred and twenty patients, 46 female and 74 male, participated in the study. The mean age of the patients was 7.5±4.7 years. A total of 51 specimens from 306 samples were found to be positive for vanA or vanB. Mean turnaround time for PCR was 0.5±0.2 days. Compared to the culture method, the RT-PCR assay had an overall sensitivity of 91.8% (34/37) and a specificity of 93.6%. The positive predictive value and negative predictive value were 66.6% and 98.8%, respectively. This study demonstrates that RT-PCR is a suitable alternative to culture-based procedures for rapid and accurate identification of VRE in hematology-oncology patients, as the overall performance of PCR is comparable to that of a chromogenic agar-based culture method for VRE screening, especially for detection of VRE-negative patients.

Objective: Although importance of expression status of caveolin-1 protein was estab - lished in different cancers including the renal cell carcinoma, its significance has not been evaluated in Wilms tumor. The aim of this study was to determine the prognostic value of caveolin-1 in Wilms tumor. Material and Methods: Immunhistochemical caveolin-1 expression was studied in a total of 50 formalin-fixed, paraffin-embedded Wilms tumor specimens and its association with different clinicopathologic parameters was evaluated. Results: Caveolin-1 expression was positive in 24 tu - mors (48%), while was absent in 26 tumors (52%). Using Mann Whitney U Analysis, Caveolin-1 ex - pression was found to be associated with the weight of the kidney (p=0.013). In addition; there was statistical significance with Caveolin-1 expression and therapy response (p=0.049). But there were no statistical relationships between with Caveolin-1 and some clinical prognosis factors such as stage (p=0.093) and survive (p=0.256). Conclusion: In the present study, it was shown that the presence of caveolin-1 expression in Wilms tumor associated with both tumor size and therapy response. These findings were thought that Caveolin-1 expression may play a role in Wilms tumor evolution.

Background Acute lymphoblastic leukemia (ALL) remains the most common pediatric malignancy in the world. Overall survival rates have increased from 30% in the 1960s to 90% in past 10 years. Enabling this dramatic improvement are the multi-agent chemotherapeutic regimens in which asparaginase is cornerstone. Unfortunately, hypersensitivity to asparaginase has been a commonly-reported adverse event, associated with inferior outcomes in ALL. Methods Between January 1, 2005 and December 31, 2012, 97 patients were enrolled in the study. Selection criteria included those were diagnosed with ALL and given the ALL-BFM-2000 protocol at Dr. Behcet Uz Children's Hospital. Data regarding age at diagnosis, sex, follow-up duration, hypersensitivity of L- asparaginase, status of relapse and outcome were gathered from the patients’ files. The event-free survival (EFS) and the overall survival (OS) of the patients who developed and did not develop hypersensitivity to L- asparaginase were compared. Peg-asparaginase was given to patients who developed L- asparaginase hypersensitivity, and erwinia asparaginase was given if the patients developed peg-asparaginase. The asparaginase activity could not be measured. Kaplan mayer test was used to calculate the EFS and the OS. Results A total of 97 patients were evaluated. Thirty-two were standard risk, 49 were moderate risk, and 15 were high risk. 61 patients were male, and 36 were female. The median age (range) at diagnosis was 5 (1-15) years. Median (range) follow-up duration was 5.1 (0.08-7.3) years. Of the 97 patients, 28 (29.2%) developed L-asparaginase hypersensitivity. The event-free survival was 84.5 ± 4.6%, and 62.6 ± 9.4% for patients who developed and did not develop hypersensitivity, respectively (p=0.01). The overall survival rates were 89.2 ± 3.9 % and 74.6 ± 8.3% for patients who developed and did not develop hypersensitivity, respectively (p=0,07). Figure 1. Event-free survival. The five years EFS±SE for the patients who developed and did not develop native L- asparaginasehypersensitivity were 84.5 ± 4.6% and 62.6 ± 9.4%, respectively (p=0.01). Figure 1. Event-free survival. The five years EFS±SE for the patients who developed and did not develop native L- asparaginasehypersensitivity were 84.5 ± 4.6% and 62.6 ± 9.4%, respectively (p=0.01). Conclusions This study demonstrated that EFS was lower in patients with L-asparaginase hypersensitivity than those without hypersensitivity, although there were no differences in terms of OS. All of the patients with hypersensitivity received a sufficient dose of peg-asparaginase or erwinia asparaginase according to the ALL-BFM-2000 protocol. The lower EFS for patients who were switched to peg-asparaginase because of L- asparaginase may be explained by silent inactivation of peg-asparaginase. If the activity of asparaginase could not be measured, it could be preferable to switch to erwinia asparaginase. Disclosures No relevant conflicts of interest to declare.

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.