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.

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.

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.

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.

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.

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.

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

In children with β-thalassemia (β-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 β-thal trait.

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).

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.

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.

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.

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.

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

Objective: Through its receptor (VDR), vitamin D 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.

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.

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 F-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), SUV normalized by weight (SUV), lean body mass (SUV), body surface area (SUV) and plasma glucose levels of tumors (SUV) were calculated using pre-treatment FDG PET/CT scan images.

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.

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

Balkaya E, Bozkurt C, Aksu AE, Özmen S, İncesoy-Özdemir S, Şahin G. Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: Report of three cases. Turk J Pediatr 2017; 59: 704-707.

Epidemiological features and risks of hemangiomas.

Şahin G, Düzcan-Kilimci D, Tanyıldız HG. Epidemiological features and risks of hemangiomas. Turk J Pediatr 2017; 59: 664-669.

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

İncesoy-Özdemir S, Ertem U, Şahin 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.

Are the methylenetetrahydrofolate reductase 1298 and 677 gene polymorphisms related to optic glioma and hamartoma risk in neurofibromatosis type 1 patients?

The methylenetetrahydrofolate reductase (MTHFR) gene plays a key role in carcinogenesis through its effects on DNA synthesis and methylation and also has a significant role in the etiology of many disorders, such as diabetes, migraine, and cardiovascular disease. Neurofibromatoses (NF) are autosomal dominant inherited diseases that can affect tissues such as bone and skin and predispose individuals to tumor development in various parts of the nervous system or body. Optic nerve glioma and brain tumors are common in children with NF, and leukemia and lymphoma incidence is also higher than normal.

Vitamin D Receptor (VDR) Polymorphisms in Pediatric Patients Presenting With Hodgkin's Lymphoma.

Vitamin D receptor (VDR) polymorphisms are found more commonly in some tumor types than in healthy individuals, suggesting that some polymorphisms (Cdx2, Fok1, Bsm1, Apa1, Taq1) contribute to tumor development. There is no previous report on VDR polymorphism in Hodgkin's lymphoma (HL) patients. VDR polymorphism patterns in 95 pediatric HL cases with 100 healthy controls were compared.

18F-FDG PET/CT Imaging of Hodgkin Lymphoma in a Child with Common Variable Immunodeficiency.

Common variable immunodeficiency is characterized by low levels of serum immunoglobulins and antibodies, recurrent infections, and a predisposition to malignancy. Here, we present the F-FDG PET/CT findings of a 7-y-old boy with common variable immunodeficiency and Hodgkin lymphoma.

Single-center experience with sirolimus therapy for vascular malformations.

Vascular malformations (VMs) are described as congenital malformations of the vasculature derived from capillaries, veins, lymphatic vessels, arteries, or a combination of these vessels. They can cause significant morbidity resulting from soft tissue hypertrophy-related disfiguration, bony abnormalities, and even organ compromise. They are usually treated with various interventional procedures to achieve local control; however, the chance of success decreases as the anatomical distribution of the malformation widens.