Oculoauriculofrontonasal Dysplasia Syndrome With Additional Clinical Features.

Oculo-auriculo-vertebral spectrum and frontonasal dysplasia are two well-known examples of dysmorphology syndromes. Oculoauriculofrontonasal syndrome (OAFNS) is a clinical entity involving the characteristics of both OAVS and FND and is thought to be a result of the abnormal development of structures in the first and the second branchial arches, including the abnormal morphogenesis of maxillary processes. Herein we report a case of OAFNS with cliteral hypertrophy, premaxillary teeth, and inguinal hernia, features not previously reported in the literature.

Diagnostic Value of Upar, IL-33, and ST2 Levels in Childhood Sepsis.

Background: The aim of this study was to evaluate the diagnostic value of Upar, IL-33, and ST2 in comparison with C-reactive protein, TNF-α, and Interleukin-6 in childhood sepsis.

Methods: A total of 128 children were included and 20 of them were the control group. We used only data showing a high probability of sepsis with blood culture positive children, because of this reason 68 children were excluded.

Evaluation of Etanercept Treatment in Newborn Rat Model with Hyperoxic Lung Injury.

Background: Many factors contribute to the development of BPD basically by increasing inflammation in preterm lungs. However, premature neonates have insufficient anti-inflammatory capacity. We aimed to evaluate the effect of etanercept, an anti-TNF agent, on BPD development in newborn rat model with hyperoxia-induced lung injury.

Interleukin-8 and Its Receptors in Human Milk from Mothers of Full-Term and Premature Infants.

In addition to its nutritional benefits, human milk also has bioactive elements. Limited immunological functions of newborns are supported and altered by the immunological elements of mother milk. Chemokines are of importance among these immune factors.

Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever--a randomized controlled noninferiority trial.

Background: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF).

Methods: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage.

Inflammatory Myofibroblastic Tumor Presenting with Diabetes Insipidus in an Eight-Year-Old Boy: A Case Report.

Inflammatory myofibroblastic tumors (IMT) develop as a non-neoplastic proliferation of myofibroblasts in a myxoid to collagenous stroma admixed with inflammatory cells. The symptoms depend on the specific location of the tumor, which can be anywhere, but is particularly in the respiratory system. Thus, patients with IMT can present with a variety of findings.

Development of a medication adherence scale for familial Mediterranean fever (MASIF) in a cohort of Turkish children.

Objectives: To develop and assess the validity and reliability of an adherence scale concerning medical treatment in paediatric FMF patients.

Methods: The Medication Adherence Scale in FMF Patients (MASIF) is a 18-item questionnaire that evaluates adherence to medication in four domains. Validation of the instrument was accomplished in paediatric FMF patients (aged 2-18 years) under medication at least for 6 months.

Endocan and Soluble Triggering Receptor Expressed on Myeloid Cells-1 as Novel Markers for Neonatal Sepsis.

Background: Neonatal sepsis is an important cause of neonatal morbidity and mortality in the neonatal intensive care unit. Soluble triggering receptor expressed on myeloid cells-1 (sTREM-1) has been evaluated in sepsis and septic shock, and it was found to be valuable in distinguishing septic cases from nonseptic cases. Endocan is constitutively expressed by endothelial cells, and high levels of endocan may be of relevance for the promotion of systemic inflammation.

Ozone Ameliorates Doxorubicine-Induced Skin Necrosis - results from an animal model.

Doxorubicin (DXR) extravasation result with serious morbidity like skin ulceration and necrosis. The purpose of this study is to determine the protective effects of ozone, olive oil, dimethyl sulfoxide (DMSO), and coenzyme Q10 in the treatment of DXR-induced skin ulcers on rats. After an intradermal injection of DXR on a basis of an animal extravasation model, the materials were topically applied.

Metabolic and Histopathological Effects of Fructose Intake During Pregestation, Gestation and Lactation in Rats and their Offspring.

Objective: Studies have demonstrated a significant relationship between maternal fructose intake and metabolic outcome in their offspring. However, there is a paucity of data about the long-term effects of fructose intake on the offspring of fructose-fed dams. Therefore, we planned a study to evaluate the long-term effects of fructose intake on the offspring of dam rats fed a high-fructose diet.

Measures of ventricles and evans' index: from neonate to adolescent.

Ventricle sizes are important for the early diagnosis of hydrocephalus or for follow-up after ventriculostomy. Diameters of ventricles may change, especially in childhood. This study aims to provide normative data about ventricle diameters.

