Neonatal Cardiac Rhabdomyoma: A Single-Center Experience.

AIM: Cardiac rhabdomyoma, known as the most common benign cardiac tumor in childhood, is strongly associated with tuberous sclerosis complex. This study aims to present our single-center experience regarding clinical observations, diagnostic approaches, and treatment modalities for cardiac rhabdomyoma identified during the neonatal period. PATIENTS AND METHODS: In this clinical observational study, we retrospectively assessed the outcomes of 12 newborn patients diagnosed with cardiac rhabdomyoma who were followed up in our neonatal intensive care unit over the past 12 years. RESULTS: The mean gestational age of the patients was 38.2±1.6 weeks, with an average birth weight of 3193±314 grams. The mean postnatal age at initial diagnosis was 12.42±15.75 days. Tuberous sclerosis complex was clinically identified in 50% of cases (six patients). Seven infants received everolimus treatment, while three infants underwent clinical monitoring without specific interventions. A significant reduction in cardiac mass size was observed in all surviving patients, leading to their subsequent discharge from the hospital. CONCLUSION: Cardiac rhabdomyomas often undergo spontaneous regression in early childhood. However, in cases with obstructive lesions or arrhythmias, they may present life-threatening consequences. Timely diagnosis, appropriate clinical management, and monitoring are crucial in optimizing outcomes for neonates with cardiac rhabdomyoma.

Treatment of tuberous sclerosis complex manifestations in children with mTOR inhibitors.

PURPOSE: Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder that affects multiple organ systems. Mutations in the TSC1 and TSC2 genes result in the constitutive hyperactivation of the mammalian target of rapamycin (mTOR) pathway, contributing to the growth of benign tumors or hamartomas in various organs. Due to the implication of mTOR pathway dysregulation in the disease pathology, increasing evidence supports the use of mTOR inhibitors for treating multiple manifestations of TSC. METHODS: In this study, we conducted a retrospective analysis of clinical findings and treatment data from 38 patients diagnosed with tuberous sclerosis who were followed up in the Pediatric Oncology Clinic between 2010 and 2020. We collected information on patients' ages, genders, affected sites, familial history, imaging findings, presence of tumors, and treatments. RESULTS: Among the patients, nine individuals with TSC manifestations were treated with mTOR inhibitors. Specifically, everolimus was successfully administered to five patients with inborn cardiac rhabdomyoma causing hemodynamic impairment. In addition, two patients with refractory seizures received everolimus in combination with anti-epileptic drugs. A patient with renal angiomyolipomas larger than 3 cm was treated with everolimus, while a patient with extensive facial angiofibroma received topical sirolimus. All patients tolerated the mTOR inhibitors well, and the side effects were deemed acceptable. CONCLUSION: The utilization of mTOR inhibition in TSC is expected to become more prevalent in clinical practice, as current research is anticipated to provide a better understanding of the therapeutic roles of these treatments in TSC.

Isocitrate Dehydrogenase 1 and 2 Mutations in Pediatric Neuroblastoma Patients.

Objective: Neuroblastoma is one of the common tumors of childhood. The demonstration of new factors such as isocitrate dehydrogenase 1 (IDH1) and isocitrate dehydrogenase 2 (IDH2) mutations will be important in the diagnosis and treatment. IDH1 and IDH2 mutations have been found in many types of cancer, such as malignant gliomas, acute myeloid leukemias, chondrosarcoma, and thyroid carcinoma. This study aimed to investigate the presence of IDH1 or IDH2 mutations in patients with neuroblastoma and to determine whether these mutations were different in terms of age, clinical findings, and response to treatment. Methods: Biopsy specimens of 25 patients with pediatric neuroblastoma patients were evaluated for IDH mutations. The clinical and laboratory features of the patients with/without mutation were retrospectively analyzed from a hospital database. Results: A total of 25 patients for whom genetic analysis could be performed were included in the study (60% male, n=15). The mean age was 32.2±25.9 months (3 days-96 months). IDH1 mutation was detected in 8 (32%) and IDH2 mutations in 5 (20%) patients. These mutations showed no statistically significant relationship with age, tumor localization, laboratory results, stage, and prognosis. However, in the case of IDH mutation, patients were diagnosed at the advanced stage. Conclusions: This study demonstrated the relationship between neuroblastoma and IDH mutation for the first time. Because to the fact that the mutation is very heterogeneous, it would be appropriate to conduct a larger series of patients in terms of the impact of the clinical significance of each mutation on the diagnosis and prognosis.

