hrp0089p2-p329 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

Gender-Related Differences in Etiological Distribution of Organic Causes of Central Precocious Puberty

Vuralli Dogus , Ozon Alev , Gonc Nazli , Kandemir Nurgun , Alikasifoglu Ayfer

Introduction: Organic lesion underlying central precocious puberty (CPP) is common in boys, and rare in girls. We aimed to compare the etiological distribution of organic causes according to gender, and define the clinical-laboratory characteristics that predict an organic cause to CPP.Subject and methods: Medical records of 260 girls and 120 boys with CPP were reviewed retrospectively to analyze the clinical, laboratory characteristics, radiological fin...

hrp0082p1-d2-154 | Growth (1) | ESPE2014

Clinical and Laboratory Parameters Predicting a Requirement for Reevaluation of GH Status During GH Treatment

Vuralli Dogus , Gonc Nazli , Ozon Alev , Alikasifoglu Ayfer , Kandemir Nurgun

Background: Reevaluation of children diagnosed as GH deficiency (GHD) showed 25–75% of cases had normal GH responses in retests after cessation of therapy. Low reproducibility and high intra-individual variability of the tests are the important problems in diagnosis. Repeat evaluation during treatment may help detect cases with normal GH status earlier.Objective and hypotheses: We repeated stimulation tests following the first year of GH treatment t...

hrp0092p2-223 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Long Term Effects of GnRH Agonist Therapy on BMI in Girls with Idiopathic Central Precocious Puberty

Vuralli Dogus , Ozon Z. Alev , Gonc E. Nazli , Alikasifoglu Ayfer , Kandemir Nurgun

Background: Studies investigating effects of GnRHa therapy on body weight (BW) and BMI in subjects with CPP are generally including short term effects.Aim: To investigate changes in BMI at the beginning, during and two years after completion of GnRHa therapy to determine influential factors in girls with idiopathic CPP.Methods: Data of 138 girls who completed GnRHa therapy for iCPP...

hrp0089p1-p154 | GH & IGFs P1 | ESPE2018

Early Onset GH Replacement in GH Deficiency: Is Neonatal Hypoglycemia Important for Long Term Follow-Up?

Alikasifoglu Ayfer , Emet Sadiye Dicle , Ozon Alev , Gonc Nazlı , Kandemir Nurgun

A small number of GH deficient patients can be recognized before age 3, and only few of them are diagnosed during work-up for hypoglycemia. Data comparing clinical and laboratory characteristics of hypoglycemic vs non-hypoglycemic population of children with early onset GH deficiency is scarce. The aim of this study is to assess long-term follow-up of growth hormone therapy in early onset GH deficiency, and compare pre-treatment and treatment related factors with respect to hi...

hrp0082p3-d1-955 | Sex Development | ESPE2014

Severe Undervirilisation in a 46,XY Case due to a Novel Mutation in HSD17B3 Gene

Alikasifoglu Ayfer , Vuralli Dogus , Hiort Olaf , Gonc E Nazli , Ozon Z Alev , Kandemir Nurgun

Background: 17-β-hydroxysteroid dehydrogenase 3 (17-β-HSD3) deficiency is a rare disorder of sex development due to impaired conversion of androstenedione to testosterone. Children with 46,XY karyotype often have female appearing external or ambiguous genitalia at birth. At the time of puberty, virilisation can occur. Therefore 46,XY patients with HSD17B3 gene defects should be raised as male.Objective and hypotheses: When a child with 46,XY ka...

hrp0084p3-645 | Bone | ESPE2015

Long Term Effects of Bisphosphonate Treatment in a Case with Infantile Onset Severe form of Juvenile Paget’s Disease

Gonc E Nazli , Ozon Z Alev , Buyukyilmaz Gonul , Alikasifoglu Ayfer , Simsek Ozlem , Kandemir Nurgun

Background: Juvenile Paget’s disease is a rare autosomal recessive osteopathy. Patients presenting in infancy develop severe bone deformities and may never walk. Bisphosphonate therapy is used to decrease bone turnover and it has generally good responses in milder forms of the disease. However there is no long-term experience of bisphosphonates in severe infantile forms.Case report: A 9 month-old boy was referred to our clinic for bone deformities. ...

hrp0094fc10.6 | Thyroid | ESPE2021

Clinical features and long-term follow up of childhood papillary thyroid cancer (PTC): a single reference-center experience

Ozon Alev , Canoruc Emet Dicle , Gonc Nazli , Vuralli Dogus , Buyukyilmaz Gonul , Kandemir Nurgun , Alikasifoglu Ayfer ,

Background: Thyroid cancer before 20 years of age constitutes less than 2% of thyroid malignancies. Adult guidelines have been used in evaluation and follow-up of children until 2015. Features and long-term results of more children with PTC are needed to improve the ATA guideline for children. The aim of this study is to evaluate children with PTC in the last 15 years in a single reference-center, and assess the convenience of ATA 2015 guideline in practice.</...

hrp0092p1-404 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Improvement of Final Height in Idiopathic Central Precocious Puberty is Associated with Delay of Bone Maturation with GnRH Agonist Therapy Under the Age of 7 Years

Vuralli Dogus , Gonc E. Nazli , Ozon Z. Alev , Kandemir Nurgun , Alikasifoglu Ayfer

Background: GnRHa therapy is shown to be beneficial in increasing final height when started before 6 years of age in girls with idiopathic CPP(iCPP). However controversial data exist in improvement of final height whose pubertal signs appear after age of 6.Aim: To investigate effect of age of onset of GnRHa therapy on final height n girls with iCPP, and to evaluate factors affecting height gain.<st...

hrp0092p2-59 | Bone, Growth Plate and Mineral Metabolism | ESPE2019

Spondyloocular Syndrome: Presentation of Two Siblings Diagnosed with The Rare Disease and The Results of Pamidronate Therapy

Vuralli Dogus , Simsek Kiper Pelin Ozlem , Utine Eda , Unsal Yagmur , Alikasifoglu Ayfer , Kandemir Nurgun

Spondyloocular syndrome (OMIM 605822) is an autosomal recessive disorder characterized by skeletal complaints (osteoporosis, platyspondyly, multiple bone fractures), hearing loss and ocular symptoms (cataracts, retinal detachment). XYLT2 gene (OMIM 608125) mutation encoding xylosyltransferase II enzyme which is responsible from the first step of proteoglycan assembly is responsible for the pathogenesis. Phenotypical variability is associated with varying genetic expre...

hrp0092p2-224 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

To Whom Should Central Nervous System Imaging be Performed in Girls with Central Precocious Puberty (CPP)?

Vuralli Dogus , Gonc E. Nazli , Alikasifoglu Ayfer , Kandemir Nurgun , Ozon Z. Alev

Background: Organic lesions are present in 5-10% of cases with CPP. Determinants of an increased risk for organic pathology underlying CPP in girls are debatable. CNS imaging is recommended for girls who have pubertal signs before 6 years of age. Although neurological findings may suggest organic pathology, other clinical features and hormonal variables have not been adequately examined.Aim: To analyze clinical&h...