Measures of pituitary gland and stalk: from neonate to adolescence.

Objective: The aim of this study is to provide normative data about pituitary diameters in a pediatric population. Pituitary imaging is important for the evaluation of the hypothalamo-pituitary axis defect. However, data about normal pituitary gland diameters and stalk are limited, especially in children.

The effect of melatonin on procarbazine induced testicular toxicity on rats.

Procarbazine (P) is an effective chemotherapeutic drug especially used in lymphoma treatment; however testicular toxicity is a limiting factor. Various ways of treatment were tried to preserve testicular function including hormonal treatment, antioxidant treatment, and sperm cryopreservation but resulted with low rates of satisfaction. Procarbazine is a well known agent causing sterility even in the first doses of chemotherapy.

Endothelial function in patients with familial Mediterranean fever-related amyloidosis and association with cardiovascular events.

Objectives: Secondary amyloidosis is the most important complication of FMF and endothelial function is more severely impaired. Elevated asymmetric dimethyl arginine (ADMA) may mediate the excess cardiovascular disease (CVD) risk of this group. We aimed to compare endothelial function characteristics, including ADMA, in patients with FMF-related amyloidosis and primary glomerulopathies and to define risk factors for a CVD event.

Diagnostic value of elevated CXCR4 and CXCL12 in neonatal sepsis.

Objective: Neonatal sepsis remains a major cause of morbidity and mortality in newborns. The chemokine CXCL12 and its receptor CXCR4 are now known to play an important role in inflammatory states. However, it is unclear how chemokines respond to late-onset neonatal sepsis.

Predictive value of soluble urokinase plasminogen activator receptor, soluble ST2, and IL-33 in bronchopulmonary dysplasia.

Background: Bronchopulmonary dysplasia (BPD) remains an important complication of preterm births. The soluble form of ST2 (sST2), interleukin-33 (IL-33), and soluble form of the urokinase plasminogen activator receptor (suPAR) have attracted increasing attention as biomarkers for different diseases. The aim of the current study was to assess the predictive value of plasma sST2, IL-33, and suPAR levels in patients with risk of BPD development.

An analysis of neonatal risk factors associated with the development of ophthalmologic problems at infancy and early childhood: a study of premature infants born at or before 32 weeks of gestation.

Background: To determine the frequency of ophthalmologic problems and the risk factors that affect the occurrence of these problems in premature newborns with a gestational age of 32 weeks or less.

Methods: Premature newborns observed at a neonatal intensive care unit between January 2002 and March 2006 were included. A control visit including an ophthalmologic examination was performed at 10 months of age or later.

Neurodevelopmental status of preterm newborns at infancy, born at a tertiary care center in Turkey.

Our objective was to determine the incidence of early neonatal problems and the neurodevelopmental status and probable risk factors associated with neurodevelopmental abnormality in preterm infants of < or = 32 weeks of gestation. Preterm newborns of < or = 32 weeks of gestation followed at the neonatal intensive care unit of the Department of Pediatrics of Gülhane Military Medical Academy, Ankara, Turkey, were evaluated with a complete neurological examination and the Bayley Scales of Infant Development at a mean age of 25.85 + or - 11.

Chitotriosidase activity in human milk from mothers of premature and full-term infants during the first month of lactation.

Objective: The objectives of the present study were to measure the activity of chitotriosidase (ChT) in human milk, to record changes in enzyme activity over time and to determine whether there are differences in activity between the milk of mothers of full-term (FT) and premature (PT) infants.

Patients And Methods: Three samples were collected from each of 28 mothers (26.9+/-4.

Genetic factors in neonatal hyperbilirubinemia and kernicterus.

Although the relationship between hyperbilirubinemia and genetic factors has long been questioned, the role of genetic factors in the development of severe hyperbilirubinemia and kernicterus has been investigated in detail in the last decade with the rapid progression in molecular medicine. Although the first historical data gathered about genetical tendency to neonatal hyperbilirubinemia dates back to description of the Crigler-Najjar syndrome in 1952, a substantial interest is currently focused on coding and promoter region mutations of uridine diphosphoglucuronate glucuronosyltransferase 1A1 gene. In this article, the role of uridine diphosphoglucuronate glucuronosyltransferase gene mutations in neonatal significant hyperbilirubinemia and kernicterus is reviewed together with the clinical presentations of the most common syndromes of bilirubin conjugation, such as Gilbert and Crigler-Najjar syndromes.