Evaluation of adolescents with heavy menstrual bleeding using the International Society of Thrombosis Haemostasis- Bleeding Assessment Tool and The Pediatric Bleeding Questionnaire scores.

BACKGROUND: Heavy menstrual bleeding (HMB) is a common condition in adolescents. However, bleeding disorders are known to be one of the causes of HMB in adolescent girls, so they should be considered. Simple methods that can be used in primary health care are needed to determine whether patients have bleeding disorders. The aim of this study was to evaluate the bleeding score of patients admitted with HMB and to determine the diagnostic value of patients who were symptomatic but whose initial hemostatic tests were normal. METHODS: A total of 113 adolescents with HMB and 20 healthy adolescent girls were included in the study. The Pediatric Bleeding Questionnaire (PBQ) and the International Society of Thrombosis Haemostasis-Bleeding Assessment Tool (ISTH-BAT) were used for evaluation. RESULTS: Overall, approximately 18% (n= 20) of the adolescents in the study were diagnosed with a bleeding disorder. The cut off value for the `clinically significant bleeding score` was found to be 3.5. CONCLUSIONS: The PBQ and ISTH-BAT can help distinguish a significant bleeding history from an otherwise trivial bleeding and can be included in the algorithm for the primary care of adolescents with HMB with suspected bleeding disorders.

Thiol disulfide homeostasis in ionizing radiation and chemotherapeutic drug exposure.

OBJECTIVE: This study aims to reveal the oxidant and antioxidant status in nurses with chemotheropathic drug exposure and radiology unit workers exposed to ionizing radiation (IR). METHODS: Nineteen radiology unit workers, 14 nurses, and 15 controls were included the study. All of the participants using antioxidants, vitamin supplements, smokers, any therapeutic drugs, and exposed therapeutic or diagnostic X-ray or chemotherapeutic drugs in 12 months were excluded from the study. Total and native thiols, disulfide/native thiol percent ratios (SS/SH), disulfide/total thiol percent ratios, disulfide amounts, and native thiol/total thiol percent ratios, ischemia-modified albumin (IMA) were determined. RESULTS: Disulfide levels, disulfide/total thiol ratio, and disulfide/native thiol ratio of serum samples of both radiology unit workers and nurses were significantly higher and ratio of native thiol/total thiol was lower than the control group. The radiation dose in radiology unit workers was mean±SD: 0.02±0.009, median (min-max): 0.02 (0.001-0.04). Thiol-disulfide homeostasis was disturbed and the balance shifted in the direction of oxidant damage, even at low-dose IR exposure and normal range. CONCLUSION: As far as we know, the current findings first demonstrate an apparent chronic oxidative stress in the subjects who were occupationally exposed to antineoplastic drugs and radiation even if annual radiation exposure dose measurements are normal.

Evaluation of the Attitude of Parents in Pediatric Oncology Towards COVID-19 Vaccine.

Severe acute respiratory syndrome, coronavirus (SARS, COVID-19) has been declared a pandemic by the World Health Organization since March 2020. Patients with active cancer should be considered especially for priority access to the COVID-19 vaccine. Therefore, our study aimed to learn parents' opinions of cancer-diagnosed patients about the COVID-19 vaccine. Between December 2021 and January 2022, 76 people were willing to answer the questionnaire from the parents of outpatient/inpatient patients at the Pediatric Hematology and Oncology Clinic of Dr. Sami Ulus Obstetrics, Child Health and Diseases Education Research Hospital were included. In our study, 18 parents (23.7%) did not get vaccinated. Among the reasons for not getting vaccinated, the most common answer for "I fear from the vaccine's side effects.", "Do you think oncology patients are at risk for COVID-19?", "Do you think the COVID-19 pandemic affects cancer treatment?" were 90.7%, 89.5% and 21% yes, respectively. Would your opinion of the vaccine be positive if the domestic vaccine was produced?"-67.4% answered yes. Vaccine hesitation and public misinformation put cancer patients at risk. Increasing awareness of the rejection of COVID-19 vaccines is important for public health and the fight against the pandemic. In addition, the doctors' recommendations for conducting oncology treatment will significantly impact parents' compliance with the COVID-19 vaccine.

Sirolimus treatment of a PTEN hamartoma tumor syndrome presenting with melena.

BACKGROUND: PTEN hamartoma tumor syndrome (PHTS) is an umbrella term including Cowden syndrome (CS), Bannayan-Riley-Ruvalcaba syndrome (BRRS), PTEN-related Proteus syndrome (PS), and PTEN-related Proteus-like syndrome. One of the disorders in PHTS spectrum, CS is characterized by macrocephaly, mucocutaneous findings, gastrointestinal system (GIS) polyposis and an increased lifetime risk of GIS, breast, thyroid and other cancers. CASE: In this study, we report an adolescent patient presenting with recurrent life-threatening upper GIS bleeding as a result of hamartomatous polyposis. Genetic studies revealed a known pathogenic nonsense mutation confirming the initial diagnosis of CS. CONCLUSIONS: Additionally, we describe our therapeutic intervention to improve the patient`s clinical symptoms with sirolimus, which its use is infrequently addressed in the literature for pediatric age group harboring PTEN mutations.

Dynamic Thiol-Disulfide Homeostasis in Children With ß-Thalassemia Trait.

In children with ß-thalassemia (ß-thal) trait, tissue damage occurs with oxidative stress due to oxygen free radicals and reactive oxygen species (ROS) production. Dynamic thiol-disulfide homeostasis (DTDH) is one of the most important indicators showing the pro-oxidant/antioxidant status in the body. In this study, we aimed to examine the status of DTDH by measuring native thiol, disulfide, and total thiol levels in children with ß-thal trait. The study included 40 children with ß-thal trait and 30 healthy controls (matched by age and gender). The DTDH parameters were measured by an automated method and results were compared between the groups. The levels of native thiol, total thiol, and disulfide in children with ß-thal trait group were statistically significantly higher than the control group (p < 0.001). There was no significant difference in disulfide/native thiol, disulfide/total thiol, and native thiol/total thiol levels between the groups. In addition, there was no correlation between hemoglobin (Hb) and serum ferritin levels with the markers of DTDH in children with ß-thal trait. In our study, a significant increase was found in native thiol, total thiol, and disulfide levels in response to oxidative stress in children with ß-thal trait compared to the healthy control group. Disulfide levels of the children with ß-thal trait were higher than the control group, showing oxidative stress is high in ß-thal trait. Accordingly, it increases the native thiol and total thiol capacity as compensation.

Intrauterine Growth Retardation is a Risk Factor for Anthracycline Toxicity.

BACKGROUND: Anthracycline chemotherapy is used to treat a variety of cancers. However, late cardiac effects of anthracycline chemotherapy, such as subclinical left ventricular dilatation and/or dysfunction, have been observed in more than half of long-term survivors of childhood cancers. A major risk factor for anthracycline cardiotoxicity is intrauterine growth restriction (IUGR). We assessed the significance of IUGR as an important risk factor for late cardiotoxic effects of anthracycline therapy in asymptomatic long-term survivors of childhood cancers. MATERIALS AND METHODS: The study included 61 survivors of childhood cancers. Cardiac functions were prospectively studied using both conventional and non-conventional echocardiographic methods (two-dimensional speckle tracking echocardiography) after completion of the treatment. The patients were divided into two groups based on their birth weights: Group 1 (patients with IUGR) and Group 2 (patients with normal birth weight). RESULTS: Conventional echocardiography revealed a similar and normal range of left ventricle systolic and diastolic functions in both groups. However, global longitudinal and circumferential strain values demonstrated subclinical left ventricular systolic dysfunction in both groups as compared with normal reference strain values. Furthermore, Group 1 patients had significantly lower global longitudinal and circumferential strain and strain rate values than those in Group 2 patients. CONCLUSION: Asymptomatic long-term survivors of childhood cancers with a history of IUGR may have an increased risk of anthracycline cardiotoxicity due to the low content of mitochondrial DNA (mtDNA). IUGR is a risk factor for late anthracycline cardiotoxicity.

Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives

Aim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey. Material and methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires. Results: Only 1 of the 21 participating centers had a separate LTFU clinic. The remaining centers provided LTFU care for childhood cancer survivors at the pediatric oncology outpatient clinic. Of these centers, 17 (80.9%) reported difficulty in transition from the pediatric clinic to the adult clinic, 14 (66.6%) reported insufficient care providers, and 12 (57.1%) reported insufficient time and transportation problems. As neglected late effects, 16 (76.1%) centers reported psychosocial and getty job problems and 11 (52.3%) reported sexual and cognitive problems. None of the centers had their own LTFU guidelines for their daily LTFU practice Conclusion: This study was the first to gain an overview of the needs of POCs and the gaps in survivorship services in Turkey. The results from this study will help to develop a national health care system and national guidelines for pediatric cancer survivors.

Evaluation of long-term cardiac side effects of anthracycline chemotherapy by conventional and non-conventional echocardiographic methods in childhood cancer survivors.

OBJECTIVE: Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiac toxicity. The aim of this study was to determine the long-term effect of anthracycline chemotherapy on the biventricular function in childhood cancer survivors using tissue Doppler imaging and two-dimensional speckle tracking echocardiography. PATIENTS AND METHODS: The study included 45 survivors of childhood cancers and 50 healthy age-matched control patients. Cardiac function was prospectively studied with conventional echocardiography, tissue Doppler imaging, and speckle tracking echocardiography after completion of treatment. The same analysis was performed on matched controls. RESULTS: There was no difference in age, gender, height, and weight between the study and control groups. The mean anthracycline dose was 240 ± 106 mg/m2 and the mean remission duration was 8.2 ± 5 years (1-20 years) in the study group. Conventional echocardiography showed similar ejection fraction, shortening fraction, and left ventricle end-diastolic diameter in both groups. Mitral lateral and septal tissue Doppler imaging showed normal but according to control group relatively sub-normal systolic and diastolic function in patient group. The global longitudinal and circumferential strain and strain rates were significantly lower in the patient group compared to control group. Correlation analysis revealed a negative and significant correlation between total anthracycline dose and global longitudinal and circumferential strain and strain rates. CONCLUSION: Sub-clinical systolic and diastolic dysfunction may not be detected by conventional echocardiographic methods which are frequently used in daily practice. Sub-clinical systolic and diastolic dysfunction may be detected more sensitively by echocardiographic method such as speckle tracking echocardiography in childhood cancer survivors.

Economic and psychosocial problems experienced by pediatric with cancer patients and their families during the treatment and follow-up process.

AIM: To identify the psychosocial and economic problems of the pediatric patients with cancer who were treated at the Dr. Sami Ulus Obstetrics and Gynecology and Child Health and Diseases Training and Research Hospital's Pediatric Oncology Department and their relatives during this process. MATERIAL AND METHODS: We interviewed a total of 100 patients who were treated at Dr. Sami Ulus Obstetrics and Gynecology and Child Health and Diseases Hospital's Pediatric Oncology Department between 1996 and 2015, and were now followed up without treatment and their relatives using survey questions on the psychosocial and economic problems they experienced. RESULTS: Most of patients were from provinces outside Ankara. The average monthly income was below the level that would meet the family needs in 80% of the families and only 16% had extra income. Additional economic support had been received by 93% of the families in the patient group. Twenty-five families (25%) had been forced to sell property during the treatment. Forty-nine (49%) families had borrowed money from acquaintances and relatives or had taken out a bank loan. Serious psychological problems were experienced during and after the treatment by 46% of the mothers; 41% of the families had used religious procedures more commonly during the treatment period to cope with the psychological problems. Education was subject to a 1-2 year pause in 83% of the patients. CONCLUSION: Childhood-age patients with cancer and their families experience significant psychosocial and economic problems during and after the treatment process. Providing medical treatment and psychosocial support in harmony is an important factor that increases the success of cancer treatment. The patient and the family will require psychosocial support mechanisms throughout life, starting from the moment they face the disease. Developing national social support programs and legal regulations to form a basis for such programs are required in our country.

AMAÇ: Dr. Sami Ulus Kadin Dogum ve Çocuk Sagligi ve Hastaliklari Egitim ve Arastirma Hastanesi Çocuk Onkoloji Klinigi'nde tedavi edilmis kanserli çocuk hastalarin ve yakinlarinin bu süreçte karsilastiklari psikososyal ve ekonomik sorunlari belirlemek. GEREÇ VE YÖNTEMLER: Dr. Sami Ulus Çocuk Sagligi ve Hastaliklari Hastanesi Çocuk Onkoloji Klinigi'nde 1996­2015 yillari arasinda tedavi görmüs ve su anda hastaliksiz olarak izlemde olan toplam 100 hasta ve yakini ile görüsülerek yasadiklari psikososyal ve ekonomik sorunlara iliskin anket sorulari yönlendirildi. BULGULAR: Hastalarimizin çogu Ankara disi illerden gelmekte idi. Hasta ailelerinin %80'inin ortalama aylik gelirinin ailenin gereksinimlerini karsilama düzeyinin altinda oldugu ve sadece %16'sinin ek geliri oldugu saptandi. Hasta grubunda ailelerimizin %93'ü ek ekonomik destek almislardi. Yirmi bes aile (%25) tedavi süresince sahip olduklari bazi mal varliklarini satmak zorunda kalmisti. Kirk dokuz (%49) aile tanidik ve akrabalarindan borç almisti ya da bankadan kredi çekmisti. Annelerin %43'ünün tedavi sirasinda ve sonrasi ciddi psikolojik sorunlar yasadigi, psikolojik sorunlarla basa çikabilmek için ailelerin %40'inda tedavi döneminde dini ibadetlere egilimde artis oldugu gözlendi. Hastalarimizin %83'ünün egitimlerinde 1­2 yil gibi bir kayip yasandigi görüldü. ÇIKARIMLAR: Çocukluk çagi kanser hastalari ve aileleri tedavi sürecinde ve sonrasinda önemli oranda psikosoyal ve ekonomik sorunlar yasamaktadir. Kanserde, tibbi tedavi ile psikososyal destegin bir uyum içerisinde yürütülmesi tedavi basarisini artiran önemli bir etmendir. Hastanin ve ailesinin hastalikla tanistigi ilk andan baslayarak tüm hayat boyunca psikososyal destek mekanizmalarina gereksinim vardir. Ülkemizde ulusal olarak bu yönde gelistirilecek sosyal destek programlari ve bu programin yürütülecegi zemini olusturacak yasal düzenlemelere gereksinim vardir.

Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. Patients diagnosed with secondary HLH, between January 2011 and December 2016, were retrospectively included in this study. We analyzed clinical and laboratory findings as well as prognostic factors from 24 pediatric patients diagnosed with secondary HLH. The mean age of patients at the time of diagnosis was 79.9 ± 68.7 months (range: 2-202) and 54.2% of the patients were male. The most frequent HLH-2004 criterion was fever (100%). Underlying triggers of HLH were as follows: 13 (54.1%) infections, juvenile idiopathic arthritis in 5 patients (20.8%), drugs in 3 patients (12.5%), malignancies in 2 (0.8%), Kawasaki disease in 1 (0.4%) patient, and 1 (0.4%) with unknown triggers. The median time of diagnosis was 3 days (1-67 days). Overall, the mortality rate was 20.8%. In our logistic regression model, factors associated with mortality were decreased albumin levels (OR1 = 2.3[1.48-3.43]) and etoposide usage (OR2 = 1.22 [1.14-1.89]). The patient's 30-day survival was inferior among patients whose albumin level was 2 g/dL or less compared to those over 2 g/dL. Increased awareness of the underlying condition is critical in HLH patients. Our study emphasizes the prognostic significance of albumin level.

Association of vitamin D receptor gene polymorphisms with osteosarcoma risk and prognosis.

OBJECTIVE: Through its receptor (VDR), vitamin D3 plays an important role in a wide variety of cellular processes. Polymorphisms in VDR gene have been linked to risk of various cancers and their prognoses. We conducted a case-control study to analyze he relationship of VDR gene polymorphisms with the occurrence and prognosis of osteosarcoma. METHODS: Fifty-eight osteosarcoma patients and 75 healthy controls were included in the study. Single nucleotide change polymorphisms (SNPs) in Cdx2, FokI, BsmI, ApaI and TaqI regions of VDR gene were examined with SNaPshot mini-sequencing technique. Allele and genotype frequencies in patients and controls were compared. The association of polymorphic genotypes with osteosarcoma was evaluated. The relationship of the presence of polymorphism to prognostic parameters and survival rates were also analyzed. RESULTS: Allele and genotype frequencies of Cdx2, FokI, BsmI, ApaI and TaqI regions in VDR gene were found to be similar in patients and controls. Polymorphisms in these regions were not associated with osteosarcoma risk. In patients having Cdx2 polymorphic allele, tumor volume was greater (p:0.041), metastasis was more common (p:0.042) and histopathological response to chemotherapy was worse (p:0.044). Good histopathological response was significantly higher in patients with BsmI homozygous polymorphism (p:0.037). In the presence of heterozygous Cdx2 and homozygous a higher three-year overall survival rate was found, while there was a higher event-free survival rate in patients with ApaI polymorphisms. CONCLUSION: Our results suggested that although polymorphisms of VDR gene are not related to the development of osteosarcoma, they may be important for prognosis. Understanding the effect of VDR polymorphisms on osteosarcoma will be important in identifying new prognostic parameters and new targets for treatment.

Kikuchi-Fujimoto disease triggered by Salmonella enteritidis in a child with concurrent auto-immune thyroiditis and papilloedema.

Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.

Vitamin D receptor polymorphisms in immune thrombocytopenic purpura.

BACKGROUND: Vitamin D receptor (VDR) polymorphisms have been studied in immune-mediated disorders, but not yet in immune thrombocytopenic purpura (ITP). We investigated whether VDR variants were associated with ITP in children. METHODS: The study included 44 children with a diagnosis of ITP and 100 healthy controls. Five VDR polymorphisms (Cdx-2, FokI, BsmI, ApaI and TaqI) were genotyped and used to evaluate the association of VDR variants with ITP. RESULTS: The distribution of the three Cdx-2 genotype groups (GG, GA, and AA) was significantly different between ITP patients and controls (P = 0.025); the homozygous GG genotype of Cdx-2 was overrepresented in ITP patients. The frequency of the A allele of Cdx-2 was significantly different between patients and controls (P = 0.01). The A allele of Cdx-2 was associated with a decreased risk of ITP (OR, 0.343; 95% CI: 0.150-0.782). No statistically significant difference was found between the ITP group and control group for Fok1, Bsm1, Apa1, and Taq1 polymorphisms (P > 0.5). CONCLUSION: There appears to be an interaction between the Cdx-2 variant of VDR and childhood immune thrombocytopenia.

The Correlation Between Pre-treatment Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Parameters and Clinical Prognostic Factors in Pediatric Hodgkin Lymphoma.

OBJECTIVE: To compare standardized uptake values (SUV) derived from pre-treatment 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) imaging and clinical prognostic factors in pediatric patients with Hodgkin lymphoma (HL). METHODS: Pre-treatment FDG PET/CT findings of 28 children with HL were evaluated in this retrospective study. Metabolic tumor volume (MTV), SUVmax normalized by weight (SUVweight), lean body mass (SUVlbm), body surface area (SUVbsa) and plasma glucose levels of tumors (SUVglucose) were calculated using pre-treatment FDG PET/CT scan images. These metabolic parameters were correlated with clinical factors [age, sex, number of lymph node groups, presence of splenic involvement, bulky mediastinal disease, Ann Arbor stage, serum white blood cell (WBC) count, erythrocyte sedimentation rate (ESR), serum albumin and hemoglobin levels]. RESULTS: SUVbsa, SUVlbm, SUVweight, SUVglucose and MTV were higher in patients with stage III-IV disease, bulky tumor and ≥3 lymph node groups (p<0.05). SUVbsa and SUVglucose were higher in patients with splenic involvement (p<0.05). There was no significant correlation between these metabolic parameters and sex, ESR, levels of albumin and WBC (p>0.05). SUVbsa and SUVlbm were higher in patients with anemia (p<0.05). Additionally, significant increases were detected in SUVweight, MTV, and SUVglucose with increasing age (p=0.005, p=0.027, and p=0.009, respectively). SUVbsa and SUVlbm had no significant correlation with age (p>0.05). CONCLUSION: Metabolic parameters derived from pre-treatment FDG PET/CT may have an important role in predicting high-risk disease in patients with HL. Also, SUVbsa and SUVlbm may be better markers than SUVweight in the quantitative evaluation of FDG PET/CT scans in pediatric patients.

Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country.

Incesoy-Özdemir S, Ertem U, Sahin G, Bozkurt C, Yüksek N, Ören AC, Balkaya E, Alkan A. Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country. Turk J Pediatr 2017; 59: 410-417. Germ cell tumor (GCT) is a rare malignancy accounting for 2-3% of all pediatric tumors. The overall survival rate of children and adolescents with GCT is more than 80% after adopting combined therapy. The aim of this study is to review clinical presentation, management, and outcome in a single-center series with extracranial GCT. Clinical characteristics, pathologic presentations, and survival outcomes of 101 children with GCT, treated at our hospital from 1988 to 2011, were analyzed. Sixty-two of patients were female and 39 of them were male. Fifty-eight (57%) patients had gonadal tumor (24 testicular, 34 ovarian), 43 (43%) extragonadal. Histologically, teratomas were found most frequently (26 mature, 10 immature), followed by yolk sac tumors (n: 33), mixed malignant tumors (n: 13), embryonal carcinoma (n: 10), disgerminoma (n: 8) and seminoma (n: 1). Twenty-six patients were diagnosed as mature teratoma and we excluded them in the evaluation of staging and survival. Five-year overall and relaps-free survival were 80.3% (mean follow-up time: 215.8 months) and 73.4% (mean follow-up time: 176.2 months), respectively. Five-year survival rates were 93.2% and 90.2% in malign GCTs diagnosed after 1999.

Epidemiological features and risks of hemangiomas.

Sahin G, Düzcan-Kilimci D, Tanyildiz HG. Epidemiological features and risks of hemangiomas. Turk J Pediatr 2017; 59: 664-669. Hemangiomas are the most common benign tumors of childhood. The known risk factors in developing hemangiomas are female gender, prematurity and low birth weight. Our study was designed to investigate prenatal and natal risk factors in addition to the above factors that would potentially affect development of hemangiomas. Two hundred forty hemangioma patients and 100 healthy controls were included in the study. Both groups were interviewed for age, gender, prenatal characteristics (presence of maternal pregnancy hypotension, hypertension and hyperemesis, parental smoking habits) as well as natal characteristics (delivery, birth weight, gestational age) and maternal-paternal age. Hemangiomas were mostly localized in the head and neck. Risk of developing hemangioma was higher in girls and in firstlings. History of hyperemesis during pregnancy increased incidence of hemangioma. Post-term delivery was found to be protective against developing hemangioma. We are in the opinion that there is a need for wider studies with a bigger sample size so as to detect other risk factors.

Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: Report of three cases.

Balkaya E, Bozkurt C, Aksu AE, Özmen S, Incesoy-Özdemir S, Sahin G. Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: Report of three cases. Turk J Pediatr 2017; 59: 704-707. Ewing`s sarcoma (ES) is the second most common childhood primary malignant tumor of the bone. The most popular locations of ES are long bones and pelvis. The involvement of the mandible is very rare in childhood. In last 10 years, we met with three cases of ES of the mandible in our department. Initially the patients had symptoms similar to periodontal inflammation. The involvement of the mandible might be considered with periodontal inflammation in its initial stages, what frequently leads to delayed treatment. Although this tumor has an aggressive clinical behavior and rapid growth, early diagnosis can reduce patient`s morbidity and mortality and thus it is important to distinguish from periodontal inflammation.